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Discriminating Value between MFS and Non-MFS Children in Different Age Groups

This study examines clinical features as first and second intention criteria for identifying children with Marfan syndrome (MFS) based on age groups. Parameters such as height, arm span to height ratio, hypermobility, pectus deformity, thumb sign, wrist sign, scoliosis angle, and striae are taken into account. Results show varying discriminatory values between MFS and non-MFS children.

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Discriminating Value between MFS and Non-MFS Children in Different Age Groups

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  1. Children between 0 and 6 years old First intention criteria 202 children Height <2.5SD Height ≥2.5SD MFS (90/139) 65% Non MFS (50/63) 79% AS/H < 0.98 AS/H ≥ 0.98 Non MFS (16/23) 70% MFS (83/116) 72% 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.

  2. Children between 0 and 6 years old First and second intention criteria 202 children No Ectopia lentis Ectopia lentis Non MFS (95/134) 71% MFS (64/68) 94% AD ≥ 3.3SD AD<3.3SD Non MFS (94/115) 82% MFS (18/19) 95% AD<1.3SD AD>1.3SD Non MFS (953/56) 95% Non MFS (41/59) 69% Height ≥2.8SD Height <2.8SD Non MFS (24/27) 89% Non MFS (17/32) 53% AS/H >0.97 AS/H <0.97 Non MFS (6/8) 75% MFS (13/24) 54% Height ≥4.6SD Height <4.6SD Non MFS (6/10) 60% MFS (9/14) 64% 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.

  3. Children between 7 and 9 years old First intention criteria 169 children Height ≥2.3SD Height <2.3SD MFS (89/115) 77% Non MFS (42/54) 78% No pectus deformity Pectus deformity Non MFS (35/37) 95% MFS (10/17) 59% MFS (37/57) 65% MFS (52/58) 90% 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.

  4. Children between 7 and 9 years old First and second intention criteria 202 children AD ≥ 2.2SD AD< 2.2SD Non MFS (60/71) 84% MFS (90/98) 92% Ectopia lentis No ectopia lentis MFS (9/11) 82% Non MFS (58/60) 97% 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.

  5. Children between 10 and 14 years old First intention criteria 294 children Height <3.2SD Height ≥3.2SD MFS (82/96) 85% Non MFS (143/198) 72% No wrist sign Wrist sign Non MFS (119/139) 86% MFS (35/59) 59% 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.

  6. Children between 10 and 14 years old First and second intention criteria 294 children Ectopia lentis No ectopia lentis Non MFS (150/196) 76% MFS (91/98) 93% AD< 3.9SD AD ≥ 3.9SD Non MFS (147/169) 87% MFS (24/27) 89% AD< 1.6SD AD≥1.6SD Non MFS (28/45) 62% Non MFS (119/124) 96% Heignt<2.6SD Heignt≥2.6SD Non MFS (23/28) 82% MFS (12/17) 71% 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.

  7. Children between 15 and 17 years old First intention criteria 308 children Height <2.5SD Height ≥2.5SD MFS (68/102) 67% Non MFS (160/206) 78% No striae Striae No striae Striae Non MFS (105/116) 91% Non MFS (55/90) 61% Non MFS (16/23) 70% MFS (61/79) 77% Scoliosis angle≥12 Scoliosis angle<12 AS/H<1 AS/H≥1 Non MFS (48/73) 66% MFS (10/17) 59% MFS (21/34) 62% MFS (40/45) 89% AS/H≥1 Scoliosis angle<5.2 Scoliosis angle≥5.2 AS/H<1 Non MFS (38/44) 86% Non MFS (10/19) 53% Non MFS (8/11) 73% MFS (18/23) 78% No wrist sign Wrist sign Non MFS (8/11) 73% MFS (6/8) 75% 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.

  8. Children between 15 and 17 years old First and second intention criteria 308 children AD<2.4SD AD≥2.4 MFS (84/95) 88% Non MFS (183/213) 86% Scoliosis angle≥3.8 Scoliosis angle<3.8 No ectopia lentis Ectopia lentis Non MFS (176/193) 92% MFS (13/20) 65% MFS (8/14) 57% MFS (76/81) 94% No valve prolaps AS/H<1 AS/H≥1 Valve prolapse Non MFS (7/14) 50% MFS (6/6) 100% Non MFS (6/9) 67% MFS (5/5) 100% AD≥0.85 AD<0.85 Non MFS (6/7) 86% MFS (6/7) 86% 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.

  9. 0-6 years 7-9 years 10-14 years 15-17 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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