Clinical cases of child progeria

Abstract

Child progeria is an extremely rare disease. Not every dermatologist is lucky enough to work with such a diagnosis during medical practice.The first case of child progeria was described in 1886 by the English doctor J. Hutchinson at a 6 years old child. 11 years later, H. Gilford entered the term «progeria» in medical terminology as a syndrome of premature aging of the human body. The etiology of this illness is not clear. The hereditary nature of the disease has not been traced, but there is an opinion about the autosomal-recessive type of inheritance. The average life expectancy of the patients with progeria is 15—16 years.Progeria is characterized by growth retardation, signs of rapid aging. At birth or in the first years of life, geroderma develops, that is, drawn, wrinkled, dry, parchment­like skin, yellow with a brown color tinge, through which the superficial venous net is often visible. The child looks like a deep elder — a small face, a beak­shaped nose. The skin hangs, the muscles are atrophied, the hair is fine, greasy, falls out. For a long time milk teeth are stored, nail plates are thin, atrophic. The genital organs are underdeveloped. On a roentgenogram of tubular bones there is osteoporosis. The intellect is developed according to the age.For more than half a century of the pediatric practice we diagnosed only three cases of child progeria. The first case was described in 1991. The reports about two more patients aged 7 and 15 with child progeria were published.Many authors attempted to classify progeria, tried to differentiate forms of this disease. In our opinion, this is inappropriate due to the large number of clinical signs and inconsistency of them. We believe that the basic diagnostic criteria for child progeria should be highlighted, namely: sclerotic changes in the heart and blood vessels, senile appearance, wrinkled atrophic skin, loss of the hair and teeth, muscle atrophy, and others. Taking into account that the symptoms of aging at progeria appear at different ages, we consider it expedient to single out two forms of progeria — early and late. In case of early form the child is born with aging symptoms, which is obviously associated with genetic factors, fetal malformations, and mother’s illnesses during pregnancy. In the late version, rapid aging begins later, at the age of 2 or 3 years, and is associated with a violation of endocrine regulation.