Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality
- 1 September 2003
- journal article
- case report
- Published by Springer Nature in Pediatric Nephrology
- Vol. 18 (9) , 952-955
- https://doi.org/10.1007/s00467-003-1192-3
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.Keywords
This publication has 11 references indexed in Scilit:
- Combined kidney and liver transplantation for familial haemolytic uraemic syndromeThe Lancet, 2002
- Hemolytic uremic syndrome: how do factor H mutants mediate endothelial damage?Trends in Immunology, 2001
- Successful plasma therapy in hemolytic uremic syndrome with factor H deficiencyPediatric Nephrology, 2001
- Targeting complement in therapyImmunological Reviews, 2001
- Familial hemolytic uremic syndrome associated with complement factor H deficiencyThe Journal of Pediatrics, 2001
- The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20.2001
- Clustering of Missense Mutations in the C-Terminal Region of Factor H in Atypical Hemolytic Uremic SyndromeAmerican Journal of Human Genetics, 2001
- Familial relapsing haemolytic uraemic syndrome and complement factor H deficiencyNephrology Dialysis Transplantation, 1999
- Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor HPediatric Nephrology, 1998
- A Simple Estimate of Glomerular Filtration Rate in Children Derived From Body Length and Plasma CreatininePediatrics, 1976