Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Life threatening mediastinitis as a complication of acute epiglottitis is very rare. A 38-year-old male in previously good health was admitted to our hospital in a state of unconsciousness. Seven days prior to admission he had complained of a sore throat, dysphagia, high fever and dyspnea. A chest X-ray on admission showed widening of the mediastinum, mediastinal emphysema, subcutaneous emphysema and left pleural effusion. Bronchoscopy showed the swelling of supraglottic structures. He was diagnosed as having acute mediastinitis and pyothorax as a complication of acute epiglottitis, but pathogens were not identified. The blood was hyperglycemic and insulin therapy was started. Though he gradually improved by massive antibiotic therapy, steroid therapy, tracheotomy and surgical drainage of both the left thoracic cavity and the mediastinum, he died suddenly of massive hemoptysis. Autopsy revealed that the acute mediastinitis had healed, but that the Aspergillus infection was present in both lungs and the pericardium. The Aspergillus infection was not lethal in the present case, and it seemed that death had resulted from arterial hemorrhage caused by erosion of the trachea. The present case suggests the need for antifungal therapy even in non-immunocompromised patients in particular when massive doses of antibiotics and steroids are administered.
 ...
PMID:[A case of mediastinitis and bilateral pyothorax, following acute epiglottitis with concurrent Aspergillus infection]. 140

Proteinase 3 (PR-3) is a human polymorphonuclear leukocyte (PMNL) serine proteinase that degrades elastin in vitro and causes emphysema when administered by tracheal insufflation to hamsters (Kao, R. C., Wehner, N. G., Skubitz, K. M., Gray, B. H., and Hoidal, J. R. (1988) J. Clin. Invest. 82, 1963-1973). We have determined the primary structure of several PR-3 peptides and have analyzed catalytic properties of the enzyme. The enzyme has considerable amino acid sequence homology with two other well characterized PMNL neutral serine proteinases, elastase and cathepsin G. Furthermore, the NH2-terminal amino acid sequence of PR-3 is identical to that of the target antigen of the anti-neutrophil cytoplasmic autoantibodies associated with Wegener's granulomatosis. PR-3 degrades a variety of matrix proteins including fibronectin, laminin, vitronectin, and collagen type IV. It shows no or minimal activity against interstitial collagens types I and III, respectively. The analysis of peptides generated by PR-3 digestion of insulin chains and the activity profile against a panel of chromogenic synthetic peptide substrates show that PR-3 prefers small aliphatic amino acids (alanine, serine, and valine) at the P1 site. The elastase-like specificity of PR-3 is consistent with its striking sequence homology to elastase at substrate binding sites. PR-3 is inhibited by alpha 1-proteinase inhibitor (ka = 8.1 x 10(6) M-1 S-1; delay time = 25 ms) and alpha 2-macroglobulin (ka = 1.1 x 10(7) M-1 S-1; delay time = 114 ms) but not by alpha 1-anti-chymotrypsin. In contrast to elastase and cathepsin G, PR-3 is not inhibited by secretory leukoprotease inhibitor and is weakly inhibited by eglin c. Thus, PR-3 is distinct from the other PMNL proteinases.
 ...
PMID:Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties. 203 50

Plasma cyclic AMP responses to adrenaline administration in normal volunteers, patients with spinocerebellar degeneration, bronchial asthma, pulmonary emphysema, and diabetes mellitus were studied. Intramuscular administration of low doses (0.1--0.4 mg/person) of adrenaline caused a dose-dependent increase in plasma cyclic AMP. The increase in cyclic AMP was completely prevented by propranolol, while it was not affected by phentolamine or atropine. In patients with spinocerebellar degeneration, the concentrations of plasma cyclic AMP both before and after adrenaline administration were lower than in normal subjects. In asthmatic patients, the plasma cyclic AMP increase after adrenaline administration was smaller than that of the healthy controls. The plasma concentration of cyclic AMP in patients with insulin-dependent diabetes reached the peak level more slowly than in diabetic patients with dietary control alone. Examining changes in the plasma cyclic AMP level after adrenaline administration appears to be a useful means for assessing the degree of beta-adrenergic dysfunction.
 ...
PMID:Plasma cyclic AMP responses to adrenaline administration in patients with spinocerebellar degeneration, bronchial asthma, and diabetes mellitus--a preliminary report of a clinical test for detecting beta-adrenergic dysfunctions. 630 12

A 56-year old diabetic patient of over 20 years duration was admitted with gangrene of right foot. About a month prior to admission he injured the fourth toe of the right leg when he cut the toe nail. Two weeks later necrotic ulcer was present on it with cellulitis and 2nd, 3rd and 5th toes were also affected. Then his entire right leg was swollen and he developed fever. In spite of the treatment with antibiotics and insulin, gangrenous lesions progressed and subcutaneous emphysema was palpable beneath the inflamed area. On the 9th hospital day amputation was carried out below the knee. He had a good recovery 6 weeks after the amputation. Aerobic and anerobic cultures from the foot yielded bacteroides and pseudomonas aeruginosa. Clostridia were not isolated. A review was also carried out on ten diabetic cases of nonclostridial gas gangrene in the lower limb in Japan.
 ...
PMID:A survived case of diabetes with nonclostridial gas gangrene. 640 67

A 47-year-old patient with panlobular emphysema and insulin-dependent diabetes had an alpha-1-antitrypsin phenotype Pi ZZ deficiency. Liver function tests were abnormal, and postmortem examination of the liver demonstrated abnormal intrahepatocytic globules of A1AT (a typical finding when the allele Z is present), but also fibrosis with steatosis. The patient's sister, Pi ZZ, had neither diabetes nor bronchopneumopathy, and no anomalies in liver function. Needle puncture biopsy of the liver had not been conducted. The phenotype Pi ZZ is typically associated with panlobular emphysema in adults, and cholestatic hepatitis in children. From reports in the published literature, it appears that isolated hepatic lesions or those associated with emphysema are rare. The fortuitous association of diabetes and hepatic lesions in this typical case of pulmonary affection in an adult is discussed.
 ...
PMID:[Pulmonary emphysema, hepatic lesions, and insulin-dependent diabetes in a patient with alpha-1-antitrypsin (Pi ZZ) deficiency (author's transl)]. 697 May 36

In order better to understand the pathophysiology of the equine form of emphysema, two elastinolytic enzymes from horse neutrophils, referred to as proteinases 2A and 2B, have been extensively characterized and compared with the human neutrophil proteinases, proteinase-3 and elastase. Specificity studies using both the oxidized insulin B-chain and synthetic peptides revealed that cleavage of peptide bonds with P1 alanine or valine residues was preferred. Further characterization of the two horse elastases by N-terminal sequence and reactive-site analyses indicated that proteinases 2A and 2B have considerable sequence similarity to each other, to proteinase-3 from human neutrophils (proteinase 2A), to human neutrophil elastase (proteinase 2B) and to a lesser extent to pig pancreatic elastase. Horse and human elastases differed somewhat in their interaction with some natural protein proteinase inhibitors. For example, in contrast with its action on human neutrophil elastase, aprotinin did not inhibit either of the horse proteinases. However, the Val15, alpha-aminobutyric acid-15 (Abu15), alpha-aminovaleric acid-15 (Nva15) and Ala15 reactive-site variants of aprotinin were good inhibitors of proteinase 2B (Ki < 10(-9) M) but only weak inhibitors of proteinase 2A (Ki > 10(-7) M). In summary, despite these differences, the horse neutrophil elastases were found to resemble closely their human counterparts, thus implicating them in the pathological degradation of connective tissue in chronic lung diseases in the equine species.
 ...
PMID:Structural and functional characterization of elastases from horse neutrophils. 751 52

A 75-g oral glucose tolerance test (OGTT) was performed on 18 patients with chronic respiratory failure and without fasting hyperglycemia, positive urine glucose, or hepatic/pancreatic disorders. Underlying diseases in these patients were pulmonary emphysema (11 cases, 61%), pulmonary tuberculosis (5 cases, 28%), and chronic bronchial asthma (2 cases, 11%). The body mass index (mean +/- SD, 17.6 +/- 2.2 kg/m2, P < 0.001) in these patients was significantly lower than that (23.8 +/- 3.1 kg/m2) in normal subjects. The OGTT results showed an impaired glucose tolerance pattern in 9 cases (50%) and a diabetes mellitus pattern in 6 cases (34%). The mean two-hour plasma glucose value in the patients was 9.8 mmol/L. However, insulin secretion responded well to glucose loading. These results suggest that a high proportion of chronic respiratory failure patients may have an intolerance for glucose loading but a normal insulin secretion pattern.
 ...
PMID:Glucose intolerance in chronic respiratory failure. 797 7

Chronic inhalation of coal dust can cause several lung disorders, including simple coal workers pneumoconiosis (CWP), progressive massive fibrosis (PMF), chronic bronchitis, lung function loss, and emphysema. This review focuses on the cellular actions and interactions of key inflammatory cells and target cells in coal dust toxicity and related lung disorders, i.e. macrophages and neutrophils, epithelial cells, and fibroblasts. Factors released from or affecting these cells are outlined in separate sections, i.e. (1) reactive oxygen species (ROS) and related antioxidant protection mechanisms, and (2) cytokines, growth factors and related proteins. Furthermore, (3) components of the extracellular matrix (ECM), including the modifying role of ROS, cytokines, proteases and antiproteases are discussed in relation to tissue damage and remodelling in the respiratory tract. It is recognised that inhaled coal dust particles are important non-cellular and cellular sources of ROS in the lung, and may be significantly involved in the damage of lung target cells as well as important macromolecules including alpha-1-antitrypsin and DNA. In vitro and in vivo studies with coal dusts showed the up-regulation of important leukocyte recruiting factors, e.g. Leukotriene-B4 (LTB4), Platelet Derived Growth Factor (PDGF), Monocyte Chemotactic Protein-1 (MCP-1), and Tumor Necrosis Factor-alpha (TNF alpha), as well as the neutrophil adhesion factor Intercellular Adhesion Molecule-1 (ICAM-1). Coal dust particles are also known to stimulate the (macrophage) production of various factors with potential capacity to modulate lung cells and/or extracellular matrix, including O2-., H2O2, and NO, fibroblast chemoattractants (e.g. Transforming Growth Factor-beta (TGF beta), PDGF, and fibronectin) and a number of factors that have been shown to stimulate and/or inhibit fibroblast growth or collagen production such as (TNF alpha, TGF beta, PDGF, Insulin Like Growth Factor, and Prostaglandin-E2). Further studies are needed to clarify the in vivo kinetics and relative impact of these factors.
 ...
PMID:Mechanisms and mediators in coal dust induced toxicity: a review. 1002 91

A 74-year-old woman with diabetes mellitus had a high fever, and was treated with antibiotics and insulin in another hospital. She was referred to our department, because CT scan showed the right hydronephrosis and the abnormal gas shadow in the right renal calyces. Ureteral catheterization was performed on the right side and cloudy urine was drained. Urine culture yielded E. coli. Since submucosal emphysematous changes were demonstrated in the bladder mucosa by cystoscopy, she was diagnosed with emphysematous pyelonephritis with emphysematous cystitis associated with diabetes mellitus. Administration of antibiotics and insulin and the ureteral catheter drainage improved her condition immediately. Abnormal gas shadow on CT scan and submucosal emphysema on cystoscopy disappeared. We reviewed 110 cases of emphysematous pyelonephritis and 23 cases of emphysematous cystitis including our case in Japan, and report their clinical characteristics.
 ...
PMID:[A case of emphysematous pyelonephritis with emphysematous cystitis]. 1054 Jul 9

We have recently identified a novel gene, klotho (kl), which may suppress several aging phenotypes. A defect of kl gene expression in the mouse results in a syndrome resembling human aging, such as arteriosclerosis, skin atrophy, osteoporosis, and pulmonary emphysema. To determine whether mouse homozygotes for the kl mutation (kl/kl) show abnormal glucose metabolism, an oral glucose tolerance test (OGTT) was performed at 6 to 8 weeks of age. Blood glucose levels during the OGTT were significantly lower in kl/kl mice versus wild-type mice. The insulin content of the pancreas was significantly lower in kl/kl mice compared with wild-type mice. Decreased insulin production was also supported by Northern blot analysis showing lower levels of insulin mRNA in kl/kl mice. To examine how lower blood glucose levels may exist in kl/kl mice despite decreased insulin production, insulin tolerance tests (ITTs) were performed. The glucose decline following insulin injection was more severe in kl/kl mice versus wild-type mice, suggesting that insulin sensitivity was higher in kl/kl mice versus wild-type mice. In kl/kl mice, an augmented expression of GLUT4 in skeletal muscle was demonstrated by both Northern blot analysis and Western blot analysis. Thus, we conclude that insulin production is decreased and insulin sensitivity is increased in the klotho mouse, a novel animal model for human aging.
 ...
PMID:Decreased insulin production and increased insulin sensitivity in the klotho mutant mouse, a novel animal model for human aging. 1101 90


1 2 3 4 Next >>