Accessible Requires Authentication Published by De Gruyter March 7, 2019

Growth hormone deficiency in children with antenatal Bartter syndrome

Inna Spector-Cohen, Ariel Koren, Waheeb Sakran, Yardena Tenenbaum-Rakover and Rephael Halevy

Abstract

Background

Bartter syndrome is a group of rare autosomal-recessive renal disorders characterized by hypokalemic hypochloremic metabolic alkalosis associated with severe growth failure; the exact causes for growth retardation are unclear. GH deficiency (GHD) has been reported in a few cases of Bartter syndrome. The aim of our study was to determine the prevalence of GHD in children with antenatal Bartter syndrome and to assess their response to GH therapy.

Methods

Ten patients aged 1.5–14.5 years and diagnosed with antenatal Bartter syndrome were enrolled. Seven children with short stature underwent GH stimulation tests.

Results

Common presenting symptoms were failure to thrive and polyuria. The mean patient height at study entry was −2.7 standard deviation (SD) (range 0.89 to −5.95) and mean weight (SD) was −1.7 (range 1.89 to −4.11). A decline in height and weight (SD) was observed over the years. GHD was diagnosed in four children and GH therapy was started in all of them. Two patients responded very well and gained >1 SD in height, one patient stopped therapy due to non-adherence and one had a poor response.

Conclusions

In addition to other important causes for poor growth in antenatal Bartter syndrome, our findings suggest that GHD should also be considered as a cause of growth retardation and therefore, clinical assessment of the GH axis is recommended. GH therapy has a role in the treatment of growth failure in some individuals with Bartter syndrome.


Corresponding author: Yardena Tenenbaum-Rakover, MD, Associate Professor in Pediatric Endocrinology, Director, Pediatric Endocrinology Institute, Ha’Emek Medical Center, Afula 1834111, Israel; and Rappaport Faculty of Medicine, Technion Institute of Technology, Haifa, Israel, Phone: +97246495203, Mobile: +972523545626, Fax: +97246495289s
aInna Spector-Cohen, Yardena Tenenbaum-Rakover and Rephael Halevy contributed equally to the study.

Acknowledgments

We are grateful to the Pediatric Endocrine Team for fruitful collaboration. We thank Camille Vainstein for professional language editing and Naama Schwartz for the statistical analysis. Thanks to the patients and their families for participating in the study.

  1. Author contributions: All the authors have accepted responsibility for the entire content of this submitted article and approved submission.

  2. Research funding: This study was supported by a grant from the Academic Committee of Ha’Emek Medical Center.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. Competing of interest: The funding organization played no role in the study design, in the collection, analysis or interpretation of data, in the writing of the report, or in the decision to submit the report for publication.

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Received: 2018-04-25
Accepted: 2018-08-06
Published Online: 2019-03-07
Published in Print: 2019-03-26

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