Transient neonatal hyperkalemia in the antenatal (ROMK defective) Bartter syndrome

Gal Finer, Hanna Shalev, Ohad S. Birk, Dalia Galron, Nikola Jeck, Levana Sinai-Treiman, Daniel Landau*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

91 Scopus citations


Objective: Identification of neonatal hyperkalemia as a complication of Bartter syndrome (BS), a disorder usually characterized by hypokalemic metabolic alkalosis. Study design: Case-series description of a group of 12 infants with mutations in the renal potassium channel ROMK, causing one of the antenatal variants of BS. Results: Prematurity, postnatal polyuria, and dehydration were seen in all cases. Plasma potassium was as high as 9.0 ± 1.2 mmol/L and sodium as low as 124 ± 3.5 mmol/L, appearing usually at day 3 of life and normalizing by the end of the first postnatal week. No hyperkalemia was found in 12 neonates with the variant of BS and deafness. The mean plasma potassium level during the first week of life among a group of very low-birth-weight infants with similar relative azotemia was 4.9 ± 1 mmol/L (P < .001). The postneonatal period in the ROMK-defective children with BS was characterized by failure to thrive, hypercalciuria, nephrocalcinosis, and minimal-to-no hypokalemia. Conclusions: Early postnatal hyperkalemia, sometimes severe, may complicate antenatal BS associated with ROMK mutations. Its association with hyponatremia and hyperreninemic hyperaldosteronism may erroneously suggest the diagnosis of pseudohypoaldosteronism type 1. The expression of ROMK in both the thick ascending limb and cortical collecting duct may explain this apparently tubular maturation phenomenon.

Original languageEnglish (US)
Pages (from-to)318-323
Number of pages6
Journaljournal of pediatrics
Issue number3
StatePublished - Mar 1 2003

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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