Abstract
Ovarian atrophy (OA), manifest as primary (PA) or secondary amenorrhea (SA) is common in APECED, being present in 64% of our postpubertal female patients. We determined in 26 female patients SCA, defined as reactive with ovarian, testicular and/or placental steroidogenic cells. SCA were found in 19 cases. 7 had PA, 6 had SA, 1 died accidentally before puberty, 1 remained prepubertal at 14.5 yrs and 4 menstruated normally. 1 of these 4 was infertile with irregular periods. The other 3 had normal periods; 2 have been pregnant (1 of them delivered a normal baby, the other chose abortion for social indications). All patients with SCA save 1 had primary adrenocortical failure (A). No patient with OA was negative for SCA (1-9 sera were studied per patient). It is unclear whether female APECED patients with SCA are bound to develop OA. Contrary to Elder et al (JCEM 1981:52:1137) it is also unclear whether patients with SCA are bound to have A.
Female APECED patients with SCA should be followed for development of OA and all patients with SCA for A. We have documented slow development of ovarian failure with serial GnRH test in 3 girls.
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Ahone, N., Miettine, N. & Perheentupa, J. Significance of steroid cell autoantibodies (SCA) in patients with APECED (autoimmune polyendocrinopathy -candidosis – ectodermal dystrophy). Pediatr Res 18, 1214 (1984). https://doi.org/10.1203/00006450-198411000-00082
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DOI: https://doi.org/10.1203/00006450-198411000-00082