Original articleDistal intestinal obstruction syndrome in adults with cystic fibrosis
Section snippets
Patient selection and CF diagnostic criteria
The study group consisted of adults with CF enrolled in the Department of Pulmonology of Cochin Hospital, Paris, France, from November 1, 1997 to November 1, 1999. Data were recorded retrospectively from the patients’ files by using Epi Info 6.0 software (Center for Disease Control, Atlanta, GA). The diagnosis of CF had been established on the basis of at least 1 typical clinical manifestation (bronchiectasis and/or steatorrhea) and at least 1 biological criterion,5, 6 namely a sweat chloride
Population
One hundred seventy-one adults with CF (89 men and 82 women) attended our department during the study period. Mean age at CF diagnosis was 8.8 years (median, 2.0 years; range, 0—57.9 years). Mean age at the first visit to our department was 24.9 years (range, 16.0–57.9 years). Follow-up in our department ranged from 0.0–15.0 years (median, 2.5 years; mean, 3.5 years). Thirteen patients (7.6%) died during the study period, 12 of respiratory complications. Mean age at the last visit was 28.9
Discussion
This study showed that episodes of intestinal obstruction are frequent in adults with CF, reaching 18.1% in our population. DIOS was the most frequent diagnosis (prevalence, 15.8%). In most cases the initial episode occurred after age 18 years, and half of the patients experienced relapse. It is difficult to compare this prevalence rate with that found in other studies because of differences in the criteria used for retrospective diagnosis of DIOS; reported rates range from 3.7%13 to 37.3%.14
References (34)
- et al.
Cystic fibrosis
Lancet
(1998) - et al.
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
Cell
(1993) - et al.
Intra-abdominal complications of cystic fibrosis
J Pediatr Surg
(1985) - et al.
Appendiceal abcess in cystic fibrosisa diagnostic challenge
Gastroenterology
(1984) - et al.
Cystic fibrosis in adults75 cases and a review of 232 cases in the literature
Am J Med
(1979) Rapport sur la situation de la mucoviscidose en France en 2001
(2004)Meconium ileus equivalent in a 15 year old patient with mucoviscidosis
Acta Paediatr Scand
(1962)- et al.
Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients
Gut
(2002) - et al.
Digestive alterations in cystic fibrosisretrospective study of a series of 46 adult patients
Gastroenterol Hepatol
(1999) The diagnosis of cystic fibrosis
N Engl J Med
(1997)
Mutation heterogeneity of cystic fibrosis in Francescreening by denaturing gradient gel electrophoresis using psoralen-modified oligonucleotide
Hum Mutat
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations
N Engl J Med
Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens
N Engl J Med
Cystic fibrosisgenotyping and phenotyping variations
Annu Rev Genet
Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients
Eur Respir J
Gastrografin in acute meconium ileus equivalent
Arch Dis Child
Appendicitis in cystic fibrosis
Arch Dis Child
Cited by (98)
Two systematic cochrane reviews of the prevention and treatment of distal intestinal obstruction syndrome in cystic fibrosis
2022, Paediatric Respiratory ReviewsDistal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis
2020, Jornal de PediatriaCitation Excerpt :Among a number of factors identified as predisposing to DIOS, past medical history of meconium ileus at birth has been consistently reported and also associated with recurrence,7 and this was observed in the majority patients in this series (seven out of ten), as well as in a significant share of those who had recurrence of DIOS (three out of five), and also among patients who required surgical treatment (two out of three). Other factors reported as associated with the occurrence of DIOS – such as dehydration, hot seasons/weather, CF pulmonary exacerbation,10 colonization by Pseudomonas aeruginosa, diabetes mellitus related to cystic fibrosis,7,11 CF-related liver disease, and post-operatory lung transplant12,13 – were not consistently present in this series. As reported in the pertinent literature, in the vast majority of episodes, the diagnosis was based on the combination of history, clinical examination, and simple abdominal radiography.
Unusual causes of large bowel obstruction
2019, Current Problems in SurgeryCystic fibrosis
2019, Emery and Rimoin's Principles and Practice of Medical Genetics and Genomics: Cardiovascular, Respiratory, and Gastrointestinal DisordersCystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer
2017, Journal of Cystic FibrosisCitation Excerpt :Previous history of MI appears to be a fairly strong predictive risk factor in several studies, as well as history of prior surgery for MI [8,11,14]. Nearly 50% of patients with CF diagnosed with DIOS had presented with MI at birth in a large European study, compared to an estimated prevalence of 10–15.8% among all patients with CF. [3,8,12]. Organ transplantation in patients with CF, notably after lung transplantation [6], appears to increase risk for DIOS; several international studies have identified an incidence of 10–20% in the post-operative period [15,16].
Abdominal Complications After Lung Transplantation in a Brazilian Single Center
2017, Transplantation Proceedings