• Tuberculosis and Respiratory Diseases
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• v.74 no.4
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• pp.151-162
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• 2013

Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.

• Tuberculosis and Respiratory Diseases
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• v.41 no.2
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• pp.152-157
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• 1994

Histiocytosis-X is a tenn used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilatera1, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X, which is confirmed by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.

• Clinical and Experimental Pediatrics
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• v.58 no.3
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• pp.112-115
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• 2015

Hand, foot, and mouth disease (HFMD) is an acute, mostly self-limiting infection. Patients usually recover without any sequelae. However, a few cases are life threatening, especially those caused by enterovirus 71 (EV71). A 12-month-old boy was admitted to a primary hospital with high fever and vesicular lesions of the mouth, hands, and feet. After 3 days, he experienced 3 seizure episodes and was referred to our hospital. On admission, he was conscious and his chest radiograph was normal. However, 6 hours later, he suddenly lost consciousness and had developed a massive pulmonary hemorrhage that continued until his death. He experienced several more intermittent seizures, and diffuse infiltration of both lung fields was observed on chest radiography. Intravenous immunoglobulin, dexamethasone, cefotaxime, leukocyte-depleted red blood cells, fresh frozen plasma, inotropics, vitamin K, and endotracheal epinephrine were administered. The patient died 9 hours after intubation, within 3 days from fever onset. EV71 subgenotype C4a was isolated retrospectively from serum and nasopharyngeal swab by real-time reverse transcription-polymerase chain reaction. Here, we report a fatal case of EV71-associated HFMD with sudden-onset massive pulmonary hemorrhage and suspected encephalitis.

• Tuberculosis and Respiratory Diseases
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• v.39 no.3
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• pp.266-270
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• 1992

Pulmonary lymphangioleiomyomatosis is a rare disease, prevalent in women of reproductive age, and characterized by proliferation of atypical smooth muscle in the lung. A 44-year-old women with pulmonary lymphangioleiomyomatosis was presented. Chest radiographs showed diffuse increased reticulonoular infiltrations on the almost entire lung and high resolution CT illustrated multiple, well defined cystic lesions which were distributed diffusely through the lung. Histologic confirmation of lymphangioleiomyomatosis was made upon open lung biopsy. Treatment with methylprogesterone was initated.

• Tuberculosis and Respiratory Diseases
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• v.42 no.3
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• pp.400-406
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• 1995

Interstitial pneumonitis may be the presenting manifestation of polymyositis-dermatomyositis (PM-DM), or may occur later in the evolution of disease. The clinical picture is characterized by non-productive cough, dyspnea and hypoxemia. The chest radiograph demonstrates interstitial infiltrates with predilection for the lung bases, often with an alveolar pattern in addition. We experienced a case of polymyositis associated with diffuse alveolar damage(DAD) that was proven in open lung biopsy. The patient was a 52 year-old woman who was presented with 6 months' duration of generalized ache, edema on ankle and wrist, non-productive cough and mild dyspnea. She had typical symptoms and physical findings of interstitial pneuminitis, and elevated muscle enzyme levels in serum with characteristic histologic findings of myositis on muscle biopsy. She also had typical interstitial lung disease pattern on high resolution CT and restrictive pattern on pulmonary function tests. The findings of open lung biopsy was compatible with diffuse alveolar damage(DAD). She failed to respond to the therapeutic trials with corticosteroid and cyclophosphamide, and finally expired due to acute respiratory distress syndrome.

• Tuberculosis and Respiratory Diseases
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• v.49 no.5
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• pp.614-623
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• 2000

Background : Diffuse alveolar hemorrhage (DAH) is rare but often fatal. To determine the clinical manifestations of DAH, its etiology, clinical course and prognosis were studied. Method : A retrospective analysis was performed in 21 patients that were diagnosed as DAH. Diagnosis of DAH was based on the presence of the "classical triad" of hemoptysis, anemia, and rapidly progressive infiltrates on chest X-ray and a finding of bronchoalveolar lavage or lung biopsy. Results : Thirteen patients (61.9%) had collagen vascular diseases (CVDs) as underlying disease and 10 patients had systemic lupus erythematosus. Females were more prevalent in CVD than in non-collagen vascular disease (NCVD). Otherwise, there were no significant differences between the two groups in terms of clinical manifestations. Dyspnea (95.2%), cough (76.2%), hemoptysis (61.9%), and fever (33.0%) were frequent symptoms. The initial creatinine level was higher in CVD than in NCVD ($3.27{\pm}3.15$ mg/dl vs. $1.19{\pm}0.94$ mg/dl, p=0.030). The corresponding drop in hemoglobin level was $2.69{\pm}1.26$ g/dl. Maximal drop in hemoglobin preceded the progression of infiltrates on the chest radiograph by $1.38{\pm}4.22$ days. The mortality rate was higher in the patients with NCVD than in those with CVD (50.0% vs. 23.1%). Conclusion : The DAH can occur not only in patients with CVD but also in those with NCVD. Higher creatinine level CVD in patients is associated with renal involvement in conjunction with DAH. The maximal drop in hemoglobin preceeding the progression of infiltrates on the chest radiograph suggests that the drop in hemoglobin is important for diagnosing DAH.

• The Korean Journal of Physiology and Pharmacology
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• v.11 no.6
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• pp.259-262
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• 2007

Diffuse panbronchiolitis (DPB) is a pulmonary disease characterized by chronic inflammation of the bronchioles and chronic infiltration of inflammatory cells in the lungs. Macrolides are effective therapeutic agents for chronic respiratory tract diseases, such as DPB. However, the mechanisms by which macrolides modulate the immune responses in patients with DPB remain unclear. To understand clinical efficacy for the treatment of DPB by macrolides, the effects of erythromycin (EM) on the expression of pro-inflammatory cytokines such as interleukin-6 (IL-6) and interleukin-8 (IL-8) by human neutrophils were examined. Pre-treatment with EM significantly decreased the expression of IL-6 and IL-8 transcripts by lipopolysaccharide (LPS)-stimulated human neutrophils. EM also reversed the enhanced survival of human neutrophils by LPS. These data indicate that EM has achieved therapeutic effect for patients with DPB, in part, through decreasing the expression of pro-inflammatory cytokines and the survival of neutrophils.

• Tuberculosis and Respiratory Diseases
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• v.49 no.1
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• pp.99-104
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• 2000

Pulmonary hemangiomatosis is a rare disease of unknown etiology characterized by diffuse proliferating microvessels that infiltrate not only the lung but also all of the thoracic tissues. The disease is rapidly progressive and usually results in death due to complications of pulmonary hypertension and pulmonary hemorrhage. There are two histologic patterns of pulmonary hemangiomatosis : capillary and cavernous. We describe a 21-year-old man with cavernous-type pulmonary hemangiomatosis presented with right side shoulder pain. He was treated with percutaneous transarterial embolization and then followed with interferon alfa-2a for one year with favorable clinical and radiological response.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.429-436
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• 1998

Approximately 100 drugs have been reported to affect the lungs adversely. Among these, pulmonary toxicity caused by antieneoplastic agent. is being recognized more frequently. Cyclophosphamide is an immunosuppressive alkylating agent used for the treatment of a wide variety of malignant and nonmalignant diseases. The incidence of pulmonary toxicity is probably less than 1 percent The first case was reported in 1967. Since then, more than 20 well-documented cases of pulmonary toxicity associated with cyclophosphamide have been reported in the literature. In Korea, three patients were identified with cyclophosphamide-induced lung disease. The typical features of toxicity include dyspnea, fever, cough, new parenchymal infiltrates, gas exchangs abnormalities on pulmonary function tests, and pleural thickening on chest roentgenogram. The best approach to management is early diagnosis, discontinuation of the offending drug and administration of corticosteroid therapy. Recently, we experienced a case of diffuse alveolar damage induced by cyclophosphamide. The patient presented with early-onset pulmonary toxicity and died of repiratory failure despite early use of corticosteroid.

• Tuberculosis and Respiratory Diseases
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• v.39 no.3
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• pp.271-277
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• 1992

Diffuse panbronchiollitis (DPB), a rare progressive disorder, has lately been receiving increasing attention. DPB is a disease of obscure etiology, characterized by chronic inflammation localized mainly in the region of respiratory bronchiole just distal to the terminal bronchioles. In 1983, Homma and coworkers reported 82 cases of a new clinicopathological entity, DPB, in Japan. Also DPB is a disease largely restricted geographically to Japan but the prevalence in other countries is extremely low. Histoloically, it is characterized by a suppurative bronchiolitis involving primarily the respiratory and terminal bronchioles with subsequent progression to bronchiectasis. The disease progresses rapidly and results in respiratory failure due to repeated respiratory infections. We experienced a cases of DPB accompanied with chronic maxillary sinusitis in both sinuses. Diagnosis of DPB was comfirmed by pathological results from thoracoscopic lung biopsy, typical radiological findings, clinical symptoms and pulmonary function test. After treatment with erythromycin for 6 months, the patient's condition and the typical micronodular densities on the chest radiography improved. A few case of DPB was reported in Korea. We report a case of DPB through thoracoscopic lung biopsy.