Acute liver failure in infancy: A 14-year experience of a pediatric liver transplantation center

被引:142
作者
Durand, P
Debray, D
Mandel, R
Baujard, C
Branchereau, S
Gauthier, F
Jacquemin, E
Devictor, D
机构
[1] Univ Paris Sud, Hop Bicetre, Assistance Publ Hop Paris, Inserm U347,Serv Reanimat, F-94275 Le Kremlin Bicetre, France
[2] Univ Paris Sud, Hop Bicetre, Assistance Publ Hop Paris, Inserm U347,Serv Chirurg, F-94275 Le Kremlin Bicetre, France
[3] Univ Paris Sud, Hop Bicetre, Assistance Publ Hop Paris, Inserm U347,Serv Hepatol Pediat, F-94275 Le Kremlin Bicetre, France
[4] Univ Paris Sud, Hop Bicetre, Assistance Publ Hop Paris, Dept Anesthesiol, F-94275 Le Kremlin Bicetre, France
关键词
D O I
10.1067/mpd.2001.119989
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Clinical charts of 80 infants 14-year period (1986 to 2000) with acute liver failure (ALF), defined as prolonged prothrombin time greater than 17 seconds and decrease of clotting factor V plasma level below 50% of normal, were reviewed retrospectively. The main causes of ALF, were inherited metabolic disorders in 42.5% of cases, including mitochondrial respiratory chain disorders in 17, type I hereditary tyrosinemia in 12, and urea cycle disorders in 2, neonatal hemochromatosis in 16% of cases; and acute viral hepatitis in 15% of cases (hepatitis B in 6, herpes virus type 6 in 4, and herpes simplex virus type I in 2). The cause of ALF remained undetermined in 16% of cases. A total of 19 (24%) infants survived without orthotopic liver transplantation; 38 (47%) infants died from sepsis, multiple organ failure, or because the underlying disease contraindicated orthotopic liver transplantation (12 [15%] infants), and 23 (29%) infants underwent orthotopic liver transplantation within 12 months from onset, 12 of whom are alive with a mean follow-up period of 5.2 years from orthotopic liver transplantation. We conclude that ALF during the first year of life is a severe condition with poor prognosis, despite the advent of liver transplantation.
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收藏
页码:871 / 876
页数:6
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