Clinical and Experimental Pediatrics

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Kyu-Hyung Lee (Lee KH)

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Case Report

A Case of Congenital Lipoid Adrenal Hyperplasia

Seo-Jeong Kim, Joo-Sik Choi, Kyu-Hyung Lee, Dong-Kyu Jin

Clin Exp Pediatr. 1996;39(4):567-571. Published online April 15, 1996

Congenital lipoid adrenal hyperplasia is the most severe form of CAH, leading to impaired production of all steroid hormones including glucocorticoids, mineralocorticoid, and sex steroids. The affected individuals are all phenotypically female with a severe salt-losing syndrome that is fatal if steroid replacement is not begun immediately after birth. The lesion of this disorder has been suggested to be in...

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