Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia
J Clin Res Pediatr Endocrinol
; 13(2): 180-186, 2021 06 02.
Article
en En
| MEDLINE
| ID: mdl-33374095
ABSTRACT
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.Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Virilismo
/
Hiperplasia Suprarrenal Congénita
/
Trastornos del Desarrollo Sexual 46, XX
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
J Clin Res Pediatr Endocrinol
Año:
2021
Tipo del documento:
Article
País de afiliación:
Turquía