STAR and Hyperkalemia: The genetic loss of steroid genesis resulting from mutation in the STAR gene is the primary defect; there is a subsequent loss of steroid genesis that is independent of STAR and which is due to cellular damage from accumulated cholesterol esters in the adrenal cortex, leading to salt wasting, hyponatremia, hypervolemia, hyperkalemia, hyperglycemia, acidosis, and death in infancy, although patients can survive to adulthood with appropriate mineralocorticoid and glucocorticoid-replacement therapy.