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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%.

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202 children

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.


202 children

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.


202 children

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.


202 children

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.


202 children

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.


202 children

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.


202 children

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.


202 children

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.


202 children

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.