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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.
Insights
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.
Aim:
Chronic intestinal pseudo-obstruction has been associated with urinary disorders, myopathy, and ophthalmoplegia in adults and cholelithiasis in children. We observed a high percentage of total-parenteral-nutrition-dependent patients with pseudo-obstruction and recurrent infections requiring gammaglobulin infusions.
Methods:
All records for 23 children with chronic intestinal pseudo-obstruction (10 females and 13 males, mean age 9.8 y +/- 4.9 y, range 4-24 y) referred for a nutritional evaluation from 1992 to 1995 were reviewed. Chronic intestinal pseudo-obstruction was diagnosed by clinical, radiographic findings and antroduodenal manometry. Intestinal full-thickness biopsies were performed in seven children.
Results:
Hypogammaglobulinemia was diagnosed in 18 patients (78%): 16 patients had various immunoglobulin deficiencies and 2 had selective antibody deficiency. Intravenous gammaglobulin was administered in 14 patients. Other medical conditions affecting the children are summarized as follows: autonomic dysfunction in 10 patients (43%), recurrent hypoglycemia in 9 (39%), asthma in 9 (39%), cholecystitis in 7 (30%), low serum carnitine level in 6 (26%), urinary dysfunction in 6 (26%), pancreatitis in 5 (22%), behavioral problems in 5 (22%), myopathy in 2 (9%), idiopathic thrombocytopenia in 2 (8%), velopharyngeal insufficiency in 1 (4%), oculocutaneous albinism in 1 (4%), Pierre-Robin syndrome in 1 (4%), and protein C deficiency in 1 (4%). Munchausen syndrome was suspected in two patients.
Conclusions:
Chronic intestinal pseudo-obstruction appears to be associated with immune deficiencies. It is unclear if the immune deficiencies, intestinal pseudo-obstruction, and the other medical conditions have a common underlying etiology. Repeated infections may be due to impaired immune function in children with chronic intestinal pseudo-obstruction. We recommend screening for immune deficiencies in children with chronic intestinal pseudo-obstruction.