• Journal of Chest Surgery
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• 제23권6호
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• pp.1263-1269
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• 1990

Two patients with uncorrectable cyanotic cardiac anomalies underwent total cavopulmonary shunt[modified Fontan operation]. Case I was a 14 years old male with dyspnea and cyanosis after birth. Aortogram showed TGA combined with overriding of aorta, pulmonary stenosis, complete atrioventricular septal defect, interruption of inferior vena cava, and situs inversus totalis. We had performed total cavopulmonary shunt using with 16 mm Gortex Graft in single atrium to bypass the hepatic vein to pulmonary artery. Postoperatively, patient sustained low PaCO2 and low cardiac output and then expired at 19th postoperative day. The cause of death of the patient would be low cardiac output. Case II was a 6 years old female with dyspnea and cyanosis after birth. Aortogram showed tricuspid atresia[Type IIb], transposition of great arteries, atrial septal defect, ventricular septal defect and pulmonary stenosis, We had performed total cavo-pulmonary shunt using intraatrial baffle[tunnel] with Goretex patch. The postoperative course of this patient was good without event.

• Journal of Chest Surgery
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• 제12권3호
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• pp.159-164
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• 1979

Kartagener`s syndrome is a clinical entity comprising a combination of situs inversus, bronchiectasis, and sinusitis or nasal polyposis. This syndrome is rare and is usually seen in a young age group. The syndrome is punctated by recurrent upper respiratory tract infection and pneumonia. This is a report of Kartagener`s syndrome found in 18 years old male and 21 years old female patients who were received surgical treatment of bronchiectasis. The male patient was performed right transposed lingular segmentectomy and lower lobectomy and female patient was perforated left transposed middle lobectomy and lower lobectomy. Both patients were discharged with good results.

• 대한구순구개열학회:학술대회논문집
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• 대한구순구개열학회 2002년도 종합학술대회
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• pp.47-47
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• 2002

• 대한기관식도과학회지
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• 제4권1호
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• pp.132-136
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• 1998

Nonrecurrence of the inferior laryngeal nerve always results from a vascular anomaly during embryonic development of the aortic arches. The nonrecurrent inferior laryngeal nerve is important clinically for two reasons, it is vulnerable during thyroid surgery and it is associated with difficulty in swallowing. It can be suspected preoperatively from signs associated with the vascular anomaly : dysphagea, thoracic x-ray images showing evidence of retroesophageal subclavian artery, or situs inversus viscerum. If such signs are noted, a barium swallow test and chest computed tomography are justified. We experienced a case of Rt. nonrecurrent inferior laryngeal nerve, which is diagnosed thyroid papillary carcinoma.

• Clinical and Experimental Pediatrics
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• 제45권5호
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• pp.622-628
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• 2002

목 적 : 저자들은 이차적인 병변으로 인한 우심증이 아닌 환아들에서 그들의 임상양상, 동반 심장 또는 심장외 기형과 예후를 분석하고자 하였다. 방 법: 1996년 1월에서 2001년 7월까지 계명대학교 동산의료원 소아과에서 흉부 방사선 소견상 심장이 우측 흉곽에 위치하였던 환아 중 폐나 흉곽 병변에 의한 우전위를 제외한 24례를 대상으로 하였다. 모든 환아들에게 심에코 검사와 복부 초음파 검사를 시행하였고 필요에 따라 심장 조영술, 염색체 검사를 시행하였으며 이들의 진단 당시의 연령과 임상 증상, 이학적 소견, 동반하는 심기형 뿐 아니라 심장외 기형, 치료 후 예후를 조사하였다. 결 과: 총 24례 중 남아가 17례(79.8%)이었으며, visceroatrial situs로 보았을 때 situs solitus가 7례, situs inversus가 10례, situs ambiguous가 7례이었다. 우심증을 처음 발견한 시기는 생후 7일 이내가 21명(87.5%)으로 대부분을 차지하였다. 진단 당시의 증상으로는 청색증과 심잡음이 각각 11례(45.8%)로 가장 많았고 11례(45.8%)는 증상 없이 우연히 발견되었다. Situs solitus와 inversus의 경우는 우연히 발견된 경우가 증상을 보여 진단된 경우보다 많았으나 situs ambiguous의 경우는 청색증(71.4%) 등의 증상으로 발견된 경우가 많았다. 19례(79.2%)에서 심기형을 동반하고 있었으며 situs solitus와 ambiguous는 전례에서 심기형을 동반하였고 situs inversus는 50%에 서 심기형을 동반하였으며 동반된 심기형 중 폐동맥 협착이나 폐동맥 폐쇄가 12례로 가장 많았다. 심장외 다른 기형이 동반된 경우는 7례(29.2%)로 원선, 비후성 유문 협착증, 요도 하열, 수신증, 다지증, 만곡지 등이었으며 염색체 검사를 시행한 4례는 모두 정상이었다. 총 24례 중 11(45.8%)례는 심기형이나 동반된 다른 선천성 기형으로 사망하였으며, 특히 situs solitus와 ambiguous에서 사망률이 높았다. 결 론 : 우심증의 첫 진단 시기는 1주 이내가 많았고, 이들 중 증상 없이 우연히 발견된 경우도 약 반수로 많았으며, situs solitus와 ambiguous는 동반된 심장기형이 100%로 situs inversus의 50%보다 많았으며 예후 또한 불량하였다.

• 한국발생생물학회지:발생과생식
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• 제14권2호
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• pp.91-97
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• 2010

FOXL2는 winged-helix/forkhead(FH) 도메인 전사인자로서 FOXL2 유전자에 돌연변이가 발생할 경우 blepharophimosis-ptosis-epicanthus inversus syndrome이라 불리는 BPES 질병이 유발되게 된다. BPES는 상염색체 우성인 유전적 질환이다. BPES type I의 환자는 조기난소부전증(POF)과 안검하수 증상이 함께 나타나는 반면, BPES type II의 경우 안검하수 및 소안검 등 안면기형만이 유발된다. FOXL2 단백질이 결여된 난소에서 granulosa 세포의 분화가 멈추는 것으로 보아 FOXL2가 정상적인 난소의 folliculogenesis에 필수적인 역할을 하고 있음을 시사한다. 이전의 연구 결과에서, 본 연구진은 FOXL2와 상호작용하는 단백질에 대한 스크리닝을 통해 스테로이드 합성효소인 CYP19 전사활성에 영향을 미치는 steroidogenic factor-1(SF-1)을 동정하였다. 이번 연구를 통해 FOXL2가 CYP19의 전사를 향상시키고, SF-1에 의한 CYP19의 전사를 더욱 촉진시킨다는 것을 증명하였다. 이와 반대로, BPES 타입 I과 II에서 발견된 FOXL2의 돌연변이형들은 SF-1에 의해 증가된 CYP19의 전사활성을 향상시키는 능력이 감소함을 보여주었다. 본 실험을 통해 FOXL2 돌연변이에 의해 유발되어지는 BPES 질환의 병리생리학적인 이해에 대해 도움을 줄 수 있는 FOXL2의 야생형과 돌연변이형 사이의 서로 다른 기능적인 차이점을 규명하였다.

• Journal of Chest Surgery
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• 제11권3호
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• pp.342-347
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• 1978

A 8 year old male was admitted to the Department of Thoracic Surgery, Korea University Hospital on June 22, 1978. The chief complaints were cyanosis and exertional dyspnea since at birth. EKG shows BVH and dextrocardia, phonocardiogram revealed the accentuation of second heart sound in aortic area. Echocardiogram from the left ventricle to the base of the heart, there is a discontinuity between the ventricular septum and the anterior aortic margin with a large aortic root & aortic overriding. His cardiac catheterization data and cardiac angiogram shows situs inversus totalis, dextrocardia, right aortic arch, large ventricular septal defect etc., and finally diagnosed Truncus Arteriosus. Edwards type IV with retrograde aortogram and selective bronchial angiogram. This is the first operative case reported as Rastelli operation for Truncus Arteriosus type IV in the literatures in Korea. Authors have experienced I case of Truncus Arteriosus, Edward type IV and Rastelli operation with Dacron Arterial Conduit Graft under cardiopulmonary bypass on July 3, 1978. The procedures were as follows; 2] Cardiopulmonary bypass: Origin of bronchial arteries excised from descending aorta bilaterally; defects in aorta closed. 2] Horizontal incision made high in right ventricle. 2] Ventricular septal defect [Kirklin type I+II] closed with Teflon patch. 4] Bifurcated dacron arterial graft with pericardial monocusp sutured to the bilateral pulmonary arteries. [Diameter 9 mm: Length 7 cm]. 5] Proximal end of the conduit graft anastomosed to right ventricle. [Diameter 19 mm: Length 5 cm]..Total perfusion time was 220 min. The result of operation was poor due to anastomotic leakage and increased pulmonary vascular resistance resulting acute right heart failure. The patient was died on the operation table. Literatures were briefly reviewed.

• Journal of Chest Surgery
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• 제11권3호
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• pp.333-341
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• 1978

A 8 year old male was admitted to the Department of Thoracic Surgery, Korea University Hospital on June 22, 1978. The chief complaints were cyanosis and exertional dyspnea since at birth. EKG shows BVH and dextrocardia, phonocardiogram revealed the accentuation of second heart sound in aortic area. Echocardiogram from the left ventricle to the base of the heart, there is a discontinuity between the ventricular septum and the anterior aortic margin with a large aortic root & aortic overriding. His cardiac catheterization data and cardiac angiogram shows situs inversus totalis, dextrocardia, right aortic arch, large ventricular septal defect etc., and finally diagnosed Truncus Arteriosus. Edwards type IV with retrograde aortogram and selective bronchial angiogram. This is the first operative case reported as Rastelli operation for Truncus Arteriosus type IV in the literatures in Korea. Authors have experienced I case of Truncus Arteriosus, Edward type IV and Rastelli operation with Dacron Arterial Conduit Graft under cardiopulmonary bypass on July 3, 1978. The procedures were as follows; 2] Cardiopulmonary bypass: Origin of bronchial arteries excised from descending aorta bilaterally; defects in aorta closed. 2] Horizontal incision made high in right ventricle. 2] Ventricular septal defect [Kirklin type I+II] closed with Teflon patch. 4] Bifurcated dacron arterial graft with pericardial monocusp sutured to the bilateral pulmonary arteries. [Diameter 9 mm: Length 7 cm]. 5] Proximal end of the conduit graft anastomosed to right ventricle. [Diameter 19 mm: Length 5 cm]..Total perfusion time was 220 min. The result of operation was poor due to anastomotic leakage and increased pulmonary vascular resistance resulting acute right heart failure. The patient was died on the operation table. Literatures were briefly reviewed.

• Journal of Chest Surgery
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• 제15권3호
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• pp.346-354
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• 1982

A Complete transposition of the great arteries combined with V.S.D, A.S.D, dextrocardia, and P.S is a rare congenital anomaly. The patient was a 10 year-old female whose complaints were frequent URI, exertional dyspnea, and cyanosis at rest since birth. Cheat X-ray films showed Dextrocardia ; situs inversus, moderate cardiomegaly, and Characteristic egg-shape heart shadow. E.K.G, Echocardiography, Cardiac Catheterization, and Angio-Cardiography were performed. Open heart Surgery was done under diagnosis of d-TGA, Dextrocardia, V.S.D, A.S.D, and P.S. At the time of Operation, Dextroeardia, T.G.A, Secndum type A.S.D, A.S.D, and P.S. At the time of Operation, Dextroeardia, T.G.A, Secndum type A.S.D. ($2.0{\times}2.0cm$. in diameter), V.S.D. type II ($1.5cm{\times}1.5cm$ in diameter), and pulmonary valvular stenosis were noted. Mustard operation using pericardial Baffle in the atrium for T.G.A. was perforsned. Teflon patch graft for closure of V.S.D. through tricuspid orifice and pulmonary Valvulotomy through pulmonary arteriotomy were performed. The post-op, low cardiac output Syndrome and dysrhythmicawere developed till Postoperative day #7, so that was controlled by inotropic and antiarrhythmic agents. After that, patient's conditions were Uneventful.

• 대한핵의학회지
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• 제20권1호
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• pp.39-43
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• 1986

[ $^{99m}Technetium-Heat$ ] damaged erythrocyte were used as spleen scanning agents in 12 patients from July, 1985 to April, 1986. We used this scan to evaluate situs inversus, asplenia, accessory spleen, hypersplenism, splenic infarction, tumor staging and evaluation of therapy, especially when the $^{99m}Tc-tin$ colloid scans were not definite for diagnosis. The techniques applied to these scans were in vivo/in vitro-labeling method and heating-method to damage the erythrocytes. Liver-to-spleen uptake ratios were increased upto 100 : 1 and interference from the left lobe of the liver was eliminated. These scans were helpful to evaluate the spleen.