• Tuberculosis and Respiratory Diseases
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• v.39 no.2
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• pp.186-193
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• 1992

Eosinophilic granuloma (histiocytosis $\times$) is an uncommon granulomatous disorder affecting the mononuclear phagocytic cell system caused by unknown etiology. When confined to the lungs, it is also known as pulmonary eosinophilic granuloma or pulmonary histiocytosis $\times$. After the first case was described in 1951 by Farinacci et ai, more 250 cases were reported in the English literature. However only 4 cases has been introduced in our country. We describe two additional cases. In both cases, open lung biopsies are performed for definite diagnosis and examined by immunohistochemical stain for S-100 protein and electron microscopy for ultrastructural identification of Birbeck granule.

• Tuberculosis and Respiratory Diseases
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• v.66 no.1
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• pp.58-61
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• 2009

Dieulafoy's disease of the bronchus is rare but potentially life-threatening, and should be considered in patients with massive hemoptysis, especially from unknown etiology. We report a case of a patient with massive hemoptysis due to bronchial Dieulafoy's disease. He underwent bronchial artery embolization and surgical resection, and the post-operative specimen revealed dilated and tortuous arteries in the submucosa that presented as Dieulafoy's disease of the bronchus.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.52-57
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• 2006

Kimura's disease is a rare chronic inflammatory disease of unknown etiology which appears primarily in young Asian males as non-tender subcutaneous swellings in the head and neck region. Histologic characteristics are the presence of lymphoid follicles, vascular proliferation and infiltration of eosinophils. Peripheral eosinophilia and elevated serum IgE are frequently combined. Systemic steroid therapy with surgical excision is the mainstay of treatment, though recurrence after surgery or discontinued steroid treatment is common. It has been known that about 16% of the cases are associated with renal diseases, particularly nephrotic syndrome. We present an 8-year-old boy with a past history of steroid-responsive, infrequently relapsing nephrotic syndrome who developed right buccal swelling and peripheral eosinophilia during the remission state. He has been managed with surgical resection, steroid and cyclosporine due to multiple recurrences.

• Tuberculosis and Respiratory Diseases
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• v.43 no.2
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• pp.285-290
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• 1996

Diffuse panbronchiolitis is a chronic inflammatory lung disease of unknown etiology which is characterized by chronic airflow limitation and airway inflammation, predominantly localized in the respiratory bronchioles with infiltration of inflammatory cells, and has typical clinical, radiological and pathological features. Obstructive respiratory functional impairment, occasional symptoms of wheezing, and also cough and sputum resemble the feature of emphysema, bronchial asthma, or chronic bronchitis, respectively. We experienced a case of pathologically proven advanced diffuse panbronchiolitis in a 55-year-old man with productive cough and exertional dyspnea. The chest radiography showed multiple tiny nodular densities on whole lung fields. It was confirmed by thoracoscopy-guided lung biopsy and the patient was improved after initiation of treatment with low-dose erythromycin.

• Tuberculosis and Respiratory Diseases
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• v.63 no.1
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• pp.88-93
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• 2007

Wegener's granulomatosis is a disease with an unknown etiology that is characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tract and the kidneys. The typical pulmonary findings are bilaterally involved multiple variable sized nodules. We report a case of Wegener's granulomatosis that presented as a single lung mass. A male patient presented with a nasal obstruction, arthralgia, cough, and intermittent dyspnea. The chest radiograph showed a mass, approximately 4.5 cm in diameter, in the right lower lobe. Lung cancer or tuberculosis was initially considered. However, the clinical, laboratory and pathological findings of the mass indicated Wegener's granulomatosis. The patient was administered prednisolone and cyclophosphamide, and improved temporarily. Unfortunately, the immunocompromised patient expired as a result of respiratory failure with pneumonia.

• Tuberculosis and Respiratory Diseases
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• v.61 no.1
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• pp.65-69
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• 2006

Behcet's disease is a systemic vasculitis of an unknown etiology involving the arteries and veins of all sizes. There are reports showing that a pulmonary artery aneurysm or thromboembolism and superior vena cava thrombosis are present in 5-10% of patients with Behcet's disease and that lung parenchymal lesions are mainly airway consolidations resulting from hemorrhage or infarction. We encountered a patient with increasing pulmonary cavitary changes and localized aspergilloma. The patient was a 43-year-old man diagnosed with Behcet's disease with a history of recurrent oro-genital ulceration and uveitis, and who was administered methotrexate, colchicines, prednisolone. During the follow up he developed progressive dyspnea upon exertion and finger clubbing. Therefore further evaluations were performed. Chest computed tomography showed more advanced consolidations and cavitations than the previous film with the previously known aspergilloma still observable. An open lung biopsy was carried out to determine the presence of malignant changes, which revealed nonspecific vasculitis. Azathioprine was added resultion in an improvement of symptoms.

• Tuberculosis and Respiratory Diseases
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• v.40 no.6
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• pp.714-718
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• 1993

Tracheobronchopathia osteoplastica(TPO) is a rare disorder characterized by submucosal cartilaginous or bony projections into the tracheobronchial lumen with sparing of the posterior membranous portion of tracheobronchial tree. The etiology of TPO is still unknown. A 44-year-old male was admitted to Seoul Paik Hospital Inje University due to left chest pain for 10 days. On the past history he had sufferred from symptoms of bronchitis for several months. He showed radiologically massive pleural effusion in left lung field. Pleural biopsy revealed chronic pleuritis with hemorrhage. Bronchoscopic findings showed multiple intraluminal portruding nodule from just below the vocal cord to carina and both main bronchi. Pathology of bronchoscopic biopsy showed abnormal proliferation of atypical bony and carilagious nodules in the tracheal submucosa. We experianced a case of tracheobronchopathia osteoplastica involving the trachea and main bronchus in 44-year old male, associated with massive pleural effusion. This report is a case of TPO with review of literature.

• Tuberculosis and Respiratory Diseases
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• v.46 no.6
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• pp.861-868
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• 1999

Relapsing polychondritis (RP) is a rare inflammatory disorder of unknown etiology, causing recurrent inflammatory and degenerative reactions involving the cartilaginous structures throughout the body. Generally, RP is known as multiorgan disease presented as auricular chondritis, arthritis, nasal chondritis, ocular inflammation, audiovestibular damage and respiratory tract inflammation. Major airway involvement occurs in more than 50% of the patient and has been reported to be the primary cause of death. Rarely, it may be presented with only respiratory symptoms without typical clinical manifestation of RP. We experienced a 64-year-old male patient with RP involving diffuse airway tract without other characteristic clinical manifestation and present here with a review of literatures.

• Pediatric Infection and Vaccine
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• v.25 no.2
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• pp.101-106
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• 2018

A 6-year-old boy with underlying hemolytic anemia of unknown etiology, atopic dermatitis, and recurrent urticaria visited our hospital because of acute respiratory failure induced by influenza A. Despite mechanical ventilation after endotracheal intubation along with inhalation of nitric oxide, respiratory acidosis and hypoxemia persisted. Veno-venous extracorporeal membrane oxygenation (VV ECMO) insertion was performed to provide respiratory support. After performing flexible bronchoscopy, we found that thick mucus plugs were obstructing the right bronchus intermedius and the upper lobe orifice. After bronchial washing and removal of the plugs, we were able to wean the patient off VV ECMO and transfer him to the general ward. He was discharged without any neurologic or pulmonary sequelae.

• Childhood Kidney Diseases
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• v.24 no.1
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• pp.47-52
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• 2020

Pneumocystis pneumonia (PCP) is a rare disease in healthy people but a potentially fatal opportunistic infection by Pneumocystis jirovecii in immunocompromised patients with organ transplantation. We present three cases of PCP after kidney transplantation in pediatric patients. First case was a 4-year-old boy diagnosed with Denys-Drash syndrome and received living-donor kidney transplantation from his mother at age of 1. Second case was a 19-year-old male, with polycystic kidney disease, who received kidney transplantation from his mother at the age of 18. Third case was a 19-year-old female with chronic kidney disease of unknown etiology, who received kidney transplantation from her father at age of 15. These three patients who were on immunosuppressive therapy and completed of routine PCP prophylaxis for 6 months had presented with cough and dyspnea more than 1 year after transplantation. Chest x-ray all showed diffuse haziness of both lung fields, and bronchoalveolar lavage from bronchoscopy revealed Pneumocystisjirovecii infection. All patients showed clinical resolution with intravenous trimethoprim-sulfamethoxazole (TMP-SMX) therapy for at least 3 weeks and had continued secondary prophylaxis for another 6-12 months. This report suggests that clinicians should have suspicion for the possibilities of opportunistic infection such as PCP after kidney transplantation in children.