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  • 1
    ISSN: 1432-2277
    Keywords: Key words Alpha-1-antitrypsin deficiency ; Liver transplantation ; Childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Alpha-1-antitrypsin (a1-AT) deficiency is an inborn error of metabolism, which can cause liver disease. The condition is one of the most common genetic disorders in the Caucasian population. Here we review our experience with 21 children suffering from end-stage liver disease due to a1-AT deficiency. All children are PIZZ homozygotes. Nineteen of them initially presented with neonatal jaundice and two with hepatosplenomegaly in childhood. Twenty-five liver transplantions were performed. All children are currently alive at a median follow-up of 40 months. Liver replacement provides the only definite treatment for children with end-stage liver disease associated with a1-AT deficiency. Excellent results can be achieved by reducing waiting time for transplantation and by early referral to a liver transplant centre.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2277
    Keywords: Key words Bile acids ; donors ; liver transplantation ; Bile flow ; liver transplantation ; donor ; Liver transplantation ; bile flow ; donor Donor bile flow ; liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Many parameters currently used for the pre-transplant assessment of liver allografts, are not reliable enough in predicting the likelihood of early graft dysfunction or non-function. It is generally accepted that bile secretion is a sign of hepatic function post-transplant and that bile flow shows a close linear relationship to the secretion of bile acids (“apparent choleretic activity”). We have studied bile flow, biliary bile acid concentrations and composition and measured apparent choleretic activity from hepatic bile collected with a new technique under controlled conditions at the time of retrieval from 18 donor livers. More than three samples were collected from each of 13 donors and a total of 65 samples of hepatic bile were analysed. Of these, ten showed typical apparent choleretic activity with a positive slope in the regression line analysis (correlation coefficient of 0.9), validating our collection technique.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Transplant international 10 (1997), S. 475-477 
    ISSN: 1432-2277
    Keywords: Key words Segmental liver transplantation ; vena cava replacement ; Vena cava replacement ; segmental liver transplantation ; Liver transplantation ; segmental ; vena cava replacement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Reduced grafts represent an important technical development in paediatric liver transplantation. The use of a left lateral segment graft has required preservation of the native inferior vena cava to “piggy-back” the graft onto it. We report four children who underwent left lateral segment transplantation with caval replacement using the donor iliac vein because the native retrohepatic inferior vena cava was small, friable or difficult to preserve. There were no caval or hepatic vein complications post-transplant and the donor iliac vein proved to be a satisfactory interpositional graft. The technique offers the advantages of a wider retrohepatic cava avoiding venous outflow or caval obstruction, provides good tissue to suture and is well suited for the triangulation technique of the left hepatic vein.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-2277
    Keywords: Key words Tacrolimus ; Absorption ; Short bowel syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two children with intestinal failure due to short or absent small bowel who underwent isolated liver transplantation for liver disease related to parenteral nutrition. Both received reduced-size liver grafts whilst awaiting a suitable small bowel donor. Immunosuppressive therapy was based on oral tacrolimus and intravenous steroids. Therapeutic levels of tacrolimus were achieved at low dosage of 0.14–0.28 mg/kg per day. Median and mean blood tacrolimus levels were 9.9 and 13.7 ng/ml (range 4.9–42.3 ng/ml) in case 1 and 5.8 and 7.2 ng/ml (range 1–30 ng/ml) in case 2 before small bowel transplantation, respectively. Following small bowel transplantation, levels were 17.1 and 20.1 ng/ml (range 9.2– 30 ng/ml), with oral doses of 0.54–1.35 mg/kg per day. Both children died of adenovirus pneumonia, with functioning grafts. Our experience demonstrates that effective levels of immunosuppression can be achieved by oral administration of tacrolimus in children with short or absent small bowel.
    Type of Medium: Electronic Resource
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