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Patients diagnosed with Marfan syndrome based on the revised Ghent criteria
| Criteria 1 | Criteria 2 | n | Explanation |
|---|---|---|---|
| Aorta diametera | Ectopia lentis | 2 | No FBN1variant |
| Aorta diametera | FBN1 variants | 27 | All have ectopia lentis |
| Aorta diameter a | Systemic score > 7 | 13 | No FBN1variant |
| Family history | Systemic score > 7 | 1 | No FBN1variant |
| Total | 43 | Marfan syndrome |
aZ-score ≥ 3 in individuals < 20 years
