• 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.52 no.1
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• pp.86-91
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• 2002

A bronchial artery aneurysm(BAA) is rare, and has an unclear etiology. However, it may be caused by congenital abnormalities and acquired diseases like as bronchiectasis, tuberculosis, and other infections. The pathogenesis of a bronchial artery dilatation and the formation of an aneurysm results in an increase in the systemic blood flow to the chronic inflammatory pathologic lungs such as bronchiectasis or tuberculosis. It can be divided into the mediastinal and intrapulmonary BAA according to their location. The most common symptom is hemoptysis. Chest computed tomography and bronchial artery angiography may be used for a diagnosis. Treatment is mainly by a surgical resection of the aneurysmal artery. However, when patient is unstable due to massive hemoptysis or recurrent hemoptysis, bronchial artery embolization is useful. Here, we experienced a case of a bronchial artery aneurysm presenting as a massive hemoptysis.

• Tuberculosis and Respiratory Diseases
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• v.65 no.2
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• pp.110-115
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• 2008

Background: Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital developmental anomaly of the lower respiratory tract. Most cases are diagnosed within the first 2 years of life, so adult presentation of CCAM is rare. We describe here six adult cases of CCAM and the patients underwent surgical resection, and all these patients were seen during a five and a half year period. The purpose of this study was to analyze the clinical, radiological and histological characteristics of adult patients with CCAM. Methods: Through medical records analysis, we retrospectively reviewed the clinical characteristics, the chest pictures (X-ray and CT) and the histological characteristics. Results: Four patients were women and the mean age at diagnosis was 23.5 years (range: 18~39 years). The major clinical presentations were lower respiratory tract infection, hemoptysis and pneumothorax. According to the chest CT scan, 5 patients had multiseptated cystic lesions with air fluid levels and one patient had multiple cavitary lesions with air fluid levels, and these lesions were surrounded by poorly defined opacities at the right upper lobe. All the patients were treated with surgical resection. 5 patients underwent open lobectomy and one patient underwent VATS lobectomy. On the pathological examination, 3 were found to be CCAM type I and 3 patients were CCAM type II, according to Stocker's classification. There was no associated malignancy on the histological studies of the surgical specimens. Conclusion: As CCAM can cause various respiratory complications and malignant changes, and the risks associated with surgery are extremely low, those patients who are suspected of having or who are diagnosed with CCAM should go through surgical treatment for making the correct diagnosis and administering appropriate treatment.

• Tuberculosis and Respiratory Diseases
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• v.57 no.5
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• pp.489-493
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• 2004

Unilateral pulmonary artery agenesis is a rare congenital anomaly usually associated with other cardiovascular anomalies such as tetralogy of Fallot or septal defect. Unilateral pulmonary artery agenesis without other coexisting cardiovascular abnormality (isolated unilateral pulmonary artery) is extremely rare and often asymptomatic until adulthood. In these patients, diagnostic clue is found in a plain chest roentgenogram, showing a hyperlucent contracted hemithorax. We have recently experienced a case of isolated right pulmonary artery agenesis, which was diagnosed by chest dynamic CT, perfusion scan, echocardiogram and 3-dimensional reconstruction cardiac CT angiography in a 50-year old female who had suffered from mild dyspnea on exertion and improved with conservative treatment. We report this case with a brief review of the relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.45 no.3
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• pp.649-653
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• 1998

Pulmonary arteriovenous fistula is a congenital vascular malformation in lung resulting from abnormal capillary development and the exact etiology is not well known but it may occur with or without hereditary hemorrhagic telangiectasia. Hereditary hemorrhagic telangiectasia (HHT) or Rendu-osler-Weber disease is an autosomal dominant vascular disorder which associates epistaxis, mucocutaneous and visceral telangicetases, and recurrent hemorrhage with chronic anemia and visceral shuntings. Recently we experienced a case of the pulmonary arteriovenous fistula in a 23 year-old woman with a family history of this disease, which was confirmed by pulmonary angiography.

• Tuberculosis and Respiratory Diseases
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• v.47 no.2
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• pp.259-264
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• 1999

The scimitar syndrome, a rare complex anomaly, is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. The shape of the Turkish curved sword (scimitar) has provided the name of this syndrome. Additional characteristics of this syndrome such as hypoplasia of the right lung and of the right pulmonary arterial tree, anomalous arterial supply of the right lung from the aorta, dextrocardia and bronchial anomalies are common. Recently we experienced a case of scimitar syndrome (adult form) in a 19-year-old woman patient, so we report the case with a brief review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.68 no.6
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• pp.358-362
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• 2010

Intralobar pulmonary sequestration is a rare congenital lung anomaly. It is defined as a portion of nonfunctioning lung parenchyma that receives its blood supply from an anomalous systemic artery. Patients often present with chronic or recurrent pneumonia. A chest radiograph may show a cystic lesion with air-fluid levels in the lung base. A high index of suspicion is needed for a diagnosis. Surgical removal of a symptomatic intralobar pulmonary sequestration is generally the treatment of choice. Identifying the aberrant artery is a difficult problem when resecting a pulmonary sequestration. The thoracic and abdominal aortas are the most common origins for the abnormal blood supply. However, arterial supply from the celiac artery is quite rare. We present a case of intralobar pulmonary sequestration with the blood supply originating from the celiac artery.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.548-553
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• 1992

The Unilateral absence of a pulmonary artery (UAPA) is an uncommon congenital anomaly. Approximately 160 cases have been reported in the literature since Frantzel's first report in 1968. Most of the patients with UAPA are asymptomatic but some patients may suffer from recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis could be suspected from the chest roentgenogram and lung scan, and definitely confirmed by pulmonary angiography. We experienced a case of UAPA in a 39-year-old male with the recurrent hemoptysis. Chest X-ray revealed that the left lung volume was moderately decreased and the heart and mediastinum were displaced to the left side. Lung perfusion scan showed that the left lung was not perfused. Pulmonary angiography revealed the absence of the left main pulmonary artery. Aortic arch and descending aorta on aortogram were right sided. Blood supply to the left lung was originated from numerous systemic collaterals from intercostal and brachiocephalic origin. No other intrinsic or internal abnormalities of the cardiac chambers were noted.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.42 no.5
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• pp.767-771
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• 1995

The bronchial artery-pulmonary vein malformation should be called the systemic artery-to-pulmonary vein arterioveonus malformation in the lung. Although pulmonary arteriovenous malformation has been well documented in intrapulmonary arteriovenous malformation, the systemic artery-to-pulmonary vein arteriovenous malformation is rare. Most patients with systemic artery-to-pulmonary vein arteriovenous malformation is asymptomatic and the diagnosis of these anomaly may be done by continuous murmur or abnormal chest X-ray on the physical examination. The pathogenesis of this condition is congenital malformation which explains these anastomoses between the pulmonary vein and accessory brachial arteries and acquired malformation which explains development of new blood vessel to supply large enough to cause significant systemic-pulmonary shunts due to inflammation secondary to infection, trauma, or previous surgery. We experienced a case of the bronchial artery-pulmonary vein malformation which was detected on angiography in 20-year-old women whose chief complain is hemoptysis. This massive hemoptysis was controlled by selective brachial artery embolization with Gelfoam and Ivalon particles.