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Titolo:
LIVER-TRANSPLANTATION IN A CASE OF HYPOPROTEINEMIA AND COAGULOPATHY
Autore:
KAWAI M; YORIFUJI T; YAMANAKA C; MIYAZAKI A; HATTORI H; UEMOTO S; INOMATA Y; TANAKA K; FURUSHO K;
Indirizzi:
KYOTO UNIV,FAC MED,DEPT PEDIAT,SAKYO KU,54 KAWAHARA CHO KYOTO 606 JAPAN KYOTO UNIV,FAC MED,DEPT TRANSPLANTAT IMMUNOL,SAKYO KU KYOTO 606 JAPAN
Titolo Testata:
Acta Paediatrica Japonica Overseas Edition
fascicolo: 1, volume: 40, anno: 1998,
pagine: 96 - 98
SICI:
0374-5600(1998)40:1<96:LIACOH>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
TYROSINEMIA;
Keywords:
COAGULOPATHY; FATTY LIVER; HYPOPROTEINEMIA; LIVER TRANSPLANTATION; TYROSINEMIA;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
8
Recensione:
Indirizzi per estratti:
Citazione:
M. Kawai et al., "LIVER-TRANSPLANTATION IN A CASE OF HYPOPROTEINEMIA AND COAGULOPATHY", Acta Paediatrica Japonica Overseas Edition, 40(1), 1998, pp. 96-98

Abstract

A female infant with hypoproteinemia and coagulopathy associated withhypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed ona low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly, hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore, LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day oflife. Thus, the disorder is designated as a new entity, namely 'congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia'.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/04/20 alle ore 23:20:17