Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 1 de 1
Filtrar
Mais filtros

Base de dados
Tipo de documento
País/Região como assunto
Ano de publicação
Intervalo de ano de publicação
1.
J Endocrinol Invest ; 44(1): 83-93, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32358738

RESUMO

OBJECTIVES: The prevalence of CAH in Egypt is reported to be ten times more than that of the worldwide prevalence. The study aimed at genetic screening of children diagnosed with 21-alpha hydroxylase deficiency congenital adrenal hyperplasia (21OHD-CAH). In addition, the study offers a rapid and easy guide for clinical reporting of common mutations for endocrinologists. METHODS: A cohort of 174 unrelated Egyptian children with 21OHD-CAH were screened for 11 common CYP21A2 gene mutations using a strip hybridization assay, and then, bioinformatics analysis was done to report the pathogenicity of the common mutations for clinical classification. RESULTS: The most common mutations were I2 splice and p.Q318X. Deletions/conversions comprised 45.9% of the cohort, whereas 7.4% of the cases were negative for all mutations. The least positively detected point mutations were p.P453S, cluster E6, p.R483P, and p.L307FS, which were detected in fewer than 5% of cases. CONCLUSION: Strip hybridization assay is a rapid screening tool for the diagnosis of CAH. The authors hypothesized an easy and rapid scheme for clinical interpretation of the strip results to gain the highest value of the strip in diagnosis.


Assuntos
Hiperplasia Suprarrenal Congênita/genética , Biomarcadores/análise , Testes Genéticos/métodos , Mutação , Fenótipo , Esteroide 21-Hidroxilase/genética , Hiperplasia Suprarrenal Congênita/epidemiologia , Hiperplasia Suprarrenal Congênita/patologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos de Coortes , Egito/epidemiologia , Feminino , Seguimentos , Genótipo , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA