Progeria natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]
Overview
The symptoms of Hutchinson-Gilford progeria syndrome (HGPS) usually develop in the first decade of life, complications of Hutchinson-Gilford progeria syndrome (HGPS) include progressive atherosclerosis and myocardial infarction. Prognosis is generally poor, in patients with Hutchinson-Gilford progeria syndrome (HGPS).
Natural History, Complications, and Prognosis
Natural History
- The symptoms of Hutchinson-Gilford progeria syndrome (HGPS) usually develop in the first decade of life, and start with symptoms as follows:[1][2]
- Failure to thrive
- Loss of subcutaneous fat
- Poor weight gain
- Weight increase of only 0.44 kg/year
- Short stature
- Decreased linear weight gain
- Alopecia
Complications
- Common complications of Hutchinson-Gilford progeria syndrome (HGPS) include:[3][4][5]
- Progressive atherosclerosis
- Myocardial infarction
- Transient ischemic attack (TIA)
- Stroke
- Exposure keratopathy
- Conductive hearing loss
Prognosis
- Prognosis is generally poor, in patients with Hutchinson-Gilford progeria syndrome (HGPS).[6]
- The presence of progressive atherosclerosis is associated with a particularly poor prognosis among patients with Hutchinson-Gilford progeria syndrome (HGPS).
- The average lifespan of patients with Hutchinson-Gilford progeria syndrome (HGPS) is about 15 years.[7][8]
References
- ↑ Gordon LB, McCarten KM, Giobbie-Hurder A, Machan JT, Campbell SE, Berns SD; et al. (2007). "Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development". Pediatrics. 120 (4): 824–33. doi:10.1542/peds.2007-1357. PMID 17908770.
- ↑ Merideth MA, Gordon LB, Clauss S, Sachdev V, Smith AC, Perry MB; et al. (2008). "Phenotype and course of Hutchinson-Gilford progeria syndrome". N Engl J Med. 358 (6): 592–604. doi:10.1056/NEJMoa0706898. PMC 2940940. PMID 18256394.
- ↑ Ahmed MS, Ikram S, Bibi N, Mir A (2018). "Hutchinson-Gilford Progeria Syndrome: A Premature Aging Disease". Mol Neurobiol. 55 (5): 4417–4427. doi:10.1007/s12035-017-0610-7. PMID 28660486.
- ↑ Ullrich NJ, Gordon LB (2015). "Hutchinson-Gilford progeria syndrome". Handb Clin Neurol. 132: 249–64. doi:10.1016/B978-0-444-62702-5.00018-4. PMID 26564085.
- ↑ Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K; et al. (1993). "GeneReviews®". PMID 20301300.
- ↑ Gordon LB, Massaro J, D'Agostino RB, Campbell SE, Brazier J, Brown WT; et al. (2014). "Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome". Circulation. 130 (1): 27–34. doi:10.1161/CIRCULATIONAHA.113.008285. PMC 4082404. PMID 24795390.
- ↑ Gordon LB, Massaro J, D'Agostino RB, Campbell SE, Brazier J, Brown WT; et al. (2014). "Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome". Circulation. 130 (1): 27–34. doi:10.1161/CIRCULATIONAHA.113.008285. PMC 4082404. PMID 24795390.
- ↑ Ahmed MS, Ikram S, Bibi N, Mir A (2018). "Hutchinson-Gilford Progeria Syndrome: A Premature Aging Disease". Mol Neurobiol. 55 (5): 4417–4427. doi:10.1007/s12035-017-0610-7. PMID 28660486.