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Effect of nonsteroidal anti-inflammatory drugs in children with Bartter syndrome

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Abstract

Background

Bartter syndrome (BS) is a salt-wasting tubulopathy with induced expression of cyclooxygenase-2 in the macula densa, leading to increased prostaglandin production and hyperreninemia. Nonsteroidal anti-inflammatory drugs (NSAIDs) are currently used in BS; however, there is limited information on the impact of NSAIDs at treatment initiation or the potential utility of plasma renin level to guide therapy in patients with BS.

Methods

We included 19 patients with BS treated with NSAIDs between 1994 and 2016. We assessed serum levels of renin, aldosterone, electrolytes, calcium, phosphorus, vitamin D, and intact parathyroid hormone (iPTH) before and after treatment initiation. We also recorded modifications in sodium and potassium supplements and changes in urine calcium.

Results

Median age at diagnosis was 0.9 months [IQR 0–6.9]. Seven patients had BS types 1 or 2, 12 had BS type 3 and two had no mutation identified. There was a trend towards a decrease in sodium chloride supplementation after initiation of NSAIDs. When defining response to treatment based on the normalization of plasma renin level, responders had a greater reduction in their electrolytes supplementation. NSAIDs treatment was associated with a reduction in urine calcium. Before treatment, half of the patients had elevated iPTH, but iPTH normalized following initiation of NSAIDs in all but one patient.

Conclusions

This study confirms that NSAIDs reduce urine wasting of sodium and calcium in patients with BS. Monitoring serum renin levels may be useful to identify the lowest effective dose of NSAIDs that optimizes reduction of urine electrolyte losses.

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Authors and Affiliations

  1. Pediatric Nephrology Department, Robert Debre University Hospital, APHP, 48, Bd Serurier, 75019, Paris, France

    Gaël Gasongo, Olivier Niel, Theresa Kwon, Marie-Alice Macher, Anne Maisin, Véronique Baudouin, Claire Dossier, Georges Deschênes & Julien Hogan

  2. Pediatric Nephrology Department, Children’s Healthcare of Atlanta, Emory School of Medicine, Atlanta, GA, USA

    Larry A. Greenbaum & Julien Hogan

Authors
  1. Gaël Gasongo
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  2. Larry A. Greenbaum
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  3. Olivier Niel
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  4. Theresa Kwon
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  5. Marie-Alice Macher
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  6. Anne Maisin
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  7. Véronique Baudouin
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  8. Claire Dossier
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  9. Georges Deschênes
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  10. Julien Hogan
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Correspondence to Julien Hogan.

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Gasongo, G., Greenbaum, L.A., Niel, O. et al. Effect of nonsteroidal anti-inflammatory drugs in children with Bartter syndrome. Pediatr Nephrol 34, 679–684 (2019). https://doi.org/10.1007/s00467-018-4135-8

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  • DOI: https://doi.org/10.1007/s00467-018-4135-8

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