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Background: Congenital adrenal hyperplasia due to 11-beta hydroxylase is a rare genetic disorder. It is transmitted as an autosomal recessive trait. We describe a novel genetic mutations, in a 19 year old untreated male with 11-Beta hydroxylase deficiency, severe CAH with late presentation and TART. Methods: A 19 years old Chinese male presented with testicular masses, hypertension and hypokalemia. Hormonal studies showed elevated 17-hydroxyprogesterone, androstenedione, DOC, ACTH and markedly elevated 11-deoxycortisol levels, low cortisol, aldosterone and plasma renin activity levels. Ultrasonographic imaging revealed markedly abnormal appearing testes, with diffuse hypervascularity and numerous confluent infiltrative nodules, suggestive of TART. Genetic analysis revealed novel R43Q in Exon1, A386V in Exon7 and G452V in Exon8 mutations on chromosome 8. Results: Normalization of ACTH, reduction in 11-deoxycortisol, DOC and additional metabolites were achieved with steroid treatment.Conclusion: This is a 19 yo male from China with CAH secondary to 11-beta hydroxylase who was found to have novel mutations