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Immune deficiencies in chronic intestinal pseudo-obstruction
M L Forchielli1, M C Young, A F Flores
1Combined Program in Gastroenterology and Nutrition, and Department of Allergy and Immunology, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Acta Paediatrica (Oslo, Norway : 1992)
|November 14, 1997
Summary
Children with chronic intestinal pseudo-obstruction often have immune deficiencies, leading to recurrent infections. Screening for these deficiencies is recommended for better management of this condition.
Area of Science:
- Pediatric Gastroenterology
- Immunology
Background:
- Chronic intestinal pseudo-obstruction (CIPO) is a rare gastrointestinal motility disorder.
- CIPO is associated with various comorbidities, including urinary disorders, myopathy, and ophthalmoplegia in adults, and cholelithiasis in children.
- A significant proportion of CIPO patients require total parenteral nutrition and experience recurrent infections.
Purpose of the Study:
- To investigate the association between chronic intestinal pseudo-obstruction and immune deficiencies in children.
- To identify other medical conditions frequently observed in children with CIPO.
- To recommend appropriate screening for immune deficiencies in pediatric CIPO patients.
Main Methods:
- Retrospective review of medical records for 23 children diagnosed with CIPO between 1992 and 1995.
- Diagnosis of CIPO confirmed by clinical, radiographic, and antroduodenal manometry findings.
- Intestinal full-thickness biopsies were performed in a subset of patients.
Main Results:
- Hypogammaglobulinemia was diagnosed in 78% of patients, with 16 having immunoglobulin deficiencies and 2 having selective antibody deficiency.
- Intravenous gammaglobulin was administered to 14 patients.
- Other prevalent conditions included autonomic dysfunction (43%), recurrent hypoglycemia (39%), asthma (39%), and cholecystitis (30%).
Conclusions:
- Chronic intestinal pseudo-obstruction in children is significantly associated with immune deficiencies.
- The underlying etiology linking CIPO, immune deficiencies, and other comorbidities remains unclear.
- Screening for immune deficiencies is crucial in children diagnosed with chronic intestinal pseudo-obstruction due to the high prevalence of infections.