• Title/Summary/Keyword: Willams syndrome

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Diffuse Supravalvar Aortic Stenosis Associated with Congenital Anomaly of the Aortic Valve(Williams Syndrome) -1 case report- (선천성 대동맥 판막 이상과 무명동맥 협착이 동반된 미만성 대동맥 판막상부 협착증 치험 1례)

  • 김수철;전순호
    • Journal of Chest Surgery
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    • v.33 no.9
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    • pp.748-751
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    • 2000
  • The diffuse form of supravalvar aortic stenosis represents a surgical challenge when the ascending aorta, aortic arch, proximal descending thoracic aorta and arch arteries are involved. It can be treated by a variety of surgical approaches. We report a case of severe diffuse supravalvar aortic stenosis combined with an aortic valve anomaly and occlusion of the right coronary artery ostium in a 14-year-old boy with Williams syndrome. We enlarged the aortic root(Nick's procedure), ascending aorta, aortic arch, proximal descending thoracic aorta, and innominate artery with patches and replaced aortic valve with 19 mm St. Jude valve. Deep hypothermic circulatory arrest and retrograde cerebral perfusion were used during repair of the arch and arch artery.

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SCISSOR BITE CORRECTION IN PATIENTS WITH WILLAMS SYNDROME: A CASE REPORT (Williams Syndrome 환자에서의 scissor bite correction: 증례보고)

  • Ji, Eun-Hye;Choi, Hyung-Jun;Kim, Seong-Oh;Son, Heung-Kyu;Lee, Jae-Ho
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.7 no.1
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    • pp.21-24
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    • 2011
  • Williams syndrome(WS) is a congenital disorder caused by a deletion of the Elastin gene and other contiguous genes at chromosome 7. Patients with WS are at a high risk of dental caries, and they also have a higher frequency of dental malocclusion compared to normal children. Malocclusion occurs in 85% of individuals with WS, which results from combined causes, such as tongue thrust, hypotonia, and connective tissue abnormality. An 11 year-old girl with WS presented scissor bite on the lower right second premolar and the first molar, and she complained of difficulty in chewing. Active lingual arch was used instead of removable appliance, considering the patient's cooperation ability. Unilateral posterior scissor bite was corrected in 7 months. Although patients with WS are sociable and friendly, dental treatment can be a fearful experience for them. Efforts to build rapport with the patients with WS resulted in improved relationship between the doctor and patient, and desired outcome of dental treatment was achieved with patient's improved cooperation.

Study on the Movement of New Qi-gong "WuQinXi" Exercise for Lumbar Spinal Disease : Based on 20 Mode, 30 Mode, 40 Mode (요추 질환에 대한 신기공 오금희의 동작연구 - 20식, 30식, 40식을 중심으로 -)

  • You, Kyung Gon;Kwon, Young Dal;Jeong, Hyun Woo
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.28 no.2
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    • pp.129-136
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    • 2014
  • The "WuQinXi" exercise, one of the medical Qi-gongs, is an exercise maximizing human's self healing power and has been confirmed to be effective significantly at several modern researches. There are many exercise therapies in western medcine, such as Willams's flexion exercise, Mckenzie's extension exercise, vertebral stabilization exercise and so on. However, there isn't a special exercise therapy which can be applied for medical practice in oriental medicine. So we selected 24 motions which are related with lumbar movements from 3 type "WuQinXi" exercises ; 20 mode, 30 mode, and 40 mode. And then, we classified them according to lumbar movements as flexion, extention, lateral bending and rotation, and also functions as stabilization and rubbing. Next, with these classifications, we assorted them by kinds of lumbar spinal disease as HIVD(herniation of intervertebral disc), spinal stenosis, spondylolysis and spondylolisthesis, facet joint syndrome, compression fracture and spondylosis. We expect that "WuQinXi" exercise be a exercise therapy for lumbar spinal disease at an oriental medical clinic in this way. Oriental medical doctors will be able to teach easily patients "WuQinXi" exercise's motions at clinic, depending on kinds of lumbar spinal disease each patient suffers from. We plan to study the effect of "WuQinXi" exercise by comparing patients who do the "WuQinXi" exercise with the patients who do the western medical exercise therapy.