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Abstract: TH-PO757

An Intriguing Case of Hypertension and Hypokalemia in a Third Trimester Pregnancy: Early-Onset Autosomal Dominant With Severe Exacerbation in Pregnancy

Session Information

Category: Women's Health and Kidney Diseases

  • 2100 Women's Health and Kidney Diseases

Authors

  • Maturostrakul, Boonyanuth N., Northwell Health, New Hyde Park, New York, United States
  • Nimkar, Abhishek, Northwell Health, New Hyde Park, New York, United States
  • Sachdeva, Mala, Northwell Health, New Hyde Park, New York, United States
  • Jhaveri, Kenar D., Northwell Health, New Hyde Park, New York, United States
Introduction

Geller et al described a familial syndrome of hypertension (HTN) and hypokalemia, exacerbated by pregnancy, caused by an activating mutation of the mineralocorticoid receptor (MR). Here we describe a case of hypokalemia and hypertension with pregnancy that is consistent with the diagnosis of Geller syndrome.

Case Description

A 35 yo woman(G1P0) was admitted at 37 weeks gestation for HTN and hypokalemia. She had no prior history of HTN prior to this and had normal K values during most of her pregnancy. She had a family history of both mother and father with HTN since their 30s. She was noted to have a low serum K of 2.9mmol/L and BP in 145/90mmHG range, highest blood pressure recorded of 156/88 mmHG, and was persistent for 2 weeks prior to admission. She denied nausea, vomiting, or diarrhea. Physical examination showed 1+ bilateral lower extremity edema. There was no proteinuria. Urine K was 19 mmol/L, urine Cr 55 mg/dl, plasma Aldosterone <3.0 ng/dL, and plasma renin activity 1.10 g/ml/hr. The urine K/crt ratio was 36.3 meq/g. Her serum cortisol was not low or too high. She was started on potassium supplements with prompt delivery. She delivered a healthy baby girl and 3 weeks post-delivery; her BP is in 90-100mmHg SBP and 50-60mmHg DBP range. She is off her K supplements as repeat K after 3 weeks post delivery was 5.1mmol/L. Her repeat aldosterone level 4 weeks post delivery was 32.3ng/dL. Genetic testing for Liddle’s syndrome and Geller’s syndrome was performed and pending results.

Discussion

Normally the MR is activated by aldosterone but inhibited by progesterone. The novel MR S810L, described by Geller et al, is activated by both aldosterone and progesterone. In the initial description, two MR L810 carriers had a pregnancy-induced exacerbation of HTN and hypokalemia in 5 pregnancies, with low aldosterone levels. High progesterone in pregnancy was implicated. Our patient experienced new HTN and new hypokalemia in pregnancy, with renal potassium wasting and low renin and aldosterone levels, consistent with autosomal dominant early onset HTN with hypokalemia exacerbated during pregnancy. This resolved few weeks post delivery as noted with labs above. Classically in pregnancy, aldosterone and renin levels are higher than baseline. Genetic testing will confirm the diagnosis.