title: Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients.
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 anti-infective 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.
authors.affiliation: Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. udayghoshal@gmail.com
authors.forename: Uday C
authors.initials: UC
authors.lastname: Ghoshal
authors.forename: Amit
authors.initials: A
authors.lastname: Goel
authors.forename: Ujjala
authors.initials: U
authors.lastname: Ghoshal
authors.forename: Manoj
authors.initials: M
authors.lastname: Jain
authors.forename: Asha
authors.initials: A
authors.lastname: Misra
authors.forename: Gourdas
authors.initials: G
authors.lastname: Choudhuri
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications* / Q000175:diagnosis; D057210:Delayed Diagnosis; D003967:Diarrhea / Q000209:etiology*; D006801:Humans; D008286:Malabsorption Syndromes / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D055815:Young Adult