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NDT Advance Access published online on June 28, 2005
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfh893
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© The Author [2005]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
Received January 12, 2005
Accepted April 13, 2005

Brief Report

Severe syncope and sudden death in children with inborn salt-losing hypokalaemic tubulopathies

Cinzia Cortesi 1, Alberto Bettinelli 2, Francesco Emma 3, Michel Fischbach 4, Paolo Bertolani 5, and Mario G. Bianchetti 1*

1 Pediatric Renal Unit, University of Milan Medical School, De Marchi Hospital, Milan, Italy; Department of Pediatrics, San Giovanni Hospital, Bellinzona, Switzerland
2 Pediatric Renal Unit, University of Milan Medical School, De Marchi Hospital, Milan, Italy; Division of Pediatrics, Leopoldo Mandic Hospital, Merate, Italy
3 Division of Nephrology, Children's Hospital Bambino Gesù, Rome, Italy
4 Division of Pediatric Nephrology, Hautepierre Hospital, Strasbourg, France
5 Department of Pediatrics, University of Modena, Italy

* To whom correspondence should be addressed.
Mario G. Bianchetti, E-mail: mario.bianchetti{at}pediatrician.ch



  Abstract

Background. Potassium deficiency may cause cardiac arrhythmias culminating in syncope or sudden death.

Methods. An inquiry performed among physicians caring for a total of 249 patients with inborn salt-losing tubulopathies revealed that acute cardiac complications occurred in seven children.

Results. Four patients died suddenly and three had severe syncope. These episodes occurred in the context of severe chronic hypokalaemia (≤2.5 mmol/l) or were precipitated by acute diseases, which exacerbated hypokalaemia (≤2.0 mmol/l).

Conclusions. In conclusion, severe chronic or acute hypokalaemia is hazardous in inborn salt-losing tubulopathies.

Keywords: arrhythmias; Bartter syndrome; Gitelman syndrome; hypokalaemia; sudden death.
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