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Diseases 2015, 3(1), 24-33; doi:10.3390/diseases3010024

Kid-Short Marfan Score (Kid-SMS) Is a Useful Diagnostic Tool for Stratifying the Pre-Test Probability of Marfan Syndrome in Childhood

1
Clinic for Pediatric Cardiology, University Heart Center, Martinistr. 52, 20251 Hamburg, Germany
2
Clinic for Cardiology, University Heart Center, Martinistr. 52, 20251 Hamburg, Germany
*
Author to whom correspondence should be addressed.
Academic Editor: Peter Varga
Received: 12 October 2014 / Revised: 13 November 2014 / Accepted: 28 February 2015 / Published: 12 March 2015
(This article belongs to the Special Issue Marfan Syndrome: Recognition, Diagnosis and Managements)
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Abstract

Due to age dependent organ manifestation, diagnosis of Marfan syndrome (MFS) is a challenge, especially in childhood. It is important to identify children at risk of MFS as soon as possible to direct those to appropriate treatment but also to avoid stigmatization due to false diagnosis. We published the Kid-Short Marfan Score (Kid-SMS) in 2012 to stratify the pre-test probability of MFS in childhood. Hence we now evaluate the predictive performance of Kid-SMS in a new cohort of children. We prospectively investigated 106 patients who were suspected of having MFS. At baseline, children were examined according to Kid-SMS. At baseline and follow-up visit, diagnosis of MFS was established or rejected using standard current diagnostic criteria according to the revised Ghent Criteria (Ghent-2). At baseline 43 patients were identified with a risk of MFS according to Kid-SMS whereas 21 patients had Ghent-2 diagnosis of MFS. Sensitivity was 100%, specificity 77%, negative predictive value 100% and Likelihood ratio of Kid-SMS 4.3. During follow-up period, three other patients with a stratified risk for MFS were diagnosed according to Ghent-2. We confirm very good predictive performance of Kid-SMS with excellent sensitivity and negative predictive value but restricted specificity. Kid-SMS avoids stigmatization due to diagnosis of MFS and thus restriction to quality of life. Especially outpatient pediatricians and pediatric cardiologists can use it for primary assessment. View Full-Text
Keywords: Marfan syndrome; revised Ghent Criteria; Kid-SMS; diagnosis; childhood; quality of life Marfan syndrome; revised Ghent Criteria; Kid-SMS; diagnosis; childhood; quality of life
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Stark, V.C.; Arndt, F.; Harring, G.; von Kodolitsch, Y.; Kozlik-Feldmann, R.; Mueller, G.C.; Steiner, K.J.; Mir, T.S. Kid-Short Marfan Score (Kid-SMS) Is a Useful Diagnostic Tool for Stratifying the Pre-Test Probability of Marfan Syndrome in Childhood. Diseases 2015, 3, 24-33.

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