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AAP Grand Rounds 2:45-46 (1999) Genotyping as Part of Congenital Adrenal Hyperplasia ScreeningSource: Nordenström A, Thilén A, Hagenfeldt L, Larsson A, Wedell A. Genotyping is a valuable diagnostic complement to neonatal screening for congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency. J Clin Endocrinol Metab. 1999;84(5):15051509.
Approximately 20 states and numerous countries perform neonatal screening for congenital adrenal hyperplasia (CAH), a disease that has an incidence of approximately 1 in 10,000. More than 95% of cases of CAH are due to 21-hydroxylase deficiency, which results in elevated levels of 17-hydroxyprogesterone (17-OHP) which is used as a marker for this condition. Most screening programs depend on analysis of filter paper blood 17-OHP concentrations obtained from heelstick sampling. Recently, genotype screening for CAH has been developed with approximately 10 gene deletions or sequence aberrations accounting for most cases. Between 1986 and 1997, 114 children were diagnosed with CAH in Sweden
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