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)

Scleroderma is an uncommon complex disease. The onset is slow and the progress is chronic. The main pathophysiological changes vary; they affect blood vessels, connective tissue, collagen fibres, cause fibrin deposition and inflammatory reactions. There may be early oedema and a wide spectrum of organic involvement. Clinically, all the fibril-containing and connective tissue organs can be attacked in various degrees. The most common organ manifestations are the Raynaud's phenomenon in the arms and hands, vascular fibrosis, stiff and hard facial skin, restriction of joint movement by pericapsular hardening, calcium deposition and capsular rigidity. In the gastrointestinal tract muscle atrophy, collagen and connective tissue damage are common, especially at the cardia. Malabsorption may occur. Progressive pulmonary fibrosis leads to cor pulmonale and respiratory insufficiency. The liver, kidneys and the endocrine glands are, however, seldom involved. Therapeutic trials have been performed using many different groups of drugs: vasodilatating agents, corticosteroids, drugs found experimentally to influence connective tissue, thyroxine and a variety of anti-rheumatic agents. In the last decade best short-term clinical results have been achieved with penicillamine, some vasodilators, chlorambucil and in recent years with cyclofenil a potent anti-oestrogen, which has marked connective tissue and collagen metabolism influencing properties. Good therapeutic effects without serious side effects have been achieved.
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PMID:Scleroderma (progressive systemic sclerosis, PSS); pathophysiological, clinical and pharmacological aspects of the syndrome. 75 10

The major difference between heart disease in the old and the young is that elderly people almost have one or more other major, even life-threateninng conditions. These associated diseases challenge the physician in establishing a diagnosis and prognosis, understanding the pathogenesis of the symptoms and signs, and determining the best managemnt. Another important difference is that old persons have senescent changes in all organ systems, whether the heart is normal or diseased. Dyspnea, especially on exertion, is common, and so is edema of the feet and ankles. Chest x-rays show changes typical of old age, e.g., small lung fields, mild to moderate pulmonary fibrosis, and calcium plaques in the aorta. In the absence of cardiac disease, the heart is normal in size or even small. Translationg experience in managing heart disease in young patients to elderly patients must be done cautiously. Old people tend to be confused, delicate, mentally and physically slow, feeble, and unreliable, and they are more sensitive to most-if not all-drugs, especially digitalis. The physician should see them often and question them routinely about their symptoms, signs, and medication. Decisions on management should not be hurried, except in an emergency situation.
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PMID:The special problems of heart disease in old people. 83 77

This study was performed to evaluate bronchopulmonary lavage and chelation therapy as a treatment method to prevent the development of radiation pneumonitis after inhalation of a radioactive aerosol. Twelve beagle dogs were exposed to an aerosol of cerium-144 in fused clay particles resulting in initial lung burdens from 47 to 64 muCi of 144-Ce per kg of body weight. Eight of the dogs were treated with a series of 10 bronchopulmonary lavages and 10 intravenous injections of calcium diethylenetriamine pentaacetate acid during the first 56 days after exposure to remove the deposited 144-Ce; the remaing 4 exposed dogs receiged no treatment. An additional 4 dogs were exposed to stable cerium and were given the course of treatment as an additional control group. Three of the 4 untreated dogs and 2 of the 8 treated dogs died 171 to 246 days after exposure with radiation pneumonitis or pulmonary fibrosis, or both. All but one of the remaining dogs were alive and apparently in good clincial health 550 dyas after exposure; the one dog had radiographic indications of pulmonary fibrosis by 365 days after exposure. The relative distribution of 144-Ce in the lungs and other major organs was similar in the treated and untreated dogs that died.
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PMID:Prevention of radiation pneumonitis from inhaled cerium-144 by lung lavage in beagle dogs. 113 48

A 26 year-old male presented with a spontaneous pathological fracture of the right femur caused by an osteolytic lesion. A chest X-ray demonstrated the coexistence of interstitial pulmonary fibrosis. Fibrocaseous tuberculosis and widespread malignancy were ruled out by appropriate investigations. The differential diagnosis, as based on the histological examination of the bone tumour, rested between hyperparathyroidism complicated by pulmonary disease and eosinophilic granuloma with lung manifestations. Hyperparathyroidism was ruled out by the absence of clinical and biochemical evidence of a disturbance of calcium metabolism. The diagnosis of histiocytosis-X was established by the histological appearance of the lung biopsy.
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PMID:[Eosinophilic granuloma (histiocytosis-X) with lung and bone manifestations]. 122 55

Cyclic nucleotides play an important role in the regulation of fibroblast proliferation and collagen metabolism. In the present study, the antifibrotic potential of dibutyrylcAMP (Bt2cAMP) was evaluated in the bleomycin (BLM)-hamster model of pulmonary fibrosis. Bt2cAMP (10 mg kg-1, s.c.) or saline (SA, s.c.) was given daily two days prior to the first intratracheal (i.t.) dose of BLM or SA and thereafter throughout the study. BLM or SA was instilled i.t. in three consecutive doses (2.5, 2.0 and 1.5 U 5ml-1 kg-1) at weekly intervals. Hamsters were killed at 7, 14 and 20 days after the third i.t. instillation. Bt2cAMP significantly reduced the contents of lung hydroxyproline and lung thiobarbituric acid reactive substance equivalents in BLM-treated animals at 7 and 14 days. Bt2cAMP significantly elevated lung superoxide dismutase activity in BLM-treated animals at 7 days. Lung prolyl hydroxylase activity was significantly elevated at 14 and 20 days in SABLM- and Bt2cAMPBLM-treated animals. The ratio of cAMP/cGMP was significantly reduced at all time points in SABLM-treated animals but only at 7 and 14 days in Bt2cAMPBLM-treated animals. Bt2cAMP caused no significant changes in lung calcium and calmodulin levels and protein content of the bronchoalveolar lavage. BLM significantly increased various inflammatory cell counts in the lavage at all three time points. The cell counts in the Bt2cAMPBLM groups were generally lower at 7 days and higher at 20 days than those of the SABLM groups. Histological evaluation showed that the lungs of Bt2cAMPBLM-treated hamsters progressed from an inflammatory cell lesion to a fibrotic lesion at a slower rate than the SABLM groups. It was concluded that Bt2cAMP attenuated BLM-induced pulmonary fibrosis in hamsters in part by delaying the acute phase of the inflammatory reaction.
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PMID:Effects of dibutyrylcyclic adenosine monophosphate on bleomycin-induced lung toxicity in hamsters. 137 23

The bleomycin (BL)-hamster model of interstitial pulmonary fibrosis (IPF) is generally associated with increased lung lipid peroxidation, measured as malondialdehyde equivalent (MDAE), calcium and collagen content; and superoxide dismutase (SOD), prolyl hydroxylase (PH) and poly(ADP-ribose) polymerase activities. We found that combined treatment with taurine in drinking water (1%) and niacin IP (250 mg/kg) daily, significantly decreased the BL-induced increases in lung MDAE and calcium content, and SOD, PH and poly(ADP-ribose) polymerase activities. This treatment almost completely ameliorated the BL-induced increases in the lung collagen accumulation as well. Findings of a similar nature were also demonstrated when taurine (2.5%) and niacin (2.5%) were supplemented in the diet of hamsters used in the same BL model of IPF. The diet supplemented with taurine (2.5%), niacin (2.5%), or taurine (2.5%) + niacin (2.5%) also reduced AD-induced increases in lung collagen accumulation, phospholipids, MDAE and SOD activity. It was concluded that diet supplemented with taurine and/or niacin would completely or partially ameliorate chemically-induced pulmonary fibrosis.
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PMID:Taurine and niacin offer a novel therapeutic modality in prevention of chemically-induced pulmonary fibrosis in hamsters. 138 Jul 62

Sixteen beagles were allocated into 4 groups, each group consisting of 2 males and 2 females, which were injected sc with 1,3,5 or 7 mg paraquat/kg. The beagles were observed for 2 w after the administration. At the end of the observation period all the dying and surviving dogs were studied pathologically. The LD50 was calculated as 1.8 (1.0-6.1) in males and 3.5 (2.4-10.1) mg/kg in females. Clinical laboratory tests showed increases in segmented neutrophils and monocytes, decreases in lymphocytes, slight decreases in chloride, moderate increases in BUN, GOT, GPT and phospholipids, slight increases in uric acid, total protein, creatine, total cholesterol and total bilirubin, and prolonged prothrombin times. Marked edema, congestion and hemorrhage of lungs, as well as slight congestion in various organs, were observed grossly. In histopathological examination, marked pulmonary hemorrhage and congestion, fibroblast-like cells in alveolar septa, breakdown of alveolar walls, thickening of alveolar walls and pleura, mild congestion and degeneration of the liver, and mild degeneration of renal tubules were observed. The cause of death was respiratory distress and renal failure. The surviving animals had mild atelectasis of the lungs. Electromicroscopic examination on the surviving animals revealed the appearance of spindle-shaped cells, proliferation of type II alveolar cells and fibroblasts, mitosis of fibroblasts, and abundant collagen fiber in the lung, calcium deposition, stratification and thickening of basement membranes, and localized necrotic epithelial cells in the proximal tubules of kidneys, and stratification of intramitochondrial cristae of the liver. Pulmonary fibrosis in the switchover stage was present with participation from type II alveolar cells, fibroblasts and myofibroblasts.
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PMID:Acute toxicological studies on paraquat: pathological findings in beagle dogs following single subcutaneous injections. 150 67

Familial hypocalciuric hypercalcemia (FHH) is usually characterized by asymptomatic hypercalcemia, mild hypermagnesemia, and low urinary calcium excretion, and is occasionally associated with pulmonary fibrosis. It is inherited as an autosomal-dominant, and no sporadic case of hypocalciuric hypercalcemia has been heretofore reported. This report describes a patient with hypocalciuric hypercalcemia completely compatible with FHH but with no family history, suggesting that the most likely diagnosis is "nonfamilial" hypocalciuric hypercalcemia. We propose that the urinary excretion of calcium be examined in all patients with hypercalcemia, hypophosphatemia, and increased PTH before neck surgery, even if patients have no family history of hypercalcemia.
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PMID:A case of hypocalciuric hypercalcemia without family history. 182 36

Clinical symptoms and signs and the frequency of abnormalities in the results of enzymatic, electromyographic, histological and serological tests were analysed in 50 patients, including 17 with polymyositis (PM) and 33 with dermatomyositis (DM), groups I and II according to Bohan's classification and followed-up for 25 years. Weakness of the proximal muscles of the extremities was present in both groups in all patients and in a high proportion of cases weakness involved also the distal muscles. Dysphagia was more frequent in DM patients (54.5%) than in PM (17.6%). In DM erythema and facial oedema as well as Gottron's sign were observed more frequently. In 11.8% of PM patients and 15.1% of DM patients deposition of calcium salts in subcutaneous tissue developed. Signs of vasculitis were found in 39.4% of DM cases and 17.6% of PM cases. In one-third of PM and in only 3 DM cases pitting oedema of the distal parts of the extremities was noted. Cardiovascular changes were disclosed in 82.3% of PM and 69.7% of DM patients. On the other hand, radiological signs of interstitial pulmonary fibrosis were noted more frequently in DM (36%) than in PM (23%). Increased serum activity of CPK, AspAT and ALAT was present with similar frequency in both groups from 71% to 81% of cases. EMG showed evidence of primary muscular changes in all PM patients and 69.6% of DM patients. Histological examination confirmed the diagnosis in the studied patients. Antinuclear antibodies were found relatively rarely, from 2% to 24% of PM/DM patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Polymyositis-dermatomyositis--a 25-year follow-up of 50 patients (analysis of clinical symptoms and signs and results of laboratory tests). 213 28

To determine whether the clinical, immunological and serological features of patients with silica-associated systemic sclerosis are different from patients with the 'idiopathic' form of systemic sclerosis (SS) we studied 22 underground coal miners who were exposed to silica dust (SD), 30 mine workers who later developed silicosis (S) and 17 mine workers exposed to silica dust who subsequently developed a systemic sclerosis-like disease (SA-SS). The patients with SA-SS had features clinically indistinguishable from individual patients with SS. They all had Raynaud's phenomenon, 14 had cutaneous sclerosis identical to that seen in acrosclerosis and three had a generalized cutaneous sclerosis. Sixteen patients had bibasilar pulmonary fibrosis, 10 had necrosis of the fingertip pulps, nine had oesophageal involvement and only one patient had renal involvement. Antinuclear antibodies and circulating immune complexes were detected in three and eight patients with SD, 14 and five patients with S and in 16 and nine patients with SA-SS, respectively. Anti-Scl-70 antibody was detected in eight of the 17 patients with SA-SS. Evidence for in vivo endothelial cell damage, as determined by elevated levels of von Willebrand factor, was found in nine patients with SD, 14 patients with S and in 10 patients with SA-SS. Following incubation of the patient's serum with confluent cultures of human umbilical vein endothelial cells there was only a significant reduction in calcium ionophore-induced release of prostacyclin with the serum from SA-SS patients compared to that with control serum (NC). The mean +/- SEM release of 6-keto-PGF1 alpha (the stable metabolite of prostacyclin expressed as ng/10(4) cells) decreased from 2.90 +/- 0.27 to 2.01 +/- 0.33 (SD), 3.34 +/- 0.42 to 1.76 +/- 0.31 (S), 1.98 +/- 0.12 to 0.64 +/- 0.07 (SA-SS) and 2.28 +/- 0.33 to 1.36 +/- 0.21 (NC) with 1 and 20% serum, respectively. This study demonstrates that immune complex and antinuclear antibody formation and in vivo endothelial cell damage occurs following occupational exposure to silica. The patients who subsequently develop a systemic sclerosis-like disease have clinical, immunological and serological features which are indistinguishable from the idiopathic form of the disease although as a group the SA-SS patients have a higher prevalence of pulmonary involvement and the anti-Scl-70 antibody.
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PMID:Silica-associated systemic sclerosis is clinically, serologically and immunologically indistinguishable from idiopathic systemic sclerosis. 217 91


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