 |
 |

Marfanoid ChildrenEtiologic Heterogeneity and Cardiac Findings
Shawky Tayel, MD;
Thaddeus W. Kurczynski, MD, PhD;
Marc Levine, MD;
Ernest Brookfield, MD;
Robert Ehrlich, MD;
James R. Hennessy, MD;
Martin M. DeBeukelaer, MD
Am J Dis Child. 1991;145(1):90-93.
Abstract
The clinical, cardiac, and echocardiographic test results of 20 children with marfanoid features are reviewed. Fifteen were diagnosed as having Marfan syndrome, two had "possible" Marfan syndrome, and three had other diagnoses. On first evaluation, eight patients with Marfan syndrome (53%) had mitral regurgitation and none had aortic regurgitation. Echocardiography showed aortic root enlargement in 12 (80%) of 15 patients and mitral valve prolapse in 12 (80%) of 15. None had a normal echocardiogram. At follow-up examination, one patient had developed aortic root enlargement, and one patient, mitral valve prolapse. Thus, although aortic root enlargement is usually present in early childhood in patients with Marfan syndrome, it is not considered specific because in this study it also occurred in one child with Alport's syndrome and in one with marfanoid features. Four patients with aortic root enlargement were treated with propranolol and their echocardiograms showed no further increase in the aortic root diameter for several years. We recommend echocardiography in the diagnosis and routine management of children in whom Marfan syndrome is suspected.
(AJDC. 1991;145:90-93)
Author Affiliations
From the Department of Pediatrics, Medical College of Ohio, Toledo.
Footnotes
Accepted for publication June 27,1990.
Presented at the First International Symposium on the Marfan Syndrome, Baltimore, Md, July 9, 1988.
Reprint requests to Department of Pediatrics, Medical College of Ohio, CS 10008, Toledo, OH 43699 (Dr Kurczynski).
CiteULike Connotea Delicious Digg Facebook Reddit Technorati Twitter
What's this?
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Aortic abnormalities in males with Alport syndrome
Kashtan et al.
Nephrol Dial Transplant 2010;25:3554-3560.
ABSTRACT
| FULL TEXT
Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions
Song et al.
Ann. Thorac. Surg. 2009;88:781-788.
ABSTRACT
| FULL TEXT
Clinical and Molecular Study of 320 Children With Marfan Syndrome and Related Type I Fibrillinopathies in a Series of 1009 Probands With Pathogenic FBN1 Mutations
Faivre et al.
Pediatrics 2009;123:391-398.
ABSTRACT
| FULL TEXT
Evolving phenotype of Marfan's syndrome
Lipscomb et al.
Arch. Dis. Child. 1997;76:41-46.
ABSTRACT
| FULL TEXT
|