지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2015.1
- 수록면
- 1,260 - 1,265 (6page)
이용수
초록· 키워드
오류제보하기
A new CT-based diagnostic method of protrusio acetabuli (PA) was introduced. However, prevalence of PA by this method and correlation between PA and other manifestations of Marfan syndrome (MFS) is unknown in Korean MFS patients. This study aimed to investigate the prevalence of PA diagnosed by a CT-based method in Korean patients with MFS, the association of PA with other manifestations of MFS, and the contribution of PA to MFS diagnosis. We retrospectively reviewed the records of 146 MFS patients with the presence of a causative FBN1 mutation and 146 age- and sex-matched controls from a single tertiary care center. All MFS patients underwent a complete assessment of criteria based on the revised Ghent nosology. PA was assessed quantitatively using a CT-based circle-wall distance (CWD) method. PA was diagnosed in 77.4% of patients in the MFS group and in 11.0% of the control group. CWD was significantly different between the two groups (1.50 mm vs. -0.64 mm, P < 0.001). The presence of PA did not correlate with the presence of ectopia lentis, aortic root diameter, or history of aortic dissection. The presence of PA did not have a significant impact on the final diagnosis of MFS. Even though the presence of PA does not related to the cardinal clinical features of MFS or influence MFS diagnosis, its presence may be helpful for the suspicion of MFS when aortic dissection or aneurysm is found on CT angiography of the aorta because of the high frequency of PA in MFS patients.
목차
등록된 정보가 없습니다.
참고문헌 (18)
참고문헌 신청
Koh KK / 1987 / Cardiovascular manifestations of marfan syndrome / Korean Circ J 17:777~782
Loeys BL / 2010 / The revised Ghent nosology for the Marfan syndrome / J Med Genet 47:476~485
De Paepe A / 1996 / Revised diagnostic criteria for the Marfan syndrome / Am J Med Genet 62:417~426
McBride MT / 2001 / Protrusio acetabuli : diagnosis and treatment / J Am Acad Orthop Surg 9:79~88
Steel HH / 1996 / Protrusio acetabuli : its occurrence in the completely expressed Marfan syndrome and its musculoskeletal component and a procedure to arrest the course of protrusion in the growing pelvis / J Pediatr Orthop 16:704~718
Loeys BL / 2010 / The revised Ghent nosology for the Marfan syndrome / J Med Genet 47:476~485
De Paepe A / 1996 / Revised diagnostic criteria for the Marfan syndrome / Am J Med Genet 62:417~426
McBride MT / 2001 / Protrusio acetabuli : diagnosis and treatment / J Am Acad Orthop Surg 9:79~88
Steel HH / 1996 / Protrusio acetabuli : its occurrence in the completely expressed Marfan syndrome and its musculoskeletal component and a procedure to arrest the course of protrusion in the growing pelvis / J Pediatr Orthop 16:704~718
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