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202 children MFS (90/139) 65% Non MFS (50/63) 79% Height <2.5SD Height 2.5SD Non MFS (16/23) 70% MFS (83/116) 72% AS/H < 0.98AS/H 0.98 Children between 0 and 6 years old First intention criteria Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters are for first intention : Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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202 children MFS (64/68) 94% Non MFS (95/134) 71% No Ectopia lentis Ectopia lentis Non MFS (94/115) 82% MFS (18/19) 95% AD<3.3SD AD 3.3SD Children between 0 and 6 years old First and second intention criteria Non MFS (41/59) 69% Non MFS (24/27) 89% Non MFS (953/56) 95% Non MFS (17/32) 53% AD<1.3SDAD>1.3SD Height <2.8SD Height 2.8SD MFS (13/24) 54% Non MFS (6/8) 75% AS/H <0.97 AS/H >0.97 MFS (9/14) 64% Non MFS (6/10) 60% Height 4.6SD Height <4.6SD Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters for first and second intention criteria are : Ectopia lentis, aortic diameter (SD), Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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169 children MFS (89/115) 77% Non MFS (42/54) 78% Height <2.3SD Height 2.3SD MFS (37/57) 65% MFS (52/58) 90% No pectus deformity Pectus deformity Children between 7 and 9 years old First intention criteria Non MFS (35/37) 95% MFS (10/17) 59% MFS (24/43) 56% MFS (13/14) 93% Non MFS (16/29) 55% MFS (11/14) 79% Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters are for first intention : Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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202 children MFS (90/98) 92% Non MFS (60/71) 84% AD< 2.2SD AD 2.2SD Non MFS (58/60) 97% MFS (9/11) 82% No ectopia lentis Ectopia lentis Children between 7 and 9 years old First and second intention criteria Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters for first and second intention criteria are : Ectopia lentis, aortic diameter (SD), Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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294 children MFS (82/96) 85% Non MFS (143/198) 72% Height <3.2SDHeight 3.2SD Non MFS (119/139) 86% MFS (35/59) 59% No wrist sign Wrist sign Children between 10 and 14 years old First intention criteria Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters are for first intention : Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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294 children MFS (91/98) 93% Non MFS (150/196) 76% No ectopia lentis Ectopia lentis Non MFS (147/169) 87% MFS (24/27) 89% AD< 3.9SD AD 3.9SD Children between 10 and 14 years old First and second intention criteria Non MFS (119/124) 96% Non MFS (28/45) 62% AD< 1.6SD AD1.6SD MFS (12/17) 71% Non MFS (23/28) 82% Heignt<2.6SDHeignt2.6SD Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters for first and second intention criteria are : Ectopia lentis, aortic diameter (SD), Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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308 children MFS (68/102) 67% Non MFS (160/206) 78% Height <2.5SDHeight 2.5SD Non MFS (105/116) 91% Non MFS (55/90) 61% No striaeStriae Children between 15 and 17 years old First intention criteria Non MFS (16/23) 70% MFS (61/79) 77% No striaeStriae MFS (40/45) 89% MFS (21/34) 62% AS/H1 AS/H<1 MFS (18/23) 78% Non MFS (8/11) 73% Scoliosis angle<5.2 Scoliosis angle5.2 Non MFS (48/73) 66% MFS (10/17) 59% Scoliosis angle<12 Scoliosis angle12 Non MFS (38/44) 86% Non MFS (10/19) 53% AS/H<1 AS/H1 Non MFS (8/11) 73% MFS (6/8) 75% Wrist sign No wrist sign Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters are for first intention : Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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Children between 15 and 17 years old First and second intention criteria 308 children MFS (84/95) 88% Non MFS (183/213) 86% AD<2.4SDAD2.4 Non MFS (176/193) 92% MFS (13/20) 65% No ectopia lentis Ectopia lentis MFS (8/14) 57% MFS (76/81) 94% Scoliosis angle<3.8 MFS (5/5) 100% Non MFS (6/9) 67% No valve prolaps Valve prolapse Non MFS (7/14) 50% MFS (6/6) 100% AS/H<1AS/H1 Non MFS (6/7) 86% MFS (6/7) 86% AD<0.85 Scoliosis angle3.8 AD0.85 Recursive partitionning of discriminating value between MFS and non MFS children of clinical features in different age groups for general practitionners at first consultation (first intention criterion) or for specialists (second intention criterion). The taken into account parameters for first and second intention criteria are : Ectopia lentis, aortic diameter (SD), Height (SD), arm span to height ratio (AS/H), hypermobility, pectus deformity, thumb sign, wrist sign, angle of scoliosis (°), striae.

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0-6 years7-9 years 10-14 years15-17 years Comparison of prevalence of different features in the Marfan and non Marfan groups in each class of age. The percentages are provided for each feature and group.

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