• Skeletal radiology · Aug 2017

    Case Reports

    Progeria: an extremely unusual disorder.

    • Gurnihal Singh Chawla, Purva Mahesh Agrawal, and Avinash Dhok.
    • Department of Radiology, NKPSIMS and Lata Mangeshkar Hospital, Hingana Road, Digdoh Hills, Nagpur, Maharashtra, 440019, India. gurnihal.chawla1@gmail.com.
    • Skeletal Radiol. 2017 Aug 1; 46 (8): 1149-1153.

    AbstractHutchinson-Gilford progeria syndrome, also known as progeria, is an extremely rare disorder with an incidence rate of 1 in 8 million. It occurs sporadically, and patients suffering from this syndrome usually exhibit premature ageing. It has an autosomal recessive inheritance with a slight male predominance. The affected children usually die early with an average life span of 13.4 years. The most common cause of death in such patients is a cardio-vascular abnormality such as myocardial infarction. We present a rare case of progeria in an 8-year-old boy who was diagnosed clinically and was referred to our department for a skeletal survey. Almost all of the typical radiological findings were present in this case, which further confirmed the clinical diagnosis of progeria.

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