Professor Icahn School of Medicine at Mount Sinai New York, New York, United States
Disclosure(s):
Rebecca McGinnis, MD: No financial relationships to disclose
Andrea Dunaif, MD: Dexcom: Consultant (); Eli Lilly & Company: Speaker (); Neurocrine Biosciences: Consultant (); Pfizer Inc: Consultant ()
Background: We describe three premenopausal women with portal hypertension and elevated testosterone levels near or above those seen in androgen-secreting neoplasms (>150 ng/dL by LC/MS). We hypothesize that portosystemic shunting has the potential to cause hyperandrogenemia in women. Case 1: A 22-year-old woman with cirrhosis due to autoimmune hepatitis presented with secondary amenorrhea. Menarche occurred at age 12 with regular menses. She developed oligomenorrhea at age 14, coincident with her liver disease diagnosis. Her menses normalized with immunosuppressive therapy at age 15. At age 20, her menses ceased. Total testosterone (T) 386 ng/dL (10-55) LC/MS, SHBG 144.0 nmol/L (24.6-122.0), and androstenedione 469 ng/dL (28-230) levels were elevated. DHEAS 129 µg/dL (110-432), LH 5.3 mIU/mL, and FSH 6.4 mIU/mL levels were normal. There were no adrenal or ovarian lesions on pelvic imaging with MRI and PET-CT showed no adrenal or ovarian lesions. There was a modest decrease in T levels to 256 ng/dL with an oral contraceptive containing norethindrone-ethinyl estradiol 1 mg-10 mcg. Case 2: A 35-year-old woman with a history of acute lymphoblastic leukemia prior to puberty complicated by chemotherapy-related non-cirrhotic portal hypertension presented with oligomenorrhea. Menarche occurred at age 16 with persistent oligomenorrhea. T 265 ng/dL (10-55) LC/MS, SHBG 178.0 nmol/L (24.6-122.0), and androstenedione 247 ng/dL (28-230) levels were elevated. DHEAS 27 µg/dL (57-279) levels were low, attributed to intralesional glucocorticoid injections for acne. LH 14.2 mIU/mL and FSH 7.2 mIU/mL levels were normal. Case 3: A reproductive-age woman with a hypercoagulable disorder and thrombus-related noncirrhotic portal hypertension, previously with regular menses, presented with new-onset oligomenorrhea following a mesocaval shunt placement. T 115 ng/dL (10-55) LC/MS and androstenedione 330 ng/dL (28-230) levels were elevated. SHBG 65.5 nmol/L (24.6 - 122.0), DHEAS 135 ug/dL (84-378), LH 18.2 mIU/mL, and FSH 6.6 mIU/mL levels were normal. She was lost to follow-up but conceived without therapy a few years following her initial evaluation.
Conclusions: We report a previously unrecognized association between portal hypertension and marked hyperandrogenemia in reproductive-age women. Hyperandrogenemia has been reported in children with congenital vascular shunts, implicating bypassed hepatic steroid metabolism. The increase in both T and androstenedione levels is consistent with increased glandular secretion. Since the unifying feature of our cases is portal hypertension, in the absence of hepatic dysfunction in cases 2 and 3, we suggest that portosystemic shunting, with reduced hepatic clearance of androgens, may contribute to the elevated androgen levels in these cases.
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