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Syrian females with congenital adrenal hyperplasia: a case series

BACKGROUND: One of the most common types of congenital adrenal hyperplasia is an autosomal recessive disorder with 21-hydroxylase deficiency. The classical form, defined by cortisol insufficiency, is accompanied by prenatal androgen excess causing variable masculinization degrees of external genital...

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Detalles Bibliográficos
Autores principales: Dehneh, Nada, Jarjour, Rami, Idelbi, Sahar, Alibrahem, Assad, Al Fahoum, Sahar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9569117/
https://www.ncbi.nlm.nih.gov/pubmed/36242011
http://dx.doi.org/10.1186/s13256-022-03609-y
Descripción
Sumario:BACKGROUND: One of the most common types of congenital adrenal hyperplasia is an autosomal recessive disorder with 21-hydroxylase deficiency. The classical form, defined by cortisol insufficiency, is accompanied by prenatal androgen excess causing variable masculinization degrees of external genitalia in babies with a 46, XX karyotype. CASES PRESENTATION: These five case reports highlight the management of Syrian females aged between 0 and 32 years with congenital adrenal hyperplasia. Two of the patients have been raised as males, while two had reconstructive surgery and one had hormonal therapy. Becoming mother was achieved by two patients CONCLUSION: The integrated treatment of females with classical congenital adrenal hyperplasia CAH, which includes appropriate surgical procedures and controlled hormonal therapy, gives these females the opportunity to live as they are, and perhaps as mothers in the future.