Immune deficiencies in chronic intestinal pseudo-obstruction.

Abstract

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.

• Authors: M L Forchielli, M C Young, A F Flores, D Richardson, C W Lo
• Journal: Acta paediatrica (Oslo, Norway : 1992) (Acta Paediatr) Vol. 86 Issue 10 Pg. 1077-81 (Oct 1997) ISSN: 0803-5253 [Print] Norway
• PMID: 9350888 (Publication Type: Journal Article)

Topics

• Adolescent
• Adult
• Agammaglobulinemia (complications)
• Child
• Child, Preschool
• Chronic Disease
• Female
• Humans
• Immune System Diseases (complications)
• Intestinal Pseudo-Obstruction (etiology, immunology, therapy)
• Male
• Munchausen Syndrome (complications)
• Parenteral Nutrition, Total
• Retrospective Studies