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| Fältnamn | Indikatorer | Metadata |
|---|---|---|
| 000 | 03479naa a2200361 4500 | |
| 001 | oai:lup.lub.lu.se:540b68b8-a0ae-442a-9b13-06df5f543c6f | |
| 003 | SwePub | |
| 008 | 160401s2007 | |||||||||||000 ||eng| | |
| 024 | 7 | a https://lup.lub.lu.se/record/6742182 URI |
| 024 | 7 | a https://doi.org/10.1093/eurheartj/ehl4572 DOI |
| 040 | a (SwePub)lu | |
| 041 | a engb eng | |
| 042 | 9 SwePub | |
| 072 | 7 | a art2 swepub-publicationtype |
| 072 | 7 | a ref2 swepub-contenttype |
| 100 | 1 | a Aburawi, Elhadiu Lund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine4 aut0 (Swepub:lu)pedi-eab |
| 245 | 1 0 | a Relation of aortic root dilatation and age in Marfan's syndrome |
| 264 | c 2007-01-11 | |
| 264 | 1 | b Oxford University Press (OUP),c 2007 |
| 520 | a Aims The main aim of this study was to describe the age at which pathological aortic root dilation occurs in patients with Marfan's syndrome (MFS). Methods and results A total of 160 patients with MFS attending a regional cardiac centre were reviewed retrospectively. Dilation of the ascending aorta was diagnosed by comparing the maximum aortic sinus measurement with control data from the literature. We employed a Kaplan-Meier survival curve to estimate the age at which dilatation occurs. The mean age of the total group at presentation was 15.5 years (range 1.5-40 years). Skeletal abnormalities were present in 95%. Eye involvement was found in 18%. In the 115/160 patients with an abnormal aortic root, 78/115 (68%) developed aortic root dilatation before 19 years of age. From the Kaplan-Meier curve, it can be estimated that about 35% of the patients have aortic root dilatation already at the age of 5 years and 70% before the age of 20 years, and at least 80% by 40 years. There were 31 patients with normal aortic root when first seen but 24/31 (77%) developed aortic root dilatation before the age of 19 years and 7/31 (22.6%) after 19 years of age. Of those (seven patients) who developed new pathological aortic root dilatation after age 19 years, the age range was between 21 and 40 years with a mean of 27 years. Overall, 13 patients (8%) had surgery for aortic root replacement. Conclusion Aortic root dilatation develops early in MFS and was present in 35% by the age of 5 years and 68% by 19 years. Even though new aortic root dilation is relatively rare, it is not possible to safely discharge patients with MFS as about one-third of the patients in our series who developed new pathological aortic root dilation did so after the age of 19 years. | |
| 650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Kardiologi0 (SwePub)302062 hsv//swe |
| 650 | 7 | a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Cardiac and Cardiovascular Systems0 (SwePub)302062 hsv//eng |
| 653 | a cohort study | |
| 653 | a puberty | |
| 653 | a Marfan's syndrome | |
| 653 | a aorta root | |
| 700 | 1 | a O'Sullivan, John4 aut |
| 710 | 2 | a Pediatrik, Lundb Sektion V4 org |
| 773 | 0 | t European Heart Journald : Oxford University Press (OUP)g 28:3, s. 376-379q 28:3<376-379x 1522-9645x 0195-668X |
| 856 | 4 | u http://dx.doi.org/10.1093/eurheartj/ehl457y FULLTEXT |
| 856 | 4 | u https://academic.oup.com/eurheartj/article-pdf/28/3/376/9627381/ehl457.pdf |
| 856 | 4 8 | u https://lup.lub.lu.se/record/674218 |
| 856 | 4 8 | u https://doi.org/10.1093/eurheartj/ehl457 |
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