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  • 1
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] The progressive familial intrahepatic cholestases (PFIC) are a group of inherited disorders with severe cholestatic liver disease from early infancy. A subgroup characterized by normal serum cholesterol and γ-glutamyltranspeptidase (γGT) levels is genetically heterogeneous with loci ...
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 171-175 
    ISSN: 1432-1076
    Keywords: Key words Children ; Liver ; transplantation ; Immunosuppression ; Viral diseases ; Development
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Orthotopic liver transplantation is now routinely performed as a cure of numerous untreatable paediatric liver diseases. Evaluation of post-transplant quality of life is subjective and very difficult. It has to take into account the pre-transplant quality of life and the emotional stress to the family. Transplantation saves life in 65%–90% of the patients. Several diseases may, however, recur after transplantation, such as hepatitis B, C or NANB, or tumours. Some metabolic diseases may also progress in other organs. Extra-hepatic manifestations or sequelae may persist after transplantation. Complications of transplantation include renal function impairment, hypertension, viral and opportunistic diseases. Of particular concern is the post-transplant lymphoproliferative syndrome. Liver transplantation is able to restore growth. Children are less frequently admitted to hospital after transplantation, take fewer medications, return to school, are less dependent and interact more normally with their peers. Quality of life may not reach perfection, and depends also on the way our society accepts these imperfections.
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 171-175 
    ISSN: 1432-1076
    Keywords: Children ; Liver transplantation ; Immunosuppression ; Viral diseases ; Development
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Orthotopic liver transplantation is now routinely performed as a cure of numerous untreatable paediatric liver diseases. Evaluation of post-transplant quality of life is subjective and very difficult. It has to take into account the pre-transplant quality of life and the emotional stress to the family. Transplantation saves life in 65%–90% of the patients. Several diseases may, however, recur after transplantation, such as hepatitis B, C or NANB, or tumours. Some metabolic diseases may also progress in other organs. Extra-hepatic manifestations or sequelae may persist after transplantation. Complications of transplantation include renal function impairment, hypertension, viral and opportunistic diseases. Of particular concern is the post-transplant lymphoproliferative syndrome. Liver transplantation is able to restore growth. Children are less frequently admitted to hospital after transplantation, take fewer medications, return to school, are less dependent and interact more normally with their peers. Quality of life may not reach perfection, and depends also on the way our society accepts these imperfections.
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  • 4
    ISSN: 1432-2277
    Keywords: Liver transplantation, rat, cervical ; Accessory liver transplantation, rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Current methods for accessory liver transplantation in the rat require a high degree of microsurgical expertise and long training before success is achieved. We present a simpler method of arterialized accessory liver transplantation using the cervical vessels for revascularization of the transplanted liver with the cuff technique, which is useful for studies of liver preservation, reperfusion injury, and liver regeneration. After classical 70% hepatectomy is performed on the graft, the right common carotid artery is anastomosed to the donor aorta, the distal right external jugular vein is anastomosed to the donor portal vein, and the proximal right external jugular vein is anastomosed to the donor supradiaphragmatic inferior vena cava. The skin is not closed over the cervically transplanted liver (CTL). This method was used 30 times for periods of up to 6 h with a 90% success rate. CTL structure and function, as revealed by histology, bile flow rates, biliary bilirubin concentrating capacity, membrane potential, enzyme activity and distribution, have shown the CTL to be a structurally normal and metabolically active graft. In conclusion, the cervical approach to arterialized accessory liver transplantation is simple, and should prove useful for studies of liver preservation, reperfusion, regeneration, physiology, and toxicology.
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  • 5
    ISSN: 1432-2277
    Keywords: Key words Pediatric liver transplantation ; Neoral ; pharmacokinetics ; Liver transplantation ; pediatric ; Neoral ; Neoral ; liver transplantation ; pediatric ; Pharmacokinetics ; Neoral ; pediatric liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pediatric liver transplant recipients constitute a population characterized by a particularly unpredictable and poor bioavailability of cyclosporin (CyA). Even though several adult studies show that the new oral formulation of CyA, Neoral (NEO), produces better bioavailability and blood level predictability, few data describe its pharmacokinetics in children. We performed a complete analysis of the pharmacokinetics of NEO in ten small children after primary liver transplantation. Three pharmacokinetic profiles were set up with data obtained from tests taken during i. v. administration of CyA, after the first oral NEO dose, and after the last NEO dose before discharge from the hospital. The mean half-lives obtained were 8.1, 7.7, and 6.9 h, respectively, and the bioavailabilities were 22 % and 21 % for the first and last NEO doses. A large interpatient variability was observed. This was due, in part, to episodes of diarrhea that interfered with the pharmacokinetic evaluation and, in part, to the variability of post-transplant hepatic function. There was a good correlation between CyA trough levels and their related AUCs for both NEO profiles (r = 0.93 and r = 0.74, respectively). We conclude that, even though the pediatric OLT population remains more unpredictable than that of adults, NEO has a relatively rapid half-life and a remarkably improved bioavailability.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 37 (1992), S. 1250-1252 
    ISSN: 1573-2568
    Keywords: hepatitis B virus ; vaccination ; children ; cirrhosis ; orthotopic liver transplantation ; recombinant HBV vaccine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty-five children with cholestatic cirrhosis due to extrahepatic biliary atresia received a course of hepatitis B vaccine. Forty-seven received a plasma-derived vaccine and eight a recombinant vaccine. Antibody determination was evaluated before and after liver transplantation in 30 patients. Twenty-five additional patients had antibody determination after transplantation only. Protection against hepatitis B was observed in 73.3% of the children evaluated prior to transplantation. One to 15 months after transplantation, 54.6% of all children studied showed protective levels of anti HBs. We conclude that hepatitis B vaccination is efficient in inducing immunity in the majority of children with cholestatic cirrhosis. Some patients will loose immunity under immunosuppression, but the protection rate remains higher than reported for patients vaccinated after transplantation.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 38 (1993), S. 1091-1098 
    ISSN: 1573-2568
    Keywords: microvillous enzymes ; secretory component ; polyamines ; intestinal maturation ; trophic hormones
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To evaluate the role of dietary polyamines in maturation of the rat small intestine, spermine was given orally twice daily to suckling pups from day 10 to day 14 postpartum at different doses: 0, 0.2, 0.5, 1, 2.5, and 5 μmol/dose. Compared, to saline treated controls, spermine (5 μmol) produced significant increases in mucosal mass parameters (+12 to +57%,P〈0.05), induced prematurely, an adult pattern of microvillous enzymes, and enhanced respectively, by 19- and 3.5-fikd (P〈0.01 vs controls) the concentration of the secretory component ofp-immunoglobulins in villous and crypt cells. The response of microvillous enzymes (lactase, sucrase, maltase, and aminopeptidase) to spermine was dose-dependent and-specific since oral administration of arginine (5 μmol) or ornithine (5 μmol) was without effect. Intestinal changes were found to be significant (P〈0.05) for doses of spermine exceeding 1 μmol/day, which is in the range of the amount of polyamines provided by solid pellets at weaning (0.4 μmol/g). However, intestinal changes were undetectable at the physiological amounts of polyamines consumed by pups from rat milk during the suckling period (less than 0.3 μmol/day). Consistent with a direct effect of spermine on the intestinal cell, the cytosolic activity of ornithine decarboxylase was depressed by 27-fold (P〈0.005 vs controls) in the jejunum, while inhibition of ornithine decarboxylase by α-difluoromethylornithine did markedly decrease but did not suppress the cell response to spermine. Alternately, plasma corticosteronemia, which was virtually, absent by day 14 in controls, ranged between 1.4 and 4.6 μg/dl in 60% (N=9) of the spermine-treated rats. These novel findings indicate that dietary polyamines exert direct and indirect trophic effects on the rat immature intestine and can trigger at a critical level of intake the adult expression of villus and crypt cell functions.
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