Abstract: PO1467
A Case of the Cons: How Contraception Confused Congenital Adrenal Hyperplasia for Conn
Session Information
- Fluid, Electrolyte, and Acid-Base Disorders: Clinical - 1
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 902 Fluid, Electrolyte, and Acid-Base Disorders: Clinical
Authors
- Lavenburg, Linda-Marie Ustaris, University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Shah, Sanjeev R., University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Aggarwal, Sandeep, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Introduction
Drospirenone is a synthetic progestin oral contraception (OCP) with anti-androgen and anti-mineralocorticoid properties. We present a case of hypokalemic alkalosis and hypertension masked by drospirenone use.
Case Description
A 21-year old female with presumed polycystic ovarian syndrome (PCOS) was referred for hematuria, facial rash, and positive ANA concerning for lupus nephritis. Her only medication was drospirenone-ethinyl estradiol for oligomenorrhea, acne and hirsutism. Blood pressure (BP) was 98/67mmhg and heart rate 88 bpm with orthostasis. She had male pattern hair loss and dense comedones on her cheeks.
Labs were significant for normal renal function, 2+ hematuria with isomorphic RBCs, negative BHCG and ANA titer 1:64. Glomerulonephritis and rheumatology work-ups were negative. Hematuria was attributed to breakthrough uterine bleeding. Gynecology held her OCP to allow for withdrawal bleeding.
At 6-week follow-up, she reported muscle cramps and increased facial hair. BP was 176/98 mmhg. Hypertension was confirmed with ambulatory BP monitoring. Labs were significant for potassium 2.8 meq/L, bicarbonate 37 meq/L, urine sodium 14 meq/L, potassium 54 meq/L, chloride 38 meq/L. Mineralocorticoid excess due to primary hyperaldosteronism (Conn's syndrome) was suspected but plasma renin activity was <1 and aldosterone was <1. Syndrome of apparent mineralocorticoid excess was considered but the presence of hirsutism prompted investigation of congenital adrenal hyperplasia (CAH). Work-up found elevated levels of ACTH (146 pg/ml), DHEA-S (694 mcg/dl), 11-deoxycortisone (452 ng/dl), and testosterone (92 ng/dl), but normal 17-OH progesterone, LH and FSH.
Ultrasound showed bilateral adrenal enlargement, normal right and left ovaries with two dominant follicles. She declined genetic testing for 11-beta hydroxylase deficiency (11BHD).
Clinical and lab findings were consistent with non-classical CAH due to 11BHD. She started hydrocortisone with suppression of ACTH, decrease in 11-deoxycortisone, and normalization of BP and electrolyte abnormalities.
Discussion
11BHD is rare form of CAH, which can be confused with PCOS and other syndromes of mineralocorticoid excess. In this case, drospirenone use controlled symptoms but potentially delayed diagnosis of 11BHD. Clinical trials should assess drospirenone in treatment of endocrinopathies.