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SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia

Subfertility rate has been observed in women with classical CAH, being inversely correlated with the severity of enzymatic defect. Psychosexual behaviors, genital abnormalities, chronic anovulation and/or excessive androgen exposure could corroborate for this finding. However, there are scarce data...

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Autores principales: Falcao, Camila, Miranda, Mirela, Gomes, Larissa, Costa, Fernanda, Madureira, Guiomar, Inacio, Marlene, Mendonca, Berenice, Bachega, Tania
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6553277/
http://dx.doi.org/10.1210/js.2019-SUN-361
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author Falcao, Camila
Miranda, Mirela
Gomes, Larissa
Costa, Fernanda
Madureira, Guiomar
Inacio, Marlene
Mendonca, Berenice
Bachega, Tania
author_facet Falcao, Camila
Miranda, Mirela
Gomes, Larissa
Costa, Fernanda
Madureira, Guiomar
Inacio, Marlene
Mendonca, Berenice
Bachega, Tania
author_sort Falcao, Camila
collection PubMed
description Subfertility rate has been observed in women with classical CAH, being inversely correlated with the severity of enzymatic defect. Psychosexual behaviors, genital abnormalities, chronic anovulation and/or excessive androgen exposure could corroborate for this finding. However, there are scarce data in the literature evaluating reproductive outcomes in CAH women. Objective: To evaluate the reproductive outcomes in women with classical CAH. Patients: 84 females with 33±11yrs old: 80 with 21-hydroxylase deficiency and 4 with 11β-hydroxylase deficiency (11OHD), were selected. Methods: Data of Prader score, age at first genitoplasty, sexual activity, sexual orientation, desire to conceive, fecundity rate and pregnancy outcomes were analyzed. Chi-square and T tests were used. Results: Prader score and age at feminizing genitoplasty (3.4±3yrs) did not differ between patients with and without desire to conceive. Among the 84 patients, 27% have never tried sexual activity, due to shame of their genitalia. Homosexual and bisexual orientations were identified in 15% and 1.2% of patients, respectively; 38% of all patients were engaged in stable relationships. Desire to conceive was expressed by 26/84 of patients, being lower in salt wasters-SW (28%) than simple virilizers (67%), and the reference population (76%, p<0.05). Fertility was achieved in 21/26 patients; the mean time to conceive was 2.2±4 yrs. Two patients were submitted to in vitro fertilization. The mean age of the first pregnancy was 29.8±6.4yrs and in the reference population was 21yrs. Full-term pregnancy occurred in 20/21 cases, one 11OHD patient had premature labor due to hypertension. Gestational diabetes was observed in only one case. Glucocorticoid doses remained stable during pregnancy, but were increased in 8% of patients in the last trimester; fludrocortisone was administered only to the SW patients. No patient had adrenal crises during gestation and all of them received stress glucocorticoid doses during delivery. All patients, except one, was submitted to cesarean section. The fecundity rate was 1.3 child/patient, lower than the reference population (1.7), p<0.05. Conclusion: We observed that the low fertility rate in CAH females is mainly secondary to lower desire to conceive, psychosexual behaviors and genital abnormalities. Regarding the fertility rate considering only those women who wanted to conceive, most of them succeed spontaneously, with low frequency of comorbidities.
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spelling pubmed-65532772019-06-13 SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia Falcao, Camila Miranda, Mirela Gomes, Larissa Costa, Fernanda Madureira, Guiomar Inacio, Marlene Mendonca, Berenice Bachega, Tania J Endocr Soc Adrenal Subfertility rate has been observed in women with classical CAH, being inversely correlated with the severity of enzymatic defect. Psychosexual behaviors, genital abnormalities, chronic anovulation and/or excessive androgen exposure could corroborate for this finding. However, there are scarce data in the literature evaluating reproductive outcomes in CAH women. Objective: To evaluate the reproductive outcomes in women with classical CAH. Patients: 84 females with 33±11yrs old: 80 with 21-hydroxylase deficiency and 4 with 11β-hydroxylase deficiency (11OHD), were selected. Methods: Data of Prader score, age at first genitoplasty, sexual activity, sexual orientation, desire to conceive, fecundity rate and pregnancy outcomes were analyzed. Chi-square and T tests were used. Results: Prader score and age at feminizing genitoplasty (3.4±3yrs) did not differ between patients with and without desire to conceive. Among the 84 patients, 27% have never tried sexual activity, due to shame of their genitalia. Homosexual and bisexual orientations were identified in 15% and 1.2% of patients, respectively; 38% of all patients were engaged in stable relationships. Desire to conceive was expressed by 26/84 of patients, being lower in salt wasters-SW (28%) than simple virilizers (67%), and the reference population (76%, p<0.05). Fertility was achieved in 21/26 patients; the mean time to conceive was 2.2±4 yrs. Two patients were submitted to in vitro fertilization. The mean age of the first pregnancy was 29.8±6.4yrs and in the reference population was 21yrs. Full-term pregnancy occurred in 20/21 cases, one 11OHD patient had premature labor due to hypertension. Gestational diabetes was observed in only one case. Glucocorticoid doses remained stable during pregnancy, but were increased in 8% of patients in the last trimester; fludrocortisone was administered only to the SW patients. No patient had adrenal crises during gestation and all of them received stress glucocorticoid doses during delivery. All patients, except one, was submitted to cesarean section. The fecundity rate was 1.3 child/patient, lower than the reference population (1.7), p<0.05. Conclusion: We observed that the low fertility rate in CAH females is mainly secondary to lower desire to conceive, psychosexual behaviors and genital abnormalities. Regarding the fertility rate considering only those women who wanted to conceive, most of them succeed spontaneously, with low frequency of comorbidities. Endocrine Society 2019-04-30 /pmc/articles/PMC6553277/ http://dx.doi.org/10.1210/js.2019-SUN-361 Text en Copyright © 2019 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Adrenal
Falcao, Camila
Miranda, Mirela
Gomes, Larissa
Costa, Fernanda
Madureira, Guiomar
Inacio, Marlene
Mendonca, Berenice
Bachega, Tania
SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia
title SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia
title_full SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia
title_fullStr SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia
title_full_unstemmed SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia
title_short SUN-361 Reproductive Outcomes in Females with Virilizing Forms of Congenital Adrenal Hyperplasia
title_sort sun-361 reproductive outcomes in females with virilizing forms of congenital adrenal hyperplasia
topic Adrenal
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6553277/
http://dx.doi.org/10.1210/js.2019-SUN-361
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