Pediatric emergency medicine trisk 1885 1885
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is present shortly after birth, but acute crisis usually does not occur until the second week of
life.
TABLE 89.6
CLINICAL AND LABORATORY FEATURES OF VARIOUS FORMS OF
CONGENITAL ADRENAL HYPERPLASIA
FIGURE 89.1 Adrenal steroid hormone biosynthesis. The pathways for the synthesis of adrenal steroid
hormones (adrenal cortex) and catecholamines (adrenal medulla) are arranged from left to right. Synthesis of all
compounds originates from cholesterol in the mitochondria of the adrenal cortex. Subsequent conversions are
shown with enzyme names located next to open arrows , and gray lines indicating enzymatic blocks in the
various forms of congenital adrenal hyperplasia (CAH). Mineralocorticoids (aldosterone) are produced in the
zona glomerulosa, glucocorticoids (cortisol) in the zona fasciculata, and androgens (testosterone) and estrogens
(estradiol) in the zona reticularis. Cortically produced cortisol is required for full induction of the medullary
conversion of norepinephrine to epinephrine. (Courtesy of Joseph Majzoub, MD, Children’s Hospital Boston.)
Triage
