Teen With Progeria, Is The Last Of 6 Siblings Afflicted With Rare Disease .

Ali Hussain Khan from Bihar, India, is only 14 years old, but the condition, called progeria, has given him the body of a 110-year-old.


His parents, who are first cousins, have eight children. Four born with progeria died between ages 12 and 24. Another child, who died shortly after birth, is also thought to have had the disease. Two of their daughters do not have progeria.

There are only about 80 cases of progeria known in the world. According to the Progeria Research Foundation, a nonprofit that seeks to cure the condition and its aging-related disorders , the children with progeria "are born looking healthy... [but] begin to display many characteristics of accelerated aging at around 18-24 months of age."

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What is Progeria ?

Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare hereditary disease that affects the skin, musculoskeletal system, and vasculature. HGPS is characterized by signs of premature aging. The term progeria is derived from the Greek word geras, meaning old age. Significant morbidity and mortality result from accelerated atherosclerosis of the carotid and coronary arteries, leading to premature death during the first or second decade of life. HGPS is considered a segmental aging syndrome, as affected patients do not manifest all of the typical features of aging, such as increased incidence of cancer and neurocognitive decline.

Clinical Presentation

Evidence of Hutchinson-Gilford progeria syndrome (HGPS) begins within the first 2 years of life. At birth, infants usually appear healthy, although sclerodermatous skin changes have been noted in some patients. Typically, the onset of the disease occurs at age 6-12 months, when skin changes and alopecia are first noted and when the infant fails to gain weight. The following are other suggestive findings :

High-pitched voice
Short stature and low weight for height, with prenatal onset of growth failure
Incomplete sexual maturation
Generalized osteoporosis and pathologic fractures
Feeding difficulties
Delayed dentition, anodontia, hypodontia, or crowding of teeth
Low-frequency conductive hearing loss.
Hypertension
Prolonged prothrombin time, elevated platelet counts, and elevated serum phosphorus levels
Emotionally, patients with HGPS share the same feelings as age-matched healthy persons with regard to expressing proper mood and affect. Patients with HGPS are keenly aware of their different appearance and remain reserved in the company of strangers; in the presence of friends, they display affection and good social interaction.


Read More About Progeria at Medscape 

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