- True Aneurysm of the Common Coronary Button in a Marfan Patient with an Anomalous Right Coronary Artery after a Bentall Procedure: A Case Report
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Jo JJ, Kim YS, Kim GJ, Kim JH
- KMID: 2530408
- J Chest Surg.
- 2022 Jun;55(3):243-245.
- doi: 10.5090/jcs.22.008
True aneurysms of the coronary artery after aortic root replacement in Marfan syndrome patients are very rare. An anomalous origin of the right coronary artery (RCA) from the left sinus... -
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- Severe laryngomalacia in a 1-month infant with Marfan syndrome
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Moon SJ, Yang HC, Song ES, Choi YY, Lee E
- KMID: 2512580
- Allergy Asthma Respir Dis.
- 2021 Jan;9(1):42-45.
- doi: 10.4168/aard.2021.9.1.42
Severe respiratory difficulty can be more prominent, when combined with severe pectus excavatum and cardiovascular manifestations in infants with Marfan syndrome. There have been few reports on severe laryngomalacia as... -
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- Weak Ligaments and Sloping Joints: A New Hypothesis for Development of Congenital Atlantoaxial Dislocation and Basilar Invagination
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Chauhan AK, Chandra PS, Goyal N, Chowdhury MR, Banerjee J, Tripathi M, Kabra M
- KMID: 2510918
- Neurospine.
- 2020 Dec;17(4):843-856.
- doi: 10.14245/ns.2040434.217
Objective: Developmental bony craniovertebral junction (CVJ) anomalies seem to have a genetic basis and also abnormal joint morphology causing atlantoaxial dislocation (AAD) and basilar invagination (BI). Methods: DNA extracted polymerase chain... -
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- Shprintzen–Goldberg syndrome with a novel missense mutation of SKI in a 6-month-old boy
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Jeon MJ, Park SG, Kim MJ, Lim BC, Kim KJ, Chae JH, Kim SY
- KMID: 2503611
- J Genet Med.
- 2020 Jun;17(1):43-46.
- doi: 10.5734/JGM.2020.17.1.43
The Shprintzen–Goldberg syndrome (SGS) is an extremely rare genetic disorder caused by heterozygous variant in SKI . SGS is characterized by neurodevelopmental impairment with skeletal anomaly. Recognition of SGS is... -
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- Minimally Invasive Mitral Valve Repair in a Woman with Marfan Syndrome and Type B Dissection
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Lim MH, Je HG, Lee SK
- KMID: 2409715
- Korean J Thorac Cardiovasc Surg.
- 2018 Feb;51(1):61-63.
- doi: 10.5090/kjtcs.2018.51.1.61
We report the case of a patient with mitral regurgitation complicated by type B dissection and Marfan syndrome (MFS) who was managed successfully with minimally invasive mitral valve repair. Without... -
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- Sequential Hybrid Repair of Aorta and Bilateral Common Iliac Arteries Secondary to Chronic Aortic Dissection with Extensive Aneurysmal Degeneration in a Marfan Patient
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Hinojosa C, Anaya-Ayala JE, Laparra-Escareno H, Lizola R, Torres-Machorro A
- KMID: 2396191
- Vasc Spec Int.
- 2017 Sep;33(3):112-116.
- doi: 10.5758/vsi.2017.33.3.112
Marfan syndrome is a connective tissue disorder associated with aortic dissection, aneurysmal degeneration and rupture. These cardiovascular complications represent the main cause of mortality, therefore repair is indicated. We present... -
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- The Prevalence of Marfan Syndrome in Korea
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Jang SY, Seo SR, Park SW, Kim DK
- KMID: 2371440
- J Korean Med Sci.
- 2017 Apr;32(4):576-580.
- doi: 10.3346/jkms.2017.32.4.576
The aim of this study was to assess the prevalence of Marfan syndrome (MFS) in Korean adults. Data were collected from the National Health Insurance Service in Korea from 2006... -
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- Atypical Neonatal Marfan Syndrome with p.Glu1073Lys Mutation of FBN1: the First Case in Korea
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Heo JS, Song JY, Choi EY, Kim EH, Kim JH, Park SE, Jeon JH
- KMID: 2417577
- J Korean Med Sci.
- 2017 Jan;32(1):1-3.
- doi: 10.3346/jkms.2017.32.1.1
Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe... -
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- Femur Neck Fracture in a Young Marfan Syndrome Patient
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Kwon YU, Kong GM, Park JH
- KMID: 2364706
- Hip Pelvis.
- 2016 Dec;28(4):264-268.
- doi: 10.5371/hp.2016.28.4.264
Marfan syndrome is an autosomal dominant and could decrease bone mineral density. So patients with Marfan syndrome could vulnerable to trauma in old ages. We present the first report, to... -
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- Clinical Characteristics of Marfan Syndrome in Korea
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Lim AY, Song JS, Kim EK, Jang SY, Chung TY, Choi SH, Sung K, Huh J, Kang IS, Choe YH, Ki CS, Kim DK
- KMID: 2355461
- Korean Circ J.
- 2016 Nov;46(6):841-845.
- doi: 10.4070/kcj.2016.46.6.841
BACKGROUND AND OBJECTIVES: Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a highly variable clinical spectrum. However, there are limited data available on the clinical... -
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- A novel FBN1 gene mutation associated with early-onset pneumothorax in Marfan syndrome
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Park MJ, Lee DH, Shin YL, Hong YH
- KMID: 2327702
- J Genet Med.
- 2016 Jun;13(1):41-45.
- doi: 10.5734/JGM.2016.13.1.41
Marfan syndrome (MFS) is an inherited connective tissue disorder with a mutation in the fibrillin-1 (FBN1) gene. Fibrillin is a major building block of microfibrils, which constitute the structural component... -
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- Estrogen-mediated Height Control in Girls with Marfan Syndrome
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Lee DY, Hyun HS, Huh R, Jin DK, Kim DK, Yoon BK, Choi D
- KMID: 2360053
- J Korean Med Sci.
- 2016 Feb;31(2):275-279.
- doi: 10.3346/jkms.2016.31.2.275
This study evaluated the efficacy of a stepwise regimen of estradiol valerate for height control in girls with Marfan syndrome. Eight girls with Marfan syndrome who had completed estrogen treatment... -
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- Infantile Marfan syndrome in a Korean tertiary referral center
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Seo YJ, Lee KE, Kim GB, Kwon BS, Bae EJ, Noh CI
- KMID: 2155924
- Korean J Pediatr.
- 2016 Feb;59(2):59-64.
- doi: 10.3345/kjp.2016.59.2.59
PURPOSE: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS... -
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- Stanford type A aortic dissection in a patient with Marfan syndrome during pregnancy: a case report
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Kim WH, Bae J, Choi SW, Lee JH, Kim CS, Cho HS, Lee SM
- KMID: 2152865
- Korean J Anesthesiol.
- 2016 Feb;69(1):76-79.
- doi: 10.4097/kjae.2016.69.1.76
Aortic dissection during pregnancy is a devastating event for both the pregnant woman and the baby. We report a case of acute aortic dissection (Stanford type A) in a pregnant... -
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- The Beneficial Effect of Renin-Angiotensin-Aldosterone System Blockade in Marfan Syndrome Patients after Aortic Root Replacement
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Lee SJ, Oh J, Ko YG, Lee S, Chang BC, Lee DY, Kwak YR, Choi D
- KMID: 2466355
- Yonsei Med J.
- 2016 Jan;57(1):81-87.
- doi: 10.3349/ymj.2016.57.1.81
PURPOSE: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy. MATERIALS AND METHODS: We reviewed Marfan syndrome (MFS) patients... -
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- Analysis of Protrusio Acetabuli Using a CT-based Diagnostic Method in Korean Patients with Marfan Syndrome: Prevalence and Association with Other Manifestations
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Chun KJ, Yang JH, Jang SY, Lee SH, Gwag HB, Chung TY, Huh J, Ki CS, Sung K, Choi SH, Kim SM, Choe YH, Kim DK
- KMID: 2344150
- J Korean Med Sci.
- 2015 Sep;30(9):1260-1265.
- doi: 10.3346/jkms.2015.30.9.1260
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... -
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- Free Vascularized Fibular Strut Autografts to the Lumbar Spine in Complex Revision Surgery: A Report of Two Cases
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Saltzman B, Levy DM, Vakhshori , DeWald CJ
- KMID: 2288347
- Korean J Spine.
- 2015 Sep;12(3):185-189.
- doi: 10.14245/kjs.2015.12.3.185
This case report presents two patients who underwent fibular strut grafting for complex revisions of previous lumbar spine arthrodeses. A case review of the Electronic Medical Record at the index... -
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- Disease-specific Growth Charts of Marfan Syndrome Patients in Korea
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Kwun Y, Kim SJ, Lee J, Isojima T, Choi DS, Kim DK, Huh J, Kang IS, Chang M, Cho SY, Sohn YB, Park SW, Jin DK
- KMID: 2164476
- J Korean Med Sci.
- 2015 Jul;30(7):911-916.
- doi: 10.3346/jkms.2015.30.7.911
Patients with Marfan syndrome (MFS) presents with primary skeletal manifestations such as tall stature, chest wall abnormality, and scoliosis. These primary skeletal manifestations affect the growth pattern in MFS. Therefore,... -
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- Surgical Management of Mitral Regurgitation in Patients with Marfan Syndrome during Infancy and Early Childhood
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Kim ER, Kim WH, Choi ES, Cho S, Jang WS, Kim YJ
- KMID: 2382220
- Korean J Thorac Cardiovasc Surg.
- 2015 Feb;48(1):7-12.
- doi: 10.5090/kjtcs.2015.48.1.7
BACKGROUND: Mitral regurgitation is one of the leading causes of cardiovascular morbidity in pediatric patients with Marfan syndrome. The purpose of this study was to contribute to determining the appropriate... -
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- Multiple Aortic Operations in Loeys-Dietz Syndrome: Report of 2 Cases
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Na KJ, Park KH
- KMID: 2382207
- Korean J Thorac Cardiovasc Surg.
- 2014 Dec;47(6):536-540.
- doi: 10.5090/kjtcs.2014.47.6.536
Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often mis-diagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology.... -
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