Immune deficiencies in chronic intestinal pseudo-obstruction.
OBJECTIVE
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.
Forchielli
M L
ML
Combined Program in Gastroenterology and Nutrition, and Department of Allergy and Immunology, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Young
M C
MC
Flores
A F
AF
Richardson
D
D
Lo
C W
CW
D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000150:complications*; D007418:Intestinal Pseudo-Obstruction / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D008297:Male; D009110:Munchausen Syndrome / Q000150:complications; D010289:Parenteral Nutrition, Total; D012189:Retrospective Studies