Progeria

PROGERIA, nanisme senile, presenile involution, senilism. A rare disease described for the first time by Gilford consists of premature aging of the organism connected with its underdevelopment. The main signs are a sharp lag in growth from the earliest age, general cachexia, wrinkled skin, an old man's appearance, absence of hair, underdevelopment of the sexual organs and secondary sexual signs. The proportions of the body remain childish. Epiphyseal seams close early. The psyche does not suffer and corresponds to the age. At autopsy, general arteriosclerosis, atheromatosis of the aorta, sclerosis of various organs, including the pituitary gland, thyroid gland, adrenal glands and sexual glands, were found. The cause of the disease is unknown. Gilford considers that in the pathogenesis of the ailment the main role belongs to damage to the pituitary gland, while Varney Pironneau give the main place to the adrenal glands. Falta refers these cases to his group of "multiple sclerosis of blood glands", considering this the consequence of general sclerosis of the whole organism. Therapy is symptomatic.