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J Nephropathol. 2017;6(4): 359-362.
doi: 10.15171/jnp.2017.59

Scopus ID: 85036593658
  Abstract View: 4059
  PDF Download: 1626

Case Report

A unique finding of normal aldosterone level in Bartter’s
syndrome

Rupesh Raina 1*, Tushar Chaturvedi 2, Shyam Polaconda 2, Amrita Siri Mukunda 2, Sidharth Kumar Sethi 3, Vinod Krishnappa 2

1 Division of Nephrology, Cleveland Clinic Akron General, Akron, Ohio, USA
2 Cleveland Clinic Akron General/Akron Nephrology Associates, Akron, Ohio, USA
3 Kidney Institute, Medanta, The Medicity Hospital, Gurgaon, Haryana, India
*Corresponding Author: *Corresponding author: Rupesh Raina, Email: rraina@chmca.org,, Email: raina@akronnephrology.com

Abstract

Background: Bartter’s syndrome is a rare autosomal recessive renal tubular disorder
characterized by hypokalemia, hypochloremia, metabolic alkalosis, hyperreninemia
and hyperaldosteronemia with normotension. Bartter syndrome has five types; type 1
(mutation in sodium/potassium chloride transporter), type 2 (mutation in voltage gated
potassium channel), type 3 (mutation on chromosome 1 that encodes Barttin and makes
only kidney-specific chloride channel B non-functional), type 4 ( mutation in BSND gene
encoding Barttin and makes both kidney-specific chloride channels A & B non-functional)
and type 5 (L125P gain in function mutation in calcium-sensing receptor).

Case Presentation: A 28-year-old male was hospitalized for evaluation of nausea, vomiting,
generalized weakness and persistent chronic hypokalemia. Bartter’s syndrome was
suspected based on clinical and laboratory evidence, however serum aldosterone level was
normal. Further genetic testing confirmed the diagnosis of Bartter’s syndrome type 3.

Conclusions: We report a case of Bartter’s syndrome type 3 with a unique finding of normal
aldosterone level.


Implication for health policy/practice/research/medical education:

Bartter’s syndrome with normal aldosterone level is a rare finding. The approach to such patients needs to be further explored.
The treatment of Bartter’s is long term potassium replacement, intravenous hydration and prostaglandin synthase inhibitors
like indomethacin and ketoprofen. COX-2 inhibitors such as rofecoxib can be substituted to avoid long-term side effects and
in patients refractory to indomethacin.

Please cite this paper as: Raina R, Chaturvedi T, Polaconda S, Mukunda AS, Sethi SK, Krishnappa V. A unique finding of normal
aldosterone level in Bartter’s syndrome. J Nephropathol. 2017;6(4):359-362. DOI: 10.15171/jnp.2017.59.

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Submitted: 18 Mar 2017
Accepted: 10 Jun 2017
ePublished: 27 Jun 2017
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