[Primary amenorrhea and hirsutism associated with hyperinsulinemia type A]

Hyperinsulinemia type A, which is a very rare disease, is often manifested in menstrual disorders and/or anovulation. Recently, a 19-year-old woman diagnosed with hyperinsulinemia and acanthosis nigricans, visited our outpatient clinic complaining primarily of amenorrhea and hirsutism. Endocrinological studies revealed normal LH and FSH levels, low estradiol (42.3pg/ml) and high androgens (testosterone: Over 150ng/ml, delta androstenedione: 4.8ng/ml) in serum. Her fasting insulin level was excessively high (over 320 microU/ml). UST showed bilateral polycystic ovaries. These data support the hypothesis of a pathogenic mechanism of ovarian dysfunction in patients with hyperinsulinemia; that is, too much insulin stimulates ovaries directly, followed by the production of androgens and the formation of polycystic ovaries. Strict dietary management of this patient failed to cause spontaneous vaginal bleeding. Progesterone administration did not cause withdrawal vaginal bleeding. These findings suggest that the higher that insulin level is, the more difficult it is to induce ovulation.