IMMUNE DEFICIENCIES IN CHRONIC INTESTINAL PSEUDOOBSTRUCTION

Aim: Chronic intestinal pseudo-obstruction has been associated with ur inary disorders, myopathy, and ophthalmoplegia in adults and cholelith iasis in children. We observed a high percentage of total-parenteral-n utrition-dependent patients with pseudo-obstruction and recurrent infe ctions 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 nutriti onal evaluation from 1992 to 1995 were reviewed. Chronic intestinal ps eudo-obstruction was diagnosed by clinical, radiographic findings and antroduodenal manometry. Intestinal full-thickness biopsies were perfo rmed in seven children. Results: Hypogammaglobulinemia was diagnosed i n 18 patients (78%): 16 patients had various immunoglobulin deficienci es and 2 had selective antibody deficiency. Intravenous gammaglobulin was administered in 14 patients. Other medical conditions affecting th e children are summarized as follows: autonomic dysfunction in 10 pati ents (43%), recurrent hypoglycemia in 9 (39%), asthma in 9 (39%), chol ecystitis in 7 (30%), low serum carnitine level in 6 (26%), urinary dy sfunction in 6 (26%), pancreatitis in 5 (22%), behavioral problems in 5 (22%), myopathy in 2 (9%), idiopathic thrombocytopenia in 2 (8%), ve lopharyngeal 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: Chro nic intestinal pseudo-obstruction appears to be associated with immune deficiencies. It is unclear if the immune deficiencies, intestinal ps eudo-obstruction, and the other medical conditions have a common under lying etiology. Repeated infections may be due to impaired immune func tion in children with chronic intestinal pseudo-obstruction. We recomm end screening for immune deficiencies in children with chronic intesti nal pseudo-obstruction.