The fetal adrenal cortex lacks expression of the enzyme early on, thus mineralocorticoids (. aldosterone ) and glucocorticoids (. cortisol ) cannot be synthesized. This is significant because cortisol induces type II pneumocytes of the lungs to synthesize and secrete pulmonary surfactant ; without pulmonary surfactant to reduce the alveolar surface tension , premature neonates may die of neonatal respiratory distress syndrome . If delivery is unavoidable (. because of placental abruption , or pre-eclampsia / HELLP syndrome ), then glucocorticoids (. cortisol) can be administered.
Al-Jurayyan (2011) conducted a retrospective study in the pediatric endocrine clinic at a university hospital Saudi Arabia during the period 1989-2008. Medical records of 81 children with ambiguous genitalia were reviewed. Fifty three (65%) of the patients were genetically females (46XX). Male genetic sex (46XY) was present in only 28 (35%) patients with a diversity of causes; multiple congenital anomalies in nine (32%), local anorectal anomalies in two (7%), congenital adrenal hyperplasia (3-beta-hydroxysteroid dehydrogenase deficiency) in two (7%), 5-alpha-reductase deficiency in four (14%), partial androgen insensitivity in three (11%), complete androgen insensitivity in four (14%), and hypogonadotrophin deficiency in four (14%). Twenty-five (47%) of females were wrongly assigned as males, where only two (7%) males were wrongly assigned as females.