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

• DOI: 10.1111/apa.12072
• Publication Date: 2012-10-30T08:52:21Z
• Authors: Goetz C Mueller; Veronika Stark; Kristoffer Steiner; Jochen Weil; Yskert von Kodoli...; Thomas S Mir
• Keywords: Male; Likelihood Functions; Adolescent; Magnetic Resonance Imaging; Risk Assessment; IGMD 1: Functional imaging; Decision Support Techniques; Marfan Syndrome; 3. Good health; 03 medical and health sciences; 0302 clinical medicine; Echocardiography; Case-Control Studies; Child, Preschool; Health Status Indicators; Humans; Female; Child

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