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  • 1
    Electronic Resource
    Electronic Resource
    Renal-hepatic-pancreatic dysplasia: an autosomal recessive disorder with renal and hepatic failure (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 791-795 
    Details
    ISSN: 1432-1076
    Keywords: Key words Renal-hepatic-pancreatic dysplasia ; Congenital ; hepatic fibrosis ; Liver cirrhosis ; Combined liver-kidney ; transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report two brothers with renal dysplasia and congenital hepatic fibrosis. One patient died shortly after birth of lung hypoplasia. The second developed end-stage renal failure at 14 months. The hepatic fibrosis progressed to cirrhosis and hepatic failure. Pancreatic function was normal, but increased echogenicity was seen on ultrasound. At age 3 years and 9 months a successful combined liver-kidney transplantation was performed. The features of our patients are compatible with the “renal-hepatic-pancreatic dysplasia” syndrome. Conclusion Renal-hepatic-pancreatic dysplasia is an autosomal recessive disorder with variable expression. Combined liver-kidney transplantation offers a new therapeutic option.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02002909
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Renal-hepatic-pancreatic dysplasia: An autosomal recessive disorder with renal and hepatic failure (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 791-795 
    Details
    ISSN: 1432-1076
    Keywords: Renal-hepaticpancreatic dysplasia ; Congenital hepatic fibrosis ; Liver cirrhosis ; Combined liver-kidney transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abstract We report two brothers with renal dysplasia and congenital hepatic fibrosis. One patient died shortly after birth of lung hypoplasia. The second developed end-stage renal failure at 14 months. The hepatic fibrosis progressed to cirrhosis and hepatic failure. Pancreatic function was normal, but increased echogenicity was seen on ultrasound. At age 3 years and 9 months a successful combined liver-kidney transplantation was performed. The features of our patients are compatible with the “renal-hepatic-pancreatic dysplasia” syndrome. Conclusion Renal-hepatic-pancreatic dysplasia is an autosomal recessive disorder with variable expression. Combined liver-kidney transplantation offers a new therapeutic option.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02002909
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Epoetin alfa in anaemic children or adolescents on regular dialysis (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 509-512 
    Details
    ISSN: 1432-1076
    Keywords: Renal anaemia ; Human recominant erythropoietin ; Epoetin alfa ; Renal hypertension ; Hyperkalaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Eighteen patients aged 5–18 years on regular dialysis had a packed cell volume (PCV)〈0.27. On treatment with epoetin alfa (EA) PCV increased by 0.05 or more in all patients. Iron supplementation was necessary in 13 patients with a ferritinaemia 〈300 μg/l before study. During treatment, plasma potassium increased significantly and more vigorous antihypertensive measures were required in 8 patients, 5 of them being already on antihypertensive drugs before EA. Iliofemoral thrombosis occurred in 1 patient 10 days after renal transplant. The data indicate that EA ameliorates the anaemia of chronic renal disease. The main concerns arising during treatment with EA are hyperkalaemia, arterial hypertension and possibly thrombosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01958435
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
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