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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introdu&#231;&#227;o</span><p id="par0005" class="elsevierStylePara elsevierViewall">A defici&#234;ncia de lipase &#225;cida lisossomal &#40;DLAL&#41; &#233; uma doen&#231;a recessiva autoss&#244;mica que pertence ao grupo de doen&#231;as de dep&#243;sito lisoss&#244;mico&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#8211;3</span></a> O fen&#243;tipo da DLAL varia de acordo com a atividade da enzima LAL e o espectro cl&#237;nico pode variar de doen&#231;a de Wolman &#40;DW&#41;&#44; uma forma grave de in&#237;cio na inf&#226;ncia&#44; a doen&#231;a do armazenamento de &#233;ster de colesterol &#40;DAEC&#41;&#44; uma doen&#231;a com sintomas mais leves&#44; por&#233;m &#224;s vezes com apresenta&#231;&#227;o grave e progn&#243;stico ruim&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#8211;6</span></a> A baixa frequ&#234;ncia da DLAL foi contestada&#44; pois os estudos populacionais prop&#245;em que sua incid&#234;ncia pode ser maior do que a descrita anteriormente&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">2&#44;3&#44;7</span></a> Adicionalmente&#44; o aumento da conscientiza&#231;&#227;o cl&#237;nica resultou em mais diagn&#243;sticos&#44; corroborou a ideia de que ela pode ser uma doen&#231;a subdiagnosticada&#46; Foram descritas muitas variantes no gene da lipase &#225;cida lisossomal &#40;LIPA&#41; e novos estudos ainda escrevem novas muta&#231;&#245;es&#59; atualmente&#44; n&#227;o foi encontrada uma correla&#231;&#227;o gen&#243;tipo&#8208;fen&#243;tipo&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#44;2&#44;8&#44;9</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Neste estudo&#44; analisamos sete pacientes com DLAL e acreditamos ser a maior s&#233;rie relatada na Am&#233;rica do Sul&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">10</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">M&#233;todos</span><p id="par0015" class="elsevierStylePara elsevierViewall">Fizemos uma an&#225;lise retrospectiva dos prontu&#225;rios m&#233;dicos dos pacientes com DLAL tratados em nossa cl&#237;nica ambulatorial&#46; Todos os dados com rela&#231;&#227;o &#224;s caracter&#237;sticas demogr&#225;ficas&#44; cl&#237;nicas&#44; laboratoriais e moleculares foram recuperados e&#44; ent&#227;o&#44; analisados&#46; Todos os pacientes e seus pais &#40;ou respons&#225;veis&#41; estavam cientes da inten&#231;&#227;o de publicar seus prontu&#225;rios&#44; concordaram com isso e assinaram um consentimento informado&#44; de acordo com as diretrizes do Comit&#234; de &#201;tica de nossa institui&#231;&#227;o&#46; Este estudo foi aprovado pelo Comit&#234; de &#201;tica&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">O Departamento de Laborat&#243;rios do Seattle Children&#39;s fez todos os testes da atividade da LAL e a an&#225;lise molecular com o uso de amostra de sangue seco &#40;DBS&#41;&#46; Os testes da atividade da LAL foram feitos com o m&#233;todo descrito por Hamilton et al&#46; em 2012&#58; foi usado 4&#8208;metilumbeliferil palmitato como substrato para a atividade da lipase total determinada&#59; como sangue total para outras lipases&#44; Lalistat 2&#44; um inibidor de LAL&#44; foi acrescentado &#224; amostra para medir a atividade da lipase pancre&#225;tica e&#44; por fim&#44; foi calculada a atividade da LAL&#46; As unidades usadas foram pmol&#47;hr&#47;spt&#46; Foi feito o sequenciamento de Sanger do gene LIPA em cada paciente com baixa atividade da LAL&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">11</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Resultados &#40;descri&#231;&#245;es dos casos&#41;</span><p id="par0025" class="elsevierStylePara elsevierViewall">Revisamos os prontu&#225;rios m&#233;dicos de sete crian&#231;as com DLAL &#40;5<span class="elsevierStyleHsp" style=""></span>M&#58; 2 F&#41;&#59; seis eram pardas e uma negra&#46; As idades m&#233;dia e mediana no in&#237;cio dos sintomas foram cinco anos &#40;quatro meses a nove anos&#41; e seis anos e no diagn&#243;stico foram 6&#44;9 a &#40;tr&#234;s a 10 anos&#41; e 6&#44;5 anos&#44; respectivamente&#46; O tempo entre o diagn&#243;stico e o in&#237;cio dos sintomas variou amplamente e &#233; mostrado na <a class="elsevierStyleCrossRef" href="#tbl0005">tabela 1</a>&#44; juntamente com outras caracter&#237;sticas cl&#237;nicas&#46; Dois pacientes eram assintom&#225;ticos e a suspeita cl&#237;nica surgiu devido &#224; hepatomegalia&#46; Seis pacientes apresentaram hepatomegalia e nenhum apresentou esplenomegalia nem desenvolveu icter&#237;cia&#46; Dois pacientes irm&#227;os apresentaram consanguinidade&#46; Cinco dos sete pacientes apresentaram hist&#243;rico familiar de dislipidemia&#46; Ap&#243;s suspeita cl&#237;nica e laboratorial em nossos pacientes&#44; foi medida a atividade da LAL para confirmar DLAL&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Uma caracter&#237;stica da DLAL &#233; a heterogeneidade cl&#237;nica dos sintomas na apresenta&#231;&#227;o&#46; &#201; obrigat&#243;ria uma descri&#231;&#227;o cl&#237;nica detalhada dos casos&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">O paciente 1 foi trazido a nossa instala&#231;&#227;o ambulatorial com dor abdominal&#44; sem sinal alarmante&#44; e tinha oito anos&#46; Ap&#243;s avalia&#231;&#227;o cont&#237;nua&#44; observamos hepatomegalia e a endoscopia digestiva alta mostrou histi&#243;citos azul marinho no exame histol&#243;gico de bi&#243;psia da mucosa do duodeno&#46; A medi&#231;&#227;o da atividade da LAL confirmou a DLAL&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">O paciente 3&#44; durante uma consulta pedi&#225;trica de rotina aos seis anos&#44; apresentou hepatomegalia&#46; Os exames iniciais mostraram um aumento de 2&#8208;3 vezes da alanina aminotransferase &#40;ALT&#41;&#44; baixo n&#237;vel de lipoprote&#237;na de alta densidade &#40;HDL&#41; e n&#237;vel elevado de lipoprote&#237;na de baixa densidade &#40;LDL&#41;&#46; A bi&#243;psia do f&#237;gado mostrou dep&#243;sitos lip&#237;dicos nos histi&#243;citos&#46; Mais tarde&#44; a atividade da LAL medida aos sete anos foi diagn&#243;stico de DLAL&#46; Sua irm&#227;&#44; a paciente 2&#44; tamb&#233;m apresentou enzimas hep&#225;ticas ligeiramente elevadas aos tr&#234;s anos&#44; sem hepatomegalia&#44; e foi diagnosticada ao mesmo tempo em que seu irm&#227;o com DLAL&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">O paciente 4&#44; que tinha quatro meses de idade&#44; apresentou diarreia progressiva e intrat&#225;vel &#40;primeira ocorr&#234;ncia em 1991&#41;&#44; d&#233;ficit de crescimento&#44; hepatomegalia&#44; dislipidemia e calcifica&#231;&#245;es adrenais&#46; Aos tr&#234;s anos&#44; uma bi&#243;psia hep&#225;tica revelou esteatose microvesicular significativa&#44; fibrose septal e agregados de macr&#243;fagos com microvacuoliza&#231;&#227;o citoplasm&#225;tica &#8211; achados sugestivos de Niemann&#8208;Pick naquela &#233;poca&#44; pois tamb&#233;m apresentou baixos n&#237;veis de esfingomielinase e n&#237;veis elevados de fosfatase &#225;cida&#46; Contudo&#44; a microscopia eletr&#244;nica mostrou imagens negativas de cristais de colesterol e vac&#250;olos lip&#237;dicos nos hepat&#243;citos e histi&#243;citos&#59; assim&#44; a DLAL foi favorecida por esse m&#233;todo&#46; Sua diarreia cr&#244;nica e doen&#231;a hep&#225;tica progressiva continuaram&#46; Ao atingir seis anos&#44; ele desenvolveu v&#225;rios epis&#243;dios de pieira&#46; Os raios X e a tomografia de t&#243;rax mostraram sinais de pneumonite intersticial&#46; A espirometria revelou um padr&#227;o restritivo&#46; Aos 13 anos&#44; desenvolveu complica&#231;&#245;es devido &#224; insufici&#234;ncia hep&#225;tica com ascite e apresentou peritonite bacteriana espont&#226;nea duas vezes&#46; Aos 18 anos&#44; no in&#237;cio de 2010&#44; foi submetido a uma cirurgia de transplante de f&#237;gado de um doador morto&#46; Seu f&#237;gado explantado &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>A e B&#41; apresentou cirrose micronodular e caracter&#237;sticas espec&#237;ficas da doen&#231;a&#44; inclusive algumas fendas em forma de agulha dentro das c&#233;lulas Kupffer microvacuoladas&#46; Antes do transplante de f&#237;gado&#44; o paciente desenvolveu pequena esplenomegalia &#40;2<span class="elsevierStyleHsp" style=""></span>cm da borda costal esquerda&#41; e a mesma apresentou redu&#231;&#227;o de cerca de 50&#37; ap&#243;s transplante de f&#237;gado&#46; Ap&#243;s transplante do f&#237;gado&#44; apresentou v&#225;rios epis&#243;dios de rejei&#231;&#227;o&#44; que melhoraram com corticosteroides&#46; Sua bi&#243;psia hep&#225;tica em 2015 mostrou rejei&#231;&#227;o cr&#244;nica com ductopenia &#40;perda de 50&#37;&#41;&#44; fibrose portal e centrolobular&#44; septos raros&#44; perivenulite central e algumas c&#233;lulas de Kupffer espumosas &#40;geralmente s&#227;o uma resposta a colestase nesse contexto&#41;&#46; Contudo&#44; sua bi&#243;psia hep&#225;tica em 2017 mostrou muitos macr&#243;fagos xantomatosos no trato portal&#44; fortemente sugestivo de recrudescimento da doen&#231;a &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>C&#41;&#44; juntamente com fibrose em ponte marcada &#40;F3&#41;&#44; rejei&#231;&#227;o cr&#244;nica e c&#233;lulas sinusoidais espumosas raras&#46; A atividade da enzima LAL medida em 2015 foi zero&#46; O perfil lip&#237;dico do paciente melhorou para n&#237;veis normais de LDL e triglicer&#237;deos &#40;TG&#41; ap&#243;s transplante de f&#237;gado&#44; por&#233;m os baixos n&#237;veis de HDL continuaram&#46; Al&#233;m disso&#44; uma bi&#243;psia duodenal feita em 2016 mostrou in&#250;meros macr&#243;fagos xantomatosos na l&#226;mina pr&#243;pria &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>D&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0050" class="elsevierStylePara elsevierViewall">O paciente 5&#44; com oito anos&#44; apresentou dor abdominal cr&#244;nica juntamente com hepatomegalia e aumento da aspartato aminotransferase &#40;AST&#41; e ALT&#44; juntamente com baixo HDL e alto LDL&#46; Ap&#243;s investiga&#231;&#227;o inicial inconclusiva&#44; uma bi&#243;psia de f&#237;gado revelou achados sugestivos de doen&#231;a de Niemann&#8208;Pick&#46; Como o curso cl&#237;nico n&#227;o foi sugestivo de Niemann&#8208;Pick&#44; a medi&#231;&#227;o da atividade da LAL diagnosticou DLAL&#46; Sua tia tamb&#233;m foi diagnosticada anteriormente com doen&#231;a de Niemann&#8208;Pick e&#44; mais tarde&#44; com DLAL&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">O paciente 6 apresentou dor abdominal cr&#244;nica por volta dos seis anos&#46; A avalia&#231;&#227;o laboratorial inicial mostrou ligeiro aumento na AST e ALT&#44; HDL baixa e LDL alta e esteatose no ultrassom do f&#237;gado&#46; Ele n&#227;o estava acima do peso e n&#227;o havia consumido altas doses de frutose&#44; sinais sugestivos de doen&#231;a hep&#225;tica gordurosa n&#227;o alco&#243;lica &#40;DHGNA&#41; ou esteatose hep&#225;tica&#46; Portanto&#44; medimos a atividade da LAL e confirmamos DLAL&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">O paciente 7&#44; um menino de dois anos&#44; foi internado em nosso hospital devido &#224; dificuldade de andar&#46; Ap&#243;s exame f&#237;sico inicial&#44; detectamos hepatomegalia&#46; A an&#225;lise laboratorial indicou claramente raquitismo&#46; Aos tr&#234;s anos&#44; o paciente foi transferido para nossa cl&#237;nica de hepatologia para investigar hepatomegalia&#46; Uma bi&#243;psia hep&#225;tica&#44; feita quando o paciente tinha seis anos&#44; sugeriu doen&#231;as metab&#243;licas&#44; principalmente glicogenose&#46; Contudo&#44; o paciente nunca desenvolveu hipoglicemia&#46; Em 2016&#44; ap&#243;s medir a atividade da LAL&#44; diagnosticamos DLAL&#46; Ap&#243;s um tempo&#44; o patologista reavaliou as l&#226;minas da bi&#243;psia hep&#225;tica&#46; Os achados histopatol&#243;gicos &#40;<a class="elsevierStyleCrossRef" href="#fig0010">fig&#46; 2</a>&#41; das poucas c&#233;lulas de Kupffer espumosas e alguns macr&#243;fagos do trato portal fracamente positivos para &#225;cido peri&#243;dico de Schiff &#40;PAS&#41; ap&#243;s digest&#227;o diast&#225;sica&#44; a aus&#234;ncia das c&#233;lulas hep&#225;ticas inchadas caracter&#237;sticas que se assemelham a c&#233;lulas vegetais&#44; sem mencionar a presen&#231;a de esteatose grau 3&#44; s&#227;o sinais de diagn&#243;stico de DLAL &#40;al&#233;m de outros diagn&#243;sticos diferenciais&#44; como mucopolissacaridoses&#41;&#46; Vale mencionar que esse caso mostrou somente mudan&#231;as sutis nas c&#233;lulas de Kupffer&#44; tornou o diagn&#243;stico mais desafiador&#44; possivelmente relacionado &#224; variabilidade de amostragem das bi&#243;psias de f&#237;gado&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">A <a class="elsevierStyleCrossRef" href="#tbl0010">tabela 2</a> resume as caracter&#237;sticas laboratoriais de nossos pacientes&#46; A an&#225;lise gen&#233;tica revelou uma rara variante p&#46;L89P &#40;c&#46;266T<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>C&#41; em tr&#234;s pacientes&#44; algo nunca descrito no Brasil&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">12</span></a> Nenhum desses pacientes estava relacionado com o outro&#46; Um era homozigoto para p&#46;L89P e os outros eram heterozigotos&#46; As variantes c&#46;894G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&#44; uma variante no local de porte na extremidade 3&#8217; do &#233;xon 8&#44; e c&#46;67G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A encontrada em nossos pacientes&#44; j&#225; foram comumente descritas&#46;</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Discuss&#227;o</span><p id="par0070" class="elsevierStylePara elsevierViewall">Relatamos sete pacientes com DLAL&#44; a maior casu&#237;stica j&#225; descrita na Am&#233;rica do Sul&#46; Os diagn&#243;sticos dos pacientes foram confirmados ap&#243;s alta suspeita cl&#237;nica que levou &#224; medica&#231;&#227;o da atividade da LAL&#44; que foi invariavelmente baixa&#46; Nossa conscientiza&#231;&#227;o cl&#237;nica da DLAL melhorou ao longo dos anos e isso explica os intervalos de tempo vari&#225;veis entre o in&#237;cio dos sintomas e o diagn&#243;stico&#46; Os tr&#234;s pacientes com rela&#231;&#227;o aos quais levamos mais de um ano para chegar ao diagn&#243;stico s&#227;o aqueles que apresentaram sintomas at&#237;picos no acometimento&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">O paciente 4 apresentou sintomas severos que se assemelham &#224; doen&#231;a de Wolman&#44; por&#233;m tamb&#233;m apresentou pneumonite intersticial&#44; baixa esfingomielinase e altos n&#237;veis de fosfatase &#225;cida&#44; que s&#227;o vari&#225;veis de confus&#227;o&#44; pois esses achados s&#227;o sugestivos de doen&#231;a de Niemann&#8208;Pick&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">O paciente 7 apresentou hepatomegalia e a bi&#243;psia hep&#225;tica inicial sugeriu uma doen&#231;a de armazenamento&#44; como glicogenose&#44; pois alguns achados &#40;por exemplo&#44; c&#233;lulas de Kupffer espumosas&#41; n&#227;o foram muito vis&#237;veis na amostra do tecido&#46; A reavalia&#231;&#227;o das l&#226;minas do f&#237;gado&#44; ap&#243;s considerar as novas informa&#231;&#245;es de DLAL&#44; foi compat&#237;vel com a doen&#231;a&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Esses 2 casos mencionados &#40;dos pacientes 4 e 7&#41; acrescentaram a DLAL como um poss&#237;vel diagn&#243;stico diferenciado de glicogenose e Niemann&#8208;Pick tipo B&#46; A doen&#231;a de Gaucher&#44; Niemann&#8208;Pick tipo C e NAFLD j&#225; foram descritas como diagn&#243;sticos diferenciais de DLAL&#46;<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">13&#44;14</span></a> Os diagn&#243;sticos diferenciais de DLAL s&#227;o amplos e geralmente sobrep&#245;em outras doen&#231;as cr&#244;nicas&#46; Consequentemente&#44; uma raz&#227;o pela qual a preval&#234;ncia real da DLAL ser desconhecida &#233; o fato de ela ser uma doen&#231;a subdiagnosticada&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">2&#44;3&#44;7&#8211;9&#44;15&#44;16</span></a> Deve haver alta suspeita cl&#237;nica de DLAL em pacientes com hepatomegalia n&#227;o explicada&#44; altos n&#237;veis de LDL e triglicer&#237;deos&#44; baixos n&#237;veis de HDL e enzimas hep&#225;ticas levemente elevadas&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#44;2&#44;8</span></a> Seis de nossos pacientes apresentaram hepatomegalia&#44; todos apresentaram um padr&#227;o de colesterol t&#237;pico e cinco apresentaram enzimas hep&#225;ticas levemente elevadas&#46; Esses resultados mostram que h&#225; um padr&#227;o de achados para diagnosticar DLAL&#44; mesmo em casos at&#237;picos&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">O LIPA&#44; o gene que codifica a LAL&#44; est&#225; localizado no cromossomo 10q23&#46;31&#46; Sabe&#8208;se que muitas variantes nesse gene est&#227;o associadas &#224; DLAL&#46; A variante c&#46;894<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>1G&#62;A&#160;&#40;E8SJM<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>1&#41; &#233; a mais comum e &#233; respons&#225;vel por mais da metade dos casos publicados&#59; tr&#234;s de nossos pacientes apresentaram essa variante&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#44;2</span></a> A outra variante&#44; c&#46;67G&#62;A&#44; j&#225; foi relatada tr&#234;s vezes no site ClinVar&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">17</span></a> Curiosamente&#44; descrevemos neste trabalho uma variante rara&#44; a c&#46;266T&#62;C &#40;p&#46;L89P&#41;&#44; que foi encontrada em tr&#234;s pacientes&#46; Essa variante patog&#234;nica foi descrita somente uma vez em uma apresenta&#231;&#227;o em p&#244;ster na Reuni&#227;o de 2014<span class="elsevierStyleHsp" style=""></span>da Sociedade Americana de Gen&#233;tica Humana&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">12</span></a> Propusemos um poss&#237;vel gen&#243;tipo brasileiro para a DLAL&#44; pois nenhum dos pacientes com essa variante estava relacionado a outro&#46; O paciente homozigoto para a &#250;ltima variante mostrou a apresenta&#231;&#227;o mais grave com envolvimento pulmonar&#44; diarreia cr&#244;nica e doen&#231;a hep&#225;tica progressiva&#44; necessitou de transplante de f&#237;gado &#40;paciente 4&#41;&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">A DLAL &#233; classicamente definida como uma doen&#231;a recessiva autoss&#244;mica&#46; Contudo&#44; os pacientes 1&#44; 5 e 6 apresentaram um alelo mutante e um alelo tipo selvagem&#46; Todos esses pacientes apresentaram sinais laboratoriais e cl&#237;nicos sugestivos de DLAL &#40;hepatomegalia&#44; baixa HDL&#44; alta LDL e baixa atividade da LAL&#41; e foram exclu&#237;das outras doen&#231;as prov&#225;veis&#46; A doen&#231;a de Niemann&#8208;Pick foi o principal diagn&#243;stico de confus&#227;o para o paciente 5&#44; pois ele tinha uma tia com a doen&#231;a de Niemann&#8208;Pick&#46; Contudo&#44; ap&#243;s nosso diagn&#243;stico&#44; investigamos sua tia e pudemos diagnostic&#225;&#8208;la com DLAL&#46; DHGNA e intoler&#226;ncia &#224; frutose foram outros diagn&#243;sticos exclu&#237;dos&#44; pois elas podem surgir com hepatomegalia&#44; esteatose hep&#225;tica e especificamente DHGNA com dislipidemia&#46; Outra hip&#243;tese &#233; que esses pacientes podem ter muta&#231;&#245;es intr&#244;nicas que n&#227;o foram reconhecidas por meio do sequenciamento de Sanger ou que a redu&#231;&#227;o da atividade da LAL em alguns pacientes heterozigotos pode causar os sintomas&#46; Pullinger et al&#46; j&#225; descreveram os pacientes heterozigotos para as variantes patog&#234;nicas do gene LIPA que apresentaram sintomas e os dados laboratoriais sugestivos de DLAL&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">2&#44;18</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">O tratamento e o progn&#243;stico da DLAL mudaram drasticamente nos &#250;ltimos anos ap&#243;s a cria&#231;&#227;o do sebelipase alfa &#40;Alexion&#44; Kanuma&#174;&#44; Kanuma&#8482;&#44; WA&#44; EUA&#41;&#44; uma terapia de reposi&#231;&#227;o enzim&#225;tica &#40;TRE&#41; que mostrou melhorias no dano hep&#225;tico e nas anomalias lip&#237;dicas&#46;<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">19&#8211;22</span></a> O transplante de f&#237;gado para DLAL &#233; outro poss&#237;vel tratamento para casos graves e foi relatado somente 20 vezes na literatura m&#233;dica&#44; por&#233;m poucos trabalhos relatam acompanhamento de longo prazo desses pacientes&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">2</span></a> Bernstein et al&#46; publicaram dois casos de pacientes com DLAL submetidos a transplante hep&#225;tico e&#44; nos dois&#44; a doen&#231;a DLAL multissist&#234;mica reapareceu ap&#243;s o transplante&#46; Eles tamb&#233;m revisaram a literatura de 18 casos de pacientes com DLAL submetidos a transplante de f&#237;gado e descreveram a progress&#227;o multissist&#234;mica da DLAL em 11 pacientes &#40;66&#37;&#41; e &#243;bito em seis pacientes &#40;33&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">23</span></a> Em nosso estudo&#44; o paciente 4 foi submetido a transplante de f&#237;gado e&#44; ap&#243;s cinco anos de acompanhamento&#44; apresentou rejei&#231;&#227;o cr&#244;nica e manteve baixos n&#237;veis de HDL&#46; Curiosamente&#44; esse paciente ainda tinha deposi&#231;&#227;o de colesterol nos macr&#243;fagos e mostrou histi&#243;citos xantomatosos na bi&#243;psia de duodeno em 2016 e&#44; mais tarde&#44; desenvolveu macr&#243;fagos espumosos no trato portal na bi&#243;psia de f&#237;gado ap&#243;s sete anos de transplante de f&#237;gado&#46; Portanto&#44; o transplante de f&#237;gado n&#227;o &#233; a cura para essa doen&#231;a&#44; pois os macr&#243;fagos continuam a ser prejudicados&#44; causam sintomas&#44; pois outros &#243;rg&#227;os&#44; como a medula &#243;ssea e o intestino delgado&#44; ainda conter&#227;o os macr&#243;fagos carregados de lip&#237;dios&#46; Por outro lado&#44; acredita&#8208;se que o transplante de medula &#243;ssea &#40;TMO&#41; corrige o defeito da enzima e&#44; portanto&#44; se feito no in&#237;cio do curso da doen&#231;a&#44; pode salvar a vida dos pacientes com a doen&#231;a de Wolman&#46; Foram relatados alguns TMOs bem&#8208;sucedidos na DLAL&#59; contudo&#44; a maior parte dos casos tem um progn&#243;stico ruim e a TER imediata &#233; a terapia de escolha&#46;<a class="elsevierStyleCrossRefs" href="#bib0250"><span class="elsevierStyleSup">24&#8211;26</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Em conclus&#227;o&#44; relatamos a maior casu&#237;stica de pacientes com DLAL na Am&#233;rica do Sul e descrevemos um gen&#243;tipo raro&#44; possivelmente um gen&#243;tipo brasileiro&#46; Enfatizamos que a DLAL pode apresentar sinais e sintomas heterog&#234;neos&#44; por&#233;m ela deve ser inclu&#237;da como um diagn&#243;stico diferenciado de hepatomegalia&#44; enzimas hep&#225;ticas elevadas e dislipidemia&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Conflitos de interesse</span><p id="par0110" class="elsevierStylePara elsevierViewall">Os autores declaram n&#227;o haver conflitos de interesse&#46;</p></span></span>"
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            0 => "Lysosomal storage diseases"
            1 => "Pediatrics"
            2 => "Metabolic diseases"
            3 => "Liver failure"
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            0 => "Doen&#231;as do armazenamento lisossomal"
            1 => "Pediatria"
            2 => "Doen&#231;as metab&#243;licas"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">To describe the demographic&#44; clinical&#44; laboratory and molecular characteristics of patients with lysosomal acid lipase deficiency&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A retrospective review of the medical records of children with the disease&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Seven children with lysosomal acid lipase deficiency &#40;5 male&#59; 2 female&#41;&#59; 6 were mixed race&#44; and 1 was black&#46; The mean ages at the first onset of symptoms and at diagnosis were 5&#46;0 years &#40;4 months to 9 years&#41; and 6&#46;9 years &#40;3&#8211;10 years&#41;&#44; respectively&#46; Symptom manifestations at onset were&#58; 3 patients had abdominal pain&#44; one had bone&#47;joint pain due to rickets&#44; and 1 had chronic diarrhea and respiratory insufficiency due to interstitial pneumonitis&#46; One was asymptomatic&#44; and clinical suspicion arose due to hepatomegaly&#46; Six patients had hepatomegaly&#44; and none had splenomegaly&#46; Two patients were siblings&#46; Enzymatic assay and molecular analysis confirmed the diagnoses&#46; Genetic analysis revealed a rare pathogenic variant &#40;p&#46;L89P&#41; in three patients&#44; described only once in medical literature and never described in Brazil&#46; None of those patients were related to each other&#46; Lysosomal acid lipase deficiency was previously described as an autosomal recessive disease&#44; but three patients were heterozygous and undoubtedly had the disease &#40;low enzyme activity&#44; suggestive lab findings and clinical symptoms&#41;&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">This case series supports that lysosomal acid lipase deficiency can present with highly heterogeneous signs and symptoms among patients&#44; but it should be considered in children presenting with gastrointestinal symptoms associated with dyslipidemia&#46; We describe a rare variant in three non&#8208;related patients that may suggest a Brazilian genotype for lysosomal acid lipase deficiency&#46;</p></span>"
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        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Objetivo</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Descrever as caracter&#237;sticas demogr&#225;ficas&#44; cl&#237;nicas&#44; laboratoriais e moleculares de pacientes com defici&#234;ncia de lipase &#225;cida lisossomal&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">M&#233;todos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">An&#225;lise retrospectiva dos prontu&#225;rios m&#233;dicos de crian&#231;as com a defici&#234;ncia de lipase &#225;cida lisossomal&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Sete crian&#231;as com defici&#234;ncia de lipase &#225;cida lisossomal &#40;5<span class="elsevierStyleHsp" style=""></span>M&#58;2F&#41;&#59; seis eram pardas e uma negra&#46; As faixas et&#225;rias no in&#237;cio dos sintomas e no diagn&#243;stico foram 5 anos &#40;4 meses a 9 anos&#41; e 6&#44;9 anos &#40;3 a 10 anos&#41;&#44; respectivamente&#46; As manifesta&#231;&#245;es dos sintomas no in&#237;cio foram as que seguem&#58; tr&#234;s pacientes apresentaram dor abdominal&#44; um apresentou dor nos ossos&#47;articula&#231;&#245;es devido a raquitismo e um apresentou diarreia cr&#244;nica e insufici&#234;ncia respirat&#243;ria devido &#224; pneumonite intersticial&#46; Os outros n&#227;o apresentaram sintomas e a suspeita cl&#237;nica surgiu devido &#224; hepatomegalia&#46; Seis pacientes apresentaram hepatomegalia e um apresentou esplenomegalia&#46; Dois pacientes eram irm&#227;os&#46; O ensaio enzim&#233;tico e a an&#225;lise molecular confirmaram os diagn&#243;sticos&#46; A an&#225;lise gen&#233;tica revelou uma variante patog&#234;nica rara &#40;p&#46;L89P&#41; em tr&#234;s pacientes&#44; descrita uma &#250;nica vez na literatura m&#233;dica e nunca descrita no Brasil&#46; Nenhum desses pacientes tinha parentesco com os outros&#46; A defici&#234;ncia de lipase &#225;cida lisossomal foi anteriormente descrita como uma doen&#231;a recessiva autoss&#244;mica&#44; por&#233;m tr&#234;s pacientes eram heterozigotos e&#44; sem d&#250;vida&#44; apresentaram a doen&#231;a &#40;atividade enzim&#225;tica baixa&#44; achados laboratoriais sugestivos e sintomas cl&#237;nicos&#41;&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclus&#227;o</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Esta casu&#237;stica afirma que a defici&#234;ncia de lipase &#225;cida lisossomal pode se manifestar com sinais e sintomas altamente heterog&#234;neos entre os pacientes&#44; por&#233;m deve ser considerada em crian&#231;as que apresentam sintomas gastrointestinais associados &#224; dislipidemia&#46; Descrevemos uma variante rara em tr&#234;s pacientes n&#227;o relacionados que pode sugerir um gen&#243;tipo brasileiro para defici&#234;ncia de lipase &#225;cida lisossomal&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0030">Como citar este artigo&#58; Benevides GN&#44; Miura IK&#44; Person NC&#44; Pugliese RP&#44; Danesi VL&#44; Lima FR&#44; et al&#46; Lysosomal acid lipase deficiency in Brazilian children&#58; a case series&#46; J Pediatr &#40;Rio J&#41;&#46; 2019&#59;95&#58;552&#8211;8&#46;</p>"
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          "pt" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Alguns aspectos histol&#243;gicos do f&#237;gado e duodeno no paciente n&#176; 4&#46; A &#8211; Cirrose micronodular avan&#231;ada observada no explante do f&#237;gado &#40;H&#38;E&#44; 25X&#41;&#59; B &#8211; Essa &#225;rea &#40;do explante do f&#237;gado&#41; descreve esteatose microvesicular e mediovesicular em muitos hepat&#243;citos e grupos de c&#233;lulas de Kupffer xantomatosas em sinusoides &#40;H&#38;E&#44; 200X&#41;&#44; al&#233;m de raras fendas em forma de agulha &#40;setas&#41; dentro das c&#233;lulas de Kupffer na inser&#231;&#227;o&#59; C &#8211; Muitos macr&#243;fagos no trato portal mostram citoplasma xantomatoso no enxerto hep&#225;tico ap&#243;s sete anos de transplante de f&#237;gado &#40;H&#38;E&#44; 400X&#41;&#59; D &#8211; A l&#226;mina pr&#243;pria &#233; infiltrada intensamente por grandes c&#233;lulas espumosas carregadas de lip&#237;dios nessa bi&#243;psia duodenal &#40;H&#38;E&#44; 400X&#41;&#46;</p>"
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          "pt" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Aspectos histol&#243;gicos da bi&#243;psia de f&#237;gado no paciente 7&#46; A &#8211; Fibrose em ponte marcada com n&#243;dulos ocasionais &#40;colora&#231;&#227;o com Picrosirius Red&#44; 25X&#41;&#59; B &#8211; Esteatose mediovesicular &#40;variante de pequenas got&#237;culas da esteatose macrovesicular&#41; e hepat&#243;citos com citoplasma p&#225;lido &#40;H&#38;E&#44; 400X&#41;&#59; C &#8211; Somente raras c&#233;lulas de Kupffer microvacuoladas foram identificadas&#44; que foram ligeiramente coradas com PAS &#40;PAS ap&#243;s digest&#227;o diast&#225;sica&#44; 400X&#41;&#59; D &#8211; Alguns macr&#243;fagos com citoplasma espumoso e corado foram detectados nos tratos portais &#40;H&#38;E&#44; 400X&#41;&#46;</p>"
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                  \t\t\t\t">27 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">31 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">24 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Sexo&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Idade no in&#237;cio dos sintomas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Idade no diagn&#243;stico&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 a 5 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tempo do in&#237;cio dos sintomas at&#233; o diagn&#243;stico&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 a 5 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 a 8 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Sintomas&#47;sinais no in&#237;cio&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor abdominal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Diarreia cr&#244;nica&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor abdominal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor abdominal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor &#243;ssea&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tamanho do f&#237;gado<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">d</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Hist&#243;rico familiar&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Pai com dislipidemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Pais eram primos de primeiro grau&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tio com DLAL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&#227;e com lupus&#59; tia com l&#250;pus e com DLAL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Pai com dislipidemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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          "leyenda" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">&#947;GT&#44; gama glutamil transferase&#59; ALT&#44; alanina aminotransferase&#59; AST&#44; aspartato aminotransferase&#59; CT&#44; colesterol total&#59; FA&#44; fosfatase alcalina&#59; HDL&#44; lipoprote&#237;na de alta densidade&#59; LDL&#44; lipoprote&#237;na de baixa densidade&#59; LIN&#44; limite inferior da normalidade&#59; LSN&#44; limite superior da normalidade&#59; RNI&#44; raz&#227;o normalizada internacional&#59; TG&#44; triglicer&#237;deos&#59; ts&#44; gene tipo selvagem&#46;</p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col">Exames&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " colspan="7" align="center" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">2<a class="elsevierStyleCrossRef" href="#tblfn0025"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">3<a class="elsevierStyleCrossRef" href="#tblfn0025"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">5&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">AST &#40;ULN&#41; U&#47;L&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">28 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">72 &#40;15&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">22 &#40;18&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">188&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">61 &#40;37&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">51 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">35 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">ALT &#40;ULN&#41; U&#47;L&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">21 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">127 &#40;17&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">38 &#40;22&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">94&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">119 &#40;41&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">53 &#40;35&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">41 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#947;GT &#40;ULN&#41;&#160;U&#47;L&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">23 &#40;22&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">9 &#40;28&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">16 &#40;28&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">68&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">118 &#40;61&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">18 &#40;22&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">26 &#40;25&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">AP &#40;ULN&#41; U&#47;L&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">236 &#40;350&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">590 &#40;400&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">205&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">256 &#40;300&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">238 &#40;300&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">3872 &#40;383&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">TC &#40;ULN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>200&#41; mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">299&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">415&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">295&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">340&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">262&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">236&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">238&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">HDL &#40;LLN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>45&#41; mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">38&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">21&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">13&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">34&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">32&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">LDL &#40;LSN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>100&#41;&#160;mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">238&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">333&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">229&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">268&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">207&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">174&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">182&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">TG &#40;LSN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>150&#41;&#160;mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">142&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">305&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">150&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">288&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">209&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">140&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t">INR&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">0&#44;98&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1&#44;3&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">An&#225;lise do DNA&nbsp;\t\t\t\t\t\t\n
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          "pt" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Trabalho laboratorial no in&#237;cio dos sintomas e an&#225;lise gen&#233;tica</p>"
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      "titulo" => "Refer&#234;ncias"
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        0 => array:2 [
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                0 => array:1 [
                  "referenciaCompleta" => "Hoffman EP&#44; Barr ML&#44; Giovanni MA&#44; Murray MF&#44; et al&#46; Lysosomal Acid Lipase Deficiency&#46; 2015&#46; p&#46; 1&#8211;14 &#91;Updated 2016 Sep 1&#93;&#46; In&#58; Adam MP&#44; Ardinger HH&#44; Pagon RA&#44; et al&#46; editors&#46; GeneReviews<span class="elsevierStyleSup">&#174;</span> &#91;Internet&#93;&#46; Seattle &#40;WA&#41;&#58; University of Washington&#44; Seattle&#59; 1993&#8211;2018&#46; Dispon&#237;vel em&#58; https&#58;&#47;&#47;www&#46;ncbi&#46;nlm&#46;nih&#46;gov&#47;books&#47;NBK305870 &#91;acesso 10&#46;10&#46;17&#93;&#46;"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cholesteryl ester storage disease&#58; review of the findings in 135 reported patients with an underdiagnosed disease"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "D&#46;L&#46; Bernstein"
                            1 => "H&#46; H&#252;lkova"
                            2 => "M&#46;G&#46; Bialer"
                            3 => "R&#46;J&#46; Desnick"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jhep.2013.02.014"
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                        "tituloSerie" => "J Hepatol"
                        "fecha" => "2013"
                        "volumen" => "58"
                        "paginaInicial" => "1230"
                        "paginaFinal" => "1243"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23485521"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
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              "identificador" => "bib0145"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Wolman&#39;s disease and cholesteryl ester storage disorder&#58; the phenotypic spectrum of lysosomal acid lipase deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
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                            1 => "C&#46; Kelly"
                            2 => "T&#46; Wang"
                            3 => "C&#46; Livingstone"
                            4 => "A&#46; Ala"
                          ]
                        ]
                      ]
                    ]
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                    0 => array:2 [
                      "doi" => "10.1016/S2468-1253(17)30052-3"
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                        "tituloSerie" => "Lancet Gastroenterol Hepatol"
                        "fecha" => "2017"
                        "volumen" => "2"
                        "paginaInicial" => "670"
                        "paginaFinal" => "679"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28786388"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
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              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Genetic and biochemical evidence that CESD and Wolman disease are distinguished by residual lysosomal acid lipase activity"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
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                            4 => "H&#46; Klima"
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                    0 => array:2 [
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                        "tituloSerie" => "Genomics"
                        "fecha" => "1996"
                        "volumen" => "33"
                        "paginaInicial" => "85"
                        "paginaFinal" => "93"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8617513"
                            "web" => "Medline"
                          ]
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Role of lysosomal acid lipase in the metabolism density lipoprotein of plasma low density lipoprotein observations in cultured fibroblasts from a patient with cholesteryl ester storage disease"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
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                        "paginaInicial" => "8487"
                        "paginaFinal" => "8495"
                        "link" => array:1 [
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                    0 => array:2 [
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                          "etal" => false
                          "autores" => array:4 [
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                          ]
                        ]
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                    0 => array:2 [
                      "doi" => "10.1016/j.ymgme.2011.11.004"
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                        "tituloSerie" => "Mol Genet Metab"
                        "fecha" => "2012"
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                            1 => "H&#46; Wiebusch"
                            2 => "M&#46; Jansen-Rust"
                            3 => "S&#46; Rust"
                            4 => "U&#46; Seedorf"
                            5 => "H&#46; Schulte"
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                        "tituloSerie" => "Arterioscler Thromb Vasc Biol"
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                        "paginaInicial" => "1866"
                        "paginaFinal" => "1868"
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                          0 => array:2 [
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                      "titulo" => "Cholesteryl ester storage disease&#58; protean presentations of lysosomal acid lipase deficiency"
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                          "etal" => false
                          "autores" => array:2 [
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                          ]
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                        "tituloSerie" => "J Pediatr Gastroenterol Nutr"
                        "fecha" => "2013"
                        "volumen" => "56"
                        "paginaInicial" => "682"
                        "paginaFinal" => "685"
                        "link" => array:1 [
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                      "titulo" => "Lysosomal acid lipase deficiency &#8211; an under&#8208;recognized cause of dyslipidaemia and liver dysfunction"
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                    0 => array:1 [
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                        "volumen" => "7"
                        "paginaInicial" => "446"
                        "paginaFinal" => "450"
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                          0 => array:2 [
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
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                          "etal" => false
                          "autores" => array:3 [
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                        "fecha" => "2015"
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                        "paginaInicial" => "696"
                        "paginaFinal" => "697"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26503675"
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                          0 => array:2 [
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                      "titulo" => "Lysosomal acid lipase deficiency allograft recurrence and liver failure &#8211; clinical outcomes of 18 liver transplantation patients"
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                        "paginaFinal" => "19"
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                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29655841"
                            "web" => "Medline"
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                      ]
                    ]
                  ]
                ]
              ]
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            23 => array:3 [
              "identificador" => "bib0250"
              "etiqueta" => "24"
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                      "titulo" => "Pathological evidence of Wolman&#39;s disease following hematopoietic stem cell transplantation despite correction of lysosomal acid lipase activity"
                      "autores" => array:1 [
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                    ]
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                    0 => array:2 [
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                        "paginaInicial" => "449"
                        "paginaFinal" => "450"
                        "link" => array:1 [
                          0 => array:2 [
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                            "web" => "Medline"
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                      ]
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              ]
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                    0 => array:2 [
                      "titulo" => "Wolman disease successfully treated by bone marrow transplantation"
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                            5 => "J&#46;E&#46; Wagner"
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                        "link" => array:1 [
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Artigo Original
Lysosomal acid lipase deficiency in Brazilian children: a case series
Deficiência de lipase ácida lisossomal em crianças brasileiras: casuística
Gabriel Nuncio Benevidesa,
Autor para correspondência
gnbenevides@gmail.com

Autor para correspondência.
, Irene Kazue Miurab, Natalia Canale Persona, Renata Pereira Sustovich Puglieseb, Vera Lúcia Baggio Danesib, Fabiana Roberto Limac, Gilda Portab
a Instituto da Criança, Hospital das Clínicas, Unidade de Gastroenterologia, Hepatologia e Nutrologia Pediátrica, São Paulo, SP, Brasil
b Hospital Menino Jesus, Departamento de Hepatologia Pediátrica, São Paulo, SP, Brasil
c Hospital das Clínicas, Departamento de Patologia, São Paulo, SP, Brasil
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pois os estudos populacionais prop&#245;em que sua incid&#234;ncia pode ser maior do que a descrita anteriormente&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">2&#44;3&#44;7</span></a> Adicionalmente&#44; o aumento da conscientiza&#231;&#227;o cl&#237;nica resultou em mais diagn&#243;sticos&#44; corroborou a ideia de que ela pode ser uma doen&#231;a subdiagnosticada&#46; Foram descritas muitas variantes no gene da lipase &#225;cida lisossomal &#40;LIPA&#41; e novos estudos ainda escrevem novas muta&#231;&#245;es&#59; atualmente&#44; n&#227;o foi encontrada uma correla&#231;&#227;o gen&#243;tipo&#8208;fen&#243;tipo&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#44;2&#44;8&#44;9</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Neste estudo&#44; analisamos sete pacientes com DLAL e acreditamos ser a maior s&#233;rie relatada na Am&#233;rica do Sul&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">10</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">M&#233;todos</span><p id="par0015" class="elsevierStylePara elsevierViewall">Fizemos uma an&#225;lise retrospectiva dos prontu&#225;rios m&#233;dicos dos pacientes com DLAL tratados em nossa cl&#237;nica ambulatorial&#46; Todos os dados com rela&#231;&#227;o &#224;s caracter&#237;sticas demogr&#225;ficas&#44; cl&#237;nicas&#44; laboratoriais e moleculares foram recuperados e&#44; ent&#227;o&#44; analisados&#46; Todos os pacientes e seus pais &#40;ou respons&#225;veis&#41; estavam cientes da inten&#231;&#227;o de publicar seus prontu&#225;rios&#44; concordaram com isso e assinaram um consentimento informado&#44; de acordo com as diretrizes do Comit&#234; de &#201;tica de nossa institui&#231;&#227;o&#46; Este estudo foi aprovado pelo Comit&#234; de &#201;tica&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">O Departamento de Laborat&#243;rios do Seattle Children&#39;s fez todos os testes da atividade da LAL e a an&#225;lise molecular com o uso de amostra de sangue seco &#40;DBS&#41;&#46; Os testes da atividade da LAL foram feitos com o m&#233;todo descrito por Hamilton et al&#46; em 2012&#58; foi usado 4&#8208;metilumbeliferil palmitato como substrato para a atividade da lipase total determinada&#59; como sangue total para outras lipases&#44; Lalistat 2&#44; um inibidor de LAL&#44; foi acrescentado &#224; amostra para medir a atividade da lipase pancre&#225;tica e&#44; por fim&#44; foi calculada a atividade da LAL&#46; As unidades usadas foram pmol&#47;hr&#47;spt&#46; Foi feito o sequenciamento de Sanger do gene LIPA em cada paciente com baixa atividade da LAL&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">11</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Resultados &#40;descri&#231;&#245;es dos casos&#41;</span><p id="par0025" class="elsevierStylePara elsevierViewall">Revisamos os prontu&#225;rios m&#233;dicos de sete crian&#231;as com DLAL &#40;5<span class="elsevierStyleHsp" style=""></span>M&#58; 2 F&#41;&#59; seis eram pardas e uma negra&#46; As idades m&#233;dia e mediana no in&#237;cio dos sintomas foram cinco anos &#40;quatro meses a nove anos&#41; e seis anos e no diagn&#243;stico foram 6&#44;9 a &#40;tr&#234;s a 10 anos&#41; e 6&#44;5 anos&#44; respectivamente&#46; O tempo entre o diagn&#243;stico e o in&#237;cio dos sintomas variou amplamente e &#233; mostrado na <a class="elsevierStyleCrossRef" href="#tbl0005">tabela 1</a>&#44; juntamente com outras caracter&#237;sticas cl&#237;nicas&#46; Dois pacientes eram assintom&#225;ticos e a suspeita cl&#237;nica surgiu devido &#224; hepatomegalia&#46; Seis pacientes apresentaram hepatomegalia e nenhum apresentou esplenomegalia nem desenvolveu icter&#237;cia&#46; Dois pacientes irm&#227;os apresentaram consanguinidade&#46; Cinco dos sete pacientes apresentaram hist&#243;rico familiar de dislipidemia&#46; Ap&#243;s suspeita cl&#237;nica e laboratorial em nossos pacientes&#44; foi medida a atividade da LAL para confirmar DLAL&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Uma caracter&#237;stica da DLAL &#233; a heterogeneidade cl&#237;nica dos sintomas na apresenta&#231;&#227;o&#46; &#201; obrigat&#243;ria uma descri&#231;&#227;o cl&#237;nica detalhada dos casos&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">O paciente 1 foi trazido a nossa instala&#231;&#227;o ambulatorial com dor abdominal&#44; sem sinal alarmante&#44; e tinha oito anos&#46; Ap&#243;s avalia&#231;&#227;o cont&#237;nua&#44; observamos hepatomegalia e a endoscopia digestiva alta mostrou histi&#243;citos azul marinho no exame histol&#243;gico de bi&#243;psia da mucosa do duodeno&#46; A medi&#231;&#227;o da atividade da LAL confirmou a DLAL&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">O paciente 3&#44; durante uma consulta pedi&#225;trica de rotina aos seis anos&#44; apresentou hepatomegalia&#46; Os exames iniciais mostraram um aumento de 2&#8208;3 vezes da alanina aminotransferase &#40;ALT&#41;&#44; baixo n&#237;vel de lipoprote&#237;na de alta densidade &#40;HDL&#41; e n&#237;vel elevado de lipoprote&#237;na de baixa densidade &#40;LDL&#41;&#46; A bi&#243;psia do f&#237;gado mostrou dep&#243;sitos lip&#237;dicos nos histi&#243;citos&#46; Mais tarde&#44; a atividade da LAL medida aos sete anos foi diagn&#243;stico de DLAL&#46; Sua irm&#227;&#44; a paciente 2&#44; tamb&#233;m apresentou enzimas hep&#225;ticas ligeiramente elevadas aos tr&#234;s anos&#44; sem hepatomegalia&#44; e foi diagnosticada ao mesmo tempo em que seu irm&#227;o com DLAL&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">O paciente 4&#44; que tinha quatro meses de idade&#44; apresentou diarreia progressiva e intrat&#225;vel &#40;primeira ocorr&#234;ncia em 1991&#41;&#44; d&#233;ficit de crescimento&#44; hepatomegalia&#44; dislipidemia e calcifica&#231;&#245;es adrenais&#46; Aos tr&#234;s anos&#44; uma bi&#243;psia hep&#225;tica revelou esteatose microvesicular significativa&#44; fibrose septal e agregados de macr&#243;fagos com microvacuoliza&#231;&#227;o citoplasm&#225;tica &#8211; achados sugestivos de Niemann&#8208;Pick naquela &#233;poca&#44; pois tamb&#233;m apresentou baixos n&#237;veis de esfingomielinase e n&#237;veis elevados de fosfatase &#225;cida&#46; Contudo&#44; a microscopia eletr&#244;nica mostrou imagens negativas de cristais de colesterol e vac&#250;olos lip&#237;dicos nos hepat&#243;citos e histi&#243;citos&#59; assim&#44; a DLAL foi favorecida por esse m&#233;todo&#46; Sua diarreia cr&#244;nica e doen&#231;a hep&#225;tica progressiva continuaram&#46; Ao atingir seis anos&#44; ele desenvolveu v&#225;rios epis&#243;dios de pieira&#46; Os raios X e a tomografia de t&#243;rax mostraram sinais de pneumonite intersticial&#46; A espirometria revelou um padr&#227;o restritivo&#46; Aos 13 anos&#44; desenvolveu complica&#231;&#245;es devido &#224; insufici&#234;ncia hep&#225;tica com ascite e apresentou peritonite bacteriana espont&#226;nea duas vezes&#46; Aos 18 anos&#44; no in&#237;cio de 2010&#44; foi submetido a uma cirurgia de transplante de f&#237;gado de um doador morto&#46; Seu f&#237;gado explantado &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>A e B&#41; apresentou cirrose micronodular e caracter&#237;sticas espec&#237;ficas da doen&#231;a&#44; inclusive algumas fendas em forma de agulha dentro das c&#233;lulas Kupffer microvacuoladas&#46; Antes do transplante de f&#237;gado&#44; o paciente desenvolveu pequena esplenomegalia &#40;2<span class="elsevierStyleHsp" style=""></span>cm da borda costal esquerda&#41; e a mesma apresentou redu&#231;&#227;o de cerca de 50&#37; ap&#243;s transplante de f&#237;gado&#46; Ap&#243;s transplante do f&#237;gado&#44; apresentou v&#225;rios epis&#243;dios de rejei&#231;&#227;o&#44; que melhoraram com corticosteroides&#46; Sua bi&#243;psia hep&#225;tica em 2015 mostrou rejei&#231;&#227;o cr&#244;nica com ductopenia &#40;perda de 50&#37;&#41;&#44; fibrose portal e centrolobular&#44; septos raros&#44; perivenulite central e algumas c&#233;lulas de Kupffer espumosas &#40;geralmente s&#227;o uma resposta a colestase nesse contexto&#41;&#46; Contudo&#44; sua bi&#243;psia hep&#225;tica em 2017 mostrou muitos macr&#243;fagos xantomatosos no trato portal&#44; fortemente sugestivo de recrudescimento da doen&#231;a &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>C&#41;&#44; juntamente com fibrose em ponte marcada &#40;F3&#41;&#44; rejei&#231;&#227;o cr&#244;nica e c&#233;lulas sinusoidais espumosas raras&#46; A atividade da enzima LAL medida em 2015 foi zero&#46; O perfil lip&#237;dico do paciente melhorou para n&#237;veis normais de LDL e triglicer&#237;deos &#40;TG&#41; ap&#243;s transplante de f&#237;gado&#44; por&#233;m os baixos n&#237;veis de HDL continuaram&#46; Al&#233;m disso&#44; uma bi&#243;psia duodenal feita em 2016 mostrou in&#250;meros macr&#243;fagos xantomatosos na l&#226;mina pr&#243;pria &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>D&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0050" class="elsevierStylePara elsevierViewall">O paciente 5&#44; com oito anos&#44; apresentou dor abdominal cr&#244;nica juntamente com hepatomegalia e aumento da aspartato aminotransferase &#40;AST&#41; e ALT&#44; juntamente com baixo HDL e alto LDL&#46; Ap&#243;s investiga&#231;&#227;o inicial inconclusiva&#44; uma bi&#243;psia de f&#237;gado revelou achados sugestivos de doen&#231;a de Niemann&#8208;Pick&#46; Como o curso cl&#237;nico n&#227;o foi sugestivo de Niemann&#8208;Pick&#44; a medi&#231;&#227;o da atividade da LAL diagnosticou DLAL&#46; Sua tia tamb&#233;m foi diagnosticada anteriormente com doen&#231;a de Niemann&#8208;Pick e&#44; mais tarde&#44; com DLAL&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">O paciente 6 apresentou dor abdominal cr&#244;nica por volta dos seis anos&#46; A avalia&#231;&#227;o laboratorial inicial mostrou ligeiro aumento na AST e ALT&#44; HDL baixa e LDL alta e esteatose no ultrassom do f&#237;gado&#46; Ele n&#227;o estava acima do peso e n&#227;o havia consumido altas doses de frutose&#44; sinais sugestivos de doen&#231;a hep&#225;tica gordurosa n&#227;o alco&#243;lica &#40;DHGNA&#41; ou esteatose hep&#225;tica&#46; Portanto&#44; medimos a atividade da LAL e confirmamos DLAL&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">O paciente 7&#44; um menino de dois anos&#44; foi internado em nosso hospital devido &#224; dificuldade de andar&#46; Ap&#243;s exame f&#237;sico inicial&#44; detectamos hepatomegalia&#46; A an&#225;lise laboratorial indicou claramente raquitismo&#46; Aos tr&#234;s anos&#44; o paciente foi transferido para nossa cl&#237;nica de hepatologia para investigar hepatomegalia&#46; Uma bi&#243;psia hep&#225;tica&#44; feita quando o paciente tinha seis anos&#44; sugeriu doen&#231;as metab&#243;licas&#44; principalmente glicogenose&#46; Contudo&#44; o paciente nunca desenvolveu hipoglicemia&#46; Em 2016&#44; ap&#243;s medir a atividade da LAL&#44; diagnosticamos DLAL&#46; Ap&#243;s um tempo&#44; o patologista reavaliou as l&#226;minas da bi&#243;psia hep&#225;tica&#46; Os achados histopatol&#243;gicos &#40;<a class="elsevierStyleCrossRef" href="#fig0010">fig&#46; 2</a>&#41; das poucas c&#233;lulas de Kupffer espumosas e alguns macr&#243;fagos do trato portal fracamente positivos para &#225;cido peri&#243;dico de Schiff &#40;PAS&#41; ap&#243;s digest&#227;o diast&#225;sica&#44; a aus&#234;ncia das c&#233;lulas hep&#225;ticas inchadas caracter&#237;sticas que se assemelham a c&#233;lulas vegetais&#44; sem mencionar a presen&#231;a de esteatose grau 3&#44; s&#227;o sinais de diagn&#243;stico de DLAL &#40;al&#233;m de outros diagn&#243;sticos diferenciais&#44; como mucopolissacaridoses&#41;&#46; Vale mencionar que esse caso mostrou somente mudan&#231;as sutis nas c&#233;lulas de Kupffer&#44; tornou o diagn&#243;stico mais desafiador&#44; possivelmente relacionado &#224; variabilidade de amostragem das bi&#243;psias de f&#237;gado&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">A <a class="elsevierStyleCrossRef" href="#tbl0010">tabela 2</a> resume as caracter&#237;sticas laboratoriais de nossos pacientes&#46; A an&#225;lise gen&#233;tica revelou uma rara variante p&#46;L89P &#40;c&#46;266T<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>C&#41; em tr&#234;s pacientes&#44; algo nunca descrito no Brasil&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">12</span></a> Nenhum desses pacientes estava relacionado com o outro&#46; Um era homozigoto para p&#46;L89P e os outros eram heterozigotos&#46; As variantes c&#46;894G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&#44; uma variante no local de porte na extremidade 3&#8217; do &#233;xon 8&#44; e c&#46;67G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A encontrada em nossos pacientes&#44; j&#225; foram comumente descritas&#46;</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Discuss&#227;o</span><p id="par0070" class="elsevierStylePara elsevierViewall">Relatamos sete pacientes com DLAL&#44; a maior casu&#237;stica j&#225; descrita na Am&#233;rica do Sul&#46; Os diagn&#243;sticos dos pacientes foram confirmados ap&#243;s alta suspeita cl&#237;nica que levou &#224; medica&#231;&#227;o da atividade da LAL&#44; que foi invariavelmente baixa&#46; Nossa conscientiza&#231;&#227;o cl&#237;nica da DLAL melhorou ao longo dos anos e isso explica os intervalos de tempo vari&#225;veis entre o in&#237;cio dos sintomas e o diagn&#243;stico&#46; Os tr&#234;s pacientes com rela&#231;&#227;o aos quais levamos mais de um ano para chegar ao diagn&#243;stico s&#227;o aqueles que apresentaram sintomas at&#237;picos no acometimento&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">O paciente 4 apresentou sintomas severos que se assemelham &#224; doen&#231;a de Wolman&#44; por&#233;m tamb&#233;m apresentou pneumonite intersticial&#44; baixa esfingomielinase e altos n&#237;veis de fosfatase &#225;cida&#44; que s&#227;o vari&#225;veis de confus&#227;o&#44; pois esses achados s&#227;o sugestivos de doen&#231;a de Niemann&#8208;Pick&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">O paciente 7 apresentou hepatomegalia e a bi&#243;psia hep&#225;tica inicial sugeriu uma doen&#231;a de armazenamento&#44; como glicogenose&#44; pois alguns achados &#40;por exemplo&#44; c&#233;lulas de Kupffer espumosas&#41; n&#227;o foram muito vis&#237;veis na amostra do tecido&#46; A reavalia&#231;&#227;o das l&#226;minas do f&#237;gado&#44; ap&#243;s considerar as novas informa&#231;&#245;es de DLAL&#44; foi compat&#237;vel com a doen&#231;a&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Esses 2 casos mencionados &#40;dos pacientes 4 e 7&#41; acrescentaram a DLAL como um poss&#237;vel diagn&#243;stico diferenciado de glicogenose e Niemann&#8208;Pick tipo B&#46; A doen&#231;a de Gaucher&#44; Niemann&#8208;Pick tipo C e NAFLD j&#225; foram descritas como diagn&#243;sticos diferenciais de DLAL&#46;<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">13&#44;14</span></a> Os diagn&#243;sticos diferenciais de DLAL s&#227;o amplos e geralmente sobrep&#245;em outras doen&#231;as cr&#244;nicas&#46; Consequentemente&#44; uma raz&#227;o pela qual a preval&#234;ncia real da DLAL ser desconhecida &#233; o fato de ela ser uma doen&#231;a subdiagnosticada&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">2&#44;3&#44;7&#8211;9&#44;15&#44;16</span></a> Deve haver alta suspeita cl&#237;nica de DLAL em pacientes com hepatomegalia n&#227;o explicada&#44; altos n&#237;veis de LDL e triglicer&#237;deos&#44; baixos n&#237;veis de HDL e enzimas hep&#225;ticas levemente elevadas&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#44;2&#44;8</span></a> Seis de nossos pacientes apresentaram hepatomegalia&#44; todos apresentaram um padr&#227;o de colesterol t&#237;pico e cinco apresentaram enzimas hep&#225;ticas levemente elevadas&#46; Esses resultados mostram que h&#225; um padr&#227;o de achados para diagnosticar DLAL&#44; mesmo em casos at&#237;picos&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">O LIPA&#44; o gene que codifica a LAL&#44; est&#225; localizado no cromossomo 10q23&#46;31&#46; Sabe&#8208;se que muitas variantes nesse gene est&#227;o associadas &#224; DLAL&#46; A variante c&#46;894<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>1G&#62;A&#160;&#40;E8SJM<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>1&#41; &#233; a mais comum e &#233; respons&#225;vel por mais da metade dos casos publicados&#59; tr&#234;s de nossos pacientes apresentaram essa variante&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">1&#44;2</span></a> A outra variante&#44; c&#46;67G&#62;A&#44; j&#225; foi relatada tr&#234;s vezes no site ClinVar&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">17</span></a> Curiosamente&#44; descrevemos neste trabalho uma variante rara&#44; a c&#46;266T&#62;C &#40;p&#46;L89P&#41;&#44; que foi encontrada em tr&#234;s pacientes&#46; Essa variante patog&#234;nica foi descrita somente uma vez em uma apresenta&#231;&#227;o em p&#244;ster na Reuni&#227;o de 2014<span class="elsevierStyleHsp" style=""></span>da Sociedade Americana de Gen&#233;tica Humana&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">12</span></a> Propusemos um poss&#237;vel gen&#243;tipo brasileiro para a DLAL&#44; pois nenhum dos pacientes com essa variante estava relacionado a outro&#46; O paciente homozigoto para a &#250;ltima variante mostrou a apresenta&#231;&#227;o mais grave com envolvimento pulmonar&#44; diarreia cr&#244;nica e doen&#231;a hep&#225;tica progressiva&#44; necessitou de transplante de f&#237;gado &#40;paciente 4&#41;&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">A DLAL &#233; classicamente definida como uma doen&#231;a recessiva autoss&#244;mica&#46; Contudo&#44; os pacientes 1&#44; 5 e 6 apresentaram um alelo mutante e um alelo tipo selvagem&#46; Todos esses pacientes apresentaram sinais laboratoriais e cl&#237;nicos sugestivos de DLAL &#40;hepatomegalia&#44; baixa HDL&#44; alta LDL e baixa atividade da LAL&#41; e foram exclu&#237;das outras doen&#231;as prov&#225;veis&#46; A doen&#231;a de Niemann&#8208;Pick foi o principal diagn&#243;stico de confus&#227;o para o paciente 5&#44; pois ele tinha uma tia com a doen&#231;a de Niemann&#8208;Pick&#46; Contudo&#44; ap&#243;s nosso diagn&#243;stico&#44; investigamos sua tia e pudemos diagnostic&#225;&#8208;la com DLAL&#46; DHGNA e intoler&#226;ncia &#224; frutose foram outros diagn&#243;sticos exclu&#237;dos&#44; pois elas podem surgir com hepatomegalia&#44; esteatose hep&#225;tica e especificamente DHGNA com dislipidemia&#46; Outra hip&#243;tese &#233; que esses pacientes podem ter muta&#231;&#245;es intr&#244;nicas que n&#227;o foram reconhecidas por meio do sequenciamento de Sanger ou que a redu&#231;&#227;o da atividade da LAL em alguns pacientes heterozigotos pode causar os sintomas&#46; Pullinger et al&#46; j&#225; descreveram os pacientes heterozigotos para as variantes patog&#234;nicas do gene LIPA que apresentaram sintomas e os dados laboratoriais sugestivos de DLAL&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">2&#44;18</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">O tratamento e o progn&#243;stico da DLAL mudaram drasticamente nos &#250;ltimos anos ap&#243;s a cria&#231;&#227;o do sebelipase alfa &#40;Alexion&#44; Kanuma&#174;&#44; Kanuma&#8482;&#44; WA&#44; EUA&#41;&#44; uma terapia de reposi&#231;&#227;o enzim&#225;tica &#40;TRE&#41; que mostrou melhorias no dano hep&#225;tico e nas anomalias lip&#237;dicas&#46;<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">19&#8211;22</span></a> O transplante de f&#237;gado para DLAL &#233; outro poss&#237;vel tratamento para casos graves e foi relatado somente 20 vezes na literatura m&#233;dica&#44; por&#233;m poucos trabalhos relatam acompanhamento de longo prazo desses pacientes&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">2</span></a> Bernstein et al&#46; publicaram dois casos de pacientes com DLAL submetidos a transplante hep&#225;tico e&#44; nos dois&#44; a doen&#231;a DLAL multissist&#234;mica reapareceu ap&#243;s o transplante&#46; Eles tamb&#233;m revisaram a literatura de 18 casos de pacientes com DLAL submetidos a transplante de f&#237;gado e descreveram a progress&#227;o multissist&#234;mica da DLAL em 11 pacientes &#40;66&#37;&#41; e &#243;bito em seis pacientes &#40;33&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">23</span></a> Em nosso estudo&#44; o paciente 4 foi submetido a transplante de f&#237;gado e&#44; ap&#243;s cinco anos de acompanhamento&#44; apresentou rejei&#231;&#227;o cr&#244;nica e manteve baixos n&#237;veis de HDL&#46; Curiosamente&#44; esse paciente ainda tinha deposi&#231;&#227;o de colesterol nos macr&#243;fagos e mostrou histi&#243;citos xantomatosos na bi&#243;psia de duodeno em 2016 e&#44; mais tarde&#44; desenvolveu macr&#243;fagos espumosos no trato portal na bi&#243;psia de f&#237;gado ap&#243;s sete anos de transplante de f&#237;gado&#46; Portanto&#44; o transplante de f&#237;gado n&#227;o &#233; a cura para essa doen&#231;a&#44; pois os macr&#243;fagos continuam a ser prejudicados&#44; causam sintomas&#44; pois outros &#243;rg&#227;os&#44; como a medula &#243;ssea e o intestino delgado&#44; ainda conter&#227;o os macr&#243;fagos carregados de lip&#237;dios&#46; Por outro lado&#44; acredita&#8208;se que o transplante de medula &#243;ssea &#40;TMO&#41; corrige o defeito da enzima e&#44; portanto&#44; se feito no in&#237;cio do curso da doen&#231;a&#44; pode salvar a vida dos pacientes com a doen&#231;a de Wolman&#46; Foram relatados alguns TMOs bem&#8208;sucedidos na DLAL&#59; contudo&#44; a maior parte dos casos tem um progn&#243;stico ruim e a TER imediata &#233; a terapia de escolha&#46;<a class="elsevierStyleCrossRefs" href="#bib0250"><span class="elsevierStyleSup">24&#8211;26</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Em conclus&#227;o&#44; relatamos a maior casu&#237;stica de pacientes com DLAL na Am&#233;rica do Sul e descrevemos um gen&#243;tipo raro&#44; possivelmente um gen&#243;tipo brasileiro&#46; Enfatizamos que a DLAL pode apresentar sinais e sintomas heterog&#234;neos&#44; por&#233;m ela deve ser inclu&#237;da como um diagn&#243;stico diferenciado de hepatomegalia&#44; enzimas hep&#225;ticas elevadas e dislipidemia&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Conflitos de interesse</span><p id="par0110" class="elsevierStylePara elsevierViewall">Os autores declaram n&#227;o haver conflitos de interesse&#46;</p></span></span>"
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    "fechaRecibido" => "2017-10-18"
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            0 => "Lysosomal storage diseases"
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            0 => "Doen&#231;as do armazenamento lisossomal"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">To describe the demographic&#44; clinical&#44; laboratory and molecular characteristics of patients with lysosomal acid lipase deficiency&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A retrospective review of the medical records of children with the disease&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Seven children with lysosomal acid lipase deficiency &#40;5 male&#59; 2 female&#41;&#59; 6 were mixed race&#44; and 1 was black&#46; The mean ages at the first onset of symptoms and at diagnosis were 5&#46;0 years &#40;4 months to 9 years&#41; and 6&#46;9 years &#40;3&#8211;10 years&#41;&#44; respectively&#46; Symptom manifestations at onset were&#58; 3 patients had abdominal pain&#44; one had bone&#47;joint pain due to rickets&#44; and 1 had chronic diarrhea and respiratory insufficiency due to interstitial pneumonitis&#46; One was asymptomatic&#44; and clinical suspicion arose due to hepatomegaly&#46; Six patients had hepatomegaly&#44; and none had splenomegaly&#46; Two patients were siblings&#46; Enzymatic assay and molecular analysis confirmed the diagnoses&#46; Genetic analysis revealed a rare pathogenic variant &#40;p&#46;L89P&#41; in three patients&#44; described only once in medical literature and never described in Brazil&#46; None of those patients were related to each other&#46; Lysosomal acid lipase deficiency was previously described as an autosomal recessive disease&#44; but three patients were heterozygous and undoubtedly had the disease &#40;low enzyme activity&#44; suggestive lab findings and clinical symptoms&#41;&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">This case series supports that lysosomal acid lipase deficiency can present with highly heterogeneous signs and symptoms among patients&#44; but it should be considered in children presenting with gastrointestinal symptoms associated with dyslipidemia&#46; We describe a rare variant in three non&#8208;related patients that may suggest a Brazilian genotype for lysosomal acid lipase deficiency&#46;</p></span>"
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        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Objetivo</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Descrever as caracter&#237;sticas demogr&#225;ficas&#44; cl&#237;nicas&#44; laboratoriais e moleculares de pacientes com defici&#234;ncia de lipase &#225;cida lisossomal&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">M&#233;todos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">An&#225;lise retrospectiva dos prontu&#225;rios m&#233;dicos de crian&#231;as com a defici&#234;ncia de lipase &#225;cida lisossomal&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Sete crian&#231;as com defici&#234;ncia de lipase &#225;cida lisossomal &#40;5<span class="elsevierStyleHsp" style=""></span>M&#58;2F&#41;&#59; seis eram pardas e uma negra&#46; As faixas et&#225;rias no in&#237;cio dos sintomas e no diagn&#243;stico foram 5 anos &#40;4 meses a 9 anos&#41; e 6&#44;9 anos &#40;3 a 10 anos&#41;&#44; respectivamente&#46; As manifesta&#231;&#245;es dos sintomas no in&#237;cio foram as que seguem&#58; tr&#234;s pacientes apresentaram dor abdominal&#44; um apresentou dor nos ossos&#47;articula&#231;&#245;es devido a raquitismo e um apresentou diarreia cr&#244;nica e insufici&#234;ncia respirat&#243;ria devido &#224; pneumonite intersticial&#46; Os outros n&#227;o apresentaram sintomas e a suspeita cl&#237;nica surgiu devido &#224; hepatomegalia&#46; Seis pacientes apresentaram hepatomegalia e um apresentou esplenomegalia&#46; Dois pacientes eram irm&#227;os&#46; O ensaio enzim&#233;tico e a an&#225;lise molecular confirmaram os diagn&#243;sticos&#46; A an&#225;lise gen&#233;tica revelou uma variante patog&#234;nica rara &#40;p&#46;L89P&#41; em tr&#234;s pacientes&#44; descrita uma &#250;nica vez na literatura m&#233;dica e nunca descrita no Brasil&#46; Nenhum desses pacientes tinha parentesco com os outros&#46; A defici&#234;ncia de lipase &#225;cida lisossomal foi anteriormente descrita como uma doen&#231;a recessiva autoss&#244;mica&#44; por&#233;m tr&#234;s pacientes eram heterozigotos e&#44; sem d&#250;vida&#44; apresentaram a doen&#231;a &#40;atividade enzim&#225;tica baixa&#44; achados laboratoriais sugestivos e sintomas cl&#237;nicos&#41;&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclus&#227;o</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Esta casu&#237;stica afirma que a defici&#234;ncia de lipase &#225;cida lisossomal pode se manifestar com sinais e sintomas altamente heterog&#234;neos entre os pacientes&#44; por&#233;m deve ser considerada em crian&#231;as que apresentam sintomas gastrointestinais associados &#224; dislipidemia&#46; Descrevemos uma variante rara em tr&#234;s pacientes n&#227;o relacionados que pode sugerir um gen&#243;tipo brasileiro para defici&#234;ncia de lipase &#225;cida lisossomal&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0030">Como citar este artigo&#58; Benevides GN&#44; Miura IK&#44; Person NC&#44; Pugliese RP&#44; Danesi VL&#44; Lima FR&#44; et al&#46; Lysosomal acid lipase deficiency in Brazilian children&#58; a case series&#46; J Pediatr &#40;Rio J&#41;&#46; 2019&#59;95&#58;552&#8211;8&#46;</p>"
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                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Idade no diagn&#243;stico&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 a 5 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tempo do in&#237;cio dos sintomas at&#233; o diagn&#243;stico&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 a 5 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 a 8 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 m&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8 a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Sintomas&#47;sinais no in&#237;cio&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor abdominal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Diarreia cr&#244;nica&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor abdominal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor abdominal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Dor &#243;ssea&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tamanho do f&#237;gado<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">d</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 cm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Hist&#243;rico familiar&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Pai com dislipidemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tio com DLAL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">M&#227;e com lupus&#59; tia com l&#250;pus e com DLAL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Pai com dislipidemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">4&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">5&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">6&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">7&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">AST &#40;ULN&#41; U&#47;L&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">28 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">72 &#40;15&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">22 &#40;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">188&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">61 &#40;37&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">51 &#40;40&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t">&#947;GT &#40;ULN&#41;&#160;U&#47;L&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">26 &#40;25&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t">AP &#40;ULN&#41; U&#47;L&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t">3872 &#40;383&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">TC &#40;ULN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>200&#41; mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">HDL &#40;LLN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>45&#41; mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">38&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">LDL &#40;LSN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>100&#41;&#160;mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">TG &#40;LSN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>150&#41;&#160;mg&#47;dl&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t">INR&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8208;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">0&#44;99&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Atividade da LAL &#40;LIN<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>40&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t  " align="left" valign="\n
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              "identificador" => "bib0135"
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                0 => array:1 [
                  "referenciaCompleta" => "Hoffman EP&#44; Barr ML&#44; Giovanni MA&#44; Murray MF&#44; et al&#46; Lysosomal Acid Lipase Deficiency&#46; 2015&#46; p&#46; 1&#8211;14 &#91;Updated 2016 Sep 1&#93;&#46; In&#58; Adam MP&#44; Ardinger HH&#44; Pagon RA&#44; et al&#46; editors&#46; GeneReviews<span class="elsevierStyleSup">&#174;</span> &#91;Internet&#93;&#46; Seattle &#40;WA&#41;&#58; University of Washington&#44; Seattle&#59; 1993&#8211;2018&#46; Dispon&#237;vel em&#58; https&#58;&#47;&#47;www&#46;ncbi&#46;nlm&#46;nih&#46;gov&#47;books&#47;NBK305870 &#91;acesso 10&#46;10&#46;17&#93;&#46;"
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              "identificador" => "bib0140"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cholesteryl ester storage disease&#58; review of the findings in 135 reported patients with an underdiagnosed disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "D&#46;L&#46; Bernstein"
                            1 => "H&#46; H&#252;lkova"
                            2 => "M&#46;G&#46; Bialer"
                            3 => "R&#46;J&#46; Desnick"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jhep.2013.02.014"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Hepatol"
                        "fecha" => "2013"
                        "volumen" => "58"
                        "paginaInicial" => "1230"
                        "paginaFinal" => "1243"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23485521"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0145"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Wolman&#39;s disease and cholesteryl ester storage disorder&#58; the phenotypic spectrum of lysosomal acid lipase deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "M&#46; Pericleous"
                            1 => "C&#46; Kelly"
                            2 => "T&#46; Wang"
                            3 => "C&#46; Livingstone"
                            4 => "A&#46; Ala"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/S2468-1253(17)30052-3"
                      "Revista" => array:6 [
                        "tituloSerie" => "Lancet Gastroenterol Hepatol"
                        "fecha" => "2017"
                        "volumen" => "2"
                        "paginaInicial" => "670"
                        "paginaFinal" => "679"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28786388"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
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              "identificador" => "bib0150"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Genetic and biochemical evidence that CESD and Wolman disease are distinguished by residual lysosomal acid lipase activity"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "C&#46; Aslanidis"
                            1 => "S&#46; Ries"
                            2 => "P&#46; Fehringer"
                            3 => "C&#46; B&#252;chler"
                            4 => "H&#46; Klima"
                            5 => "G&#46; Schmitz"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1006/geno.1996.0162"
                      "Revista" => array:6 [
                        "tituloSerie" => "Genomics"
                        "fecha" => "1996"
                        "volumen" => "33"
                        "paginaInicial" => "85"
                        "paginaFinal" => "93"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8617513"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0155"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Role of lysosomal acid lipase in the metabolism density lipoprotein of plasma low density lipoprotein observations in cultured fibroblasts from a patient with cholesteryl ester storage disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46;L&#46; Goldstein"
                            1 => "S&#46;E&#46; Dana"
                            2 => "J&#46;R&#46; Faust"
                            3 => "A&#46;L&#46; Beaudet"
                          ]
                        ]
                      ]
                    ]
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                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "J Biol Chem"
                        "fecha" => "1975"
                        "volumen" => "250"
                        "paginaInicial" => "8487"
                        "paginaFinal" => "8495"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/172501"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
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              "identificador" => "bib0160"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Structural bases of Wolman disease and cholesteryl ester storage disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "S&#46; Saito"
                            1 => "K&#46; Ohno"
                            2 => "T&#46; Suzuki"
                            3 => "H&#46; Sakuraba"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.ymgme.2011.11.004"
                      "Revista" => array:6 [
                        "tituloSerie" => "Mol Genet Metab"
                        "fecha" => "2012"
                        "volumen" => "105"
                        "paginaInicial" => "244"
                        "paginaFinal" => "248"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22138108"
                            "web" => "Medline"
                          ]
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              ]
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              "identificador" => "bib0165"
              "etiqueta" => "7"
              "referencia" => array:1 [
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                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Prevalence of cholesteryl ester storage disease"
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                        0 => array:2 [
                          "etal" => true
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                            0 => "S&#46; Muntoni"
                            1 => "H&#46; Wiebusch"
                            2 => "M&#46; Jansen-Rust"
                            3 => "S&#46; Rust"
                            4 => "U&#46; Seedorf"
                            5 => "H&#46; Schulte"
                          ]
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                    0 => array:2 [
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                        "tituloSerie" => "Arterioscler Thromb Vasc Biol"
                        "fecha" => "2007"
                        "volumen" => "27"
                        "paginaInicial" => "1866"
                        "paginaFinal" => "1868"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17634524"
                            "web" => "Medline"
                          ]
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cholesteryl ester storage disease&#58; protean presentations of lysosomal acid lipase deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "B&#46; Zhang"
                            1 => "A&#46;F&#46; Porto"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1097/MPG.0b013e31828b36ac"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Pediatr Gastroenterol Nutr"
                        "fecha" => "2013"
                        "volumen" => "56"
                        "paginaInicial" => "682"
                        "paginaFinal" => "685"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23403440"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
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              "identificador" => "bib0175"
              "etiqueta" => "9"
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                0 => array:2 [
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                    0 => array:2 [
                      "titulo" => "Lysosomal acid lipase deficiency &#8211; an under&#8208;recognized cause of dyslipidaemia and liver dysfunction"
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                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "Reiner &#381;"
                            1 => "O&#46; Guardamagna"
                            2 => "D&#46; Nair"
                            3 => "H&#46; Soran"
                            4 => "K&#46; Hovingh"
                            5 => "S&#46; Bertolini"
                          ]
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                    0 => array:2 [
                      "doi" => "10.1016/j.atherosclerosis.2014.04.003"
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                        "tituloSerie" => "Atherosclerosis"
                        "fecha" => "2014"
                        "volumen" => "235"
                        "paginaInicial" => "21"
                        "paginaFinal" => "30"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24792990"
                            "web" => "Medline"
                          ]
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              "identificador" => "bib0180"
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                    0 => array:2 [
                      "titulo" => "Cholesterol ester storage disease&#58; clinical&#44; biochemical&#44; and pathological studies of four new cases"
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                          "etal" => false
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                            2 => "G&#46;C&#46;N&#46; Gallo"
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                      "Revista" => array:6 [
                        "tituloSerie" => "J Pediatr Gastroenterol Nutr"
                        "fecha" => "1988"
                        "volumen" => "7"
                        "paginaInicial" => "446"
                        "paginaFinal" => "450"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/3290419"
                            "web" => "Medline"
                          ]
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                  ]
                ]
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              "identificador" => "bib0185"
              "etiqueta" => "11"
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                    0 => array:2 [
                      "titulo" => "A new method for the measurement of lysosomal acid lipase in dried blood spots using the inhibitor Lalistat 2"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46; Hamilton"
                            1 => "I&#46; Jones"
                            2 => "R&#46; Srivastava"
                            3 => "P&#46; Galloway"
                          ]
                        ]
                      ]
                    ]
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                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.cca.2012.03.019"
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                        "tituloSerie" => "Clin Chim Acta"
                        "fecha" => "2012"
                        "volumen" => "413"
                        "paginaInicial" => "1207"
                        "paginaFinal" => "1210"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22483793"
                            "web" => "Medline"
                          ]
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                    0 => array:2 [
                      "titulo" => "Novel mutations in human lipase A gene"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "J&#46; Huang"
                            1 => "Z&#46; Wu"
                            2 => "C&#46;R&#46; Scott"
                          ]
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                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "LibroEditado" => array:2 [
                        "titulo" => "Poster presentation in the am assoc hum genet meeting"
                        "serieFecha" => "2014"
                      ]
                    ]
                  ]
                ]
              ]
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              "identificador" => "bib0195"
              "etiqueta" => "13"
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                0 => array:2 [
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                    0 => array:2 [
                      "titulo" => "Lysosomal acid lipase deficiency unmasked in two children with nonalcoholic fatty liver disease"
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                          "etal" => false
                          "autores" => array:6 [
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                            2 => "K&#46; Bove"
                            3 => "N&#46;M&#46; Quintanilla"
                            4 => "R&#46; Sheridan"
                            5 => "R&#46; Kohli"
                          ]
                        ]
                      ]
                    ]
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                    0 => array:2 [
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                        "tituloSerie" => "Pediatrics"
                        "fecha" => "2016"
                        "volumen" => "138"
                        "paginaInicial" => "e20160214"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27624512"
                            "web" => "Medline"
                          ]
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              ]
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              "etiqueta" => "14"
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                    0 => array:2 [
                      "titulo" => "Lysosomal acid lipase deficiency&#58; expanding differential diagnosis"
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                          "autores" => array:4 [
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                            2 => "A&#46; Brassier"
                            3 => "G&#46; Grabowski"
                          ]
                        ]
                      ]
                    ]
                  ]
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                        "tituloSerie" => "Mol Genet Metab"
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                        "volumen" => "120"
                        "paginaInicial" => "62"
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                        "tituloSerie" => "Nat Rev Endocrinol"
                        "fecha" => "2015"
                        "volumen" => "11"
                        "paginaInicial" => "696"
                        "paginaFinal" => "697"
                        "link" => array:1 [
                          0 => array:2 [
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                          ]
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                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Prevalence of lysosomal storage disorders"
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                        "fecha" => "1999"
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                        "paginaInicial" => "249"
                        "paginaFinal" => "254"
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                    ]
                  ]
                ]
              ]
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            16 => array:3 [
              "identificador" => "bib0215"
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                0 => array:1 [
                  "referenciaCompleta" => "ClinVar&#46; NM&#95;000235&#46;3&#40;LIPA&#41;&#58;c&#46;67G&#62;A &#40;p&#46;Gly23Arg&#41;&#46; NCBI&#46; 2016&#46; Dispon&#237;vel em&#58; https&#58;&#47;&#47;www&#46;ncbi&#46;nlm&#46;nih&#46;gov&#47;clinvar&#47;variation&#47;255612&#47; &#91;acesso 26&#46;10&#46;17&#93;&#46;"
                ]
              ]
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              "etiqueta" => "18"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Identification and metabolic profiling of patients with lysosomal acid lipase deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "C&#46;R&#46; Pullinger"
                            1 => "E&#46;O&#46; Stock"
                            2 => "I&#46; Movsesyan"
                            3 => "M&#46;J&#46; Malloy"
                            4 => "P&#46;H&#46; Frost"
                            5 => "R&#46; Tripuraneni"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jacl.2015.07.008"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Clin Lipidol"
                        "fecha" => "2015"
                        "volumen" => "9"
                        "paginaInicial" => "716"
                        "paginaFinal" => "726"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26350820"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            18 => array:3 [
              "identificador" => "bib0225"
              "etiqueta" => "19"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sebelipase Alfa&#58; a review in lysosomal acid lipase deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "J&#46;E&#46; Frampton"
                          ]
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                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1007/s40256-016-0203-2"
                      "Revista" => array:6 [
                        "tituloSerie" => "Am J Cardiovasc Drugs"
                        "fecha" => "2016"
                        "volumen" => "16"
                        "paginaInicial" => "461"
                        "paginaFinal" => "468"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27878737"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
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              "identificador" => "bib0230"
              "etiqueta" => "20"
              "referencia" => array:1 [
                0 => array:2 [
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                    0 => array:2 [
                      "titulo" => "Novel treatment options for lysosomal acid lipase deficiency&#58; critical appraisal of sebelipase alfa"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "K&#46; Su"
                            1 => "E&#46; Donaldson"
                            2 => "R&#46; Sharma"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.2147/TACG.S86760"
                      "Revista" => array:6 [
                        "tituloSerie" => "Appl Clin Genet"
                        "fecha" => "2016"
                        "volumen" => "9"
                        "paginaInicial" => "157"
                        "paginaFinal" => "167"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27799810"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
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              "etiqueta" => "21"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "A Phase 3 trial of sebelipase alfa in lysosomal acid lipase deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "B&#46; Burton"
                            1 => "M&#46; Balwani"
                            2 => "F&#46; Feillet"
                            3 => "I&#46; Baric"
                            4 => "T&#46; Burrow"
                            5 => "C&#46; Camarena Grande"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
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                        "tituloSerie" => "NEJM"
                        "fecha" => "2015"
                        "volumen" => "373"
                        "paginaInicial" => "1010"
                        "paginaFinal" => "1020"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26352813"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
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              "etiqueta" => "22"
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                0 => array:2 [
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                    0 => array:2 [
                      "titulo" => "Enzyme replacement therapy in fibroblasts from a patient with cholesteryl ester storage disease"
                      "autores" => array:1 [
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                          "etal" => false
                          "autores" => array:2 [
                            0 => "M&#46;J&#46; Poznansky"
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                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1096/fasebj.3.2.2644147"
                      "Revista" => array:6 [
                        "tituloSerie" => "FASEB J"
                        "fecha" => "1989"
                        "volumen" => "3"
                        "paginaInicial" => "152"
                        "paginaFinal" => "156"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/2644147"
                            "web" => "Medline"
                          ]
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                      ]
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                  ]
                ]
              ]
            ]
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              "etiqueta" => "23"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Lysosomal acid lipase deficiency allograft recurrence and liver failure &#8211; clinical outcomes of 18 liver transplantation patients"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
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                            1 => "S&#46; Lobritto"
                            2 => "A&#46; Iuga"
                            3 => "H&#46; Remotti"
                            4 => "T&#46; Schiano"
                            5 => "M&#46;I&#46; Fiel"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.ymgme.2018.03.010"
                      "Revista" => array:6 [
                        "tituloSerie" => "Mol Genet Metab"
                        "fecha" => "2018"
                        "volumen" => "124"
                        "paginaInicial" => "11"
                        "paginaFinal" => "19"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29655841"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            23 => array:3 [
              "identificador" => "bib0250"
              "etiqueta" => "24"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Pathological evidence of Wolman&#39;s disease following hematopoietic stem cell transplantation despite correction of lysosomal acid lipase activity"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "M&#46;M&#46; Gramatges"
                            1 => "C&#46;C&#46; Dvorak"
                            2 => "D&#46;P&#46; Regula"
                            3 => "G&#46;M&#46; Enns"
                            4 => "K&#46; Weinberg"
                            5 => "R&#46; Agarwal"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1038/bmt.2009.57"
                      "Revista" => array:6 [
                        "tituloSerie" => "Bone Marrow Transplant"
                        "fecha" => "2009"
                        "volumen" => "44"
                        "paginaInicial" => "449"
                        "paginaFinal" => "450"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19308038"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            24 => array:3 [
              "identificador" => "bib0255"
              "etiqueta" => "25"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Wolman disease successfully treated by bone marrow transplantation"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "W&#46; Krivit"
                            1 => "C&#46; Peters"
                            2 => "K&#46; Dusenbery"
                            3 => "Y&#46; Ben-Yoseph"
                            4 => "N&#46;K&#46; Ramsay"
                            5 => "J&#46;E&#46; Wagner"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1038/sj.bmt.1702557"
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                        "tituloSerie" => "Bone Marrow Transplant"
                        "fecha" => "2000"
                        "volumen" => "26"
                        "paginaInicial" => "567"
                        "paginaFinal" => "570"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11019848"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            25 => array:3 [
              "identificador" => "bib0260"
              "etiqueta" => "26"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Long&#8208;term metabolic&#44; endocrine&#44; and neuropsychological outcome of hematopoietic cell transplantation for Wolman disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "J&#46; Tolar"
                            1 => "A&#46; Petryk"
                            2 => "K&#46; Khan"
                            3 => "K&#46;J&#46; Bjoraker"
                            4 => "J&#46; Jessurun"
                            5 => "M&#46; Dolan"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1038/bmt.2008.273"
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                        "tituloSerie" => "Bone Marrow Transplant"
                        "fecha" => "2009"
                        "volumen" => "43"
                        "paginaInicial" => "21"
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                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18776925"
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