• Title/Summary/Keyword: 선천성 기형

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Clinic Analysis of Heart Surgery -110 cases- (심장수술 110례 임상고찰)

  • Chang, Hoon;Shin, Sung-Aia;No, Joong-Kee;Rho, Jun-Ryang
    • Journal of Chest Surgery
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    • v.34 no.8
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    • pp.597-603
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    • 2001
  • Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

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Clinical Characteristics and Associated Anomalies in Children with Solitary Kidney (단일신을 가진 소아의 임상적 특징과 동반기형)

  • Kim, Joo-Whee;Lee, Se-Eun;Jung, Yun-Hye;Han, Kyung-Hee;Lee, Hyun-Kyung;Kang, Hee-Gyung;Ha, Il-Soo;Cheong, Hae-Il;Choi, Yong
    • Childhood Kidney Diseases
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    • v.14 no.1
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    • pp.42-50
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    • 2010
  • Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

DENTAL TREATMENT OF A PATIENT WITH CATCH22 SYNDROME UNDER GENERAL ANESTHESIA: A CASE REPORT (CATCH22 syndrome 환아의 전신마취 하 치아우식 치료: 증례보고)

  • Kim, Min Jin;Song, Ji-Soo;Shin, Teo Jeon;Kim, Young-Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Hyun, Hong-Keun
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.14 no.1
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    • pp.36-40
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    • 2018
  • CATCH22 syndrome or DiGeorge syndrome is a medical acronym of cardiac defects, abnormal facial appearances, thymic hypoplasia, cleft palate, and hypocalcemia. Patients with CATCH22 syndrome are susceptible to infection due to an absent or hypoplastic thymus and often have difficulties in maintaining good oral hygiene, which may require dental treatment. We present a case of dental treatment for the uncooperative child with CATCH22 syndrome under general anesthesia. A 4-year-old, 14.8 kg boy with CATCH22 syndrome visited Seoul National University Dental Hospital for dental check up. The patient had multiple caries requiring dental treatment. He experienced the corrective heart surgery due to Tetralogy of Fallot a few years ago. General anesthesia was planned because his heart rate and vital sign had shown unstable during the previous conscious sedation procedure. Dental restorative treatments were successfully performed and no complications were observed during and after the procedure. Safe and effective dental management of the patients with CATCH22 syndrome could be performed with the help of general anesthesia and careful monitoring.

Study on the Evaluation of Renal Function According to Set a Partial Region of Interest in 99mTc-DMSA scan of the Pediatric Patient with a Duplicated Ureter (중복요관을 가진 소아환자의 99mTc-DMSA 검사에서 부분적 관심영역 설정에 따른 신기능 평가에 관한 연구)

  • Nam-Koong, Hyuk;Oh, Shin Hyun;Kim, Jung Yul;Choi, Yoon Jung;Kim, Jae Sam;Lee, Chang Ho
    • The Korean Journal of Nuclear Medicine Technology
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    • v.17 no.1
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    • pp.43-47
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    • 2013
  • Purpose: A duplicated ureter is congenital renal malformations with ureter in two. Patients with duplicated ureter are in force to $^{99m}Tc-DMSA$ scan at surgery before and after. In existing examination, at produce result after $^{99m}Tc-DMSA$ scan, didn't compare to upper pole and lower pole with malformed kidney and compared to only relative uptake ratio. Therefore, this study will examine about utility of set a partial region of interest and to functional recovery of renal cell through change of upper pole uptake ratio of malformed kidney by setting each partial region of interest in upper pole and lower pole of malformed kidney in $^{99m}Tc-DMSA$ examination in surgery before and after. Materials and Methods: Pediatric patients with malformed kidney of incomplete duplicated ureter, 15 patients were enrolled in this study. Scanning were scan 3 to 4 hours after injection of $^{99m}Tc-DMSA$ 1.5 ~ 1.9 MBq/kg. Region of interest were each set in normal kidney, upper pole and lower pole with malformed kidney. Region of interest were set with same condition and method to images of surgery before and after that radio technologist 1 person, resident of nuclear medicine 1 person and doctor of urology together. Therefore, this study were compared to uptake ratio (A: B: C) that normal kidney (A), lower pole of malformed kidney (B) and upper pole of malformed kidney (C) about uptake ratio changes of malformed kidney in follow-up examination of surgery before and after. Results: When compared to 15 patients, uptake ratios were increased 7 persons and decreased 8 persons. Among increased 7 persons, it were periods of follow-up examination that 2 persons were 14 months, 4 persons were 12 months and 1 person was 8 months after surgery. Among decreased 8 persons, it were periods of follow-up examination that 4 persons were 12 months 3 persons were 6 months and 1 persons were 4 months after surgery. Conclusion: Existing study could not see the exact uptake ratio changes of malformed kidney because using only the overall Left-Right kidney uptake ratios. But a setting partial region of interest was able to see exactly what changes in the uptake of each upper pole and lower pole of malformed kidney. Because recovery of renal parenchymal cells is difficult in an evaluation of short period of time, follow-up examination should be made in long period of time. How to set up partial region of interest be thought that it would be useful.

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MEDIAN CLEFT OF THE LOWER LIP AND MANDIBLE;A CASE REPORT (하순 및 하악골 정중열의 치험례)

  • Cha, Doo-Won;Kim, Hyun-Soo;Baek, Sang-Heum;Kim, Chin-Soo;Byeon, Ki-Jeong
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.23 no.3
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    • pp.263-269
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    • 2001
  • Median cleft of the lower lip and/or mandible is a rare congenital anomaly, first mentioned by Couronne in 1819. Monroe(1966), Fujino(1970), Ranta(1984) and Oostrom(1996) conducted comprehensive reviews and list cases in literature. Median cleft varies greatly, from a simple vermilion notch to a complete cleft of the lip involving the tongue, the chin, the mandible, the supporting structures of the median of the neck, and the manubrium sterni. The associated anomalies include ankyloglossia, cleft tongue, neck contraction, heart lesion, absence of hyoid bone, and so on. The etiology of median cleft is unknown. Various possibilities, such as failure of mesodermal penetration into the midline, failure of fusion of mandibular processes, external factors apart from the embryogenic pattern such as pressure, position in utero, circulatory failure caused placental adhesion, diseases in pregnancy, and so on, have been discussed. A 8-year-old girl was referred to the Dept. of Oral & Maxillofacial Surgery, Kyungpook National University Hospital and had been aware of the fact that at birth "she had something wrong with her mouth." Shortly after birth she had been examined by a plastic surgeon and at that time surgical procedure had been performed to release the tongue from the lower jaw and lip at local hospital. On admission, she had a slight notching of lower lip and two fibrous frenum ran from the lip along the ventral surface of the tongue, diastema between her mandibular central incisors, and slightly constricted bifid mandible associated independent movement of the two halves of mandible. The patient had autogenous iliac bone graft to reconstruct the mandibular midline defect. The postoperative result was uneventful. In future, the correction of the soft tissue deformities such as notching of the lower lip and partial ankyloglossia will be required for the esthetic and functional improvement.

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Reoperation for congenital heart disease (선천성 심장기형에 대한 2차수술로서의 개심술)

  • Ahn, H.;Sung, S.W.;Kim, Y.J.;Roh, J.R.;Suh, K.P.
    • Journal of Chest Surgery
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    • v.19 no.2
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    • pp.280-287
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    • 1986
  • Between March 1978 and August 1985, 29 cases at various congenital heart diseases were reoperated because of remnant shunt of residual anomalies at Seoul National University Hospital. They were consisted of 10 cases of Tetralogy, 4 simple VSD, 6 complicated VSD. 3 partial ECD, and 5 other rare congenital anomalies. The interval between the initial and the second procedure ranged from 1 day to 122 months [mean; 26.9 months]. In 4 cases of them, the second procedure was done during initial hospitalization within 3 weeks post-operatively. The primary operation intended to be corrective surgery except four whose primary operation was palliative or exploratory one even though it was done with extracorporeal circulation. The indication for second operation was mainly residual shunt or valvular obstruction due to patch detachment or inadequate relief of stenotic lesion. Others were paravalvular leak, valvuloplasty failure, prosthetic valve failure, and inadequate primary diagnosis. Four patients were dead [14.3%]; three complicated VSD`s and one Tetralogy. There were 7 cases of nonfatal complication with subsequent improvement except one [diffuse cerebral dysfunction].

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Pulmonary Trunk Translocation from LV to RV in Double-Outlet Left Ventricle -A case report- (양대혈관 좌심실기시에서 좌심실에서 우심실로의 폐동맥간 전위술의 치험 -1례 보고-)

  • 김상익;최진호;전태국;박표원
    • Journal of Chest Surgery
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    • v.34 no.11
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    • pp.854-857
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    • 2001
  • Double-outlet left ventricle(DOLV) is a rare congenital cardiac malformation, defined as the origin of both the aorta and the pulmonary artery being entirely or predominantly above the morphologically left ventricle, which is difficult to diagnose accurately. A 3-year old male was admitted for cyanosis and dyspnea. At the age of 2 months, he had undergone pulmonary artery banding and coarctoplasty. He was diagnosed as DOLV with subaortic ventricular septal defect(VSD). Biventricular repair was achieved by patch closure of VSD, primary closure of PFO, and pulmonary trunk translocation from left ventricle to right ventricle. The advantages of this procedure using native tissue for right ventricular outflow tract reconstruction are growth potential and preserved valve function, which contribute to a decreased likelihood of reoperation related to the right ventricular dysfunction related to pulmonary insufficiency.

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One-stage Repair of Truncus Arteriosus with Interrupted Arch (대동맥궁 차단증를 동반한 동맥간의 일차 완전교정 - 1예 보고-)

  • 성시찬;박준호;이형두;김시호;우종수;이영석
    • Journal of Chest Surgery
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    • v.36 no.10
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    • pp.759-765
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    • 2003
  • Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25 ∼ 50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

Experimental Study on Congenital Malformations of the Heart and Great Vessels in Rat Fetuses Induced by Nitrofen (임신랫트 태자에서 Nitrofen에 의해 유발된 선천성 심혈관 기형에 관한 실험연구)

  • 김원곤
    • Journal of Chest Surgery
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    • v.20 no.4
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    • pp.659-672
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    • 1987
  • Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

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The Study on Trends and Factors of inpatient care of the province residents provided in Seoul (지방 환자의 서울 지역 입원진료의 추이 및 요인에 관한 연구)

  • Kim, Yoo-Mi
    • Proceedings of the KAIS Fall Conference
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    • 2010.11b
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    • pp.755-758
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    • 2010
  • 본 연구의 목적은 지방 환자의 서울 지역 입원진료의 추이를 파악하고 그 요인을 규명하는 데 있다. 이를 위해 2005년 및 2008년 환자조사 입원자료를 이용하였으며, 서울지역 거주 환자를 제외하고 2005년 333,280명, 2008년 419,873명을 연구대상으로 하였다. 자료분석은 기술통계, 카이제곱 검정, 로지스틱 회귀분석을 실시하였다. 2005년 대비 2008년 성별, 연령별, 의료기관 유형 등 일반적 특성의 분포는 유사한 것으로 나타났다. 지방 환자의 서울지역 이용은 다소 증가한 것으로 나타났으며, 서울 지역 입원진료는 남자, 중장년층 건강보험환자가 타기관에서 의뢰되어 외래를 통해 입원하며, 주 거주지가 경기, 강원, 충북, 충남, 제주지역 순으로, 광역시는 상대적으로 낮았다. 질병군별로는 선천성 기형, 신생물, 종양이나 수술후 추후치료, 눈 질환, 혈액 조혈 면역기 질환, 근골격계 질환 순으로 지방환자의 서울지역 의료기관 입원 이용률이 높았다. 그러나 상대적으로 지방 입원진료 확률이 높은 노년층, 의료급여, 응급경유, 질병군별로 중증도가 높은 환자가 혼재되어 있어 있을 가능성이 있어 향후 중증도 보정에 대한 심층 연구가 필요한 것으로 판단된다.

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