ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume: 15 Issue: 1 Year: 2023
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Turkish Society for Pediatric Endocrinology and Diabetes
Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia [J Clin Res Pediatr Endocrinol]
J Clin Res Pediatr Endocrinol. 2021; 13(2): 180-186 | DOI: 10.4274/jcrpe.galenos.2020.2020.0216

Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia

Şenay Savaş-Erdeve1, Zehra Aycan2, Semra Çetinkaya1, Ayşe Pınar Öztürk3, Firdevs Baş3, Şükran Poyrazoğlu3, Feyza Darendeliler3, Elif Özsu2, Zeynep Şıklar2, Meliha Demiral4, Edip Unal4, Mehmet Nuri Özbek4, Fatih Gürbüz5, Bilgin Yüksel5, Olcay Evliyaoğlu6, Nesibe Akyürek7, Merih Berberoğlu2
1University of Health Sciences Turkey, Dr. Sami Ulus Obstetrics and Gynecology, Children’s Health and Disease Training and Research Hospital, Clinic of Pediatric Endocrinology, Ankara, Turkey
2Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, Turkey
3İstanbul University Faculty of Medicine, Department of Pediatric Endocrinology, İstanbul, Turkey
4University of Health Sciences Turkey, Diyarbakır Gazi Yaşargil Training and Research Hospital, Clinic of Pediatric Endocrinology, Diyarbakır, Turkey
5Çukurova University Faculty of Medicine, Department of Pediatric Endocrinology, Adana, Turkey
6İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Pediatric Endocrinology, İstanbul, Turkey
7University of Health Sciences Turkey, Konya Training and Research Hospital, Clinic of Pediatric Endocrinology, Konya, Turkey

Objective: To retrospectively evaluate the follow-up data in patients with 46,XX congenital adrenal hyperplasia (CAH) who were raised male.
Methods: A national database was created. The data of patients were asked to be recorded in the data form.
Results: The median (range) age of diagnosis was three (0.1-18.3) years in 44 patients. Twenty nine cases were diagnosed after the age of two years. Most (95.4%) cases were stage 4-5 virilized. Hysterectomy and bilateral salpingoopherectomy, at a median age of 7.25 (2.4-25.3) years, was performed in 35 cases. Testicular prostheses were placed in 11 (25%) cases at a median age of 11.2 (2.8-17) years. The median final height was 149.2 (132.8-172) cms in 38 patients, including simple virilizing (n=18), salt-wasting (n=6), and 11-beta hydroxylase (n=12). Of the 16 patients above the age of eighteen, university education was completed in 25%.
Conclusion: It was seen that most (65.9%) of the 46,XX CAH cases raised male were diagnosed after two years of age. In these cases, hysterectomy and bilateral salpingoopherectomy, genital corrective surgeries and testicular prosthesis operations were performed in a very wide age rage.

Keywords: 46,XX, congenital adrenal hyperplasia, final height

Şenay Savaş-Erdeve, Zehra Aycan, Semra Çetinkaya, Ayşe Pınar Öztürk, Firdevs Baş, Şükran Poyrazoğlu, Feyza Darendeliler, Elif Özsu, Zeynep Şıklar, Meliha Demiral, Edip Unal, Mehmet Nuri Özbek, Fatih Gürbüz, Bilgin Yüksel, Olcay Evliyaoğlu, Nesibe Akyürek, Merih Berberoğlu. Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia. J Clin Res Pediatr Endocrinol. 2021; 13(2): 180-186
Manuscript Language: English
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