Disease Information for Progeria of child (Hutchinson-Gilford)

Hutchinson-Gilford disease; Gilford’s syndrome; Hutchinson-Gilford progeria; Hutchinson-Gilford syndrome;Souques-Charcot syndrome (a variant of this syndrome); Premature ageing syndrome, premature senility syndrome, progeria, progeria infantum, progeria infantilis, senilism syndrome;

A very rare disease of premature aging in young children, characterized mainly by a birdlike, "wizened old man" facial appearance, premature bodily ageing (progeria) and dwarfism. The children have large skull, bird-like features, atrophy of muscles and skin, loss of subcutaneous fat, high serum lipid levels and early atherosclerotic changes in the vessels; The child is normal at birth; Intelligence is usually normal; The average age of death is 16 years, but survival until 26 years has been recorded; Occurs in children of both sexes; Death is usually caused by the effects of arteriosclerosis; Etiology and mode of genetic transmission (if any) is unknown; Hutchinson-Gilford progeria was discovered in 1886 by Jonathan Hutchinson; He documented the clinical features of a boy aged 6 years who had congenital absence of the hair and atrophy of the skin; Hastings Gilford followed up this patient and another, and in 1897 recognized it as a clinical entity and introduced the term «progeria» from the Greek word meaning "prematurely old"; In his case report published in 1904 he provided a set of pictures of the disorder from infancy to the age of 17 years, in which the characteristic alopecia, fat loss and premature ageing are clearly depicted;

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