• Journal of Chest Surgery
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• v.26 no.8
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• pp.646-649
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• 1993

Bronchiectasis in infant is very rare. We have experienced an infant who underwent bilobectomy due to bronchiectasis. This patient had immunoglubulin A deficiency, cytomegalovirus infection and recurrent aspiration. We review literatures and present here.

• The Korean Chronic Disease News
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• no.116
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• pp.9-9
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• 1990

• The Korean Chronic Disease News
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• no.61
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• pp.7-7
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• 1986

• Tuberculosis and Respiratory Diseases
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• v.54 no.6
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• pp.628-634
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• 2003

Bronchiectasis is defined as an abnormal, irreversible dilatation of the bronchi, which may result from a number of possible causes, and the recognition of these causes may lead to a specific management strategy. Immunodeficiency is known as one of the conditions associated with bronchiectasis. X-linked agammaglobulinemia is a rare inheritable immunodeficiency disorder, caused by a differentiation block, leading almost to the complete absence of B lymphocytes and plasma cells. The affected protein is a cytoplasmic protein tyrosine kinase, Bruton's tyrosine kinase. The early detection and treatment with immunoglobulin replacement are most important for the management of recurrent infections and for reducing severe complications. We report a 20-year-old male patient, with X-linked agammaglobulinemia associated with bilateral bronchiectasis, carrying a missense mutation(R520P) in the BTK gene.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.510-513
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• 2003

Annuloaortic ectasia, aortic regurgitation(AR), and ventricular septal defect(VSD) in patients with right ventricular hypoplasia is a very rare condition. We report a patient who underwent aortic root replacement with a composite graft for annuloaortic ectasia associated with VSD and AR in right ventricular hypoplasia. The patient was a 19 year-old male. Transthoraic echocardiogram and cardiac catheterization revealed a perimembranous VSD (2 cm in diameter), severe AR, annuloaortic ectasia, bipartite right ventricle with hapoplasia, and hypoplastic tricuspid valve. Operative findings showed that free margins of the right and noncoronary cusps were markedly elongated, thickened, and retracted, and commissure between the right coronary cusp and the noncoronary cusp was fused and calcified. VSD was closed with an autologous pericardial patch and composite graft aortic root replacement using direct coronary button reimplantation was performed, and the hypertrophic muscle of the right ventricular outflow tract was resected. The patient had transient weaning failure of cardiopulmonary bypass and was discharged at the postoperative 14 days without any problems.

• Journal of Chest Surgery
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• v.37 no.4
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• pp.364-368
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• 2004

Congenital pulmonary vein stenosis is a rare anomaly and related to high mortality due to progressive pulmonary hypertension and heart failure in infancy. Aggressive anti-failure medication and surgical treatment is recommended. Surgical options are balloon dilatation, endovascular stent, pneumonectomy, lung transplantation, patch grafting, and sutureless repair. We report a case of congenital pulmonary vein stenosis with normal anatomical connection successfully treated with sutureless technique and using pulmonary vasodilators, such as Sildenafil, lloprost and iNO postoperatively.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.847-850
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• 1999

Tracheobronchomegaly or Mounier-Kuhn syndrome a is rare disease characterized by marked dilatation of the trachea and main bronchi. It is thought to be due to a congenital anomaly, but is uncertain. It has variable clinical manifestations from causing chronic respiratory infections and bronchiectasis to being asymptomatic for the lifetime. Recently, we experienced a case of Mounier-Kuhn syndrome patient with tracheal diverticulum, who had lung cancer. Our case is reported with literature reviews.

• Journal of Chest Surgery
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• v.11 no.2
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• pp.181-184
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• 1978

A case of idiopathic dilatation of Pulmonary artery with calcification is presented. The idiopathic dilatation of pulmonary artery has been recognized as a clinical entity since 1923, when Wessler and Jaches described the first case. Recently we experienced one case of Idiopathic Dilatation of Pulmonary artery with calcification which was very rare, and corrected with surgical treatment successfully.

• Proceedings of the KTCVS Conference
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• 1998.10a
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• pp.60-60
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• 1998

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.253-259
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• 2004

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.