x-linked adrenal hypoplasia congenita x-linked adrenal hypoplasia congenita x-linked adrenal hypoplasia congenita is a genetic disorder that mainly affects males. it involves many endocrine tissues in the body, especially the adrenal glands. x-linked adrenal hypoplasia congenitaspecialtyendocrinology presentation one of the main characteristics of this disorder is adrenal insufficiency, which is a reduction in adrenal gland function resulting from incomplete development of the gland's outer layer (the adrenal cortex). adrenal insufficiency typically begins in infancy or in childhood and can cause vomiting, difficulty with feeding, dehydration, extremely low blood sugar (hypoglycemia), low sodium levels, and shock. however, adult-onset cases have also been described.