Endocrine Abstracts

JOINT2358

Introduction: The liver plays a vital role in the endocrine system. In patients with portosystemic shunting, such as congenital portosystemic shunts or acquired portosystemic shunting because of collateral formation after portal vein thrombosis, several endocrine disturbances can occur. We present a case of a girl with primary amenorrhea after portal vein thrombosis.

Case report: A female,16 year of age, presented with primary amenorrhea and mild hirsutism. Height was 157 cm (-2,0 SD) and BMI 22,8 kg/m² (+0,7 SD). Her biochemistry showed hyperandrogenism with an androstenedione level of 19 nmol/l(1.35 – 7.82 nmol/l)and a testosterone level of 6.5 nmol/l(0.100 – 1.70 nmol/L), along with an LH to FSH ratio of 2.2 (LH 14,4 U/land FSH 6,6 U/l; PCOS-like fenotype). Her medical history included hematemesis at the age of 9 years due to esophageal varices, revealing an idiopathic portal vein thrombosis with porto-systemic shunting. She was also known to have mild primary hypothyroidism. In addition to hyperandrogenism, her biochemistry showed increased ammonia (176 µmol/l(15 - 45 µmol/L)), thrombocytopenia and mildly elevated bile salts. A retrograde portogram showed an intact portal bifurcation, after which a MesoRex bypass was performed successfully. This surgical procedure restored the portal flow from the mesentery to the liver, reconstituting the first pass mechanism. During follow-up, androstenedione and testosterone levels normalized (1.1 nmol/land 5.1 nmol/l, respectively), as did the LH to FSH ratio (LH 4,6 U/land FSH 6,2 U/l). Three months postoperatively, menarche occurred and her menses have remained regular since.

Conclusion: Loss of the liver’s first pass metabolism due to a diverted portal flow can lead to several endocrine abnormalities, as illustrated in this case. Clinicians should be aware that porto-systemic shunting can mimic polycystic ovary syndrome (PCOS). More information on congenital portosystemic shunts can be found on the website from the International Registry of Congenital Porto-Systemic Shunts: https://ircpss.com.