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06:29
Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
Published on: January 29, 2014
Familial hemolytic uremic syndrome associated with complement factor H deficiency
D Landau1, H Shalev, G Levy-Finer
1Department of Pediatrics, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel.
The Journal of Pediatrics
|March 10, 2001
Summary
Factor H deficiency-hemolytic uremic syndrome (FHD-HUS) often recurs after kidney transplants. Prophylactic plasma therapy can prevent relapses and preserve kidney function in children with FHD-HUS.
Area of Science:
- Nephrology
- Hematology
- Genetics
Background:
- Atypical hemolytic uremic syndrome (HUS) associated with factor H deficiency (FHD) presents significant challenges due to poor prognosis.
- FHD-HUS often leads to end-stage renal disease and recurrent thrombotic microangiopathy.
Observation:
- A pediatric case of FHD-HUS experienced recurrent HUS and cerebral infarction post-renal transplantation, despite plasma transfusions.
- Another pediatric case with FHD-HUS showed successful relapse prevention and renal function preservation with prophylactic, twice-weekly plasma therapy.
Findings:
- Renal transplantation does not correct the underlying factor H deficiency, leading to recurrence of FHD-HUS.
- Prophylactic plasma therapy can elevate subnormal serum factor H levels and prevent HUS relapses.
Implications:
- FHD-HUS management requires addressing the underlying factor H deficiency, not just the renal manifestations.
- Prophylactic plasma therapy represents a potential strategy to improve outcomes for children with FHD-HUS.
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