| disease | Premature Puberty with Genital Enlargement in Males |
Adrenal cortical reticular zone lesions, characterized by excessive secretion of sex hormones leading to specific manifestations, are termed adrenal sexual differentiation disorders. This is the most common cause of female pseudohermaphroditism and male precocious puberty. Lesions originating in the fetal period, with symptoms appearing in infancy or early childhood, are referred to as congenital adrenal cortical hyperplasia. This condition arises due to deficiencies in certain enzymes that affect cortisol biosynthesis, leading to increased ACTH secretion and continuous stimulation of the cortex. C21 hydroxylase deficiency is the most common cause. If the disease onset occurs after birth, adrenal cortical tumors are more frequently observed in childhood or later stages, while hyperplasia is less common. Clinical manifestations vary depending on the patient's gender, disease cause, and time of onset.
bubble_chart Clinical Manifestations
1. Early development of the penis, often resembling that of an adult, capable of erection and with pubic hair.
2. Accelerated development of muscles and bones, appearance of beard and armpit hair, body shape similar to that of an adult.
3. Enlargement of the Adam's apple, deep and rough voice, and acne on the skin.
4. The prostate may develop early, but the testes do not enlarge. {|103|}
1. The penis develops particularly early, often resembling that of an adult, capable of erection, accompanied by the appearance of pubic hair.
2. Muscles and bones develop rapidly, with the emergence of facial hair and underarm hair, and the body shape becomes similar to that of an adult.
4. The prostate may develop early, but the testes do not enlarge; instead, their development is inhibited. Although semen is present, it contains no sperm.
5. Ultrasound and CT scans can detect the presence of tumors in both adrenal glands.
bubble_chart Treatment Measures
Treatment 1. If it is an adrenal tumor, surgical removal should be performed.
2. If it is adrenal cortical hyperplasia, cortisol suppression therapy can be administered, with the dose being the same as that used for treating female pseudohermaphroditism.
