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Abstract: SA-PO378

Hypokalemic Periodic Paralysis and Hypertension in Pregnancy: A Diagnostic Challenge

Session Information

Category: Trainee Case Report

  • 2000 Women's Health and Kidney Diseases

Authors

  • Yalamarti, Tanuja, Pennsylvania hospital of UPENN, Philadelphia, Alabama, United States
  • Ntoso, K. Adu, Pennsylvania Nephrology Associates, P.C., Philadelphia, Pennsylvania, United States
  • Patrascu, Carmen, Pennsylvania Nephrology Associates, P.C., Philadelphia, Pennsylvania, United States
  • Zonoozi, Shahrzad, Pennsylvania Hospital, Philadelphia, Pennsylvania, United States
  • Barry, Timothy, UPHS, Philadelphia, Pennsylvania, United States
Introduction

Geller syndrome is a rare autosomal dominant syndrome that causes new or worsening hypertension (HTN) during pregnancy associated with hypokalemia and metabolic alkalosis.

Case Description

We report the case of a 37-year-old Burmese female who presented at 24 weeks’ gestation with a 3-day history of progressive lower extremity weakness leading to inability to walk. Her blood pressure (BP) was elevated at 180/90. Initial labs revealed serum potassium (K) 1.6 mMol/L with ECG changes of prolonged QT interval and U waves. Initial spot urine potassium was 5.7 mMol/L and sodium 54 mMol/L. A 24-hour urine potassium was 19.8 mMol/L. Following repletion of K both orally and intravenously, paralysis resolved. Initial diagnosis was hypokalemic periodic paralysis. She was started on nifedipine for HTN. Thyroid function was intact. Cortisol 3.4 mcg/dl (3.5-19.5 mcg/dl), renin <2.5 pg/ml (2.5-45.7 pg/ml), aldosterone <3.0ng/dl (4.0-31 ng/dl), and aldosterone/renin activity (A/RA) <3.8. Catecholamines were all normal. Moderate to severe hypokalemia recurred several times during the pregnancy, and urine K was not suppressed. BP remained high and she required addition of labetalol. She underwent cesarean delivery at 33 weeks’ pregnancy due to severe pre-eclampsia. Amiloride was started postpartum. Hypokalemia and hypertension improved.

Discussion

Low renin and high aldosterone related HTN is mainly due to excess mineralocorticoid activity. Our patient experienced worsening HTN and severe hypokalemia during pregnancy, with low renin and low aldosterone levels. The improvement of HTN and hypokalemia postpartum suggests pregnancy-specific factors. Normally, activation of mineralocorticoid receptor (MR) causes renal salt reabsorption through the epithelial sodium channel (ENaC) activity. Progesterone binds to but does not normally activate the MR. Geller syndrome is caused by an activating mutation in the gene encoding the MR S810L. This allows progesterone (as well as spironolactone) to function as agonist, causing increases in BP, and hypokalemia during pregnancy. Liddle’s syndrome was also considered as she responded well to amiloride. However, the age of the patient and the lack of family history argue against it. Geller syndrome is rare but should be considered in women with HTN and hypokalemia in pregnancy. Genetic testing is pending.