LIVER-TRANSPLANTATION IN A CASE OF HYPOPROTEINEMIA AND COAGULOPATHY

Citation
M. Kawai et al., LIVER-TRANSPLANTATION IN A CASE OF HYPOPROTEINEMIA AND COAGULOPATHY, Acta Paediatrica Japonica Overseas Edition, 40(1), 1998, pp. 96-98
Citations number
8
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Pediatrics
ISSN journal
0374-5600
Volume
40
Issue
1
Year of publication
1998
Pages
96 - 98
Database
ISI
SICI code
0374-5600(1998)40:1<96:LIACOH>2.0.ZU;2-P
Abstract
A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver tr ansplantation (LRLT). On the 12th day of life plasma amino acid analys is revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly , hypotonia, alopecia, eczema and psychomotor delay did not improve an d recurrent episodes of disseminated intravascular coagulation (DIC) c aused her condition to deteriorate. Liver biopsy on the 230th day reve aled marked fatty change accompanied by mild to moderate cholestasis. Therefore, LRLT from her father was performed on the 286th day resulti ng in improvement of all the aforementioned signs and symptoms. Despit e a thorough examination, no diagnosis of a known disorder could be es tablished. However, her elder brother had also been born with severe h ypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely 'congen ital hypoproteinemia and coagulopathy associated with hypertyrosinemia '.