Progeria

Abstract

Progeria is a rare disorder characterised by generalised undergrowth, premature ageing, bird like facies, baldness, absence of eye lashes, slender pinched nose under developed mandible and thin atrophic skin. Intelligence is normal or above normal. Diagnosis is usually confirmed by radiological features which include: (1) coxa valga, thin slender, long bones, hypoplastic mandible and maxillae, wormian bones; (2) fibrous displacement of clavicles and atrophy of distal phalanges. The patients are usually of low birth weight but have no other abnormality. Usually death occurs in second decade of life from atherosclerosis and myocardial infarction. There are no abnormalities found in thyroid, parathyroid, pituitary or adrenal glands on postmortem. A seven-year-old case is reported.