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Progeria natural history, complications and prognosis

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

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K; et al. (1993). “GeneReviews®”. PMID 20301300.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.

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