Acute hemolytic crisis with fulminant hepatic failure as the first manifestation of Wilson's disease: a case report.

We report a 27-year-old woman who developed Coombs' negative hemolytic anemia and fulminant hepatic failure as the initial manifestation of Wilson's disease. Unmeasurably low level of serum alkaline phosphatase provided a clue to the diagnosis of Wilson's disease. The diagnosis was es...

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Detalles Bibliográficos
Autores principales: Lee, J. J., Kim, H. J., Chung, I. J., Kook, H., Byun, J. R., Kwon, S. Y., Park, M. R., Choi, K. S., Hwang, T. J., Ryang, D. W.
Formato: Texto
Lenguaje:English
Publicado: Korean Academy of Medical Sciences 1998
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3054522/
https://www.ncbi.nlm.nih.gov/pubmed/9811188
Descripción
Sumario:We report a 27-year-old woman who developed Coombs' negative hemolytic anemia and fulminant hepatic failure as the initial manifestation of Wilson's disease. Unmeasurably low level of serum alkaline phosphatase provided a clue to the diagnosis of Wilson's disease. The diagnosis was established with the presence of Kayser-Fleischer ring, decreased serum ceruloplasmin level, and elevated urine and serum copper levels. In spite of repeated plasmapheresis, she died of multiorgan failure on the fifth hospital day.

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