• 제목/요약/키워드: Multiple Defect

검색결과 270건 처리시간 0.116초

Pierre Robin sequence with severe scoliosis in an adult: A case report of clinical and radiological features

  • Kim, Jae-Jun;Choi, Dong-Soon;Jang, Insan;Cha, Bong-Kuen;Park, In-Woo
    • Imaging Science in Dentistry
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    • 제49권4호
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    • pp.323-329
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    • 2019
  • Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. PRS does not have a single pathogenesis, but rather is associated with multiple syndromes. This report presents the case of a 35-year-old woman with PRS and scoliosis. Among the syndromes related to PRS, cerebro-costo-mandibular syndrome (CCMS), which is characterized by posterior rib gap defects and vertebral anomalies, was suspected in this patient. However, no posterior rib gap defect was detected on radiological examinations. Although over 80 cases of CCMS have been reported to date, few cases of PRS with scoliosis alone have been reported. Therefore, this report demonstrated the clinical, radiological, and cephalometric characteristics of an adult patient with PRS and scoliosis, but without rib anomalies.

Sorting of the Human Folate Receptor in MDCK Cells

  • Kim, Chong-Ho;Park, Young-Soon;Chung, Koong-Nah;Elwood, P.C.
    • BMB Reports
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    • 제37권3호
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    • pp.362-369
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    • 2004
  • The human folate receptor (hFR) is a glycosylphosphatidylinositol (GPI) linked plasma membrane protein that mediates delivery of folates into cells. We studied the sorting of the hFR using transfection of the hFR cDNA into MDCK cells. MDCK cells are polarized epithelial cells that preferentially sort GPI-linked proteins to their apical membrane. Unlike other GPI-tailed proteins, we found that in MDCK cells, hFR is functional on both the apical and basolateral surfaces. We verified that the same hFR cDNA that transfected into CHO cells produces the hFR protein that is GPI-linked. We also measured the hFR expression on the plasma membrane of type III paroxysmal nocturnal hemoglobinuria (PNH) human erythrocytes. PNH is a disease that is characterized by the inability of cells to express membrane proteins requiring a GPI anchor. Despite this defect, and different from other GPI-tailed proteins, we found similar levels of hFR in normal and type III PNH human erythrocytes. The results suggest the hypothesis that there may be multiple mechanisms for targeting hFR to the plasma membrane.

Influence of Heat-Treatment on the Adhesive Strength between a Micro-Sized Bonded Component and a Silicon Substrate under Bend and Shear Loading Conditions

  • Ishiyama, Chiemi
    • 비파괴검사학회지
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    • 제32권2호
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    • pp.122-130
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    • 2012
  • Adhesive bend and shear tests of micro-sized bonded component have been performed to clarify the relationship between effects of heat-treatment on the adhesive strength and the bonded specimen shape using Weibull analysis. Multiple micro-sized SU-8 columns with four different diameters were fabricated on a Si substrate under the same fabrication condition. Heat-treatment can improve both of the adhesive bend and shear strength. The improvement rate of the adhesive shear strength is much larger than that of the adhesive bend strength, because the residual stress, which must change by heat-treatment, should effect more strongly on the shear loading. In case of bend type test, the adhesive bend strength in the smaller diameters (50 and $75\;{\mu}m$) widely vary, because the critical size of the natural defect (micro-crack) should vary more widely in the smaller diameters. In contrast, in case of shear type test, the adhesive shear strengths in each diameter of the columns little vary. This suggests that the size of the natural defects may not strongly influence on the adhesive shear strength. All the result suggests that both of the adhesive bend and shear strengths should be complicatedly affected by heat-treatment and the bonded columnar diameter.

개와 사람의 선천성 심장 전도장애에 대한 분자 유전학적 이해 (Molecular Genetics of Inherited Cardiac Conduction Defects in Humans and Dogs)

  • Hyun, Changbaig
    • 한국임상수의학회지
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    • 제21권2호
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    • pp.219-228
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    • 2004
  • 심장 전도계에 관련된 심장 질환은 심장 전도계의 원발성 결함이나 형태학적 심장 질환 및 약물 중독 같은 속발성 원인에 의해 발생한다. 특히 선천성 심장 전도 장애의 경우, 이온 채널, 이온 채널에 관련한 세포질성 단백질, gap-junction 단백질, transcription factors (심장 발생에 관련된 유전자들) 및 kinase subunit을 전사하는 유전자의 돌연변이가 원발성 선천성 심장 전도장애의 원인으로 밝혀지고 있다. 사람의 부정맥 발생에 관련된 발병기전에 커다란 진보가 이루어지고 있음에도 불구하고, 개의 경우, 저먼 세퍼드의 급사 증후군 (sudden death syndrome)을 제외한 다른 전도 장애에 대한 연구는 극히 미비한 상태이다. 본 종설에서는 지금까지 밝혀진 심장 부정맥 발생의 분자 유전학적 고찰과 이와 관련된 사람의 유전학적 질환들과 이와 유사한 개의 선천성 심장 전도 장애에 대해 연구 고찰하였다.

심근경색후 발생한 좌심실류 및 심실중격결손의 외과적 치료 (6례 보고) (Surgical Repair of Left Ventricular Aneurysm and Postinfarction Ventricular Septal Defect with Myocardial Revascularization (A report of 6 cases])

  • 조범구
    • Journal of Chest Surgery
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    • 제21권6호
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    • pp.996-1002
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    • 1988
  • A clinical analysis was performed on 115 cases of -patent ductus arteriosus treated surgically during the period of 11 years from Aug. 1977 to Jul. 1988. at the Department of Thoracic and Cardiovascular Surgery, Chungnam National University Hospital. Among 115 cases, male was 38 and female was 77 and ages ranged 12 days to 27 years old with the average of 8 7/12 years. The major clinical symptoms on admission were frequent URI attack[77.4%], dyspnea on exertion[32.2%] and palpitation[13%]. On auscultation, continuous machinery murmurs were detected in 97 cases[84.3%] and loud systolic murmurs were detected in 18 cases[15.7%]. Preoperative electrocardiographic findings were as follows: LVH 59[51.3%], RVH 12[10.4%], BVH 16[13.9%] and WNL 28[24.3%]. Radiologically, there were increased pulmonary vascularity in 104[90.4%] and cardiomegaly 62[53.9%]. Cardiac catheterization were performed in 101 cases and mean systolic pulmonary arterial pressure was 49.84*29.7mmHg and mean Qp/Qs was 2.95k1.8. Methods of operation were multiple ligation in 96, division in 11 and transpulmonary arterial repair using cardiopulmonary bypass in 8. Complication were recannalization in 2, temporary hoarseness due to left recurrent laryngeal nerve paralysis in 3 and respiratory distress in 1 and overall mortality rate was 1.7%[2 cases].

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대동맥판막 폐쇄부전이 동반된 심실중격결손증의 외과적 치료 (Ventricular Septal Defect Associated with Aortic Insufficiency)

  • 두홍서
    • Journal of Chest Surgery
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    • 제21권6호
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    • pp.1111-1116
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    • 1988
  • Neurofibromatosis is very rare syndrome characterized by abnormal cutaneous pigmentation and numerous skin tumors was described by Smith in 1849, which is inherited as an autosomal dominant trait. Von Recklinghausen reported 2 cases of multiple skin and subcutaneous tumors in 1982. Malignant peripheral nerve tumors, although generally rare, are one of the most characteristic malignant tumors associated with Neurofibromatosis. We have experienced 3 cases of malignant Schwannoma in neurofibroma patients from 1982 to 1988 for 6 years at Thoracic and Cardiovascular surgery department, College of Medicine, Yonsei University, Seoul, Korea. l. One is 62 years old female who was taken total hysterectomy followed by irradiation treatment due to Uterine Carcinoma 21 years ago. She had a large bulging mass on left anterior chest wall and was taken enbloc resection of tumor including rib confirmed malignant Schwannoma. 2. Another is 18 years old female who had large bulging mass on right chest wall and pleural effusion in right thoracic cavity. Thoracentesis revealed a large amount of lymphocytes misdiagnosed of Tbc, pleurisy with Neurofibromatosis. We performed tissue biopsy on bulging mass and the specimen was confirmed malignant Schwannoma 2 months after first diagnosis of Tbc. pleurisy. She was not accessible to radical resection because of far advanced malignant Schwannoma at that time. 3. Third case is 28 years old male who was taken enbloc resection of tumor including rib due to Neurofibroma with Neurofibromatosis at M. hospital 6 months ago. But he had rapid growing mass at operation site again and taken tissue biopsy confirmed of malignant Schwannoma. He was not accessible to enbloc resection due to malignant Schwannoma extending to mediastinal structures.

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VATER 증후군에 동반된 선천성 심장기형 치험 1례 (Congenital Cardiac Anomaly Combined with the VATER Association - A case report -)

  • 반동규;김혁;지행옥;정원상;강정호;김영학;이철범;함시영;김남수
    • Journal of Chest Surgery
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    • 제34권11호
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    • pp.858-860
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    • 2001
  • VATER 증후군은 심장의 기형을 동반하기 쉬운 복합기형이지만 적절하고 적극적인 수술적 치료에 의해 완치에 이를 수 있는 기형이다. 저자들은 VATER 증후군에 심장기형이 동반된 여아에서 적극적인 수술적 치료로 만족할만한 성과를 얻어 이 증례를 고찰과 함께 보고하는바이다.

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거골 골연골병변의 수술적 치료: 관절경적 골수 자극술(다발성 천공 또는 미세 골절술) (Operative Treatment of Osteochondral Lesion of the Talus: Arthroscopic Bone Marrow Stimulation (Multiple Drilling or Microfracture))

  • 곽희철;은일수
    • 대한족부족관절학회지
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    • 제24권2호
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    • pp.48-54
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    • 2020
  • Osteochondral lesion of the talus (OLT) is a broad term used to describe an injury or abnormality of the talar articular cartilage and adjacent bone. Various terms are used to describe this clinical entity, including osteochondritis dissecans, osteochondral fractures, and osteochondral defects. Several treatment options are available; the choice of treatment is based on the type and size of the defect and the treating clinician's preference. Arthroscopic microfracture (a bone marrow stimulation technique) is a common and effective surgical strategy in patients with small lesions or in those in whom non-operative treatment has failed. This study had the following aims: 1) to review the historical background, etiology, and classification systems of OLT; 2) to describe a systematic approach to arthroscopic bone marrow stimulation for OLT; and 3) to determine the characteristics that are useful for assessing osteochondral lesions, including age, size, type (chondral, subchondral, cystic), stability, displacement, location, and containment of the lesion.

Atlantoaxial Rotatory Fixation in Adults Patient

  • Jeon, Sei-Woong;Jeong, Je-Hoon;Moon, Seung-Myung;Choi, Sun-Kil
    • Journal of Korean Neurosurgical Society
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    • 제45권4호
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    • pp.246-248
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    • 2009
  • Atlantoaxial rotatory fixation (AARF) in adult is a rare disorder that occurs followed by a trauma. The patients were presented with painful torticollis and a typical 'cock robin' position of the head. The clinical diagnosis is generally difficult and often made in the late stage. In some cases, an irreducible or chronic fixation develops. We reported a case of AARF in adult patient which was treated by immobilization with conservative treatment. A 25-year-old female was presented with a posterior neck pain and limitation of motion of cervical spine after a traffic accident. She had no neurological deficit but suffered from severe defect on the scalp and multiple thoracic compression fractures. Plain radiographs demonstrated torticollis, lateral shift of odontoid process to one side and widening of one side of C1-C2 joint space. Immobilization with a Holter traction were performed and analgesics and muscle relaxants were given. Posterior neck pain and limitation of the cervical spine' motion were resolved. Plain cervical radiographs taken at one month after the injury showed that torticollis disappeared and the dens were in the midline position. The authors reported a case of type I post-traumatic AARF that was successfully treated by immobilization alone.

기계학습을 이용한 태양광 발전량 예측 및 결함 검출 시스템 개발 (Development of a System for Predicting Photovoltaic Power Generation and Detecting Defects Using Machine Learning)

  • 이승민;이우진
    • 정보처리학회논문지:컴퓨터 및 통신 시스템
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    • 제5권10호
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    • pp.353-360
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    • 2016
  • 여러 개의 태양전지들이 붙어 있는 태양광 패널을 이용하여 전력을 생산하는 태양광 발전은 최근 신재생 에너지 기술로 빠르게 성장하고 있는 분야이다. 하지만 태양광발전의 단점 중 하나인 불규칙한 전력 생산문제로 인해, 장비 및 패널 결함에 빠르게 대응하지 못하는 문제가 발생한다. 이 연구에서는 다양한 기후데이터와 패널 정보를 이용하여 태양광발전량 예측 방법들을 비교하여 최적의 예측 알고리즘을 평가하고 이를 기반으로 태양광발전소 결함 검출 시스템을 개발하여 국내 태양광 발전소에 적용한 사례를 기술한다.