Disease Information for Progeria of child (Hutchinson-Gilford)

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Clinical Manifestations
Signs & Symptoms
Old-man facies/infant
Baldness/Alopecia
Gray Hair
Gray hair Childhood
Gray hair, premature
Hair loss
Increased wrinkling/skin
Non-scarring Alopecia/balding
Silvery gray hair/child
White Hair
Bossing forehead,infants
Premature Aging
Short stature
Short stature Child
Typical Clinical Presentation
Presentation/Coronary Heart Disease Young Age
Clinical Presentation & Variations
Presentation/Arteriosclerosis Young Age
Presentation/Myocardial Infarction in a Child
Presentation/Juvenile Cataract
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Diagnostic Test Results
X-RAY
Xray/Subcutaneous ossification centers/calcification
Xray/Soft tissue calcification/muscle/subcutaneous
Xray/Distal Clavicle Bone Resorption/Chest
Xray/Frontal bossing, infant/Skull
Xray/Parietal bossing/Skull
Associated Diseases & Rule outs
Associated Disease & Complications
Arteriosclerosis
Arteriosclerotic heart disease
Cataract
Growth retardation/failure
Ischemia, cardiac
Nail dystrophy
Premature aging/senescence
Progeria
Progeria of child (Hutchinson-Gilford syndrome)
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Systemic/no comment (category)
Process
PROCESS/Congenital/developmental (category)
PROCESS/Genetic disorder/Spontaneous mutations/sporadic
Synonyms
Synonym
Gilford Hutchinson, Gilford's syndrome, HGPS, Hutchinson Gilford, Hutchinson Gilford Disease, HUTCHINSON GILFORD PROGERIA SYNDROME, Hutchinson Gilford Syndrome, Hutchinson Gilford syndrome (disorder), Premature senility syndrome, Progeria, Progeria (disorder), Progeria syndrome, Progeria syndrome (disorder), Senilism syndrome, Syndrome Hutchinson Gilford, syndrome premature senility, Synonym/Hutchinson-Gilford syndrome
Definition

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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