Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients.
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Date
2011-07
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Abstract
Hypogammaglobulinemic sprue (HGS), which
may predispose to infection, is uncommon. Twelve patients (all men; median age 29 years, 15–50) with HGS (4%) of 296 with chronic small bowel diarrhea and malabsorption
syndrome (MAS) during a 10-year period were analyzed.
Treatment of HGS was delayed due to misdiagnosis as
intestinal tuberculosis (n=7) and diarrhea-predominant
irritable bowel syndrome (n=1). All had diarrhea and
weight loss (median loss 12 Kg). Associated conditions
were clubbing, bronchiectasis, and seizure (2 patients each),
and hypothyroidism (n=1). Laboratory parameters were
urinary D-xylose median 0.46 g/5 g/5 h (range 0.2–1.6;
normal ≥1), fecal fat 11.9 g/day (3.8–16.7; normal ≤7 g),
serum IgA, IgG, and IgM: 23.5 mg/dL (17–114; normal
90–450), 584 mg/dL (145–1051; normal 800–1800), and
23 (0–40.3; normal 60–280). IgA, IgG, and IgM were low
in 10, 10, and 11, respectively. Duodenal biopsy was
normal in 6 patients and showed partial villous atrophy in 6
and nodular lymphoid hyperplasia in two. Associated
infections were giardiasis (n=1), disseminated strongyloidiasis
(1), small intestinal bacterial overgrowth (3), septicemia
(2), and septic arthritis (1). Two patients died of
sepsis, five are well on immunoglobulin and specific antiinfective
treatment, and five are lost to follow up.
Approximately 4% patients with MAS have hypogammaglobulinemia, which is often associated with infection and
is diagnosed late.
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Keywords
Common variable immunodeficiency syndrome, Gastrointestinal infection, Hypogammaglobulinemic sprue
Citation
Ghoshal Uday C, Goel Amit, Ghoshal Ujjala, Jain Manoj, Misra Asha, Choudhuri Gourdas. Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients. Indian Journal of Gastroenterology. 2011 Jul-Aug; 30(4): 170-174.