pediatrics
Progeria
Other progeroid syndromes
Premature aging is a feature of other rare progeroid syndromes. Werner syndromeis premature aging after puberty with hair thinning and development of conditions of old age (eg, cataracts, diabetes, osteoporosis, atherosclerosis).Rothmund-Thomson syndromeis premature aging with increased susceptibility to cancer. Both are caused by gene mutations leading to defective RecQ DNA helicases, which normally repair DNA. Cockayne syndromeis an autosomal recessive disorder caused by mutation in theERCC8gene, which is important in DNA excision repair. Clinical features include severe growth failure, cachectic appearance, retinopathy, hypertension, renal failure, skin photosensitivity, and intellectual disability. Neonatal progeroid syndrome (Wiedemann-Rautenstrauch syndrome)is a recessively inherited syndrome of aging causing death by age 2 years. Other syndromes (eg, Down Syndrome (Trisomy 21) , 