CASE SERIES Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients Uday C Ghoshal & Amit Goel & Ujjala Ghoshal & Manoj Jain & Asha Misra & Gourdas Choudhuri Received: 13 June 2010 /Accepted: 6 July 2011 /Published online: 23 July 2011 # Indian Society of Gastroenterology 2011 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 strongyloi- diasis (1), small intestinal bacterial overgrowth (3), septi- cemia (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 hypogamma- globulinemia, which is often associated with infection and is diagnosed late. Keywords Common variable immunodeficiency syndrome . Gastrointestinal infection . Hypogammaglobulinemic sprue Introduction Chronic small bowel diarrhea and malabsorption syndrome (MAS) are common disorders all over the world. MAS results from several diseases [1]. Hypogammaglobulinemic sprue (HGS) in adults has been described mostly as case reports and small series from temperate countries [2–4] and only as case report from India [5]. Though hypogamma- globulinemia predisposes to gastrointestinal and systemic infection and infestations [6], which are likely to be more common in developing countries, there is no systematic study on frequency and nature of infection and infestations in patients with HGS from developing countries. There- fore, we present a series of twelve consecutive adult patients with HGS from a tertiary referral hospital in northern India and frequency and nature of infection and infestation among them. One of these cases has been published earlier [7]. U. C. Ghoshal (*) : A. Goel : A. Misra : G. Choudhuri Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226 014, India e-mail: udayghoshal@gmail.com U. Ghoshal Department of Microbiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226 014, India M. Jain Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226 014, India Indian J Gastroenterol (July–August 2011) 30(4):170–174 DOI 10.1007/s12664-011-0111-y