The deficiency results in impaired synthesis of all three categories of adrenal steroids (cortisol, mineralocorticoids, sex steroids) and high levels of adrenocorticotropic hormone (ACTH). A low level of steroid synthesis proceeds even without efficient transport, but is rarely enough to prevent the consequences of deficiency. While severe loss of steroid production results in manifestation of the disease within a few weeks of birth, milder forms (late onset) can present years after birth. Unlike in models of the disease in mice, patients with lipoid CAH do not always have enlarged adrenals due to lipid accumulation. This may in part be due to hormone replacement used to keep them alive preventing hyperstimulation of the gland by the pituitary.