Literature DB >> 24336584

Marfan syndrome.

Eesha Jain1, Ramesh Kumar Pandey.   

Abstract

Marfan syndrome is a rare autosomal dominant disorder of the connective tissue, with skeletal, ligamentous, orooculofacial, pulmonary, abdominal, neurological and the most fatal, cardiovascular manifestations. It has no cure but early diagnosis, regular monitoring and preventive lifestyle regimen ensure a good prognosis. However, the diagnosis can be difficult as it is essentially a clinical one, relying on family history, meticulous physical examination and investigation of involved organ systems. Patients of Marfan syndrome portray very typical physical and orofacial characteristics, suggesting obvious recognition, but due to variable phenotypic expression, cases often go unnoticed unless a full range of attributing features is apparent. Dental practitioners are very likely to encounter patients of Marfan syndrome at an early age as they frequently present for dental treatment. The present case report illustrates the preliminary screening of Marfan syndrome in a dental office followed by timely diagnosis and appropriate referrals.

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Mesh:

Year:  2013        PMID: 24336584      PMCID: PMC3863051          DOI: 10.1136/bcr-2013-201632

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  11 in total

1.  Influence of maxillary morphology on nasal airway resistance in Marfan's syndrome.

Authors:  P A Cistulli; C E Sullivan
Journal:  Acta Otolaryngol       Date:  2000-03       Impact factor: 1.494

2.  Severe periodontitis in Marfan's syndrome: a case report.

Authors:  Antje M Straub; Rodney Grahame; Crispian Scully; Maurizio S Tonetti
Journal:  J Periodontol       Date:  2002-07       Impact factor: 6.993

3.  Guidelines for the diagnosis and management of Marfan syndrome.

Authors:  Lesley Ades
Journal:  Heart Lung Circ       Date:  2006-12-26       Impact factor: 2.975

Review 4.  Marfan syndrome: an update of genetics, medical and surgical management.

Authors:  Yskert von Kodolitsch; Peter N Robinson
Journal:  Heart       Date:  2007-06       Impact factor: 5.994

5.  Prevalence of pulp calcifications in patients with Marfan syndrome.

Authors:  Oskar Bauss; Daniel Neter; Alexander Rahman
Journal:  Oral Surg Oral Med Oral Pathol Oral Radiol Endod       Date:  2008-09-20

Review 6.  Exercise and the Marfan syndrome.

Authors:  A C Braverman
Journal:  Med Sci Sports Exerc       Date:  1998-10       Impact factor: 5.411

7.  The diagnostic value of the facial features of Marfan syndrome.

Authors:  Beverlie L Ting; Deepti Mathur; Bart L Loeys; Harry C Dietz; Paul D Sponseller
Journal:  J Child Orthop       Date:  2010-10-31       Impact factor: 1.548

8.  Orofacial manifestations of congenital fibrillin deficiency: pathogenesis and clinical diagnostics.

Authors:  Peter J De Coster; Luc C Martens; Anne De Paepe
Journal:  Pediatr Dent       Date:  2004 Nov-Dec       Impact factor: 1.874

9.  Systemic management of Marfan's syndrome during dental treatment: a case report.

Authors:  Y Hirota; K Sugiyama; H Niwa; H Matsuura
Journal:  Anesth Pain Control Dent       Date:  1993

10.  Temporomandibular joint dysfunction in Marfan syndrome.

Authors:  Oskar Bauss; Reza Sadat-Khonsari; Christian Fenske; Werner Engelke; Rainer Schwestka-Polly
Journal:  Oral Surg Oral Med Oral Pathol Oral Radiol Endod       Date:  2004-05
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  1 in total

1.  Oral manifestations of a rare variant of Marfan syndrome.

Authors:  Abhishek Sinha; Sandeep Kaur; Syed Ahmed Raheel; Kirandeep Kaur; Mohammed Alshehri; Omar Kujan
Journal:  Clin Case Rep       Date:  2017-07-18
  1 in total

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