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- Title
Hereditary ADAMTS 13 deficiency presenting as recurrent acute kidney injury.
- Authors
E. Jamale, T.; K. Hase, N.; Kulkarni, M.; M. Iqbal, A.; Rurali, E.; G. Kulkarni, M.; Shetty, P.; J. Pradeep, K.
- Abstract
We report here a case of 26-year-old male who presented with history of recurrent acute renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia. ADAMTS 13 deficiency due to mutation in the gene encoding for ADAMTS 13 was identified as the cause. After eight episodes of acute kidney injury (AKI), patient started developing hypertension, proteinuria, and renal insufficiency. Treatment with regular monthly plasma infusions prevented further episodes of AKI and stabilized the renal function. Hypertension and proteinuria are controlled with angiotensin II receptor blockers.
- Subjects
KIDNEY failure; BLOOD testing; HEMOLYTIC anemia; HYPERTENSION; GENETIC mutation; PERITONEAL dialysis; PROTEINURIA; THROMBOCYTOPENIA; GENETICS
- Publication
Indian Journal of Nephrology, 2012, Vol 22, Issue 4, p298
- ISSN
0971-4065
- Publication type
Case Study
- DOI
10.4103/0971-4065.101257