• Clinical and Experimental Pediatrics
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• v.48 no.3
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• pp.327-332
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• 2005

Interstitial lung disease refers to a group of pulmonary disorders characterized by inflammation of the interstitium, derangements and loss of alveolar capillary units leading to disruption of alveolar gas exchange, which induces symptoms of restrictive lung disease. Cases of interstitial pneumonia in children are uncommon and mostly have unknown causes. We have experienced an 8-year old boy who had symptoms of cyanosis, dry cough, dyspnea and abrupt weight loss. He had not been exposed to organic dusts, allergens or any other systemic disease infections. Chest radiology showed diffuse ground glass opacity in both lung fields. High resolution computed tomography(HRCT) showed multiple small patchy areas of consolidation with an underlying ground glass appearance in both lungs. The pathologic findings of lung biopsy tissue showed patchy areas of interstitial fibrosis, alveolar obliteration and nodular fibrotic areas, strongly suggesting interstitial pneumonia. No specific finding of viral inclusion or any other evidence of infection was found under electromicroscopy. We used peak flow meters to compare functional improvement. Forced expiratory volume in one second ($FEV_1$) was decreased to 25 percent of predicted value. The boy was given treatment with prednisone and showed improvements in HRCT findings after two months. He was able to tolerate easy exercise in school and showed clinical improvements after one year of follow up.

• Tuberculosis and Respiratory Diseases
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• v.62 no.2
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• pp.134-139
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• 2007

Gefitinib is a novel drug used to treat advanced non-small cell lung cancer. However, drug-related interstitial pneumonia is a major life-threatening side effect, which has a worldwide prevalence of 0.3-0.4%. In Japan, the prevalence is high as 3-4% but the actual frequency in Korea has not been officially assessed. We report two cases of gefitinib-induced interstitial lung disease during the treatment of non-small cell lung cancer. High-resolution computerized tomography (HRCT) of one case showed nonspecific ground glass opacity and the chest x-ray of another case showed diffuse bilateral ground glass opacity. The former patient showed a rapid good response to corticosteroid treatment whereas the latter died despite receiving aggressive treatment with high dose corticosteroid and empirical antibiotics.

• Tuberculosis and Respiratory Diseases
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• v.51 no.6
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• pp.597-602
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• 2001

A 47-years-old woman presented with a 2-month history of a dry mouth and dry cough. The patient had been taking medication for Sj$\ddot{o}$gren's syndrome for approximately 7 years. The chest radiography showed multiple cystic lesions and a hazy density in both lower lung fields. The HRCT showed a diffuse ground glass like appearance and multiple variable sized cystic lesions in both lung fields. After medication, the symptoms were aggravated. Bronchoscopy was preformed with a transbronchial lung biopsy. The biopsies showed an infiltration of lymphocytes, neutrophils, monocytes and histiocytes through the interstitial space of the alveola and a widening of the alveolar septa. However, the histological findings of the cysts were not obtained. Sj$\ddot{o}$gren's syndrome is a slowly progressive inflammatory autoimmune disease, which is characterized by lymphocyte mediated destruction of the exocrine glands, with pulmonary involvement in approximately 19-65%, High-resolution CT is a sensitive technique for assessing the pulmonary involvement in patients with Sj$\ddot{o}$gren's syndrome. Although a lung biopsy is not always necessary for establishing a diagnosis of an interstitial lung disease in Sj$\ddot{o}$gren's syndrome. A lung biopsy may reveal a wide spectrum of changes ranging from a mild inflammatory response to end stage fibrosis with honeycombing. Because of the predominantly peribronchiolar inflammatory infiltration and inspissated secretions the cysts were suspected to have been formed by the ball-valve phenomen. However, no definite evidence was obtained.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.444-450
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• 1998

PCP remains the leading cause of deaths in patients with AIDS. As familiarity with PCP increases, atypical manifestations of the diseases are being recognized with greater frequency. There are following "atypical" manifestations of PCP ; 1) interstitial lung response that include diffuse alveolar damage, bronchiolitis obliterance, interstitial fibrosis, and lymphoplasmocytic infiltrate 2) striking localized process frequently exhibiting granulomatous features 3) extensive necrosis & cavitation 4) extrapulmonary dissemination of the disease. A wide variety of pathologic manifestations may occur in PCP in human immunodeficiency virus-infected patienst and that atypical features should be sought in lung biopsies from patients at risk for PCP. We had experienced a case of PCP, which presented with severe hypoxia, progressive dyspnea and fine crackles. It was diagnosed as PCP in AIDS with manifestation of BOOP by open lung biopsy and showed good response to Bactrim & corticosteroid therapy.

• Tuberculosis and Respiratory Diseases
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• v.38 no.1
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• pp.1-7
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• 1991

To study the frequency of the underlying disease of the diffuse pulmonary infiltrates in korea, we ansalyzed retrospectively 982 patients who were seen at nine university hospitals and one general hospital in Seoul area. The results are folloing: 1) Among the total 982 patients, 490 patients were male and 492 patients were female. The mean age was 44.3 years. 2) The most common etiology was milliary tuberculosis (38%), which was followed by, idopathic pulmonary fibrosis (27%), pulmonary fibrosis associated with collagen-vascular disease (15%), and diffuse pulmonary infiltrates by malignancy (10%). 3) Amon the connective tissue disease which was accompanied by the interstitial lung disease, rheumatoid arthritis was the most common disease (43%), systemic lupus erythematosus was the 2nd (28%), and progressive systemic sclerosis was the 3re (16%). 4) Among 101 cases of malignant disease, lung was the most frequent primary site (31%), which was followed by stomach (28%), thyroid (16%), and breast (6%). 5) For the diagnosis of the underlying disease of pulmonary infiltrates, the transbronchial lung biopsy was performed in 21% of the patients and open lung biopsy was done in 7%.

• Journal of Chest Surgery
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• v.33 no.9
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• pp.773-776
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• 2000

Accidental or suicidal fatalities of paraquat(Gramoxon) poisong are occasionally seen in the emergency room or intensive care unit in this country. In most cases, respiratory symptoms and eventual death by respiratory distress occur within several days. The most striking pathologic change is fibrosis of the lung due to widespread proliferation of fibroblastic cell. We experience a 21-year-old woman with huge bulla on left lung and diffuse fibrosis in other site, who ingested paraquat 10 months ago. After thoracoscopic removal of bulla, the patient survive without progression of pulmonary complication till now.

• Tuberculosis and Respiratory Diseases
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• v.40 no.4
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• pp.357-366
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• 1993

Background: Lung clearance of inhaled $^{99m}Tc$-DTPA reflects alveolar epithelial permeability and it had been reported as more sensitive than conventional pulmonary function tests in detecting lung epithelial damage. However, measuring lung clearance of inhaled $^{99m}Tc$-DTPA by gamma camera may not always reflect alveolar epithelial permeability exactly because it is influenced by mucociliary clearance depending on the site of particle deposition. Moreover, this method takes much time and patient's effort because he has to sit or lie still in front of the camera for a prolonged period. Most of the absorbed DTPA is excreted in urine within 24 hours and the amount of excreted DTPA in urine during the first few hours after inhalation is influenced by absorption rate which is correlated with the alveolar-epithelial permeability suggesting that the urinary excretion, especially in first few hours, may be an alternate index for lung clearance. The purpose of this study was to evaluate the usefulness of ratio of excreted $^{99m}Tc$-DTPA in 2 hour and 24 hour urine as an index of alveolar-epithelial damage. Methods: Pulmonary function tests including diffusing capacity and lung clearance of $^{99m}Tc$-DTPA measured by gama camera ($T_{1/2}$) and 2hr/24hr urine excretion ratio (Ratio) of inhaled $^{99m}Tc$-DTPA in 8 normal subjects and 14 patients with diffuse interstitial lung disease were compared. Results: 1) In the normal control, there was significant negative correlation between the $T_{1/2}$ and the Ratio (r=-0.77, p<0.05). In patients with diffuse interstitial lung disease, there also was significant negative correlation between $T_{1/2}$ and Ratio(r=-0.63, p<0.05). 2) In diffuse interstitial lung disease patients, the $T_{1/2}$ was $38.65{\pm}11.63$ min which was significantly lower than that of normal control, $55.53{\pm}11.15$ min and the Ratio was $52.15{\pm}10.07%$ also signifantly higher than that of the normal control, $40.43{\pm}5.53%$ (p<0.05). 3) There was no significant correlations between $T_{1/2}$ or Ratio and diffusing capactiy of lung in both patients and controls (p>0.05). Conclusion: These results suggests that 2hr/24hr urine excretion ratio of inhaled $^{99m}Tc$-DTPA is a useful simple bedside test in assessing alveolar epithelial permeability and that it may be used as an additive follow-up test in patients with diffuse interstitial lung disease complementing conventional pulmonary function tests.

• Tuberculosis and Respiratory Diseases
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• v.66 no.3
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• pp.230-235
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• 2009

Idiopathic bronchiolocentric interstitial pneumonia is one of idiopathic interstitial pneumonia, which has a relatively aggressive course and poor prognosis. It is characterized by diffuse centrilobular nodules radiologically with mainly bronchiolocentric inflammation and fibrosis associated with patchy alveolitis lacking interstitial granuloma histologically. This disorder is a recently classified disease category, and to our knowledge, there is no case report in Korea. We present a case of idiopathic bronchiolocentric interstitial pneumonia. A 62-year-old man presented with exertional dyspnea with a 1 month duration. The radiological findings showed extensive centrilobular lesions at both lungs. The surgical lung biopsy specimen demonstrated a centrilobular inflammatory process with small airway fibrosis and inflammation partially radiating into the interstitium. Therefore, the patient was diagnosed with idiopathic bronchiolocentric interstitial pneumonia. He was treated with immunosuppressants including steroids and azathioprine. However, his symptoms did not improve and he expired 7 months later due to an acute exacerbation of the interstitial pneumonia and probable infectious pneumonia.

• Tuberculosis and Respiratory Diseases
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• v.52 no.5
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• pp.550-556
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• 2002

Microscopic polyangiitis is a systemic small-vessel vasculitis that is associated primarily with necrotizing glomerulonephritis and pulmonary capillaritis. A recurrent and diffuse alveolar hemorrhage due to pulmonary capillaritis is the main clinical manifestation of lung involvement. Recently, and interstitial lung disease that mimics idiopathic pulmonary fibrosis was reported to be rarely associated with microscopic polyangiitis. Here we report two patients with microscopic polyangiitis who showed a honeycomb lung at the time of the initial diagnosis with a brief review of relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.624-628
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• 1995

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. We experienced a 32-year-old female who was admitted because of worsening exertional dyspnea after hormonal therapy with Clomifen for five months and intermittent hemoptysis. Chest PA showed diffuse ground glass appearance with some reticular infiltrations. High resolution CT scan showed multiple small thin walled cysts distributed homogeneously throughout the entire lung. Pulmonary function test showed characteristic obstructive pattern despite the restrictive interstitial pattern of chest radiography. Thoracoscopic lung biopsy specimen showed abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and pulmonary vessels typical of pulmonary lymphangioleiomyomatosis. Hormonal therapy with medroxyprogesterone was initiated.