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Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality

Lookup NU author(s): Professor Judith Goodship

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Abstract

We report a patient with continuously recurring hemolytic-uremic syndrome due to factor H deficiency. First at the age of 3 months he showed signs of hemolytic anemia, thrombocytopenia and renal insufficiency, often recurring concomitantly with respiratory tract infections, despite weekly to twice weekly plasma substitution (20 ml/kg body weight). Now at the age of 3.5 years glomerular filtration rate is approximately 50 ml/min/1.73 m2 and psychomotoric development is normal. Since factor H is mainly synthesized in the liver, hepatic transplantation has been proposed as curative treatment. Before justification of liver transplantation as the ultimate treatment for these patients, an international registry should be developed to optimize and standardize therapeutic alternatives.

Publication metadata

Author(s): Gerber A, Kirchhoff-Moradpour AH, Obieglo S, Brandis M, Kirschfink M, Zipfel PF, Goodship JA, Zimmerhackl LB

Publication type: Article

Publication status: Published

Journal: Pediatric Nephrology

Year: 2003

Volume: 18

Issue: 9

Pages: 952-955

ISSN (print): 0931-041X

ISSN (electronic): 1432-198X

Publisher: Springer

URL: http://dx.doi.org/10.1007/s00467-003-1192-3

DOI: 10.1007/s00467-003-1192-3

PubMed id: 12836093

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