Text Box: GALACTOSEMIA FOR PORTO-HEPATIC VENOUS SHUNT WITH HEPATIC HEMANGIOMA Nishimura Y1, Ono H1, Sakura N1, Mizoguchi N1, Naito K2 1Department of Pediatrics and 2Department of Radiology, Hiroshima University School of Medicine, Hiroshima, Japan Objective: Galactosemia is caused by deficiency of galactose-metabolizing enzymes and various liver diseases and portal-systemic (PS) shunts. We report that 10 cases galactosemia for porto-hepatic venous (PV) shunt with hepatic hemangioma. Method: Ten patients were diagnosed as having PV shunts with hepatic hemangioma.These patients were screened from about 410,000 neonates between 1987 and 2001 in Hiroshima. Galactose in blood was estimated by Paigen method. Hereditary galactosemias were excluded by estimate of galactose metabolizing enzyme activities in red blood cell. Detection of hepatic hemangioma was performed by using ultrasonography. Plasma total bile acids (TBA) were estimated using commercial kit method. Results: All patients showed hypergalactosemia at the age of 5days by neonatal mass -screening test. Their galactose level were various with PVshunt flow by hepatic hemangioma. All patients showed increased level of TBA (total bile acid).Their TBA levels based on shunt flow. The majority of the patients showes normal galactose metabolizing enzyme activities.In 7cases,hepatic hemangiomas regressed as well as did PVshunts. 2cases are now under observation.One patient developed liver function failure due to enlarged multiple hepatic hemangioma,and he was treated by liver transplantation. Conclusion: In this study,we report 10cases of galactosemia for PVshunt caused by hepatic hemanngioma.During the same period,we identified 8 patients with hereditary galactosemia.In Hiroshima,the major course of galactosemia is PVshunt caused by the hepatic hemangioma. 2492