Suspected progeria in Nigeria: a case report

BACKGROUND: Progeria is a rare fatal genetic condition characterized by an appearance of accelerated aging in children. It has an incidence of 1 in 8 million and results from a mutation of the LMNA gene causing nuclear instability. Clinical diagnosis is based on recognition of common clinical features and definitive diagnosis is by identifying the mutation in the LMNA gene. Affected children usually have a median life span of 13 years. There is no known cure but research is ongoing. Currently about 80 children have had a definitive diagnosis worldwide with the exclusion of Nigeria. There was however a case report of 3 siblings in the University of Benin Teaching Hospital, Benin City, Nigeria in 1990.