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The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome

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The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome. / Mueller, Goetz C; Stark, Veronika; Steiner, Kristoffer; Weil, Jochen; von Kodolitsch, Yskert; Mir, Thomas S.

in: ACTA PAEDIATR, Jahrgang 102, Nr. 2, 02.2013, S. 84-89.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Mueller, GC, Stark, V, Steiner, K, Weil, J, von Kodolitsch, Y & Mir, TS 2013, 'The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome', ACTA PAEDIATR, Jg. 102, Nr. 2, S. 84-89. https://doi.org/10.1111/apa.12072

APA

Mueller, G. C., Stark, V., Steiner, K., Weil, J., von Kodolitsch, Y., & Mir, T. S. (2013). The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome. ACTA PAEDIATR, 102(2), 84-89. https://doi.org/10.1111/apa.12072

Vancouver

Mueller GC, Stark V, Steiner K, Weil J, von Kodolitsch Y, Mir TS. The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome. ACTA PAEDIATR. 2013 Feb;102(2):84-89. https://doi.org/10.1111/apa.12072

Bibtex

@article{5c233420066e40f8910a90f5f50d13a6,
title = "The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome",
abstract = "AIM: Due to age-dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long-term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified.METHODS AND RESULTS: Sixty paediatric patients with Marfan were subject to a standardized diagnostic programme. All clinical symptoms of the revised Ghent nosology were analysed concerning age at first clinical manifestation, prevalence and likelihood ratio for MFS. Symptoms with early onset, high prevalence and high positive likelihood ratio were identified and combined for a risk score called Kid-Short Marfan Score (Kid-SMS). Three risk categories for suspicion of Marfan syndrome were developed. Finally, the Kid-SMS was operated in 130 paediatric patients with suspected MFS. Kid-SMS identified significantly more suspected patients with Marfan compared with Ghent nosology, revised Ghent and genetics alone without oversensitivity.CONCLUSION: Whereas diagnosis of MFS in childhood is sophisticated, Kid-SMS is a useful tool for risk stratification of suspected paediatric patients with Marfan by easy executable diagnostics, especially for paediatricians and paediatric cardiologists.",
keywords = "Adolescent, Case-Control Studies, Child, Child, Preschool, Decision Support Techniques, Echocardiography, Female, Health Status Indicators, Humans, Likelihood Functions, Magnetic Resonance Imaging, Male, Marfan Syndrome/diagnosis, Risk Assessment",
author = "Mueller, {Goetz C} and Veronika Stark and Kristoffer Steiner and Jochen Weil and {von Kodolitsch}, Yskert and Mir, {Thomas S}",
note = "{\textcopyright}2012 The Author(s)/Acta Paediatrica {\textcopyright}2012 Foundation Acta Paediatrica.",
year = "2013",
month = feb,
doi = "10.1111/apa.12072",
language = "English",
volume = "102",
pages = "84--89",
journal = "ACTA PAEDIATR",
issn = "0803-5253",
publisher = "Wiley-Blackwell",
number = "2",

}

RIS

TY - JOUR

T1 - The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome

AU - Mueller, Goetz C

AU - Stark, Veronika

AU - Steiner, Kristoffer

AU - Weil, Jochen

AU - von Kodolitsch, Yskert

AU - Mir, Thomas S

N1 - ©2012 The Author(s)/Acta Paediatrica ©2012 Foundation Acta Paediatrica.

PY - 2013/2

Y1 - 2013/2

N2 - AIM: Due to age-dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long-term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified.METHODS AND RESULTS: Sixty paediatric patients with Marfan were subject to a standardized diagnostic programme. All clinical symptoms of the revised Ghent nosology were analysed concerning age at first clinical manifestation, prevalence and likelihood ratio for MFS. Symptoms with early onset, high prevalence and high positive likelihood ratio were identified and combined for a risk score called Kid-Short Marfan Score (Kid-SMS). Three risk categories for suspicion of Marfan syndrome were developed. Finally, the Kid-SMS was operated in 130 paediatric patients with suspected MFS. Kid-SMS identified significantly more suspected patients with Marfan compared with Ghent nosology, revised Ghent and genetics alone without oversensitivity.CONCLUSION: Whereas diagnosis of MFS in childhood is sophisticated, Kid-SMS is a useful tool for risk stratification of suspected paediatric patients with Marfan by easy executable diagnostics, especially for paediatricians and paediatric cardiologists.

AB - AIM: Due to age-dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long-term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified.METHODS AND RESULTS: Sixty paediatric patients with Marfan were subject to a standardized diagnostic programme. All clinical symptoms of the revised Ghent nosology were analysed concerning age at first clinical manifestation, prevalence and likelihood ratio for MFS. Symptoms with early onset, high prevalence and high positive likelihood ratio were identified and combined for a risk score called Kid-Short Marfan Score (Kid-SMS). Three risk categories for suspicion of Marfan syndrome were developed. Finally, the Kid-SMS was operated in 130 paediatric patients with suspected MFS. Kid-SMS identified significantly more suspected patients with Marfan compared with Ghent nosology, revised Ghent and genetics alone without oversensitivity.CONCLUSION: Whereas diagnosis of MFS in childhood is sophisticated, Kid-SMS is a useful tool for risk stratification of suspected paediatric patients with Marfan by easy executable diagnostics, especially for paediatricians and paediatric cardiologists.

KW - Adolescent

KW - Case-Control Studies

KW - Child

KW - Child, Preschool

KW - Decision Support Techniques

KW - Echocardiography

KW - Female

KW - Health Status Indicators

KW - Humans

KW - Likelihood Functions

KW - Magnetic Resonance Imaging

KW - Male

KW - Marfan Syndrome/diagnosis

KW - Risk Assessment

U2 - 10.1111/apa.12072

DO - 10.1111/apa.12072

M3 - SCORING: Journal article

C2 - 23110520

VL - 102

SP - 84

EP - 89

JO - ACTA PAEDIATR

JF - ACTA PAEDIATR

SN - 0803-5253

IS - 2

ER -