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)

Intersitial pneumonia and pulmonary fibrosis developed in a 72-year-old man during therapy with cyclophosphamide, vincristine, and prednisone. After extensive investigations, including an open lung biopsy, cyclophosphamide appeared to be the cause of the pulmonary disease. Complete disappearance of tachypnea and the pulmonary infiltrates occurred after the discontinuation of cyclophosphamide and the institution of prednisone therapy. We concluded that the diffuse pulmonary disease in this patient was a result of cyclophosphamide therapy. The clinical and pathologic findings in this case and a review of the literature of cyclophosphamide pulmonary toxicity are reported.
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PMID:Cyclophosphamide therapy and interstitial pulmonary fibrosis. 106 40

Eleven Friesian steers were given 3, methyl indole (3MI) orally at dose rates ranging from 0.1 to 0.3 g/kg. Three of these (group B) received a single oral dose of 0.2 g/kg and subsequently developed respiratory distress. Their plasma 3MI concentrations six hours after dosing were between 2.25 and 7.23 micrograms/ml. The steer with the highest six-hour plasma value died at this stage and the dominant pathological feature was severe pulmonary oedema. The other two steers survived until they were slaughtered 96 hours after dosing; the major pathological findings in them were interstitial emphysema, hyaline membranes and alveolar epithelial hyperplasia. The other eight steers (group C) each received weekly oral doses of 0.1 g 3MI/kg for 10 weeks. One animal died after developing severe respiratory distress following its third dose. Thereafter, the others developed two separate patterns of response. Three steers (subgroup C1) became progressively more tolerant to oral 3MI, even in the face of dose rates increased to 0.2 and 0.3 g/kg during the 11th to 14th weeks of the study and also in the presence of relatively high plasma 3MI concentrations after dosing. One animal was slaughtered after its 10th dose and two after their 14th dose of 3MI; post mortem examinations revealed that their lungs were macro- and microscopically normal. The other steers (subgroup C2) all continued to react after each weekly oral dose of 3MI and their post-dosing plasma 3MI concentrations consistently remained relatively low. Latterly, each of the three steers which survived to the 14th week also exhibited persistent tachypnoea and marked hyperpnoea between dosings. On post mortem examination, in addition to the signs generally associated with acute 3MI toxicity (see above), each of the subgroup C2 steers were found to have diffuse pulmonary fibrosis and an alveolitis. While certain cattle appear to become tolerant to the effects of repeated doses of 3MI, the results of this study clearly demonstrated that, in others, such treatment eventually gives rise to diffuse pulmonary fibrosis and alveolitis.
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PMID:Experimental production of diffuse pulmonary fibrosis and alveolitis in cattle: the effects of repeated dosage with 3, methyl indole. 683 86

A pediatric patient is reported who experienced fatal progressive pulmonary fibrosis as a complication of 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) therapy. The patient received a cumulative dosage of 1.29 g (1.72 g/m2) over a two-year period as adjuvant therapy for a medulloblastoma. Two and one-half years after cessation of therapy, cough, tachypnea and fatigue were noted. Progressive pulmonary insufficiency developed. Pulmonary pathologic findings included interstitial fibrosis and alveolar dysplasia. Other cases of BCNU pulmonary toxicity are cited from the medical literature.
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PMID:Pulmonary fibrosis: a complication of 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) therapy. 727 34

A progressive pulmonary disease resulting in severe respiratory failure and death in an average of 3 weeks was diagnosed in 11 young Dalmatian dogs. The dogs were from 4 litters, all genetically related by a common ancestor. The initial clinical signs were tachypnea and noisy respiration. Respiratory distress developed shortly before death and was characterized by strenuous and rapid respirations, along with cyanosis and vomiting. On blood gas analysis, there were severe arterial hypoxemia, hypercapnia, and marked alveolar-arterial oxygen difference. Radiographically, a diffuse pattern of alveolar, interstitial, and peribronchial densities was observed in the lungs. Most dogs developed pneumomediastinum and gastroesophageal intussusception in the terminal phase of the disease. There was no response to treatment with antibiotics, corticosteroids, diuretics, or oxygen. At necropsy, the lungs were wet, heavy, and relatively airless. Absence of 1 kidney in 2 dogs and severe internal hydrocephalus in 2 dogs were additional necropsy findings. Pulmonary histopathology included metaplasia and atypia of the alveolar and bronchiolar epithelium, a nonpurulent inflammatory reaction characterized mainly by mononuclear cells and macrophages, eosinophilic hyaline membrane formation, and focal pulmonary fibrosis. The histological manifestations were typical of acute lung injury. Clinically, the findings were consistent with adult respiratory distress syndrome (ARDS), except for the relatively long course. No known risk factors for ARDS, such as trauma, toxin exposure, infection, or endotoxemia could be identified. The relationship of the other abnormalities (ie, renal aplasia, hydrocephalus) to the pulmonary disease also remains obscure. An inherited defect is suspected, because segregation analysis of the 4 litters suggests autosomal recessive inheritance.
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PMID:Lung injury leading to respiratory distress syndrome in young Dalmatian dogs. 767 17

A 58 year old man with arthralgia had been treated with antiinflammatory drugs since November, 1987. On March 1988, he was admitted to a local hospital because of jaundice. On April 18, exploratory laparotomy was done to look for the cause of continued jaundice. Macro-pathological and micropathological findings of the liver were compatible with those of intrahepatic cholestasis. On April 29, he was transferred to Kyushu University Hospital because of persistent jaundice. Physical examination showed jaundice, tachypnea and low grade fever. Laboratory examination showed elevated level of serum bilirubin, marked hypoxia and endotoxemia. Plain chest radiogram revealed ground-glass appearance in bilateral lung fields. He was diagnosed to be complicated with adult respiratory distress syndrome (ARDS). Glucocorticoid therapy and oxygen administration were started immediately. But pulmonary fibrosis developed and he died of respiratory failure. Histological findings of lung necropsy specimen were compatible with those of ARDS. These data strongly suggested that endotoxemia and reticuloendothelial dysfunction due to intrahepatic cholestasis had played important roles to develop ARDS.
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PMID:[A case of intrahepatic cholestasis following with adult respiratory distress syndrome]. 837 98

Idiopathic pulmonary fibrosis is a poorly characterized disease in infants. In the present report, we reviewed our experience with 10 infants during a 10-year period. In 9 patients, onset of symptoms occurred before the age of 2 months and included tachypnea, cough, and inadequate weight gain. However, despite the presence of these symptoms, diagnosis was delayed for 3 months at which time the infants were referred to the pediatric pulmonary department, when the diagnosis was confirmed by open lung biopsy. At the time of admission, bronchoscopy with alveolar lavage was performed in 9 children and showed severe alveolitis with an increase in the neutrophil count. Nine infants were treated with prednisone alone or in combination with chloroquine, colchicine, or cyclophosphamide; all these patients died despite treatment. One infant was treated with pulses of methylprednisolone because of a failure in response to oral prednisone. This girl who displayed similar clinical, radiological, and histological abnormalities as the other children at the time of diagnosis is the only child still alive after 3 years of follow-up. She is now free of respiratory symptoms and has a normal growth curve. The present report raised two important points: (1) a thorough evaluation of characteristic symptoms should lead to an early diagnosis of pulmonary fibrosis in infants; and (2) administration of pulse therapy using corticosteroids has been helpful and needs to be evaluated further.
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PMID:Idiopathic pulmonary fibrosis in infants. 903 92

Tropical pulmonary eosinophilia (TPE) is considered to be a variant of human filarial infection. The pulmonary manifestations of TPE have been well described. Extra-pulmonary features of the disease, although not commonly seen, have been reported previously. A 9-year-old Malay girl with a history of recurrent cough and wheezing was admitted because of cardiac failure. Physical examination revealed a very sick girl with tachypnoea, central cyanosis, finger clubbing, elevated jugular venous pulse, generalized crackles and rhonchi in the chest, a loud second heart sound and hepatosplenomegaly. A chest radiograph showed cardiomegaly and right pleural effusion. Laboratory investigations revealed hypochromic, microcytic anaemia with persistent blood eosinophilia (absolute eosinophil counts varied from 1.9 to 5.5 x 10(9)/1). The ELISA test for antifilarial IgG antibodies was strongly positive. She responded promptly to treatment with diethylcarbamazine. In summary, this is a patient with TPE who presented with cor pulmonale, probably due to late-stage interstitial pulmonary fibrosis. In order to prevent the long term morbidity of cardiorespiratory disability, the early signs of TPE should be recognized and the infection treated.
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PMID:Cor pulmonale: an unusual presentation of tropical eosinophilia. 917 82

A 44-year-old non-smoking patient with longstanding ankylosing spondylitis presented in marked respiratory distress with tachypnea, fever, cough, greenish sputum, night sweats, dyspnea and weight loss. Computed tomography showed traction bronchiectases and cavities associated with scarring. The findings were most pronounced in the upper lobes which contained multiple cavities up to 8 cm in diameter harboring fungus balls. The superior segment of the left lower lobe showed two additional cavities. Tuberculosis and atypical mycobacteria were ruled out. Antibiotic therapy resulted in transient improvement. Five months after this acute exacerbation the patient expired from massive haemoptysis. Pulmonary fibrosis is a rare manifestation of ankylosing spondylitis, may be complicated by infection and haemorrhage and determine the dismal prognosis of these patients.
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PMID:Case report and review of the literature. Fatal pulmonary complication in ankylosing spondylitis. 945 16

Two warmblood horses with a history of chronic weight loss and inappetence were referred to the Faculty of Veterinary Medicine, Ludwig-Maximilians University of Munich, Germany, for further examination. The clinical signs in horse 1 were fever, tachycardia and tachypnoea, and chronic ulcerative keratopathy of both eyes. Horse 2 had severe oral ulcerations and was coughing during feeding. In both horses, increased bronchovesicular sounds were heard during auscultation of the lungs. Laboratory findings included mild anaemia, lymphopenia and hypoalbuminaemia. Radiographic examination of the thorax revealed a severe nodular interstitial pattern. Multiple nodular lesions on the surface of the lung were observed by ultrasonographic examination. Light microscopy of lung biopsy specimens obtained from horse 1 revealed a severe chronic fibrosing interstitial pneumonia. Both horses were eventually euthanased because of a poor prognosis. Postmortem examination confirmed severe multinodular fibrosing interstitial pneumonia in both horses, and lung tissue yielded positive results for equine herpesvirus type 5 DNA using PCR assay. On the basis of the clinical, radiographic and pathological findings, as well as the PCR results, the diagnosis of equine multinodular pulmonary fibrosis was established.
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PMID:Clinical aspects of multinodular pulmonary fibrosis in two warmblood horses. 2036 10

Combined pulmonary fibrosis and emphysema (CPFE) syndrome is an uncommon entity characterised by emphysema of the upper lobes and diffuse fibrosis of the lower lobes and carries a bad prognosis with the onset of pulmonary hypertension. Lung involvement due to exposures suffered by welders is generally considered benign though, rarely, a diffuse interstitial fibrotic disease has been reported. CPFE syndrome has however never been reported in welders. A 65-year-old man, welder by occupation and an ex-smoker, presented with progressive exertional dyspnoea associated with dry cough noticed for the last four months. On examination, there was mild tachypnea, clubbing and bilateral basal velcro crepitations on chest auscultation. Lung function test revealed mild mixed ventilatory impairment with severe diffusion defect. HRCT chest showed bilateral upper lobe emphysema and diffuse interstitial fibrosis in the lower lobes. Transbronchial lung biopsy revealed interstitial fibrosis, chronic inflammation and iron deposits. A diagnosis of combined pulmonary fibrosis with emphysema (CPFE) with interstitial pulmonary siderofibrosis (IPS) was established. A review of literature did not show any other report of a similar nature.
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PMID:Combined pulmonary fibrosis and emphysema in a welder. 2266 43


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