Option of pre-emptive nephrectomy and renal transplantation for Bartter's syndrome
Abanti Chaudhuri,
Oscar Salvatierra Jr,
Steven R. Alexander,
Minnie M. Sarwal,
Abanti Chaudhuri
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorOscar Salvatierra Jr
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorSteven R. Alexander
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorMinnie M. Sarwal
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorAbanti Chaudhuri,
Oscar Salvatierra Jr,
Steven R. Alexander,
Minnie M. Sarwal,
Abanti Chaudhuri
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorOscar Salvatierra Jr
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorSteven R. Alexander
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorMinnie M. Sarwal
Department of Pediatrics and Surgery, Stanford University, CA, USA
Search for more papers by this authorMinnie M. Sarwal, G306, 300 Pasteur Drive, Stanford University, CA 94304, USA
Tel.: 540 723 7903
Fax: 650 498 6714
E-mail: [email protected]
Abstract
Abstract: Bartter's syndrome (BS) is an incurable genetic disease, with variable response to supportive therapy relating to fluid and electrolyte management. Poor control or therapy non-compliance may result in frequent life threatening episodes of dehydration, acidosis and hypokalemia, with resultant adverse effects on patient quality of life (QOL). We report, for the first time, pre-emptive bilateral native nephrectomies and successful renal transplantation, prior to the onset of ESRD, for severe, clinically brittle, neonatal BS, resulting in correction of metabolic abnormalities and excellent graft function. We propose that fragile BS should be considered as a possible indication for early native nephrectomies and pre-emptive renal transplantation, procedures that results in a ‘cure’ for the underlying disease and significant improvements in patient QOL.
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