Progeria natural history, complications and prognosis: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
 
(10 intermediate revisions by the same user not shown)
Line 5: Line 5:


==Overview==
==Overview==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
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]]).<br />
 
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, Complications, and Prognosis==


===Natural History===
===Natural History===
*The symptoms of Hutchinson-Gilford progeria syndrome (HGPS) usually develop in the first decade of life, and start with symptoms as follows:<ref name="pmid17908770">{{cite journal| author=Gordon LB, McCarten KM, Giobbie-Hurder A, Machan JT, Campbell SE, Berns SD et al.| title=Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development. | journal=Pediatrics | year= 2007 | volume= 120 | issue= 4 | pages= 824-33 | pmid=17908770 | doi=10.1542/peds.2007-1357 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17908770  }}</ref>
*The symptoms of Hutchinson-Gilford progeria syndrome (HGPS) usually develop in the first decade of life, and start with symptoms as follows:<ref name="pmid17908770">{{cite journal| author=Gordon LB, McCarten KM, Giobbie-Hurder A, Machan JT, Campbell SE, Berns SD et al.| title=Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development. | journal=Pediatrics | year= 2007 | volume= 120 | issue= 4 | pages= 824-33 | pmid=17908770 | doi=10.1542/peds.2007-1357 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17908770 }}</ref><ref name="pmid18256394">{{cite journal| author=Merideth MA, Gordon LB, Clauss S, Sachdev V, Smith AC, Perry MB et al.| title=Phenotype and course of Hutchinson-Gilford progeria syndrome. | journal=N Engl J Med | year= 2008 | volume= 358 | issue= 6 | pages= 592-604 | pmid=18256394 | doi=10.1056/NEJMoa0706898 | pmc=2940940 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18256394 }}</ref>
**Failure to thrive
**Failure to thrive
**Loss of subcutaneous fat
**Loss of subcutaneous fat
Line 24: Line 16:
**Short stature
**Short stature
***Decreased linear weight gain
***Decreased linear weight gain
**Alopecia


===Complications===
===Complications===
*Common complications of [disease name] include:
*Common complications of Hutchinson-Gilford progeria syndrome (HGPS) include:<ref name="pmid286604862">{{cite journal| author=Ahmed MS, Ikram S, Bibi N, Mir A| title=Hutchinson-Gilford Progeria Syndrome: A Premature Aging Disease. | journal=Mol Neurobiol | year= 2018 | volume= 55 | issue= 5 | pages= 4417-4427 | pmid=28660486 | doi=10.1007/s12035-017-0610-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28660486  }}</ref><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>
**[Complication 1]
**Progressive atherosclerosis
**[Complication 2]
**Myocardial infarction
**[Complication 3]
**Transient ischemic attack (TIA)
**Stroke
**Exposure keratopathy
**Conductive hearing loss


===Prognosis===
===Prognosis===
*Prognosis is generally poor, in patients with Hutchinson-Gilford progeria syndrome (HGPS).<ref name="pmid24795390">{{cite journal| author=Gordon LB, Massaro J, D'Agostino RB, Campbell SE, Brazier J, Brown WT et al.| title=Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome. | journal=Circulation | year= 2014 | volume= 130 | issue= 1 | pages= 27-34 | pmid=24795390 | doi=10.1161/CIRCULATIONAHA.113.008285 | pmc=4082404 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24795390  }}</ref>
*Prognosis is generally poor, in patients with Hutchinson-Gilford progeria syndrome (HGPS).<ref name="pmid24795390">{{cite journal| author=Gordon LB, Massaro J, D'Agostino RB, Campbell SE, Brazier J, Brown WT et al.| title=Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome. | journal=Circulation | year= 2014 | volume= 130 | issue= 1 | pages= 27-34 | pmid=24795390 | doi=10.1161/CIRCULATIONAHA.113.008285 | pmc=4082404 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24795390  }}</ref>
*The presence of progressive atherosclerosis is associated with a particularly poor prognosis among patients with Hutchinson-Gilford progeria syndrome (HGPS).
*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.<ref name="pmid247953902">{{cite journal| author=Gordon LB, Massaro J, D'Agostino RB, Campbell SE, Brazier J, Brown WT et al.| title=Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome. | journal=Circulation | year= 2014 | volume= 130 | issue= 1 | pages= 27-34 | pmid=24795390 | doi=10.1161/CIRCULATIONAHA.113.008285 | pmc=4082404 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24795390  }}</ref><ref name="pmid28660486">{{cite journal| author=Ahmed MS, Ikram S, Bibi N, Mir A| title=Hutchinson-Gilford Progeria Syndrome: A Premature Aging Disease. | journal=Mol Neurobiol | year= 2018 | volume= 55 | issue= 5 | pages= 4417-4427 | pmid=28660486 | doi=10.1007/s12035-017-0610-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28660486  }}</ref>


==References==
==References==

Latest revision as of 16:28, 9 August 2019

Progeria Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Progeria from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Progeria natural history, complications and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Progeria natural history, complications and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Progeria natural history, complications and prognosis

CDC on Progeria natural history, complications and prognosis

Progeria natural history, complications and prognosis in the news

Blogs on Progeria natural history, complications and prognosis

Directions to Hospitals Treating Psoriasis

Risk calculators and risk factors for 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.

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Progeria_natural_history,_complications_and_prognosis&oldid=1578916"