Biliary atresia with hyperandrogenic amenorrhea

The etiology of hyperandrogenic amenorrhea in a patient with biliary atresi a successfully treated by a Kasai operation was unclear. Delayed puberty an d menarche were evident at 16 years of age. Investigations showed no lutein izing hormone (LH)-follicle-stimulating hormone surge. A LH-releasing hormo ne provocative test showed a normal response. Peripheral aromatization of a ndrogens appeared to function normally. Free testosterone (T) was normal, h owever, plasma levels of sex-hormone-binding globulin and total T were high . After menarche at 18 years of age, anovulatory menstrual cycles continued . A combination of estrogen and progesterone therapy was effective. A possi ble explanation may be that metabolic clearance of T is reduced in the pres ence of liver cirrhosis and a portosystemic shunt.