• 제목/요약/키워드: Congenital anomaly

검색결과 492건 처리시간 0.036초

활로씨 4징증의 좌심실용적이 수술후 심장기능에 미치는 영향 (The Effect of Left Ventricular Volume on Postoperative Cardiac Function in Tetralogy of Fallot)

  • 김응중;노준량
    • Journal of Chest Surgery
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    • 제21권2호
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    • pp.211-222
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    • 1988
  • Tetralogy of Fallot is a cyanotic congenital heart disease characterized by large ventricular septal defect[VSD] and stenosis of right ventricular outflow tract[RVOT] and the degree of RVOT stenosis and the state of pulmonary arteries are the major determinant of prognosis of this anomaly after operation. The sum of blood flow through RVOT and collateral flow from systemic arteries determine the total pulmonary blood flow and it is drained to left atrium and left ventricle. Therefore the degree of development of left ventricle not only reflects pulmonary blood flow and the status of peripheral pulmonary arteries but also affects postoperative prognosis as a systemic ventricle. In this article, left ventricular volume and its influence on postoperative cardiac function in tetralogy of Fallot were studied in 34 patients operated on at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital in 1985. Mean age of the patients was 5 1/12*3 9/12 years[range 9/12 - 14 8/12 years], mean body surface area[BSA] 0.65*0.20m2[range 0.38 - 1.22m2], mean body weight 15.6k6.48kg[range 7.0 - 36kg]and mean hematocrit 50.6*9.77%[range 32.0 - 73.5%]. Left ventricular end-diastolic volume[LVEDV] of them were from 11.2 to 113cc and there was a good linear correlation between BSA[m2, X]and LVEDV[cc, Y][Y= - 20.0+923x, r= 0.84, p < 0.005]. Mean LVEDV/m2 was [57.6 * 18.3 cc / m2[range 28.7 - 95.8 cc / m2] and there was a significant reduction of volume compared with normal value. As body surface increases, there was a increasing tendency in LVEDV/m2 but there was no statistical significance. Mean total amount of postoperatively infused dopamine in these 33 patients[except one who expired postoperatively] was 65.6*74.5mg / kg and it was 40.6*44.0mg / kg in routine RVOT widening group [Group I] and 205*49.3mg / kg in transannular RVOT widening group[Group II]. There was a statistically significant difference between two groups. In group I patients there was a good linear inverse correlation between dopamine total amount[mg / kg, Y] and LV volume[cc / m2, X] [Y = 150 - 1.89 X, r = - 0. 77, p < 0.005]. But there were no correlations between dopamine total amount and Hct, cardiopulmonary bypass time and aorta cross clamp time. In conclusion, the patient with small preoperative left ventricular volume required more amount of dopamine as an inotropic agent for the maintenance of a cardiac function in postoperative period. But this is a result of immediate postoperative period and does not reflect the long term effect of left ventricular volume in tetralogy of Fallot. There must be more study for the evaluation of its long term effect.

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Conotruncal 기형 평가에서 전자선 단층 촬영 (EBT)의 정확성 (Evaluation of Conotruncal Anomalies by Electron Beam Tomography)

  • 최병욱;박영환;최병인;최재영;김민정;유석종;이종균;설준희;이승규
    • Journal of Chest Surgery
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    • 제33권4호
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    • pp.290-300
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    • 2000
  • Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

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쇄골두개 이형성증 환자에 관한 증례보고 (CLEIDOCRANIAL DYSPLASIA : REPORT OF A CASE)

  • 이연호;유승훈;김종수
    • 대한소아치과학회지
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    • 제32권4호
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    • pp.703-708
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    • 2005
  • 쇄골두개 이형성증은 구강내에서 치아의 맹출 지연과 함께. 두개골 쇄골 및 안면의 비정상적인 성장을 보이는 상염색체 우성 유전 증후군이다. 이 증후군의 특징적인 소견으로, 쇄골이 양측성으로 존재하지 않거나 혹은 불완전한 구조로 존재하는 것을 들 수 있다. 또한 두개골 성장에 있어서 전두골, 후두골판은 다른 골과는 다르게 크기가 증가하여 비정상적 인 형태의 머리모양을 보인다. 두개골의 어떤 부위에는 충분한 골성장이 결여되어 봉합선이 매우 넓어져 있으며 방사선 사진상에서 봉합이 열려있는 상태로 관찰된다. 코는 대개 편평하고 넓으며, 일반적으로 상악의 성장이 부족하여 상대적으로 하악이 커 보인다. 구강내 소견으로는, 유치의 흡수가 늦어지며, 그 결과 영구치도 정상인보다 늦게 맹출되는 양상이 관찰된다. 20세 이상의 쇄골두개 이형성증 환자의 구강내에서 유치를 흔히 발견할 수 있다. 또한 다수의 과잉치가 존재하는데, 이는 구강내 전체에 걸쳐 존재할 수도 있다. 대부분의 쇄골두개 이형성증 환자는, 영구치 맹출 장애가 일어날 때까지 자신의 치과적 문제점을 인식할 수 없기 때문에, 적절한 치료시기를 지나서 치과병원에 내원하는 경우가 많다. 소아환자를 치료하는 치과의사는 쇄골두개 이형성증 환자의 치열 발육에 관한 지식을 숙지하여, 이러한 환자들이 적절한 시기에 치료를 받을 수 있도록 한다.

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폐분리증의 치료와 흉강경적 절제술의 경험 (Treatment of Pulmonary Sequestration with Thoracoscopic Approach)

  • 조민정;김태훈;김대연;김성철;김인구
    • Advances in pediatric surgery
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    • 제16권2호
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    • pp.154-161
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    • 2010
  • Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The anomaly is characterized by absence of communication with the tracheobronchial tree and isolated blood supply from an anomalous systemic vessels. With the utilization of antenatal ultrasound, the diagnosis of asymptomatic neonatal PS has increased. Treatment options include observation, arterial embolization and surgical resection. The aim of the present study is to review the clinical course of PS and to share our experience with thoracoscopic resection. A total of 96 patients with PS were treated at Asan Children's Hospital between 1999 and 2010. The diagnosis of PS was established by CT in the cases managed by observation or embolization, and by tissue pathology in the surgical cases. Medical records and radiographic images were retrospectively reviewed. Thirty-nine patients were managed by embolization and 30 patients by surgery. The remaining 27 patients have been under observation without any procedures. Among 27 observation patients, 1 patient regressed completely and 10 patients were lost to follow up. Of the 39 embolizations patients, 2 had their lesion regress and sepsis was suspected after embolization. In 1 patient, the microcoil migrated to the iliac artery during the embolization procedure, and another patient developed renal abscess caused by renal artery embolization. Among 30 surgical cases, resection by thoracotomy was performed in 27 at the Department of Thoracic Surgery, and thoracoscopic resection in 3 at the Division of Pediatric Sugery. Only one wound complication ocurred. We conclud that surgical excision should be recommended for pulmonary sequestration, whether the sequestration is symptomatic or not because of the risk of infection, the low rate of natural regress, poor compliance, severe complications after embolization, and to exclude other pathology. In summary, thoracoscopic resection of the pulmonary sequestration is feasible, efficacious, safe and cosmetically superior even in neonatal period.

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1세 전후로 진단된 장 회전이상증의 임상적 특징 (Comparison of the Clinical Characteristics of Intestinal Malrotation in Infants and Children)

  • 허정민;문석배;정수민;신현백;서정민;이석구
    • Advances in pediatric surgery
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    • 제16권2호
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    • pp.126-133
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    • 2010
  • Malrotation is a congenital anomaly that becomes symptomatic more frequently during infancy. The indication for surgical treatment at that age is straightforward. In older children, the diagnosis may be more difficult because of chronic and vague complaint. The aim of this study is to compare the symptoms, rate of volvulus and surgical findings in children younger and older than one year. A retrospective study of 40 patients in a a single medical center diagnosed with malrotation from April 1996 to May 2010 was performed. There were 20 (50 %) boys and 20 (50 %) girls. At the time of operation, 27 (67.5 %) patients were younger and 13 (32.5 %) were older than 1 year. Vomiting was seen in 20 cases (74.1 %) of the younger group compared to 2 cases (15.4 %) of the older group. Abdominal sonography and upper gastrointestinal series showed a sensitivity of 100%. Operative findings: 12 (44.4 %) of the younger group presented with volvulus compared to none of the older group. The Ladd's procedure was routinely performed with appendectomy in all cases and bowel resection was requires when volvulus included bowel necrosis or other anomalies were found. After definite procedures, surgical correction for adhesive obstruction was necessary in 5 menbers (18.5 %) of the younger group and 1 patient (7.7 %) in the older group. There was 1 death due to respiratory failure and pneumonia. Abdominal pain was more frequent symptom and bilious vomiting was less frequent. Volvulus did not occur in the older group. Malrotation should be diagnosed promptly in children over 1 year of age by upper gastrointestinal series and abdominal ultrasonography even though symptoms are not as clear cut as in infants.

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소아 멕켈씨 게실의 합병증 (Complications of Meckel's Diverticulum in Children)

  • 전흥만;남소현;김대연;김성철;김인구
    • Advances in pediatric surgery
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    • 제13권2호
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    • pp.127-134
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    • 2007
  • 소아 멕켈씨 게실의 가장 흔한 합병증은 장출혈이었고, 장중첩, 장폐쇄, 장천공 등도 있었다. 멕켈씨 게실의 조직검사상 이소성위조직이 가장 많았고, 이소성 췌장조직도 있었다. 소아 급성 복증의 원인으로 멕켈씨 게실에 의한 합병증을 염두에 두어야겠으며, 진단이 불명확한 경우, 복강경을 통한 진단이 도움이 될 수 있을 것이다. 또한 멕켈씨 게실의 경우 복강경하 절제술로 복강경 수술의 장점을 충분히 살릴 수 있을 것으로 사료된다.

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새로운 적응증에 다양한 방법으로 실시한 정맥피판 (Venous Flaps Applied for New Clinical Indications with using Various Methods)

  • 김남균;최재훈;최태현;이경석;김준식;이혁구
    • Archives of Plastic Surgery
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    • 제34권1호
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    • pp.52-59
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    • 2007
  • Purpose: The venous flap is used as an alternative method to the standard free flap for the management of small and thin soft tissue defects. Especially, the venous flap has advantages of being thin, easy harvest and various donor sites, as well as it having lower morbidity. Yet their clinical applications have been limited by their unstable postoperative course and also by their complications such as partial necrosis. The aim of this study is to extend the clinical indications of venous flaps with using various methods. Methods: From May 2005 to March 2006, total of 19 patients(21 cases) underwent various venous flaps for soft tissue defects as a result of trauma(15 cases), facial skin cancer(3 cases), chronic ulcer(1 case) and surgical wound for congenital anomaly(2 cases). The arterialized venous free flap were applied in 18 cases, the pure venous free flap was applied in 1 case and the pure venous pedicled flap were applied in 2 cases. Among them, two flow-through arterialized venous free flaps were applied that used the great saphenous vein to reconstructed major arteries as well as the injured skin and soft tissues in the arm. All the flap were harvested from the volar wrist(11 cases), dorsum of foot(5 cases), thenar(2 cases), and medial thigh(3 cases). Results: The sizes of the flap ranged from $0.75cm^2$ to $264cm^2$(mean size: $40.06cm^2$). The follow-up period ranged from two to twelve months. In the majority of cases, we obtained satisfying results, which was the excellent reconstruction of skin and soft tissue defects and especially in the case of limb salvage, replantation and cancer reconstruction. However, there were 5 cases of partial necrosis and 2 cases of complete failure. The donor sites were closed primarily in 7 cases and wound closure with skin graft were in 14 cases. Conclusion: We conclude that the venous flap will not only be useful for reconstruction of small defect in the hand and foot, but also be useful for various other clinical indications.

발열성 요로 감염 환아에서 발견된 수신증의 임상적 의의 (Clinical Significance of Hydronephrosis in Febrile Urinary Tract Infection)

  • 오정민;이나라;임형은;유기환;홍영숙;이주원
    • Childhood Kidney Diseases
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    • 제14권1호
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    • pp.71-78
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    • 2010
  • 목 적 : 수신증은 요로 감염 환아의 30%에서 발견된다. 수신증은 다양한 원인에 의해 발생할 수 있으나, 대부분은 선천성인 것으로 알려져 있다. 저자들은 발열성 요로 감염과 수신증과의 관련성을 알아보았다. 방 법 : 2007년 1월부터 2009년 5월까지 고대구로병원에서 요로 감염 진단을 받은 183명의 의무 기록을 후향적으로 분석하였다. 결 과 : 수신증이 있는 환아는 80명, 수신증이 없는 환아는 103명이었다. 수신증이 있는 환아 중 58명이 왼쪽, 8명이 오른쪽, 14명이 양쪽에 수신증이 관찰되었으며, 대부분의 수신증 등급은 경도였다. 수신증이 없는 군과 비교 시, 신스캔 상의 초기 신결손이 수신증이 있는 군에서 유의하게 관찰되었다(수신증이 있는 군 37.5%, 수신증이 없는 군 16.5%, P<0.05). 방광 요관 역류의 발병률은 두 군 간에 차이를 보이지 않았다(수신증이 있는 군 22%, 수신증이 없는 군 12.1%). 백혈구 수치와 C 반응 단백 수치는 두 군 간에 유의한 차이가 없었다. 요로 감염을 앓은 후 약 6개월 째 시행한 신스캔 추적 검사 상에서도 신결손의 호전 여부 및 신반흔 발생은 두 군 간의 유의한 차이를 보이지 않았다. 결 론 : 저자들은 열성 요로 감염이 있는 환아의 수신증이 초기 신결손을 예측하는데 임상적으로 유용하나, 추적 검사 상의 신 반흔과는 관련이 없음을 제시하는 바이다.

임산부의 구강보건관리 실태와 구강보건 인지도에 관한 연구 -인구사회학적 변인을 중심으로- (A Study on the Oral Health Behavior Status and Oral Health Awareness of Pregnant Women -Demographic Socialogical Variables-)

  • 이숙정;최규일
    • 한국산학기술학회논문지
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    • 제12권11호
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    • pp.5049-5055
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    • 2011
  • 본 논문은 10개월의 임신기간을 통해 새로운 생명 탄생을 준비하는 임산부의 육체적, 정신적 부분에서의 변화 중 신체에서의 구강은 태아의 건강까지 책임져야하는 영양분 섭취에서의 첫 관문이라는 의미에서 잘 관리되어져야 한다. 이에 임산부의 구강보건관리 실태와 구강보건 인지도를 조사한 결과, 잇몸 출혈 시 칫솔질 여부는 '이행' 54.4%였고, '불이행' 45.6%로 나타나고 있다. 임신 중 치과치료 경험유무는 전체적으로 '아니오'의 응답이 164명(91.1%)로 높게 나타났다. 보조구강위생용품 사용종류에 대한 다중응답으로 조사한 결과로 가장 많이 사용한 보조구강위생용품은 '이쑤시개' 43.2%였고, 다음으로 2순위는 '치실' 26.2%였으며, 3순위는 '구강양치액' 12.6% 순이었다. 구강보건교육에 대한 경험유무는 전체적으로 구강보건교육을 받은 경험은 대다수가 '없다(92.8%)'였다. 구강보건교육 필요성은 전체적으로 '필요하다'의 응답이 92.8%로 나타났다. 임산부의 구강건강에서 가장 고민되는 부분에 대해서 자유형 개방형으로 조사한 결과로 가장 많은 고민은 '입덧으로 인한 칫솔질의 어려움'이었고, 다음으로 '잇몸출혈', '치은염과 구취', '충치'의 순이었다.

뇌실 복강간 단락 부전의 기여인자 (The Contributory Factors of CSF Shunt Failure)

  • 김영돈;황성규;황정현;성주경;함인석;박연묵;김승래
    • Journal of Korean Neurosurgical Society
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    • 제30권sup1호
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    • pp.79-84
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    • 2001
  • 목 적 : 뇌실 복강간 단락술 부전의 기여인자를 조사 및 분석하여 뇌실 복강간 단락술의 합병증 및 재수술의 가능성을 줄이는 데에 목적이 있다. 대상 및 방법 : 1995년 1월부터 1998년 12월까지 본원에서 뇌실 복강간 단락술을 시행 받은 237명의 수두증 환자를 후향적 방법으로 고찰하여 뇌실 복강간 단락 부전의 기여인자 및 단락기 생존율을 통계학적으로 분석하였다. 결 과 : 수두증의 원인은 종양, 출혈, 감염, 선천성 기형, 정상 뇌압 수두증, 외상 등이었다. 68명의 환자에서 109회의 재수술이 이루어졌다. 재수술의 이유는 폐색, 근위부 카세타의 위치이상, 감염 등이었다. 단락기 생존율은 1년, 2년, 3년에 각각 77.1%, 75.4%, 74.1%이었다. 10세 이하의 환자에서 재수술의 빈도가 통계학적으로 의의 있게 높았다. 그리고 수두증의 원인에 따라서 통계학적으로 의의 있게 재수술의 빈도 차이가 있었다. 결 론 : 대부분의 뇌실 복강간 단락 부전은 술후 1년내에 발생하였다. 환자의 나이 및 수두증의 원인이 뇌실 복 강간 단락 부전의 주요 기여인자였다.

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