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  • 1
    Electronic Resource
    Electronic Resource
    Liver transplantation after “successful” Kasai portoenterostomy: evidence that liver decompensation results from fibrotic encasement of central normal liver (1993)
    Springer
    Pediatric surgery international 8 (1993), S. 303-305 
    Details
    ISSN: 1437-9813
    Keywords: Biliary atresia ; Cirrhosis ; Liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two anicteric children were transplanted for hepatic decompensation 5 and 7 years after an initial “successful” Kasai portoenterostomy. Examination of the hepatectomy specimens showed two distinct zones: a central zone of apparently normal parenchyma with draining ductules surrounded by a peripheral zone of fibrotic tissue with no ductules. This is in contrast with the completely fibrotic explanted livers from children who are persistently jaundiced after an unsuccessful Kasai procedure. It is possible that the peripheral fibrotic tissue might have prevented the normal growth of the draining central segment, leading to hepatic decompensation at a later age. It may also contribute to the development of the portal hypertension seen so often in these patients. The earliest evidence of impending hepatic decompensation in these two cases was a steady decline in serum albumin level.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00173350
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Infantile hepatic haemangioendothelioma: an association with hemihypertrophy (1993)
    Springer
    Pediatric surgery international 8 (1993), S. 505-506 
    Details
    ISSN: 1437-9813
    Keywords: Hemihypertrophy ; Haemangioendothelioma ; Hepatic vascular tumours ; Pancreas ; Cyst
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 3-month-old infant with obvious hypertrophy affecting the right arm, leg, and face presented with a haemangioendothelioma arising from the right lobe of the liver that was not associated with cardiac failure. At diagnostic laparotomy the nature of the mass was confirmed and the right hepatic artery was ligated. Follow-up examination at 10 months revealed complete resolution of the intrahepatic mass. This appears to be the first report of an association between hemihypertrophy and hepatic haemangioendothelioma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00180356
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Anti-thymocyte globulin, cyclosporin A and granulocyte colony-stimulating factor for severe aplastic anaemia complicating paediatric liver transplantation (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 107-108 
    Details
    ISSN: 1432-1076
    Keywords: Key words Non A-E hepatitis ; Aplastic anaemia ; Orthotopic liver transplantation ; Anti-thymocyte globulin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a sustained trilineage haematopoietic response to intensified immunosuppressive therapy with anti-thymocyte globulin, cyclosporin A and recombinant human granulocyte colony-stimulating factor in a 4-year-old girl, who developed severe aplastic anaemia after orthotopic liver transplantation for fulminant liver failure induced by non A-E hepatitis. The outcome was successful and allows the following. Conclusion Intense immunosuppression in combination with haemopoetic growth factors and antimicrobial prophylaxis should be considered as first line therapy in severe aplastic anaemia after orthotopic liver trans plantation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050779
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Diaphragmatic nerve palsy in young children following liver transplantation (1998)
    Springer
    Transplant international 11 (1998), S. 281-283 
    Details
    ISSN: 1432-2277
    Keywords: Key words Liver transplantation ; child ; diaphragmatic nerve palsy ; Diaphragma ; nerve palsy ; liver transplantation ; Nerve palsy ; diaphragma ; liver transplantation ; Child ; diaphragmatic nerve palsy ; liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Diaphragmatic paralysis was identified in four children after liver transplantation. All presented with persistent right upper lobe atelectasis, pleural effusion and recurrent respiratory infections and could not be weaned from mechanical ventilatory support. Fluoroscopy and real-time ultrasound confirmed paradoxical right diaphragmatic movements. Diaphragmatic plication was undertaken and enabled rapid and sustained weaning from respiratory support in all four cases. Vascular clamping of the suprahepatic vena cava seems to be the cause. Diaphragmatic plication allows optimal recruitment of the respiratory muscles with a favourable impact on lung mechanics and gas exchange.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001470050142
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Bowel perforation after paediatric orthotopic liver transplantation (1998)
    Springer
    Transplant international 11 (1998), S. 301-304 
    Details
    ISSN: 1432-2277
    Keywords: Key words Liver transplantation ; intestinal perforation ; Intestinal perforation ; liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Bowel perforation is a well-recognized complication following orthotopic liver transplantation. Of 194 paediatric liver transplantations performed in our hospital, 13 patients (6.7 %) developed bowel perforation post-transplantation. Contributory factors included previous operation, steroid therapy and viral infection. The incidence was higher in children who underwent transplantation for biliary atresia after a previous Kasai portoenterostomy. Seven patients (53 % of this group) reperforated. Diagnosis may be difficult and a high index of suspicion is needed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001470050146
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    Paper (German National Licenses)
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  • 6
    Electronic Resource
    Electronic Resource
    α1-antitrypsin deficiency and liver disease: Clinical presentation, diagnosis and treatment (1991)
    Springer
    Journal of inherited metabolic disease 14 (1991), S. 497-511 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The α1-antitrypsin deficient subject (protease inhibitor (PI) phenotype ZZ) has an increased susceptibility to liver disease. The condition is most commonly identified in early infancy as a conjugated hyperbilirubinaemia with hepatitis (11%) or a bleeding state due to vitamin K malabsorption (2%). 50% of cases have cirrhosis and 25% die in the first decade of life. A further 2% present with cirrhosis in later childhood. Adult males are at risk of hepatoma development with or without cirrhosis. Diagnosis is by isoelectric focussing or allele-specific oligonucleotide hybridization. The treatment is that of cholestasis and cirrhosis including transplantation. The pathobiology of the deficiency state, the mechanism of liver damage and the vulnerability of the newborn liver are discussed in this review. A plea is made for a trial of infusions of α1-antitrypsin in early infancy, as is used safely but without proven efficacy in the emphysematous PIZZ subject. Prospects of therapy by gene modification are also reviewed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01797920
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    Paper (German National Licenses)
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