Document Detail

Resolution of hypertension during pregnancy in familial hyperkalemia and hypertension with the WNK4 Q565E mutation.
MedLine Citation:
PMID:  15696009     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Secondary hypertension during pregnancy usually carries high maternal and fetal morbidity and mortality rates. A rare form of monogenic hypertension is familial hyperkalemia and hypertension, which is caused by mutations in the kinases WNK1 or WNK4 and other unknown molecular defects. The purpose of the study was to examine the course of pregnancy in hypertensive women with familial hyperkalemia and hypertension. STUDY DESIGN: We prospectively studied 2 pregnancies of a woman with familial hyperkalemia and hypertension and the Q565E WNK4 mutation (pregnancies 1 and 2) and retrospectively studied the course of 2 pregnancies in another woman who was an affected member of this largest family described in the literature. RESULTS: Both women had hypertension (170-190/105-110 mm Hg), hyperkalemia (5.3-6.0 mmol/L), and hypercalciuria, all of which were well controlled by thiazides. During pregnancies, thiazides were discontinued; throughout the pregnancy, the blood pressure remained normal at 120 to 130/75 to 85 mm Hg; however, hyperkalemia and hypercalciuria, which were documented in pregnancies 1 and 2, persisted. Renin and aldosterone levels (which were measured in pregnancies 1 and 2) rose towards their end. Four normal infants were born. A woman with familial hyperkalemia and hypertension of unknown molecular defect who had 2 pregnancies with hypertension exacerbation and premature deliveries was described previously. CONCLUSION: In familial hyperkalemia and hypertension with the WNK4 mutation, pregnancy ameliorates hypertension; however, hyperkalemia and hypercalciuria persist. This dissociation may shed light on the pathogenesis of familial hyperkalemia and hypertension, on pregnancy-related hypertension, and on the mechanism of action of WNK4 kinase, a major regulator of cellular ion transport.
Haim Mayan; Meir Mouallem; Miriam Shaharabany; Rachel Pauzner; Zvi Farfel
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of obstetrics and gynecology     Volume:  192     ISSN:  0002-9378     ISO Abbreviation:  Am. J. Obstet. Gynecol.     Publication Date:  2005 Feb 
Date Detail:
Created Date:  2005-02-07     Completed Date:  2005-03-10     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0370476     Medline TA:  Am J Obstet Gynecol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  598-603     Citation Subset:  AIM; IM    
Department of Medicine E,Sheba Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Hashomer, Israel.
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MeSH Terms
Hyperkalemia / genetics*,  physiopathology
Hypertension / genetics*,  physiopathology
Middle Aged
Pregnancy Complications, Cardiovascular / physiopathology*
Protein-Serine-Threonine Kinases / genetics*
Reg. No./Substance:
EC Kinases; EC protein, human

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