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Saudi Medical Journal. 2015; 36 (1): 113-116
de Anglais | IMEMR | ID: emr-159969

RÉSUMÉ

The aim of this study is to determine congenital adrenal hyperplasia [CAH] with the pattern of CYP21A2 gene-mutations in Saudi children. Between January 2011 and March 2014 at King Fahad Military Complex, Dhahran, Saudi Arabia, we thoroughly examined 11 patients with CAH and 2 asymptomatic individuals with a history of affected siblings. Additionally, we sequenced the full coding regions of the CYP21A2 gene and screened the gene for deletion[s]/duplication[s] using the multiplex ligation-dependent probe amplification [MLPA] technique. Nine patients had classic CAH and presented with ambiguous genitalia and/or salt losing crisis. Two patients had the non-classic form of CAH and presented with precocious puberty. The remaining 2 subjects were asymptomatic. Screening the CYP21A2 gene, we detected p.Gln318X mutation in 4 patients, c.290 -13 C>G [IVS2-13C>G] in another 4, and a common deletion, involving exons 6 and 8 in 3 patients. Our strategy of Sanger sequencing followed by MLPA was very successful in detecting CYP21A2 mutations in all patients with CAH


Sujet(s)
Humains , Biologie moléculaire , Steroid 21-hydroxylase , Mutation
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