• Title/Summary/Keyword: situs inversus

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Clinical Study of 61 cases Benign Lung Tumor (폐양성 종양 61례의 외과적 고찰)

  • 박종원
    • Journal of Chest Surgery
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    • v.22 no.2
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    • pp.272-289
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    • 1989
  • Benign lung tumors compose a heterogeneous group of solid growths that present variations in clinical features, depending on whether the origin is within the bronchus on lung parenchyma or from visceral pleura. Benign tumors of the lung are relatively uncommon, and series are to be found in the literature and the classification of benign tumors of the lung continues to be controversial because of disagreement concerning the origin and prognosis of many common lesions. We adopt Liebows original classification but excluded bronchial adenoma which no longer considered as benign tumor and added pulmonary A-V fistula and congenital cystic adenomatoid malformation. We analyzed 61 cases of benign tumors which were composed of 16 original Korean cases and 45 cases which were reported on journal of Thoracic & Cardiovascular Surgery. The results were. [1] Incidence; Of 61 cases, chondromatous hamartoma was 2 cases [41 %], congenital cystic adenomatoid malformation 10 cases [16.4 %], pulmonary A-V fistula 5 cases [8.1 %], sclerosing hemangioma 4 cases [6.5 %], teratoma, plasma cell granuloma & mesothelioma were 3 cases [4.9%], Castlemans disease 2 cases [3.3%], and mucous gland adenoma, paraganglioma, and leiomyoma 1 case [1.6 %]. [2] Age & Sex distribution; Male 30 cases and female 31 cases. Mean age was 31.4 years old. [3] Main symptom; was coughing, 32.8%, and no symptom, 24.6%. [4] Sixty eight percentage of chest film showed mass density, and 4 cases showed calcification, 2 cases had lobulation. [5] Size of mass was large and multiple mass was 2 cases. Endobronchial tumors were 9 cases, 14.9 %. [6] Three cases of endobronchial tumor were preoperatively diagnosed by bronchoscopy and 2 cases of pulmonary A-V fistula were diagnosed by pulmonary arteriography. [7] Seven cases, 11.5%, had associating diseases such as bronchogenic cyst, thymic cyst, Schwannoma, situs inversus, bronchiectasis and bronchogenic carcinoma. [8] Minor resection such as excision 8i: wedge resection were 15 cases, 26.2 %, and 6 cases, 75.4 %, of lobectomy were performed including 5 cases of pneumonectomy 5 cases had. [9] Postoperative complications; One case, 1.6 %, expired due to respiratory insufficiency. Two cases had re-operation due to bleeding and hemoptysis.

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A Case of Kartagener's Syndrome Presenting as Respiratory and Right Heart Failures (호흡부전과 우심부전을 동반한 Kartagener 증후군 1예)

  • Yang, Suck-Chul;Lee, Kyung-Sang;Yoon, Ho-Joo;Shin, Dong-Ho;Park, Sung-Soo;Lee, Jung-Hee;Park, Choong-Ki
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.2
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    • pp.251-256
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    • 1996
  • Kartagener's syndrome, a congenital disease transmitted as an autosomal recessive illness with a prevalence of approximately 1:20,000 persons, is characterized by the triple association of situs inversus, bronchiectasis, and sinusitis. Affected persons have an incoordination of ciliary motility that leads to defective mucociliary transport, chronic bronchial infections. Kartagener's syndrome is a subset of the immotile cilia syndrome and therefore all patients with Kartagener's syndrome have immotile cilia with obvious ultrastructural defects in the ciliary axoneme. In the respiratory tract this inability presumably causes impaired clearance of mucus and inhaled particles and results in the chronic infections of the sinuses and bronchial trees that are characterized of the disease. The end-stage phenomenon in Kartagener's syndrome, respiratory or heart failure is a less common event and heart-lung transplantation is becoming an accepted therapy for patients with end-stage pulmonary disease in Kartagener's syndrome in many institutes. We report one case of Kartagener's syndrome in a 25-year-old young woman who was presented as respiratory and right heart failures, with review of literatures.

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Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome

  • Kim, Jung Hoon;Song, Won Jun;Jun, Ji Eun;Ryu, Duck Hyun;Lee, Ji Eun;Jeong, Ho Jung;Jeong, Suk Hyeon;Kang, Hyung Koo;Kim, Jung Soo;Lee, Hyun;Chon, Hae Ri;Jeon, Kyeongman;Kim, Dohun;Kim, Jhingook;Koh, Won-Jung
    • Tuberculosis and Respiratory Diseases
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    • v.77 no.3
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    • pp.136-140
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    • 2014
  • Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.

Operative Treatment of Congenitally Corrected Transposition of the Great Arteries(CCTGA) (교정형 대혈관 전위증의 수술적 치료)

  • 이정렬;조광리;김용진;노준량;서결필
    • Journal of Chest Surgery
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    • v.32 no.7
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    • pp.621-627
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    • 1999
  • Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

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