• 제목/요약/키워드: cause of defects

검색결과 623건 처리시간 0.025초

신생아 대사질환 선별검사에서 발견된 갈락토스혈증의 감별진단 (Differential Diagnosis of Galactosemia Detected by Neonatal Screening)

  • 최성윤;송웅주;임한혁;길홍량;김숙자
    • 대한유전성대사질환학회지
    • /
    • 제13권2호
    • /
    • pp.89-97
    • /
    • 2013
  • Purpose: We retrospectively investigated individuals who hadbeen identified by neonatal screening as potential galactosemia patients to determine the etiology of galactosemia. Methods: One hundred fifty-three patients referred to Korea Genetics Research Center due to high galactose level detected by neonatal screening test between February 2005 and May 2013 were examined. Galactose and galactose-1-phosphate levels were measured by using a fluoro metric microplate reader. Lactose free diet was initiated immediately after confirmed by urine Clinitest. If reducing sugar was negative, we employed abdominal sonogram and echocardiogram to check for possible porto-systemic shunt. Results: Fifteen patients were diagnosed with galactosemia. One patient had galactokinase (GALK) deficiency; four had UDP galactose-4-epimerase (GALE) deficiency; two had citrin deficiency; and four had porto-systemic shunt. Two had unknown causes of galactosemia. Conclusion: In addition to genetic defects of GALT, GALK and GALE, citrin deficiency or porto-systemic shunt could also cause galactosemia. It is crucial to carry out differential diagnosis to determine the cause of galactosemia.

  • PDF

터널 유지관리를 위한 안전진단시스템 개발에 관한 연구 (Development of Inspection and Diagnosis System for Safety and Maintenance in Tunnel)

  • 김영근;백기현
    • 한국터널지하공간학회 논문집
    • /
    • 제3권1호
    • /
    • pp.37-50
    • /
    • 2001
  • 최근 터널구조물에서 결함이나 변상이 많이 발생하고 있지만 터널구조물의 특수성으로 인하여 그 원인을 평가하거나, 상태 및 안전성 평가에 있어 많은 어려움을 겪고 있다. 따라서 보다 효율적인 안전진단 및 유지관리대책이 요구되고 있다. 본 연구에서는 터널에서의 정밀안전진단을 효과적으로 수행하기 위하여 터널 라이닝과 주변지반에 대한 비파괴 조사기술, 터널 라이닝의 구조적 안정성을 평가할 수 있는 해석기술, 그리고 터널의 변상원인 및 건전도를 판단할 수 있는 평가기술을 개발하여 터널의 열화 및 손상정도를 진단하고 터널의 유지관리를 위한 적절한 보수 보강대책을 제시함으로서 체계적인 터널 안전진단업무에 활용하도록 하였다.

  • PDF

승모판막 협착증의 외과적 요법 (Surgical Treatment of Mitral Stenosis)

  • 김용진
    • Journal of Chest Surgery
    • /
    • 제10권2호
    • /
    • pp.241-249
    • /
    • 1977
  • Atrial septal defect is one of the most frequently encountered congenital heart disease. Up to December 31, 1976, 1682 cardiac patients received cardiac catheterization in the cardiac department of Yonsei university medical college. Out of the 1682 cardiac patients 723 cases had congenital heart disease and only 116 cases had congetial atrial septal defect. This amounted to 16.04% of all those with congenital heart disease. 58 cases of congenital atrial septal defect operated in the chest surgery department were presented. Of these 58 cases of atrial septal defect, 27 cases were male and 31 cases were female. Their ages ranged from 5 years to 54 years. The systolic pressure of the main pulmonary artery of 40 out of the 58 cases of atrial septal defect was below 40% of that of the systemic blood pressure: in 6 cases, the range of the systolic pressure of the main pulmonary artery was 50-90mmHg; in 12 cases, the range of the systolic pressure of the main pulmonary artery was 40-50mmHg. Average age of these was 30. 1 years. This study tends to show that Korean patients with atrial septal defect even though younger have a slight higher systolic pressure of the main pulmonary artery than Western patients have. The pulmonary blood is 1.5-2.5 times of systemic blood flow in 52 cases out of 58 cases of atrial septal defect.In only one of the 58 cases of atrial septal defect, the Rp was found to be as high as 45% of Rs. All other cases were below this level.51 cases had ostium secundum defect, 4 out of these cases had ostium secundum defect combined with mitral incompetence and 6 out of them had double ostium secundum defect. The remaining 7 cases had ostium primum defect. Their atrial defects were repaired under direct vision utilizing extracorporeal circulation, by hemodilution technic combined with moderate hypothermia. 44 cases [2nd atrial septal defect] were repaired by direct sutures while 14 cases, including the 7 cases ostium primum defects needed patches [1 pericardium and 13 teflon patch]. In 4 cases there were single defects while showed two defects. However the associated septal defect was so small that it could be closed by direct sutures. The size of the defect ranged between 6.0cm2and 10.0cm2 in 19 cases[33.7%]: the smallest being 0. 5cm2 and the largest 24cm2. The surgical mortality was 2 cases [3.4%]. These one case with ostium primum defect, could not be resuscitated on operation table. The cause of death in this case was myocardial failure and MI. The other, a case of ostium primum defect had a second operation on the first operative day due to massive bleeding from LV vent-line insertion site.The patient died on 26th post-operative day due to sepsis.

  • PDF

Print Mottle : Causes and Solutions from Paper Coating Industry Perspective

  • Lee, Hak-Lae
    • 펄프종이기술
    • /
    • 제40권5호
    • /
    • pp.60-69
    • /
    • 2008
  • The principal reasons for applying a pigment coating to paper are to improve appearance and printability. The pigment coating provides a surface that is more uniform and more receptive to printing ink than are the uncoated fibers and, in turn, both facilitates the printing process and enhances the graphic reproduction. The improvement in print quality is readily apparent, especially in image areas or when multiple colors are involved. Although pigment coating of paper is to improve the printability, coated paper is not completely free from printing defects. Actually there are a number printing defects that are observed only with the coated papers. Among the printing defects that are commonly observed for coated papers, print mottle during multi-color offset printing is one of the most concerned defects, and it appears not only on solid tone area but also half dot print area. There are four main causes of print mottle ranging from printing inks, dampening solution, paper, and printing press or its operation. These indicates that almost every factors associated with lithographic printing can cause print mottle. Among these variation of paper quality influences most significantly on print mottle problems in multicolor offset printing, and this indicates that paper is most often to be blamed for its product deficiency as far as print mottle problems are concerned. Furthermore, most of the print mottle problems associated with paper is observed when coated papers are printed. Uncoated papers rarely show mottling problems. This indicates that print mottle is the most serious quality problems of coated paper products. Overcoming the print mottle is becoming more difficult because the operating speeds of coating and printing machines are increasing, coating weights are decreasing, and the demands on high-quality printing are increasing. Print mottle in offset printing is caused by (a) nonuniform back trap of ink caused by a nonuniform rate of ink drying, referred as "back trap mottle, and (b) nonuniform absorption of the dampening solution. Furthermore, both forms of print mottle have some relationship to the structure of the coated layer. The surest way of eliminating ink mottling is to eliminate unevenness in the base paper. Coating solutions, often easier to put into practice, should, however, be considered. In this paper the principal factors influencing print mottle of coated papers will be discussed. Especially the importance of base paper roughness, binder migration, even consolidation of coating layers, control of the drying rate, types of binders, etc. will be described.

임플란트 주위 골 결손 부위에 Choukroun's Platelet-rich-fibrin와 실크 분말 복합 이식재 사용 (Effect of Combination Graft of Choukroun's Platelet-rich-fibrin with Silk Fibroin Powder in the Peri-implant Defects)

  • 장은식;이형석;이희성;이희종;박기유;박영욱;윤연진;홍순민;박준우
    • Maxillofacial Plastic and Reconstructive Surgery
    • /
    • 제33권2호
    • /
    • pp.103-111
    • /
    • 2011
  • Purpose: Choukroun's platelet-rich-fibrin (PRF) is composed of platelets, white blood cells and fibrin matrix. It does not induce enough bone formation by itself but it can improve bone formation with calcium. Silk fibroin does not cause inflammatory reactions because it is bio-compatible and degradable. The purpose of this study was to exam the bone formation when a combination of Choukroun's PRF and silk fibroin was used. Methods: In this study, cell reactions to silk powder with differing molecular weights was first tested to select the appropriate silk powder. Then we applied these bone graft materials on defects of skull and in a peri-implant bony defect model in New Zealand rabbits. The results between the experimental and control s (non-grafted) group were analyzed. Results: The small sized silk fibroin powder showed increased cellular proliferation for bone-regeneration. There was no statistically significant difference between the experimental group and the control group at 6 weeks, but more new bone formation was observed in the combination graft group at 12 weeks (P<0.05). And in the dental implant model, the combination bone graft group showed much improved torque test results, which was statistically significant. Histomorphometric analysis showed more regenerated cortical bone and a higher mean bone to implant in the experimental group. Both were statistically significant. Conclusion: The combination graft of Choukroun's platelet-rich-fibrin (PRF) and silk fibroin powder can successfully restore the bony defects in a skull defected model and a peri-implant bony defects model.

ZnO-Co3O4-Cr2O3-La2O3 세라믹스의 결함과 입계 특성에 미치는 CaCO3의 영향 (Effects of CaCO3 on the Defects and Grain Boundary Properties of ZnO-Co3O4-Cr2O3-La2O3 Ceramics)

  • 홍연우;하만진;백종후;조정호;정영훈;윤지선
    • 한국전기전자재료학회논문지
    • /
    • 제31권5호
    • /
    • pp.307-312
    • /
    • 2018
  • Liquid phases in ZnO varistors cause more complex phase development and microstructure, which makes the control of electrical properties and reliability more difficult. Therefore, we have investigated 2 mol% $CaCO_3$ doped $ZnO-Co_3O_4-Cr_2O_3-La_2O_3$ (ZCCLCa) bulk ceramics as one of the compositions without liquid phase sintering additive. The results were as follows: when $CaCO_3$ is added to ZCCLCa ($644{\Omega}cm$) acting as a simple ohmic resistor, CaO does not form a secondary phase with ZnO but is mostly distributed in the grain boundary and has excellent varistor characteristics (high nonlinear coefficient ${\alpha}=78$, low leakage current of $0.06{\mu}A/cm^2$, and high insulation resistance of $1{\times}10^{11}{\Omega}cm$). The main defects $Zn_i^{{\cdot}{\cdot}}$ (AS: 0.16 eV, IS & MS: 0.20 eV) and $V_o^{\bullet}$ (AS: 0.29 eV, IS & MS: 0.37 eV) were found, and the grain boundaries had 1.1 eV with electrically single grain boundary. The resistance of each defect and grain boundary decreases exponentially with increasing the measurement temperature. However, the capacitance (0.2 nF) of the grain boundary was ~1/10 lower than that of the two defects (~3.8 nF, ~2.2 nF) and showed a tendency to decrease as the measurement temperature increased. Therefore, ZCCLCa varistors have high sintering temperature of $1,200^{\circ}C$ due to lack of liquid phase additives, but excellent varistor characteristics are exhibited, which means ZCCLCa is a good candidate for realizing chip type or disc type commercial varistor products with excellent performance.

항공기 연료흐름분배기 장착 구조물 결함개선 연구 (Study on the Defect Improvement of Fuel Flow Proportioner Install Structure on Aircraft)

  • 최형준;이진원;최재호;박성제
    • 한국산학기술학회논문지
    • /
    • 제21권4호
    • /
    • pp.558-567
    • /
    • 2020
  • 본 연구는 항공기 운용 중 발생하는 구조 결함의 원인을 분석하기 위해 연료흐름분배기 장착 구조물의 결함특성을 확인하고자 한다. 일반적으로 항공기 운용 중 발생하는 체계 진동과 구성품 단일 진동에 의해 구조 결함이 야기된다. 결함을 정의하고 설계 및 운용 분석으로 이어지는 과정을 통해 원인 규명 방법을 제시하고자 한다. 연료흐름분배기는 급격한 유량의 증가로 인한 압력의 변화로 발생되는 진동에 의해 지지구조의 결함이 발생되었다. 지지구조에 발생하는 하중 특성 및 파단면 분석을 통해 초기 균열이 반복하중으로 인해 피로균열이 발생하였다. 연료흐름분배기 지지구조 결함은 항공기 운용분석 결과 기종별 기동 및 비행시간은 균열과 직접적인 상관관계가 적고, 후기연소(A/B) 사용과 관련이 있다. 결함에 대한 개선을 위해 연료흐름분배기 하부 장착 Bracket의 균열 부위에 정적 및 수명해석을 통해 구조 보강형상을 확정하였다. 보강에 대한 분석 결과 구조 강도 최소 마진이 +0.15로 구조 건전성을 확인하였고, 구조 수명 분석결과 해당 부위에 응력이 15Ksi 이하로 작용하였으며 피로 수명이 7,700 Cycle 이상임을 확인하였다.

활로 4징증의 근치수술후의 원격 방실전도 차단 (Complete A-V Block 3 Months after Total Correction of Tetralogy of Fallot)

  • 송요준
    • Journal of Chest Surgery
    • /
    • 제11권3호
    • /
    • pp.326-332
    • /
    • 1978
  • There appears some conduction defects frequently after total correction of Tetralogy of Fallot. Common defect is right bundle branch block due to surgical intervention. We experienced complete A-V block which occured 3 months later after total correction of Tetralogy of Fallot in a 8 year old boy. The patient was completely free of any A-V block after the operation for 3 months, and sudden onset of A-V block with coupled premature ventricular contractions resulted him in shock state during the attack of severe bronchopneumonia for 4 days prior to the second visit. Emergency implantation of Cordis demand type temporary pacemaker was necessary to control the complete heart block with bradyarrhythmia and frequent ventricular fibrillation. Permanent cardiac pacemaker was implanted two weeks later as indicated with Cordis Stanicor lambda demand pacemaker, and the patient was discharged uneventfully on the 8th post implantation day with the heart rate of 72/min. Another 3 months after the implantation, the patient was transported to this hospital as dead on arrival after an accidental fall from a 2 meter height, and all possible cardiopulmonary resuscitation was performed for 60 minutes at the emergency room in vain. Autopsy was done to find out the cause of sudden death and the etiology of complete heart block. Microscopic focal infarctions with scar formation were noted along the course of conduction system in the interventricular septum, which might be the main cause of complete heart block during the attack of severe bronchopneumonia complicated with acute bacterial endocarditis. The tip of the pacemaker wire was slipped from the granulation scar at the apex of the right ventricular cavity, and this might be the direct cause of pacing failure and death.

  • PDF

The Influence of Pituitary Adenoma Size on Vision and Visual Outcomes after Trans-Sphenoidal Adenectomy : A Report of 78 Cases

  • Ho, Ren-Wen;Huang, Hsiu-Mei;Ho, Jih-Tsun
    • Journal of Korean Neurosurgical Society
    • /
    • 제57권1호
    • /
    • pp.23-31
    • /
    • 2015
  • Objective : The aims of this study were to investigate the quantitative relationship between pituitary macroadenoma size and degree of visual impairment, and assess visual improvement after surgical resection of the tumor. Methods : The medical records of patients with pituitary adenoma, who had undergone trans-sphenoidal adenectomy between January 2009 and January 2011, were reviewed. Patients underwent an ocular examination and brain MRI before and after surgery. The visual impairment score (VIS) was derived by combining the scores of best-corrected visual acuity and visual field. The relationship between VIS and tumor size/tumor type/position of the optic chiasm was assessed. Results : Seventy-eight patients were included (41 male, 37 female). Thirty-two (41%) patients experienced blurred vision or visual field defect as an initial symptom. Receiver operating characteristic curve analysis showed that tumors <2.2 cm tended to cause minimal or no visual impairment. Statistical analysis showed that 1) poor preoperative vision is related to tumor size, displacement of the optic chiasm in the sagittal view on MRI and optic atrophy, and 2) poorer visual prognosis is associated with greater preoperative VIS. In multivariate analysis the only factor significantly related to VIS improvement was increasing pituitary adenoma size, which predicted decreased improvement. Conclusion : Results from this study show that pituitary adenomas larger than 2 cm cause defects in vision while adenomas 2 cm or smaller do not cause significant visual impairment. Patients with a large macroadenoma or giant adenoma should undergo surgical resection as soon as possible to prevent permanent visual loss.

마이코플라즈마 폐렴에 의해 용혈성 빈혈이 발현된 유전성 구상 적혈구증 1례 (A Case of Hereditary Spherocytosis with Hemolytic Anemia due to Mycoplasma pneumonia)

  • 나혜연;신선희;이규만;김광남
    • Pediatric Infection and Vaccine
    • /
    • 제16권2호
    • /
    • pp.215-219
    • /
    • 2009
  • 5-14세에 호발하는 마이코플라즈마 폐렴은 20-25%에서 폐외 증상을 일으키는 것으로 알려져 있으며 이 중 혈액계 질환에는 용혈성 빈혈, 혈소판 감소증, 혈구포식세포 증식증(hemophagocytosis) 등이 있다. 마이코플라즈마 폐렴에서의 냉항체에 의한 자가면역성 용혈로 용혈성 빈혈이 발생할 수 있으며, 이 때 항체의 역가와 용혈의 정도에 상관관계가 있을 수 있다. 저자들은 마이코플라즈마 폐렴에 의해 용혈성 빈혈이 발현된 유전성 구상 적혈구증 1례를 경험하였기에 보고하는 바이다.

  • PDF