ArticleJornal de pediatria
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis.
Article in Jornal de pediatria. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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4 citing papers in PubMed, 14 citations in OpenAlex.
- The current state of gastrointestinal motility evaluation in cystic fibrosis: a comprehensive literature review.Translational gastroenterology and hepatology · 2024Review
- Effectiveness of an Oral Gastrografin Regimen for Complete Distal Intestinal Obstruction Syndrome.Annals of surgery open : perspectives of surgical history, education, and clinical approaches · 2023Article
- Dietary recommendations for people with cystic fibrosis, a relevantly changing field.Jornal de pediatriaArticle
- A 20-year case-series of distal intestinal obstruction syndrome at a state-wide cystic fibrosis service.ANZ journal of surgeryArticle
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Authors and funding
7 authors at 2 institutions in 2 countries.
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Abstract
objectiveTo evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence.
methodsCase series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up.
resultsAll patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients.
conclusionIn this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.
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