Abstract
Pubertal progression in the presence of abnormally elevated serum gonadotropins was followed over several years in two girls with multiple endocrine deficiencies (hypoadrenalism and hypoparathyroidisirm and mucocutaneous candidiasis. Accelerated height velocity and development of breasts and pubic hair were noted in both girls. A serum level of progesterone (8 ng/ml) consistent with ovulation was documented in one of the girls who has had regular monthly menses since age 15. High serum levels of FSH(30-60 mIU/ml) coincident with serum estradiol levels in the normal pubertal range(100-200 pg/ml) indicated partial ovarian end-organ resistance or failure. Basal levels of serum FSH were higher than LH, but the response of LH to intravenous leuteotropin releasing factor was relatively greater. Elevation of gonadotropins was progressive in both patients. Since premature menopause is associated with this syndrome, we postulate that we are observing progression through puberty in girls whose ovaries are in the early stages of a destructive process which may eventually result in irreversible ovarian failure. A possible explanation is increased resistance to gonadotropins at the ovarian level because of gonadotropin receptor antibodies or an inherent receptor defect.
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Lucky, A., Rebar, R., Blizzard, R. et al. PUBERTAL PROGRESSION WITH ELEVATED GONADOTROPINS IN GIRLS WITH MULTIPLE ENDOCRINE DEFICIENCIES. Pediatr Res 11, 428 (1977). https://doi.org/10.1203/00006450-197704000-00353
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DOI: https://doi.org/10.1203/00006450-197704000-00353