New Alagille syndrome study results reported from University of Paris
2007 OCT 30 -- "'Alagille syndrome (AGS; Online Mendelian Inheritance in Man no. 118450) is a multisystem autosomal dominant disorder with highly variable expression characterized by chronic cholestasis caused by a paucity of interlobular bile ducts, skeletal abnormalities, peculiar facies, ocular abnormalities, and cardiovascular disorders," scientists writing in the American Journal of Kidney Diseases report. "AGS is diagnosed almost exclusively in children in the setting of predominant liver manifestations or, more rarely, in their adult relatives. We report 2 patients in whom AGS was diagnosed in adulthood during the workup of renal disease in the absence of a well-defined familial history," wrote A. Jacquet and colleagues, University of Paris. The researchers concluded: "Renal disease caused by AGS probably is underdiagnosed in adult patients." Jacquet and colleagues published their study in American Journal of Kidney Diseases (Alagille syndrome in adult patients: It is never too late. American Journal of Kidney Diseases, 2007;49(5):705-709). Additional information can be obtained by contacting F. Fakhouri, University of Paris, Hopital Necker, AP HP, Dept. of Nephrology, Paris, France. The publisher of the American Journal of Kidney Diseases can be contacted at: W B Saunders Co-Elsevier Inc., 1600 John F Kennedy Boulevard, Ste. 1800, Philadelphia, PA 19103-2899, USA. Keywords: France, Paris, Alagille Syndrome, Kidney Disease, University of Paris. This article was prepared by Science Letter editors from staff and other reports. Copyright 2007, Science Letter via NewsRx.com.
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