Volume 16, Issue 2 (8-2002)                   Med J Islam Repub Iran 2002 | Back to browse issues page

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VAKILI R. CONGENITAL ADRENAL HYPERPLASIA IN NORTH•EAST OF IRAN: A REVIEW OF 47 PATIENTS AND THE ROLE OF PARENTAL CONSANGUINITY IN THE OCCURRENCE OF DISEASE. Med J Islam Repub Iran 2002; 16 (2) :75-78
URL: http://mjiri.iums.ac.ir/article-1-755-en.html
CONGENITAL ADRENAL HYPERPLASIA IN NORTH•EAST OF IRAN: A REVIEW OF 47 PATIENTS AND THE ROLE OF PARENTAL CONSANGUINITY IN THE OCCURRENCE OF DISEASE
R VAKILI
From the Pediatric Endocrinology Ward, Imam Reza Hospital, Mashhad University of Medical Sciences,Mashhad, I.R. Iran.
Abstract:   (3979 Views)
In this study the clinical and epidemiological characteristics of congenital adrenal hyperplasia were evaluated prospectively in 47 patients admitted in Imam Reza Hospital in Mashhad during a 4 year period. 21-hydroxylase deficiency was present in 42 patients (89.3%), the simple virilizing form in 6 and the salt-losing form in 36 of them. 11b hydroxylase deficiency was present in 5 patients (10.7%). The median chronological age at diagnosis in the salt-losing form was 68 and 47 days in boys and girls respectively. 7 girls were considered to be male before the diagnosis was established. Parental consanguinity rate among families of patients was higher than the general population in Mashhad (82% vs. 35%). In 16.2% of patients the history of disease was positive in siblings. This study showed that the incidence of congenital adrenal hyperplasia is expected to be high due to a high rate of consanguinity in our population, hence genetic counseling before marriage would definitely be beneficial in our population.
Keywords: Congenital adrenal hyperplasia, Iran, Parental consanguinity.
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Type of Study: Original Research | Subject: Pediatric
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