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Title: | Congenital adrenal hyperplasia in adolescence - a gynecological perspective | Authors: | Oliveira, Joana Correia Sousa, Filipa Costa Campos, Sara Teixeira Geraldes, Fernanda Bento Belo, Joana Lopes Leite, Maria Helena Mirante, Maria Alice Águas, Maria Fernanda |
Keywords: | congenital adrenal hyperplasia; hyperandrogenism; steroid 21-hydroxylase; menstruation disorders; amenorrhea | Issue Date: | 22-Mar-2022 | Publisher: | Via Medica | Serial title, monograph or event: | Ginekologia Polska | Volume: | 94 | Issue: | 3 | Abstract: | Objectives: Analysis of congenital adrenal hyperplasia (CAH) cases, gynaecological implications, referral reasons to gynaecologist and treatment. Material and methods: Retrospective, longitudinal, single-centre study with female CAH paediatric patients ≥ 10 years-old, followed between 1998–2018 in gynaecology and endocrinology departments at a public university tertiary hospital. Results: 47 patients, 34.0% (n = 16) with classic, 66.0% (n = 31) with non-classic forms (NCAH), CYP21 deficit and 46,XX karyotype. We found a normal median menarche age [11.5 IQR 2 (6–15) years-old], but significantly earlier in NCAH (p = 0.003). Precocious puberty occurred in 48.9%, n = 23. Primary amenorrhea occurred in salt-wasting form (21.4%, n = 3). Oligomenorrhea and hirsutism were significantly more prevalent in NCAH (p = 0.018, p = 0.014 respectively) and acanthosis nigricans and virilization signs in classic forms (p = 0.05, p = 0.000 respectively). Sixteen patients (34.0%) were referred to gynaecology, mostly due to menstrual irregularities (50.0%, n = 8). Medical treatment with isolated or combined corticoids, oestrogen and progestogen were chosen in all but one case. Gonadotropin-releasing hormone analogues were used in 19.0% (n = 9). Surgery was performed in 34.0% (n = 16) patients, median age 2.0 IQR 2.5 (0.6–90) years-old. Conclusions: This paper highlights the importance of a multidisciplinary approach. Early treatment contributes to a phenotypical feminine differentiation and normalization of the hypothalamus-pituitary-ovarian axis, which is essential given the gynaecologic and obstetric consequences of untreated cases. | URI: | https://hdl.handle.net/10316/114620 | ISSN: | 2543-6767 0017-0011 |
DOI: | 10.5603/GP.a2021.0248 | Rights: | openAccess |
Appears in Collections: | FMUC Medicina - Artigos em Revistas Internacionais |
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