Biliary atresia with hyperandrogenic amenorrhea: case report

The etiology of hyperandrogenic amenorrhea in a 16 year-old girl after a su ccessful Kasai operation for biliary atresia was unclear. Delayed puberty a nd menarche were observed. There was no luteinizing hormone-follicular stim ulating hormone surge. A provocative luteinizing hormone-releasing hormone test showed a normal response. Peripheral aromatization of androgens appear ed to function normally. However, plasma levels of sex hormone-binding glob ulin and total testosterone were high and the free testosterone level was n ormal. The anovulatory menstrual cycle continued after menarche at 18 years of age. A combination of estrogen and progesterone therapy was effective. The etiology might be associated with delayed metabolic clearance of testos terone accompanied by the compensatory cirrhosis and portosystemic shunt.