Biliary atresia with hyperandrogenic amenorrhea.
The etiology of hyperandrogenic amenorrhea in a patient with biliary atresia successfully treated by a Kasai operation was unclear. Delayed puberty and menarche were evident at 16 years of age. Investigations showed no luteinizing hormone (LH)-follicle-stimulating hormone surge. A LH-releasing hormone provocative test showed a normal response. Peripheral aromatization of androgens appeared to function normally. Free testosterone (T) was normal, however, 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 possible explanation may be that metabolic clearance of T is reduced in the presence of liver cirrhosis and a portosystemic shunt.
Hebiguchi
T
T
Department of Pediatric Surgery, Akita University School of Medicine, 1-1-1 Hondo, Akita 010, Japan.
Kato
T
T
Yoshino
H
H
Mizuno
M
M
Takahashi
T
T
Fukuda
J
J
Tanaka
T
T
D000568:Amenorrhea / Q000209:etiology*; D000858:Anovulation / Q000209:etiology; D001656:Biliary Atresia / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017588:Hyperandrogenism / Q000209:etiology*; D007223:Infant; D008103:Liver Cirrhosis / Q000209:etiology; D008657:Metabolic Clearance Rate / Q000502:physiology; D011183:Postoperative Complications / Q000209:etiology*; D012738:Sex Hormone-Binding Globulin / Q000378:metabolism; D013739:Testosterone / Q000097:blood
D012738:Sex Hormone-Binding Globulin; D013739:Testosterone