OBJECTIVES This study sought to determine the prevalence of hepatocellular carcinoma and other premalignant lesions in children with hereditary tyrosinemia type 1 who had undergone an orthotopic liver transplant at the Shiraz Transplant Center, in Shiraz, Iran. MATERIALS AND METHODS Between September 2006, and June 2011, thirty-six patients with hereditary tyrosinemia type 1 received a liver ...

H· EREDITARY TYROSINEMIA is a metabolic disorder characterized by deficiency of the enzyme fummarylacetoacetate, resulting in the accumulation of tyrosine metabolites, which are toxic to the liver. Hepatic dysfunction, associated with tyrosinemia, varies from an acute form of liver failure to a chronic, progressive form that leads to cirrhosis and hepatocellular carcinoma (HCC).1.2 Liver transp...

Organic extracts of six urine samples from children treated with nitisinone, a medicine against tyrosinemia type I, were investigated by (1)H and (19)F NMR spectroscopy. The presence of unchanged 2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione (NTBC), 6-hydroxy-2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione (NTBC-OH) and 2-nitro-4-trifluoromethylbenzoic acid (NTFA) as w...

objective: studies reported conflicting results’ regarding the status of depression and glycemic control in patients with type ii diabetes(t2dm), therefore, this study was performed to determine the relationship between depression and glycemic control in t2dm patients. materials and methods: this cross-sectional study was performed on 150 t2dm patients referred to yazd diabetes research center,...

Inborn errors of amino-acids metabolism and other inherited Mendeliandisorders are common in the MiddleEast.The number of diagnosed inborn errors of amino acid metabolism is growing constantly on account of and availability and improved of analytical techniques.  The aim of this work was to determine a rough estimate of the incidence rates of phenylketonuria (PKU), tyrosinemia, and maple syrup ...

background and objective: several studies have found that diazepam decreases serum glucose. meanwhile, glibenclamide is commonly used in diabetes treatment. the objective of this study was to survey the treatment impact of diazepam and glibenclamide on blood glucose and serum lipids in mice with type ii diabetes. materials and methods: in this study, 32 male rats were divided into four groups, ...

by a gas-liquid chromatographic method. J. Lab. Clin. Med., 74:185, 1969. 22. Kang, E. S., and Gerald, P. S. : Hereditary tyrosinemia and abnormal pvrrole metabolism. J. Pediat., 77:397, 1970. 23. Gentz, J., Johansson, S., Lindblad, B., Lindstedt, S., and Zetterstr#{246}m, R. : Excretion of daminolevulinic acid in hereditary tyrosinemia. Clin. Chim. Acta, 23:257, 1969. 24. Fellman, J. H., Vanbe...