Literature DB >> 8880576

A clinical severity grading scale for Marfan syndrome.

J R Gray1, S J Davies.   

Abstract

There is considerable interest in assessing patients with Marfan syndrome at the clinical, protein, and genetic levels. Clinical assessments are inconsistent between centres and between clinicians. There are no satisfactory clinical grading scales against which to analyse the molecular information, limiting the scope of clinical/ molecular correlations. We present grading scales for the major clinical systems involved in Marfan syndrome and suggest that their widespread use will facilitate collaborative approaches to clinical/ molecular correlations and international assessments of therapy.

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Year:  1996        PMID: 8880576      PMCID: PMC1050730          DOI: 10.1136/jmg.33.9.758

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  4 in total

1.  Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome.

Authors:  D W Hollister; M Godfrey; L Y Sakai; R E Pyeritz
Journal:  N Engl J Med       Date:  1990-07-19       Impact factor: 91.245

2.  International Nosology of Heritable Disorders of Connective Tissue, Berlin, 1986.

Authors:  P Beighton; A de Paepe; D Danks; G Finidori; T Gedde-Dahl; R Goodman; J G Hall; D W Hollister; W Horton; V A McKusick
Journal:  Am J Med Genet       Date:  1988-03

3.  A molecular approach to the stratification of cardiovascular risk in families with Marfan's syndrome.

Authors:  L Pereira; O Levran; F Ramirez; J R Lynch; B Sykes; R E Pyeritz; H C Dietz
Journal:  N Engl J Med       Date:  1994-07-21       Impact factor: 91.245

4.  Two-dimensional echocardiographic aortic root dimensions in normal children and adults.

Authors:  M J Roman; R B Devereux; R Kramer-Fox; J O'Loughlin
Journal:  Am J Cardiol       Date:  1989-09-01       Impact factor: 2.778

  4 in total
  3 in total

Review 1.  Decellularized matrices for cardiovascular tissue engineering.

Authors:  Francesco Moroni; Teodelinda Mirabella
Journal:  Am J Stem Cells       Date:  2014-03-13

2.  Marfan Syndrome and Quality of Life in the GenTAC Registry.

Authors:  Judith Z Goldfinger; Liliana R Preiss; Richard B Devereux; Mary J Roman; Tabitha P Hendershot; Barbara L Kroner; Kim A Eagle
Journal:  J Am Coll Cardiol       Date:  2017-06-13       Impact factor: 24.094

3.  Prevalence data on all Ghent features in a cross-sectional study of 87 adults with proven Marfan syndrome.

Authors:  Svend Rand-Hendriksen; Rigmor Lundby; Lena Tjeldhorn; Kai Andersen; Jon Offstad; Svein Ove Semb; Hans-Jørgen Smith; Benedicte Paus; Odd Geiran
Journal:  Eur J Hum Genet       Date:  2009-03-18       Impact factor: 4.246

  3 in total

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