Abstract
The causes of primary ovarian failure except for Turner's syndrome are extremely rare. We have experienced a 16 5/12-year-old girl who presented with short stature (137.5 cm, -3 S.D. ) and lack of secondary sexual development. Physical examination revealed immature face, typical skin changes of fading stage of incontinentia pigmenti, hemihypertrophy of extremities, ptosis of left eye lid, low set and asymmetrical ears, low posterior hair lines and atrophic nails. Bone age was 10 6/12 years. Lab data showed low estradiol (E2, 10.0pg/ml ) with high FSH (139 mIU/ml) and LH (105.8 mIU/ml). FSH and LH responded excessively to LH-RH and primary ovarian failure was suspected. Chromosome analysis revealed normal 46,X X karyotype by peripheral lymphocytes and skin fibroblasts. Laparoscopic examination revealed bilateral streak ovaries and a hypoplastic uterus. IVP demonstrated no urinary abnormalities. Bilateral perceptive deafness were found and her IQ was 104. She was placed on estrogen therapy.
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Takahashi, H. 173 A CASE OF PRIMARY OVARIAN FAILURE ASSOCIATED WITH INCONTINENTIA PIGMENTI AND MULTIPLE MINOR ANOMALIES. Pediatr Res 19, 632 (1985). https://doi.org/10.1203/00006450-198506000-00193
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DOI: https://doi.org/10.1203/00006450-198506000-00193