Other Free Encyclopedias » Medicine Encyclopedia » Genetics in Medicine - Part 1 » Accelerated Aging: Progeria - Progeria's Effects, Inheritance Of Progeria, Germinal Mosaicism

Accelerated Aging: Progeria - Progeria's Effects

age clinical children skin hutchinson gilford

There is considerable controversy as to whether or not progeria is a form of aging at all. Most clinicians believe that progeria is truly a form of early aging, although only a segmental form in which only certain specific tissues and cell types of the body age early. Hutchinson-Gilford children show what appears to be early aging of their skin, bones, joints, and cardiovascular system, but not of their immune or central nervous systems.

Clinical problems parallel this observation: They suffer from thin skin and poor skin healing, osteoporosis, arthritis, and heart disease, but do not have more infections than normal children and they do not have early dementia. Death is usually due to cardiovascular disease, especially heart attacks and strokes, yet Hutchinson-Gilford children lack normal risk factors associated with these diseases, such as smoking, high cholesterol, hypertension, or diabetes.

Clinically, the children appear old, with thin skin, baldness, swollen joints, and short stature. They do not go through puberty. The face is strikingly old in appearance. The typical Hutchinson-Gilford child looks more like a centenarian than like other children, and may look more like other progeric children than like members of their own families. There is no effective clinical intervention.

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