Fluid hydration in the form of IV fluid boluses should be given, with up to 2-3 L often required in the initial resuscitation stages to treat any concurrent dehydration. Afterwards, IV hydration with D5 NS can be beneficial, as it will help correct the hypoglycemia and hyponatremia often seen in these patients. Vasopressors can be started as needed, although these patients’ hypotension can be refractory to both fluids and vasopressors. Steroids are ultimately needed for vascular tone. The precipitating cause should be treated as indicated (1,2).
Much of our modern understanding and treatment of CAH comes from research conducted at Johns Hopkins Medical School in Baltimore in the middle of the 20th century. Lawson Wilkins , "founder" of pediatric endocrinology , worked out the apparently paradoxical pathophysiology: that hyperplasia and overproduction of adrenal androgens resulted from impaired capacity for making cortisol. He reported use of adrenal cortical extracts to treat children with CAH in 1950. Genital reconstructive surgery was also pioneered at Hopkins. After application of karyotyping to CAH and other intersex disorders in the 1950s, John Money , JL Hampson, and JG Hampson persuaded both the scientific community and the public that sex assignment should not be based on any single biological criterion, and gender identity was largely learned and has no simple relationship with chromosomes or hormones. See Intersex for a fuller history, including recent controversies over reconstructive surgery.