• Journal of Chest Surgery
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• v.30 no.1
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• pp.55-60
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• 1997

We reviewed 40 cases of primary mediastinal tumors which were operated on at Seoul Paik Hospital from September, 1987 to December, 1995. Of these, 18 were male and 22 were female. The patient ranged in age from 4 years to 68 years with a mean age of 34.1 years. The most common symptoms included chest pain(12.5%), cough(12.5%), dyspnea(7.5%). and palpable neck mass(7.5%), and symptoms were absent at the time of diagnosis in 37.5% of cases. Chest roentgenography and computed tomography(CT) were performed in all patients, and magnetic resonance imaging(MRI) in 5 patients, and transthoracic needle aspiration (TTNA) performed In 22 patients. The sensitivity of TTNA was 72.7%(16 of 22 patients). The lesion was located 60% in the anterosuperior mediastinum, 35% in the posterior mediastinum, and 5% in the middle mediastinum. The primary tumors included thymic neoplasms(11 cases), germ cell tumors(7 cases), neurogenic tumors(10 cases) and a miscellaneous group. The malignant tumors(12.5%) were invasive thymoma(3 cases), spindle cell sarcoma(1 case), and non-Hodgkin's Iymphoma(1 case). A complete excision was done in all 35 benign tumors and 3 malignant tumors. There was no operative mortality, and postoperative complications occurred in 3 cases.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.141-147
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• 2016

Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

• Pediatric Infection and Vaccine
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• v.9 no.2
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• pp.201-207
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• 2002

Purpose : We analyzed clinical features and causal viruses of acute lower respiratory tract infections(LRTIs) in order to improve the management of these infections. Methods : From end of April to early May 2001, amongst 30 children at a local institute for children's adoption and welfare, 13 were admitted to the hospital with the diagnostic impression of acute LRTIs. Nasopharyngeal aspirates were sent in Seoul National University Hospital for viral culture of respiratory syncytial virus(RSV), adenovirus, parainfluenza virus. Results : One or more viral agents were identified in 4 cases(30.7%) : were RSV(15.4%), adenovirus(7.7%), and a mix of these two viruses(7.7%). Initial symptoms were fever(69%), cough(100%), tachypnea(54%), chest retraction(69%), rale(85%) and wheezing(15%). Leukocytosis was noted in 23%, CRP increased more than 10 mg/L in 46%. Chest X-ray abnormalities were 69%. Conclusion : Although viruses were identified in 30.7%, further studies should be made for prevention and treatment of acute viral LRTIs.

• Journal of Chest Surgery
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• v.29 no.5
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• pp.564-568
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• 1996

Pseudoaneurysm of the ascending aorta following cardiac surgery is very unusual and it is poten- tially fatal. We report here a fourteen year-old female patient with pseudoaneurysm of the ascending aorta following a repair of a congenital ventricular septal defect at other hospital 50 months ago. Although she had a mild superficial wound infection postoperatively, she enjoyed uneventful. life until she visited our hospital for a generalized weakness and exertional dyspnea which developed a month ago. Chest CT and echocardiogram showed partially calcified pseudoaneurysm of the ascending aorta. Two aortic defects were located on the anterolateral ascending aortic wall wkere it was suspected as a previous sites of aortic and cardioplegic cannulation. The internal wall of the pseudoaneurysm was covered with neoendothelium and intervened by septal tissue. Two defects on he aortic wall were oval in shape and about 1.5cm in the greatest diameter The defects were trimmed to make a one large de- fect and it was reconstructed with patch designed from 22mm collagen impregnated double velour Dacron graft. The postoperative course was uneventful.

• Journal of Chest Surgery
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• v.39 no.11 s.268
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• pp.822-827
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• 2006

Background: Lung transplantation is a definitive therapy for a variety of end stage lung diseases. Since 1996, we have performed thirteen cases of lung transplantation including two retransplantations, and we analyzed the outcomes, complications, and survivals of these patients. Material and Method: We retrospectively analyzed the medical records of thirteen cases from July, 1996 to July, 2005. Result: During the period, 11 patients had undergone 43 lung and heart-lung transplantations, and two patients had retransplantation due to allograft failure. Mean age of recipients were $45.2{\pm}10.7$ years(range, $25{\sim}59$). Early complications were bleeding, reperfusion injury, and infection and late complications were mainly infection and post-transplantation lymphoproliferative disease. Excluding the operative mortality, the mean survival period was 16.5 months($2{\sim}60$ months). Two retransplantations had been performed 2 weeks and 13 months after single lung transplantations. Conclusion: In order to achieve long term survival, early detection of complications and proper treatment in addition to surgical skills are necessary, and these efforts can promote better lung transplantation programs in the near future.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.574-577
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• 2007

Primary malignant neoplasm of the pericardium is very rare, Neoplastic involvement of the pericardium may result in rapidly developing hemorrhagic effusion. A 30-year-old male who occasionally suffered from chest tightness was referred to our hospital under the diagnosis of unstable angina. He presented with acute chest pain and severe dyspnea that had developed one day previously. The diagnostic investigations such as echocardiography, chest CT and magnetic resonance image suggested cardiac tamponade that was caused by rupture of the pericardial teratoma. An operation to remove the tumor and effusion was performed. The pericardial mass was completely excised, and the result of the frozen biopsy favored malignancy. The final pathologic report was malignant fibrosarcoma of the pericardium and no malignant cells were found on the cytology of the pericardial effusion. The patient had a smooth postoperative course and was referred to another hospital for additional radiation therapy. We report here on this case of cardiac tamponade that was caused by primary pericardial fibrosarcoma, and this required urgent diagnosis and surgical management.

• Tuberculosis and Respiratory Diseases
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• v.46 no.4
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• pp.455-465
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• 1999

Background: Even though lung cancer has become a major cancer in Korea, national survey for lung cancer has not been available except several reports from individual hospitals. Methods: Korean Academy of Tuberculosis and Respiratory Diseases retrospectively investigated the characteristics of lung cancer diagnosed from January 1997 to December 1997 at general hospitals over 400 beds. Results: Among 3,794 patients, 76.8% are smokers and 89.8% of male patients are smokers. Squamous cell carcinoma is the leading type of lung cancer(44.7%) followed by adenocarcinoma(27.9%). Smoking rate in adenocarcinoma was significantly lower than in squamous cell carcinoma and small cell cancer. Cough is the most common symptom, however, 7.2% are asymptomatic. Bronchoscopic biopsy has a main role in the diagnosis of squamous cell carcinoma and small cell cancer but percutaneous needle biopsy has more important role in adenocarcinoma. Two-thirds of NSCLC patients were found in unresectable advanced stages. Conclusion: In contrast to other countries, squamous cell carcinoma is still the most frequent type of lung cancer. High proportions of smoker and advanced, unresectable lung cancer urge us to develop the program for cessation of smoking and early detection.

• Tuberculosis and Respiratory Diseases
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• v.53 no.3
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• pp.337-343
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• 2002

A tracheal bronchus, an aberrant bronchus arising directly from the trachea, is an infrequent congenital anomaly. The incidence of this anomaly ranges from 0.5 to 5%. It usually originates from the right lateral wall of the trachea at the level <2 cm above the tracheal bifurcation. These patients usually are asymptomatic, but some patients may experience recurrent pneumonia, chronic bronchitis, bronchiectasis, or asthmatic episodes. A tracheal bronchus may be associated with other anomalies such as a tracheal stenosis, pulmonary agenesis, pulmonary sequestration, congenital heart disease, a pulmonary venous anomaly and Down's syndrome. This anomaly is usually diagnosed incidentally during bronchoscopy in patients with respiratory problems. Here we report a case of a 20-year-old man with a past history of bronchial asthma, which was incidentally diagnosed as a tracheal bronchus during a medical examination prior to military service, and was associated with a bilateral superior vena cava anomaly.

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.103-109
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• 1999

Respiratory bronchiolitis-associated interstitial lung disease has been described among current or former smokers and has features consistent with interstitial lung disease. Symptoms include cough, dyspnea on exertion. Lung pathology is characterized by the accumulation of pigmented macrophages within respiratory bronchioles and adjacent air spaces associated with mild thickening of the peribronchiolar interstitium. It must be separated from the other interstitial lung disease because of marked differences in treatment and prognosis. Recently we experienced a case of respiratory bronchiolitis-associated interstitial lung disease in a 48-year-old man. As far as we know, this is the first case in Korea.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.805-811
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• 1996

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.