Progeria : Pathogenesis and Oral Manifestation- A Review

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Progeria: pathogenesis and oral manifestation--a review.

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 re...

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Progeria, also known as the Hutchinson-Gilford syndrome, is an extremely rare condition that was initially reported by Jonathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. Hutchinson Gilford progeria syndrome is a disorder characterized by premature aging of postnatal onset. The main clinical and radiological features include alopecia, thin skin hypoplasia of nails, lo...

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Hutchinson– Gilford Progeria Syndrome is a very rare disorder characterized by premature ageing caused due to mutation in LMNA gene. The child born with this disorder shows features of old age from first year of birth and generally dies in teenage. The clinical symptoms include alopecia, thin skin, stiffness of joints etc. All of the children suffering from this disease appear identical. The pa...

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We are presenting two cases, which we consider to be examples of a syndrome allied to progeria, in some detail because no exactly similar cases appear to have been recorded. These children were first seen at the Queen Elizabeth Hospital, Hackney, by Dr. Helen Mackay in June, 1947. They present strikingly similar clinical pictures, the outstanding features being dwarfism, microcephaly, a fine di...

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ژورنال

عنوان ژورنال: Kathmandu University Medical Journal

سال: 2012

ISSN: 1812-2078,1812-2027

DOI: 10.3126/kumj.v10i1.6919