Most common Enzyme Deficiency leading to childhood hypeension is
Most common enzyme deficiency causing hypeension in pediatric child group is APPARENTMINERALOCOICOID EXCESS. Apparent mineralocoicoid excess (AME) is an autosomal recessive disorder caused by deficiency of 11 beta hydroxysteroid dehydrogenase 2 (11-beta HSD 2) enzyme which is encoded by a gene in chromosome 16q22. Congenital adrenal hyperplasia due to 11b-hydroxylase deficiency is a form of congenital adrenal hyperplasia (CAH) which produces a higher than normal amount of androgen,resulting from a defect in the gene encoding the enzyme steroid 11b-hydroxylase (11b-OH) which mediates the final step of coisol synthesis in the adrenal. 11b-OH CAH results in hypeension due to excessive mineralocoicoid effects. It also causes excessive androgen production both before and after bih and can virilize a genetically female fetus or a child of either sex. Reference: GHAI Essential pediatrics, 8th edition
17- Alpha hydroxylase
21- Beta hydroxylase
11- Beta hydroxylase
3- Beta hydroxy steroid dehydrogenase
Genetic and genetic disorders