Progeria natural history, complications and prognosis: Difference between revisions
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===Complications=== | ===Complications=== | ||
*Common complications of Hutchinson-Gilford progeria syndrome (HGPS) include:<ref name="pmid26564085">{{cite journal| author=Ullrich NJ, Gordon LB| title=Hutchinson-Gilford progeria syndrome. | journal=Handb Clin Neurol | year= 2015 | volume= 132 | issue= | pages= 249-64 | pmid=26564085 | doi=10.1016/B978-0-444-62702-5.00018-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26564085 }}</ref> | *Common complications of Hutchinson-Gilford progeria syndrome (HGPS) include:<ref name="pmid26564085">{{cite journal| author=Ullrich NJ, Gordon LB| title=Hutchinson-Gilford progeria syndrome. | journal=Handb Clin Neurol | year= 2015 | volume= 132 | issue= | pages= 249-64 | pmid=26564085 | doi=10.1016/B978-0-444-62702-5.00018-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26564085 }}</ref><ref name="pmid20301300">{{cite journal| author=Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K et al.| title=GeneReviews® | journal= | year= 1993 | volume= | issue= | pages= | pmid=20301300 | doi= | pmc= | url= }}</ref> | ||
**Progressive atherosclerosis | **Progressive atherosclerosis | ||
**Myocardial infarction | **Myocardial infarction | ||
Revision as of 17:35, 25 July 2019
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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
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
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]
- 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).[5]
- The presence of progressive atherosclerosis is associated with a particularly poor prognosis among patients with Hutchinson-Gilford progeria syndrome (HGPS).
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.
- ↑ 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.