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Bartter syndrome complicated by immune complex nephropathy

Case report and literature review

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Abstract

The unusual coincidence of Bartter syndrome and C1q nephropathy is described and the literature reviewed. An African-American girl presented at 4 years of age with acute hyponatremic dehydration and failure to thrive. Persistent hypokalemic alkalosis and secondary hyperaldosteronism were found. The case was atypical for Bartter syndrome in that proteinuria (0.19 g/day) was present. Renal biopsy showed juxtaglomerular hyperplasia and C1q nephropathy. Molecular analysis showed deletion of the renal chloride channel gene (CLCNKB) typical of autosomal recessive childhood Bartter syndrome. Chronic sodium and potassium chloride replacement therapy together with indomethacin normalized her metabolic status, and she experienced catch-up growth. Proteinuria persisted, however. This is the first documentation of C1q nephropathy, in mild form, complicating autosomal recessive Bartter syndrome. This case shows the importance of the renal biopsy and of molecular analysis in delineating the cause of atypical nephropathy associated with Bartter syndrome. These findings add to the evidence of a possible association between the congenital syndrome and acquired immune complex nephropathy.

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Acknowledgements

Dr. Rosenfield's research is supported in part by the University of Chicago General Clinical Research Center (USPHS RR-00055). We thank Connie Robinson, RN, for her help in data collection.

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Author notes

  1. Yahya Sardani

    Present address: Children's Hospital of Austin, 1400 IH 35, Austin, TX 78701, USA

  2. Mark Haas

    Present address: Department of Pathology, Johns Hopkins University School of Medicine, 712 Pathology Bldg., 600 N. Wolfe St., Baltimore, MD 21287, USA

  3. Andrew J. Aronson

    Present address: Hope Children's Hospital, 4440 West 95th Street, Oak Lawn, IL 60453, USA

Authors and Affiliations

  1. Department of Pediatrics, The University of Chicago Pritzker School of Medicine, 5839 S. Maryland Avenue, Chicago, IL 60637-1470, USA

    Yahya Sardani, Kenan Qin, Andrew J. Aronson & Robert L. Rosenfield

  2. Department of Pathology, The University of Chicago Pritzker School of Medicine, 5839 S. Maryland Avenue, Chicago, IL 60637-1470, USA

    Mark Haas

  3. Section of Pediatric Endocrinology, The University of Chicago Children's Hospital, 5839 S. Maryland Avenue (M/C 5053), Chicago, IL 60637-1470, USA

    Robert L. Rosenfield

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  1. Yahya Sardani
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Correspondence to Robert L. Rosenfield.

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Sardani, Y., Qin, K., Haas, M. et al. Bartter syndrome complicated by immune complex nephropathy. Pediatr Nephrol 18, 913–918 (2003). https://doi.org/10.1007/s00467-003-1194-1

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  • DOI: https://doi.org/10.1007/s00467-003-1194-1

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