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Immune deficiencies in chronic intestinal pseudo‐obstruction
Author(s) -
Forchielli ML,
Young MC,
Flores AF,
Richardson D.,
Lo CW
Publication year - 1997
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1997.tb14811.x
Subject(s) - medicine , intestinal pseudo obstruction , hypogammaglobulinemia , gastroenterology , etiology , pancreatitis , airway obstruction , surgery , immunology , antibody , airway
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: AH 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.