Primary Hypogammaglobulinemia and Malabsorption

  1. WILLIAM S. HUGHES, M.D.;
  2. JAMES J. CERDA, M.D., F.A.C.P.;
  3. PHILIP HOLTZAPPLE, M.D.; and
  4. FRANK P. BROOKS, M.D., F.A.C.P.
  1. Philadelphia, Pennsylvania

    Abstract

    Intestinal absorption was investigated in six patients with a diagnosis of primary hypogammaglobulinemia. Malabsorption was found in four patients. Low serum vitamin E levels, decreased D-xylose absorption, and increased 5-hydroxyindoleacetic acid excretion in the urine correlated with malabsorption with minor exceptions. Five patients were subjected to jejunal biopsies, and nodular lymphoid hyperplasia was found on at least one examination in each of these patients. In addition, partial to complete mucosal atrophy characterized biopsy specimens from four patients and correlated with steatorrhea with one exception. Although gastric achlorhydria (two patients), minimal to moderate pancreatic insufficiency (two patients), significant intestinal intraluminal bacterial overgrowth (three patients), and Giardia lamblia (five patients) were found, the evidence suggests that the most significant cause of malabsorption in these hypogammaglobulinemic patients is an intestinal mucosal lesion. Reversibility of total villus atrophy and steatorrhea was clearly demonstrated in one patient over the course of a 13-year study.

    Article and Author Information

    • ▸From the Gastrointestinal Clinic of the Hospital of the University of Pennsylvania; The Gastroenterology Research Laboratory, Pennsylvania Hospital; The University of Pennsylvania Medical Division, Veterans Administration Hospital; and the Department of Medicine, University of Pennsylvania School of Medicine; Philadelphia, Pa.

    • Supported in part by grants 5T01-AM5462, Fr-5590, and 5M01 RR 40, U.S. Public Health Service, Washington, D.C. Dr. Cerda is the recipient of a Clinical Investigator award, Veterans Administration, Washington, D.C.

    • ▸Requests for reprints should be addressed to James J. Cerda, M.D., GI Section, University of Pennsylvania Medical Division, Veterans Administration Hospital, Philadelphia, Pa. 19104

      • Received November 25, 1970.
      • Accepted February 22, 1971.
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