Document Detail


Transient neonatal hyperkalemia in the antenatal (ROMK defective) Bartter syndrome.
MedLine Citation:
PMID:  12640382     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Gal Finer; Hanna Shalev; Ohad S Birk; Dalia Galron; Nikola Jeck; Levana Sinai-Treiman; Daniel Landau
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  142     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-03-17     Completed Date:  2003-04-10     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  318-23     Citation Subset:  AIM; IM    
Affiliation:
Departments of Pediatrics and Genetics, Soroka University Medical Center, Ben Gurion University of the Negev, Beer Sheva, Israel.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Bartter Syndrome / complications,  congenital*,  genetics,  metabolism
Child
Child, Preschool
Creatinine / blood
Female
Follow-Up Studies
Gestational Age
Humans
Hyperkalemia / complications*
Infant
Infant, Newborn
Infant, Very Low Birth Weight
Male
Mutation
Nephrocalcinosis / complications
Potassium / blood
Potassium Channels / genetics*
Potassium Channels, Inwardly Rectifying / genetics
Urea / blood
Chemical
Reg. No./Substance:
0/KCNJ1 protein, human; 0/Potassium Channels; 0/Potassium Channels, Inwardly Rectifying; 57-13-6/Urea; 60-27-5/Creatinine; 7440-09-7/Potassium

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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