Hereditary ADAMTS 13 deficiency presenting as recurrent acute kidney injury

N. K. Hase; A. M. Iqbal; T. E. Jamale; M. Kulkarni; M. G. Kulkarni; K. J. Pradeep; E. Rurali; P. Shetty

doi:10.4103/0971-4065.101257

Hereditary ADAMTS 13 deficiency presenting as recurrent acute kidney injury

N. K. Hase, A. M. Iqbal, T. E. Jamale, M. Kulkarni, M. G. Kulkarni, K. J. Pradeep, E. Rurali, P. Shetty

Centro Cardiologico Monzino

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Pages (from-to)	298-300
Number of pages	3
Journal	Indian Journal of Nephrology
Volume	22
Issue number	4
DOIs	https://doi.org/10.4103/0971-4065.101257
Publication status	Published - Jul 2012

Keywords

ADAMTS 13 deficiency
chronic kidney disease
plasma therapy
proteinuria
recurrent acute kidney injury

ASJC Scopus subject areas

Nephrology

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10.4103/0971-4065.101257

Cite this

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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.",

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AU - Hase, N. K.

AU - Iqbal, A. M.

AU - Jamale, T. E.

AU - Kulkarni, M.

AU - Kulkarni, M. G.

AU - Pradeep, K. J.

AU - Rurali, E.

AU - Shetty, P.

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AB - 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.

KW - ADAMTS 13 deficiency

KW - chronic kidney disease

KW - plasma therapy

KW - proteinuria

KW - recurrent acute kidney injury

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Hereditary ADAMTS 13 deficiency presenting as recurrent acute kidney injury

Abstract

Keywords

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