KUMJ | VOL. 10 | NO. 1 | ISSUE 37 | JAN-MAR, 2012
Progeria : Pathogenesis and Oral Manifestation- A Review
Saigal S, Bhargava A
Abstract: Our life span is genetically programmed and it is possible that a defect in produced
proteins encoded by the ‘longevity’ gene is a cause of aging. Progeria which is a
rare, fatal genetic condition which affects between one in four million and one in
eight million children of both sexes equally and characterized by premature and
accelerated aging. The appearance and physiology of these children resembles to
elderly people but they typically have life span to their mid teens. It is also known
as the Hutchinson-Gilford syndrome, which was initially reported by Johnathan
Hutchinson in 1886 and further described by Hastings Gilford in 1904. It is an
autosomal recessive disorder, which means an individual has inherited a mutated
gene from both parents. It is added to the expanding catalogue of ‘laminopathies’,
diseases caused by mutations affecting nuclear lamina proteins known as lamin A
(LMNA). In oral manifestation primary finding is micrognathia with delayed tooth
eruption and incomplete formation of root of permanent tooth. Presently there are
no known cures for this abnormality.
Keyword : Hutchinson–Gilford Progeria Syndrome (HGPS), Premature aging, Progeria.
