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Pulmonary Manifestations of Marfan Syndrome

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Diagnosis and Management of Marfan Syndrome

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Abstract

While the cardiovascular features of Marfan syndrome most frequently require medical intervention, it is recognised that pulmonary complications occur in up to 10 % of patients [1, 2]. Lung involvement may present in infancy or childhood [3], Complications may arise in adolescence or adult life that have serious consequences and are potentially fatal [4, 5].

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References

  1. McKusick VA. Heritable disorders of connective tissue. 4th ed. Oxford: CV Mosby, Blackwell Scientific Publications; 1972.

    Google Scholar 

  2. Foster ME, Foster DR. Bronchiectasis and Marfan’s syndrome. Postgrad Med J. 1980;56(660):718–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Dominguez R, Weisgrau RA, Santamaria M. Pulmonary hyperinflation and emphysema in infants with the Marfan syndrome. Pediatr Radiol. 1987;17:365–9.

    Article  CAS  PubMed  Google Scholar 

  4. Takanami I, Morota A, Shikata J. A case report of spontaneous pneumothorax in Marfan syndrome and an immunohistochemical study of type 1 collagen in lung tissue. Japan J Thoracic Dis. 1991;29:1186–9.

    CAS  Google Scholar 

  5. Saito H, Lijima K, Dambara T, Shiota J, Hirose S, Uekusa T, et al. An autopsy case of Marfan syndrome with bronchiectasis and multiple bullae. Japan J Thoracic Dis. 1992;30:1315–21.

    CAS  Google Scholar 

  6. Katz H. Thoracic manifestations in Marfan syndrome (arachnodactyly). Q Bull Sea View Hosp. 1952;13:95–106.

    CAS  PubMed  Google Scholar 

  7. Fuleihan FJD, Suh SK, Shepard RH. Some aspects of pulmonary function in the Marfan syndrome. Bull John Hopkins Hosp. 1963;113:320–9.

    CAS  PubMed  Google Scholar 

  8. Streeten EA, Murphy EA, Pyeritz RE. Pulmonary function in the Marfan syndrome. Chest. 1987;9:408–12.

    Article  Google Scholar 

  9. Corsico AG, Grosso A, Tripon B, Albicini F, Gini E, Mazzetta A, Di Vincenzo EM, Angesi ME, Tsana Tegomo E, Ronzoni V, Arbustini E, Cervieri I. Pulmonary involvement in patients with Marfan syndrome. Panminerva Med. 2014;56(2):177–82.

    CAS  PubMed  Google Scholar 

  10. Hall JR, Pyeritz RE, Dudgeon MDG, Haller A. Pneumothorax in the Marfan syndrome; prevalence and therapy. Ann Thorac Surg. 1984;37:500–5.

    Article  CAS  PubMed  Google Scholar 

  11. Dwyer EM, Troncale F. Spontaneous pneumothorax and pulmonary disease in the Marfan syndrome. Ann Intern Med. 1965;62:1285–92.

    Article  PubMed  Google Scholar 

  12. Wood J, Bellamy D, Child A, Citron KM. Pulmonary disease in patients with Marfan syndrome. Thorax. 1984;39:780–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Turner JA, Stanley NN. Fragile lung in Marfan syndrome. Thorax. 1976;31:771–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Yellin A, Shiner RJ, Lieberman Y. Familial multiple bilateral pneumothorax associated with Marfan syndrome. Chest. 1991;100:577–8.

    Article  CAS  PubMed  Google Scholar 

  15. Suzuki T, Akiba T, Miyake R, Marushima H, Morikawa T. Familial spontaneous pneumothorax in two adult siblings with Marfan syndrome. Ann Thorac Cardiovasc Surg. 2010;16(5):362–4.

    PubMed  Google Scholar 

  16. Dyhdalo K, Farver C. Pulmonary histologic changes in Marfan syndrome: a case series and literature review. Am J Clin Pathol. 2011;136(6):857–63.

    Article  PubMed  Google Scholar 

  17. Sayers CP, Goltz RW, Mottiaz J. Pulmonary elastic tissue in generalised elastocytosis (cutis laxa) and Marfan syndrome; a light and electron microscopic study. J Invest Dermatol. 1975;65:451–7.

    Article  CAS  PubMed  Google Scholar 

  18. Savini R, Cervellati S, Beroaldo E. Spinal deformities in Marfan syndrome. Ital J Orthop Traumatol. 1980;6:l9–40.

    Google Scholar 

  19. Kelly RE, Goretscky MJ, Obermeyer R, Kuhn MA, Redinger R, Haney TS, Moskowitz A, Nuss D. Ann Surg. 2010;252(6):1072–81.

    Article  PubMed  Google Scholar 

  20. Teoh PC. Bronchiectasis and spontaneous pneumothorax in Marfan syndrome. Chest. 1977;72:672–3.

    Article  CAS  PubMed  Google Scholar 

  21. Hwang HS, Yi CA, Yoo H, Yang JH, Kim ~D-K, Koh W-J. The prevalence of bronchiectasis in patients with Marfan syndrome. Int J Tuberc Lung Dis. 2014;18(8):995–7(3).

    Article  CAS  PubMed  Google Scholar 

  22. Lipton RA, GreenwaId A, Seriff NS. Pneumothorax and bilateral honeycombed lung in Marfan syndrome. Am Rev Respir Dis. 1971;104:924–8.

    CAS  PubMed  Google Scholar 

  23. Rybczynski M, Koschyk D, Karmeier A, Gessler N, Sheikhzadeh S, Bernhardt AM, Habermann CR, Treede H, Berger J, Robinson PN, Meinertz T, von Kodolitsch Y. Frequency of sleep apnea in adults with the Marfan syndrome. Am J Cardiol. 2010;105(12):1836–41. Erratum in: Am J Cardiol. 2010;106(5):755.

    Google Scholar 

  24. Kohler M, Blair E, Risby P, Nickol AH, Wordsworth P, Forfar C, Stradling JR. The prevalence of obstructive sleep apnoea and its association with aortic dilatation in Marfan’s syndrome. Thorax. 2009;64(2):162–6.

    Article  CAS  PubMed  Google Scholar 

  25. Kai Y, Yamaoka A, Irita K, Zaitsu A, Takahashi S. Transient tracheal obstruction during surgical correction of scoliosis in a patient with Marfan syndrome. Japan J Anaesthesiol. 1995;44:868–73.

    CAS  Google Scholar 

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Authors and Affiliations

  1. Cardiovascular and Cell Sciences Research Institute, St George’s, University of London, London, UK

    Anne H. Child MD, FRCP

  2. NIHR Respiratory Biomedical Research Unit, Royal Brompton & Harefield NHS Foundation Trust, Sydney Street, London, SW3 6NP, UK

    Anita K. Simonds MD, FRCP

Authors
  1. Anne H. Child MD, FRCP
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  2. Anita K. Simonds MD, FRCP
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Correspondence to Anne H. Child MD, FRCP .

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Editors and Affiliations

  1. Cardiovascular & Cell Sciences Research, St George's, University of London, London, United Kingdom

    Anne H. Child

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Child, A.H., Simonds, A.K. (2016). Pulmonary Manifestations of Marfan Syndrome. In: Child, A. (eds) Diagnosis and Management of Marfan Syndrome. Springer, London. https://doi.org/10.1007/978-1-4471-5442-6_15

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  • DOI: https://doi.org/10.1007/978-1-4471-5442-6_15

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  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-5441-9

  • Online ISBN: 978-1-4471-5442-6

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