Literature DB >> 1613770

Marfan syndrome in a large family: response of family members to a screening programme.

A B Bridges1, M Faed, M Boxer, J R Gray, C Bundy, A Murray.   

Abstract

Reaction to medical, social, and genetic implications of Marfan syndrome was evaluated by means of two questionnaires, the first after various tests before discussion of the diagnosis, the second after full discussion of the patient's diagnosis. Thirty-seven members of a family known to be at risk for Marfan syndrome attended for both questionnaires. All patients claimed to be satisfied with the way they were informed of the results of screening; 41% of patients were more worried about their health and 48% were more worried about the future after diagnosis. Apart from 50% of the smokers reducing or stopping their intake of cigarettes there were only very minor changes in lifestyle over the first month despite the increased level of expressed anxiety. If a definitive screening test was available, 96% of patients claimed they would have chosen it, 45% felt it would have an influence on their future plans, and 78% would choose to use a method of prenatal diagnosis for Marfan syndrome if it were available.

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Mesh:

Year:  1992        PMID: 1613770      PMCID: PMC1015844          DOI: 10.1136/jmg.29.2.81

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


  11 in total

1.  Marfan syndrome is closely linked to a marker on chromosome 15q1.5----q2.1.

Authors:  P Tsipouras; M Sarfarazi; A Devi; B Weiffenbach; M Boxer
Journal:  Proc Natl Acad Sci U S A       Date:  1991-05-15       Impact factor: 11.205

2.  Marfan's syndrome: natural history and long-term follow-up of cardiovascular involvement.

Authors:  D L Marsalese; D S Moodie; M Vacante; B W Lytle; C C Gill; R Sterba; D M Cosgrove; M Passalacqua; M Goormastic; A Kovacs
Journal:  J Am Coll Cardiol       Date:  1989-08       Impact factor: 24.094

3.  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

Review 4.  The Marfan syndrome: diagnosis and management.

Authors:  R E Pyeritz; V A McKusick
Journal:  N Engl J Med       Date:  1979-04-05       Impact factor: 91.245

5.  Diagnosing Marfan's syndrome.

Authors:  A H Child
Journal:  Br Med J (Clin Res Ed)       Date:  1988-06-11

6.  Diagnosing Marfan syndrome.

Authors:  M Super
Journal:  Br Med J (Clin Res Ed)       Date:  1988-05-14

7.  Location on chromosome 15 of the gene defect causing Marfan syndrome.

Authors:  K Kainulainen; L Pulkkinen; A Savolainen; I Kaitila; L Peltonen
Journal:  N Engl J Med       Date:  1990-10-04       Impact factor: 91.245

8.  Marfan syndrome affecting four generations of a family without ocular involvement.

Authors:  A B Bridges; M Faed; M Boxer; W M Haining; T H Pringle; G P McNeill
Journal:  Postgrad Med J       Date:  1991-06       Impact factor: 2.401

9.  The clinical course and echocardiographic features of Marfan's syndrome in childhood.

Authors:  T Geva; J Hegesh; M Frand
Journal:  Am J Dis Child       Date:  1987-11

10.  Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes.

Authors:  B Lee; M Godfrey; E Vitale; H Hori; M G Mattei; M Sarfarazi; P Tsipouras; F Ramirez; D W Hollister
Journal:  Nature       Date:  1991-07-25       Impact factor: 49.962

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  2 in total

Review 1.  Marfan syndrome.

Authors:  J R Gray; S J Davies
Journal:  J Med Genet       Date:  1996-05       Impact factor: 6.318

2.  Prenatal diagnosis and a donor splice site mutation in fibrillin in a family with Marfan syndrome.

Authors:  M Godfrey; N Vandemark; M Wang; M Velinov; D Wargowski; P Tsipouras; J Han; J Becker; W Robertson; S Droste
Journal:  Am J Hum Genet       Date:  1993-08       Impact factor: 11.025

  2 in total

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