New pathology research from University of California described
2007 APR 30 -- A new study, "Enteroendocrine cell dysgenesis and malabsorption, a histopathologic and immunohistochemical characterization," is now available. According to recent research from the United States, "Enteroendocrine cell dysgenesis was observed in 3 patients with intestinal failure of unknown cause. Enteroendocrine cell dysgenesis is a congenitally acquired life-threatening malabsorptive condition with a unique clinical phenotype paired with a histologically identifiable disease pattern." "Two cases were first presented at the Ninth International Small Bowel Transplantation Symposium, Brussels 2005, and were subsequently published (N Engl J Med 2006;355:270). We now present the histopathologic and immunohistochemical findings of the gastric antrum, small bowel, and colon in greater detail. The clinical phenotype of the patients was unusual in that the affected patients demonstrated profound malabsorption of all nutrients, except water, from birth. The small intestine in each patient demonstrated almost no abnormality, except a near absence of endocrine cells in the mucosa. The colon appeared similarly affected. Known causes of congenital malabsorption, inflammatory, and infectious causes of diarrhea were excluded. The defect is secondary to point mutations in NEUROG3, which result in an arrest of endocrine cell development in the small intestine and colon. This work describes the pathologic characterization of enteroendocrine cell dysgenesis using routine techniques. The pattern of injury is distinct from other histopathologically assessed congenital malabsorptive conditions such as microvillus inclusion disease, tufting enteropathy, and abetalipoproteinemia. It is also easily distinguished from inflammatory conditions such as food allergy, gluten-sensitive enteropathy, autoimmune enteropathy, IPEX (immune dysfunction, polyendocrinopathy, enteropathy, and X-linked inheritance), and inflammatory bowel disease," wrote G. Cortina and colleagues, University of California. The researchers concluded: "The histopathology of disease is similar to what has been found transiently in a single patient with autoimmune polyglandular syndrome type I." Cortina and colleagues published their study in Human Pathology (Enteroendocrine cell dysgenesis and malabsorption, a histopathologic and immunohistochemical characterization. Human Pathology, 2007;38(4):570-80). For additional information, contact G. Cortina, University of California at Los Angeles, Dept. of Pathology and Laboratory Medicine, David Geffen School of Medicine, Los Angeles, CA 90095 USA. Publisher contact information for the journal Human Pathology is: W B Saunders Co., Independence Square West Curtis Center, Ste. 300, Philadelphia, PA 19106-3399, USA. Keywords: United States, Los Angeles, Gastroenterology, Immunology, Pathology, Transplantation. This article was prepared by Gastroenterology Week editors from staff and other reports. Copyright 2007, Gastroenterology Week via NewsRx.com.
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