Literature DB >> 31656420

Severe aortic root dilatation in infantile Marfan syndrome.

Renita A Thomas1, Chikamuche T Anyanwu1, Maria Blazo1,2, Saradha Subramanian1,3.   

Abstract

Cardiovascular manifestations of Marfan syndrome are associated with increased mortality, especially in the pediatric population. Early recognition is critical to long-term management. We present two cases of genetically defined "classical" Marfan syndrome presenting with severe infantile aortic root dilatation among siblings and discuss options for therapy.
© 2019 Baylor University Medical Center.

Entities:  

Keywords:  Aortic valve dilatation; FBN1; classical Marfan syndrome; fibrillin; neonatal Marfan syndrome

Year:  2019        PMID： 31656420      PMCID： PMC6793975          DOI： 10.1080/08998280.2019.1646594

Source DB:  PubMed          Journal:  Proc (Bayl Univ Med Cent)        ISSN： 0899-8280


  12 in total

1.  Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?

Authors:  Hideyuki Shimizu; Hirofumi Kasahara; Atsushi Nemoto; Kentaro Yamabe; Toshihiko Ueda; Ryohei Yozu
Journal:  Interact Cardiovasc Thorac Surg       Date:  2011-11-30

2.  Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial.

Authors:  Maarten Groenink; Alexander W den Hartog; Romy Franken; Teodora Radonic; Vivian de Waard; Janneke Timmermans; Arthur J Scholte; Maarten P van den Berg; Anje M Spijkerboer; Henk A Marquering; Aeilko H Zwinderman; Barbara J M Mulder
Journal:  Eur Heart J       Date:  2013-09-02       Impact factor: 29.983

3.  Multisegment coloboma in a case of Marfan syndrome: another possible effect of increased TGFβ signaling.

Authors:  Shannon K LeBlanc; Deepa Taranath; Scott Morris; Christopher P Barnett
Journal:  J AAPOS       Date:  2014-02       Impact factor: 1.220

Review 4.  A systematic review of the pharmacological management of aortic root dilation in Marfan syndrome.

Authors:  Varsha Thakur; Kathryn N Rankin; Lisa Hartling; Andrew S Mackie
Journal:  Cardiol Young       Date:  2012-10-19       Impact factor: 1.093

5.  UMD (Universal Mutation Database): 2005 update.

Authors:  Christophe Béroud; Dalil Hamroun; Gwenaëlle Collod-Béroud; Catherine Boileau; Thierry Soussi; Mireille Claustres
Journal:  Hum Mutat       Date:  2005-09       Impact factor: 4.878

Review 6.  Marfan's syndrome and the heart.

Authors:  Alan Graham Stuart; Andrew Williams
Journal:  Arch Dis Child       Date:  2007-04       Impact factor: 3.791

Review 7.  Aortopathy in Marfan syndrome: an update.

Authors:  Federico Romaniello; Donatella Mazzaglia; Antonio Pellegrino; Susanna Grego; Roberto Fiorito; Amedeo Ferlosio; Luigi Chiariello; Augusto Orlandi
Journal:  Cardiovasc Pathol       Date:  2014-05-06       Impact factor: 2.185

8.  Losartan therapy for cardiac disease in paediatricMarfan syndrome.

Authors:  Lisa J Ewans; Phillip Roberts; Lesley Adès
Journal:  J Paediatr Child Health       Date:  2015-09       Impact factor: 1.954

9.  A Case of Neonatal Marfan Syndrome: A Management Conundrum and the Role of a Multidisciplinary Team.

Authors:  Elliott J Carande; Samuel J Bilton; Satish Adwani
Journal:  Case Rep Pediatr       Date:  2017-01-11

Review 10.  A novel fibrillin-1 gene missense mutation associated with neonatal Marfan syndrome: a case report and review of the mutation spectrum.

Authors:  Qian Peng; Yan Deng; Yuan Yang; Hanmin Liu
Journal:  BMC Pediatr       Date:  2016-04-30       Impact factor: 2.125
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