摘要:

AbstractBetween March 1984 and August 1994, 13 orthotopic liver transplantations were performed in 13 patients ≦ 25 years of age. The indications included Wilson's disease (n= 7), biliary atresia (n= 4), choledochal cyst (n=1) and hepatitis C cirrhosis (n=1). Technical variants included full‐size (n=11), reduced‐size (n=1) and living‐related (n=1) liver transplantation. These recent technical innovations have offered an expanded donor pool for earlier transplantation, shorter waiting times and excellent quality grafts. Surgical complications occurred in six patients; all required additional surgery. Biliary complications were encountered more commonly in our earlier patients. Our actuarial patient and graft survival rate is 92% at 2 years. The long‐term follow‐up of our liver‐transplanted Wilson's disease patients provides confirmatory evidence that orthotopic liver transplantation cures the underlying metabolic defect with complete normalization of biochemical abnormalities of copper metabolism, reversal of neurological impairments and the disappearance of Kayser‐Fleischer corneal rings. The high rate of patient survival and excellent rehabilitation indicate that with prudent clinical judgement, liver transplantation can be achieved with an acceptable rate of morbidity, mortality and cost in a setting where manpower and donor organs a

ISSN:0815-9319    

DOI:10.1111/j.1440-1746.1996.tb01773.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractUse of long‐term total parenteral nutrition (TPN) is often presumed to be associated with serious hepatic dysfunction. In this retrospective study, we reviewed the complete charts of patients who had received TPN for more than 2.5 years, starting in infancy or childhood, for evidence of liver dysfunction. There were 16 male and 10 female patients with a total of 254.5 patient years on TPN. Seventeen patients have been on TPN since birth or early infancy. Thirteen of 26 patients derive ≧90% of their calorie intake from TPN. Six patients had hepatomegaly; two of them also had splenomegaly. Twenty‐one patients had normal transaminases, nine have had past episodes of raised enzymes ranging from 2.5 to 7.5 times normal. Seventeen patients always had normal bilirubin levels, five had past episodes of hyperbilirubinaemia, while four patients had persistently raised bilirubin levels (range 1.5–20.7 g/dL). Alkaline phosphatase was normal for age in all patients except two. Hepatic synthetic function, as measured by albumin, pre‐albumin levels and prothrombin time, was within the normal range in all patients except one. Liver biopsies were performed in eight patients. Two biopsies showed cirrhosis, one showed chronic active hepatitis (CAH) with cholestasis, two patients had fibrosis, one showed cholestasis and two biopsies were normal. One patient with cirrhosis and one with CAH were positive for hepatitis C antibody. Another asymptomatic patient was positive for hepatitis B. Only the patient with CAH had hepatic decompensation. We conclude that clinical hepatic failure is uncommon in our group of patients on long‐term TPN for 2.5 years or more. Cirrhosis and fibrosis, when found, could not be solely attrib

ISSN:0815-9319    

DOI:10.1111/j.1440-1746.1996.tb01774.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

AbstractOrthotopic liver transplantation (OLT) is now a definitive treatment option for most cases of endstage liver disease (ESLD) in children. Efforts now focus on active supportive treatment to maintain, if not improve, the patient's clinical status before OLT and to ensure normal patterns of growth and development after OLT. Malnutrition adversely affects the outcome of OLT and is probably the single area in pre‐operative management where the largest potential improvement can be made. Our studies indicate significant abnormalities of protein energy metabolism and body composition in children referred for OLT. We have shown that the use of enteral formulae, enriched with branched‐chain amino acids, have significant advantages. Other adjunctive therapy, such as growth hormone, is the subject of current investigation. Following transplantation, catch‐up weight and growth does occur with the advent of normal liver functioning, but patients at continuing risk for undernutrition, such as those with rejection and/or chronic infection, need to be targeted for specific nutritional th

ISSN:0815-9319    

DOI:10.1111/j.1440-1746.1996.tb01775.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0815-9319    

DOI:10.1111/j.1440-1746.1996.tb01834.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0815-9319    

DOI:10.1111/j.1440-1746.1996.tb01835.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY