Long-term results of liver transplantation for Wilson's disease.

UNLABELLED: Wilson's disease is a hereditary defect in hepatic copper metabolism, causing hepatic, neurological and/or psychiatric manifestations. For patients with severe disease, liver transplantation is the treatment of choice. The aim of this study was to report the long-term outcome of patients who underwent liver transplantation for Wilson's disease. PATIENTS AND METHODS: Thirteen patients with Wilson's disease, transplanted in Lyon France between January 1987 and May 2006, were including in this study: eight women and five men, aged eight to 53 years (median 20 years, seven children and six adults). The diagnosis of Wilson's disease was established before liver transplantation. RESULTS: The indication for liver transplantation was chronic (69%) or fulminant liver failure (31%). The median follow-up after liver transplantation was 10 years with 100% patient survival. Copper metabolism returned to normal in all patients. None of the patients with exclusive liver disease required chelation treatment after liver transplantation and none developed neurological symptoms of Wilson's disease. CONCLUSION: Liver transplantation totally reverses the abnormalities of copper metabolism and subsequent hepatic failure, but the course of neurological symptoms remains unpredictable. Long-term patient survival can be excellent without occurrence of neurological complications.

Interactions between 4-HPR and diet in NMU-induced mammary tumorigenesis.

The present study was designed to determine whether the chemopreventive effect of the synthetic retinoid N(4-hydroxyphenyl)retinamide (4-HPR) on mammary tumorigenesis was influenced by diet. Three diets were used: the closed-formula grain-based Wayne Lab Blox, the open-formula grain-based NIH-07, and the casein-based semipurified AIN-76A. Groups of 25 virgin female F-344 rats were fed the experimental diets beginning one week before a single injection of N-methyl-N-nitrosourea (NMU, 45 mg/kg body wt i.v.) at 50 days of age. The experimental design was as follows: Group 1, unsupplemented AIN-76A; Group 2, AIN-76A supplemented with 4-HPR starting seven days before NMU until termination (-7); Group 3, AIN-76A supplemented with 4-HPR seven days after NMU until termination (+7); Group 4, Wayne (no 4-HPR); Group 5, Wayne (4-HPR, -7); Group 6, Wayne (4-HPR, +7); Group 7, NIH-07; Group 8, NIH-07 (4-HPR, -7). 4-HPR [782 mg/kg diet (2 mM)] was given to all supplemented groups. Termination was 25 weeks post-NMU. Analysis of tumor incidence, multiplicity, and latency indicated that 1) control rats fed the AIN-76A diet exhibited significantly higher mammary tumor yields than rats fed unsupplemented natural-ingredient diets (Wayne and NIH-07) and 2) 4-HPR inhibited mammary tumor development in the two grain-based diets but enhanced tumor development in the AIN-76A diet. Animals fed the AIN-76A diet gained weight to a greater extent than those fed the Wayne or NIH-07 diets and exhibited lower levels of circulating 4-HPR.

Desarrollo de un metodo de analisis para la determinación de mefloquina en sangre humana secada sobre papel de filtro por cromatografía liquida de alta resolucion con deteccion ultravioleta / Development of an analysis method for determination of mefloquine in whole blood splotted on filter paper by high-performance liquid chromatography with ultraviolet detection

Se valida el método de análisis para determinar mefloquina en sangre humana secada sobre papel de filtro Wathman No. 3 por HPLC con detección ultravioleta. Se hace extracción líquido-líquido y la separación se realiza con SPE un cartucho C18 por elución isocrática; la fase móvil utilizada fue acetonitrilo : fosfato de potasio monobàsico (40:60) y el analito fue monitoreado a 222 nm. Los límites de detección y cuantificación son de 97,5 ng / mL y 136,2 ng / mL respectivamente. La separación cromatográfica del analito demora 9 minutos y la cuantificación se hace por el método del estándar externo. Este método es relativamente simple, rápido y sensible y puede utilizarse para el monitoreo de estudios clínicos y farmacocinéticos.