Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient who had a pneumonectomy for lung carcinoma was treated with carmustine when brain metastases developed. His pulmonary function was mildly compromised prior to the pneumonectomy by many years of smoking. After six months of carmustine therapy [total dose: 2,250 mg (1,200 mg/m2)] he developed interstitial pulmonary fibrosis with histologic changes consistent with drug toxicity. With seven previously reported cases of this drug-effect and the addition of our case, carmustine must be added to the list of cancer chemotherapeutic agents that can cause pulmonary toxicity.
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PMID:Pulmonary toxicity from carmustine (BCNU): a case report. 47 Aug 40

A patient with diffuse eosinophilic gastroenteritis, associated with progressive chronic interstitial pulmonary fibrosis, was followed for 29 years. His major symptoms were recurrent gastrointestinal bleeding, anemia, diarrhea, malabsorption, and increasing respiratory insufficiency. Multiple jejunal biopsies revealed eosinophilic infiltration and extensive villous atrophy. Results of a lung biopsy and pulmonary function tests were consistent with chronic interstitial pulmonary fibrosis. His gastrointestinal symptoms responded to steroid therapy, but he died of progressive respiratory insufficiency. At autopsy, villous atrophy was noted to extend throughout the whole extent of the small intestine in association with multiple "pseudopolypoidal" inflammatory lesions.
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PMID:Eosinophilic gastroenteritis with malabsorption, extensive villous atrophy, recurrent hemorrhage and chronic pulmonary fibrosis. 108 12

Jo-1 syndrome is a disease recently described, included on the list of connective tissue diseases. Its clinical features are myositis and/or pulmonary fibrosis associated to the presence of precipitant antibodies against intracellular enzyme call histidine T-RNA synthetase. This antibody is related to pulmonary fibrosis associated to myositis and some scientist gave predictive value on the onset of pulmonary fibrosis in patients with myositis. However, isolated association of pulmonary fibrosis have been exceptionally described. A patient with severe interstitial pulmonary affliction and positive Jo-1 antibody without myositis is presented. The actual knowledge of the disease and its association is reviewed.
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PMID:[Pulmonary fibrosis as a presentation form of the Jo-1 syndrome]. 176 50

Two cases of pulmonary alveolar proteinosis (PAP) with increased CEA were reported. Case 1 was a 62-year-old male with suspected pulmonary fibrosis, who was transferred to our hospital. Laboratory findings on admission revealed 31.3 ng/ml of CEA. Because he had severe dyspnea, lung biopsy was not carried out. His condition gradually deteriorated and he died of respiratory failure. Autopsy revealed he had PAP and no malignancy. Case 2 was a 48 year-old male referred to our hospital because of dyspnea. Serum CEA was 52.8 ng/ml. Microscopic examination of a transbronchial lung biopsy showed PAP. The level of CEA in bronchoalveolar lavage fluid was 151 ng/ml. Unilateral whole lung lavage was performed twice. With the improvement of chest X-ray findings, serum levels of CEA fell to normal level. The molecular weight of CEA in bronchoalveolar lavage fluid was 180,000. Immunochemical staining of CEA in lung revealed nonspecific findings.
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PMID:[Two cases of pulmonary alveolar proteinosis with increased CEA]. 223 73

Up to 1980, less than 40 lung transplants had been reported worldwide without any success. The factors influencing these poor results were related to complications at the bronchial anastomosis and ineffective immunosuppressive regimens. The development of new immunosuppressive drugs has permitted the reevaluation of lung transplantation as a therapeutic option. The success with heart-lung transplantation stimulated the development of clinical human single-lung and double-lung transplantation. However the world experience is still scarce. In our institution we have developed experimental work leading to the establishment of a lung transplant program. This paper describes our first single lung transplant patient. The patient, a 33 year old man with end-stage pulmonary fibrosis, was totally oxygen dependant, maintaining arterial blood oxygen levels below 40 mmHg without oxygen supplementation and confined to a wheelchair. A single left lung transplant was performed from a young brain-dead donor. The bronchial anastomosis was protected with an omental flap. The immunosuppressive regimen was based on cyclosporin A and azathioprine from the beginning, adding prednisone on the third postoperative week. There has been only one episode suggestive of acute rejection which was managed with methylprednisolone. On the 9th postoperative week the patient developed a bronchial stenoses at the anastomotic site which required dilation and stenting with an endobronchial silastic stent. His clinical course has been uneventful since then. His ventilatory parameters showed an increase of vital capacity from 900 to 2100 mL and his FEV1 from 700 to 1500 mL. His gas exchange has been normal with arterial blood gas oxygen above 60 mmHg and oxygen saturation above 94%.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Unilateral lung transplant in a case of terminal pulmonary fibrosis]. 226 45

This is a report on two brothers with the Hermansky-Pudlak syndrome who developed pulmonary fibrosis. In the first, a gradual course lasting 13 years was observed, the patient finally died of long-standing colitis. His brother died of progressive respiratory failure 15 months after the subjective onset of the disease. Immunosuppressive therapy proved ineffective.
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PMID:[Hermansky-Pudlak syndrome in 2 brothers with lung fibrosis]. 236 70

Three patients with chronic respiratory failure of various etiology, who have (had) been in the hospital for some years, were presented. The case 1 is male and was 57 years old on admission. He has been suffered from right thoracic emphysema from October '83, which is under chronic infection of Pseudomonas aeruginosa, with bronchial fistula and aortic valve insufficiency. His pulmonary function is severely restrictive and the grade of his dyspnea has been V of Hugh-Jone's criteria and he is now unable to leave from bed. The case 2 is male and was 41 y.o. on admission. He has been ill with diffuse cystic bronchiectasis from 33 y.o. and bronchorrhea (greater than 200 ml/day) with chronic infection of Pseudomonas aeruginosa has been lasting and recurrent attacks of infection have progressively worsened of his pulmonary and cardiac functions. He is now indispensable to assist ventilation by artificial respirator every 2-3 days. The case 3 was male and 27 y.o. on admission. He had admitted because of severe dyspnea due to familial pulmonary fibrosis on August '86. His disorder had been progressive and resistant against repeated corticosteroid therapy. He died of respiratory failure at 30 years old. The transplantations of lung and heart-lung for critical patients with respiratory failure have been challenged in North America and Europe, but in Japan, many social and medical problems about transplantation have yet been unresolved. The indications for and against lung or heart-lung transplantation to these three patients was discussed with reference to English literatures.
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PMID:[Three cases of chronic respiratory failure]. 262 Jan 34

We report a 79-year-old man who developed progressive gait disturbance and sensory loss. He had been doing well except for hepatitis B virus hepatitis until 72 years of age when he developed angina pectoris for which aorto-coronary bypass operation was performed when he was 73-year-old (1986). In 1990, he developed pulmonary fibrosis for which prednisolone was prescribed. His liver function deteriorated, and the liver function tests suggested liver cirrhosis. He noted an onset of gait disturbance in the middle of June in 1992 when he was 79-year-old. His gait disturbance deteriorated progressively, and he developed edema and loss of sensation in his both legs. He became unable to walk unassisted in the beginning of July. He fractured his right external malleolus after falling down from a chair. He became unable to stand by himself, and he was admitted to the cardiology service of our hospital on July 18, 1992, and the neurology service was asked to see the patient on July 30 of the same month. The patient was well developed and well nourished man in no acute distress. General physical examination revealed slight jaundice, left carotid bruit, and slight pitting pretibial edema. His temperature was 37.3 degrees C. On neurologic examination, he was alert and mentally sound without dementia. He showed a slight weakness in the facial muscles bilaterally and mild dysarthria and dysphagia, however, the other cranial nerves appeared intact. He was unable to stand unassisted. The muscle tone was hypotonic, however, no focal muscle atrophy was noted, nor was observed fasciculatory twitches.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A 79-year-old man with rapidly progressive tetraparesis]. 829 70

Pulmonary involvement of Langerhans cell histiocytosis (LCH) is an uncommon but important cause of pulmonary fibrosis and honeycombing in young adults. Rarely, pulmonary LCH may be complicated by spontaneous pneumothorax. It may be isolated or associated with multiple organ involvement. We describe here a case of LCH with diabetes insipidus, skin lesions and pulmonary involvement in a 15-year-old boy. The case was complicated by four episodes of spontaneous pneumothorax with typical radiologic findings of pulmonary LCH. His presentation, radiologic findings, treatment and clinical course during three-years of follow-up are discussed.
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PMID:Langerhans cell histiocytosis associated with recurrent pneumothorax: a case report. 881 33

A 56-year-old male carpenter had a history of glass fiber inhalation for 41 years without any protective device. His chest radiograph showed small nodular opacities in lower lung fields and multiple cystic lesions and low attenuation areas in upper lung fields. Light and polarizing microscopic examinations of his transbronchial lung biopsy specimen revealed mild interstitial fibrosis and mononuclear cell infiltration in alveolar walls without birefringent substances. However, widespread depositions of small glass fibers (< 2.5 microns in length and 0.3 micron in diameter) were detected by analytical electron microscopy, which suggested their possible contribution to the development of his pulmonary fibrosis.
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PMID:Pulmonary fibrosis in a carpenter with long-lasting exposure to fiberglass. 890 8


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