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Atypical hemolytic uremic syndrome: a case report
B M D B Basnayake1, A W M Wazil2, N Nanayakkara2
1Department of Nephrology and Renal Transplant, Teaching Hospital Kandy, Kandy, Sri Lanka. bmdbbasnayake@yahoo.com.
Journal of Medical Case Reports
|January 14, 2020
Summary
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy. Long-term therapeutic plasma exchange led to complete remission in a Sri Lankan girl with severe aHUS complications.
Area of Science:
- Nephrology
- Hematology
- Immunology
Background:
- Thrombotic microangiopathy (TMA) involves microvascular thrombosis, leading to thrombocytopenia, hemolytic anemia, and organ damage.
- Atypical hemolytic uremic syndrome (aHUS) is a severe TMA form caused by complement dysregulation, primarily affecting the alternative pathway.
- Clinical presentation and outcomes in aHUS vary based on underlying genetic or acquired mutations.
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