вот эволюция симптомов с дошкольного возраста до взросления: рост, эктопия хрусталика и расширение корня аорты - главные дискриминанты, но последие два глазом не видны
Prevalence of skeletal features changed with aging: prevalence of
pectus deformity increased from 43% at 0-6 years to 62% at 15-17 years,
wrist signs increased from 28 to 67%, and
scoliosis increased from 16 to 59%.
Hypermobility decreased from 67 to 47% and
pes planus decreased from 73 to 65%.
Striae increased from 2 to 84%.
Prevalence of ectopia lentis remained stable, varying from 66 to 72%, similar to aortic root dilatation (varying from 75 to 80%).
Aortic root dilatation remained stable during follow-up in this population receiving β-blocker therapy.
When comparing Marfan syndrome children with non-Marfan syndrome children,
height appeared to be a simple and discriminant criterion when it was >3.3 SD above the mean.
Ectopia lentis and aortic dilatation were both similarly discriminating.
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Искренне,
Вадим Валерьевич.
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