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1/8. Angioneurotic edema of the upper airways and antihypertensive therapy.

    Angioneurotic edema is a non-pitting edema which is usually limited to the skin and the mucous membranes of the face and upper aerodigestive tract. The risk of acute upper airway obstruction makes angioneurotic edema a concern for emergency room physicians, internists and otolaryngologists because prompt recognition of the condition and immediate institution of therapy is essential for proper airway management. Angiotensin-converting enzyme (ACE) inhibitors have recently been associated with angioneurotic edema, the probable link being the reduction in angiotensin ii and the potentiation of bradykinin, resulting in vasodilatation, increased vascular permeability and angioedema. We report four cases of acquired angioneurotic edema, which were probably related to ACE inhibitor use. These cases are discussed, including a review of the literature, methods of diagnosis, pathophysiology and treatment of angioedema. Care should be taken when antihypertensive ACE inhibitor treatments are started and patients should be warned of the potential risk of angioneurotic edema.
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ranking = 1
keywords = mucous membrane, membrane
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2/8. Nine cases of accidental exposure to dimethyl sulphate--a potential chemical weapon.

    Dimethyl sulphate (DMS) is an innocuous appearing, widely used, and highly toxic chemical. It is used both as a methylating agent in industrial chemical synthesis and in medical laboratories for chemical cleavage of dna. It is readily absorbed through the skin, mucous membranes, and gastrointestinal tract. Delayed toxicity allows potentially fatal exposures to occur prior to development of any warning symptoms. Toxicity is manifested initially by mucosal inflammation of eyes, nose, oropharynx, and airways. This can progress to severe airway oedema and necrosis, and non-cardiogenic pulmonary oedema. Other systemic effects include convulsions, delirium, coma, and renal, hepatic, and cardiac failure. All these features make DMS a potential chemical weapon.We report nine cases of varying degrees of inhalational exposure to DMS, occurring as a result of a single chemical spillage incident in the United Kingdom. Industrial poisoning is surprisingly rare and there are few previous reports in the literature outside china.
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ranking = 1
keywords = mucous membrane, membrane
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3/8. A novel CFTR mutation found in a Chinese patient with cystic fibrosis.

    BACKGROUND: Cystic fibrosis (CF) is rare in Chinese. We investigated the mutations in the gene of cystic fibrosis transmembrane conductance regulator (CFTR) in a Chinese CF patient and reviewed the clinical features, gene mutations in Chinese CF cases. methods: blood samples were collected from a previously reported CF girl and her parents. The 24 coding exons of CFTR of the proband were amplified and sequenced. RESULTS: A Chinese girl of 16 years old was diagnosed as CF at the age of 14. She had recurrent productive cough with bronchiectasis in bilateral upper lobes, parasinusitis and otitis media, but without pancreatic involvement. Her sweat chloride was (108.9 /- 3.3) mmol/L. A heterozygous novel missense mutation of 699 C --> A which results in the amino acid change of N189K was identified in exon 5. In addition, a heterozygous 3821 - 3823 delT mutation in exon 19 was found in CFTR. The mutation 699C --> A was inherited from her father, and the 3821 - 3823 delT mutation was from her mother. Twenty patients with CF in Chinese reported from 1974 to 2004 were also reviewed. DelF508 mutation was not found in the nine cases whose CFTR mutations were analyzed. CONCLUSIONS: The CF proband carries two heterozygous mutations (699C --> A and 3821 - 3823 delT) in CFTR. 699C --> A mutation is a novel mutation which is not reported previously. review of reported Chinese cases suggests that the genotype of Chinese CF may be different from those of white cases. More studies are needed to understand the spectra of CFTR and clinical CF features in Chinese.
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ranking = 0.0031627486968667
keywords = membrane
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4/8. Benign mucous membrane pemphigoid of the upper aero-digestive tract: rare paraneoplastic syndrome presentation in renal cell carcinoma.

    Benign mucous membrane pemphigoid is a rare autoimmune disorder affecting the upper aero-digestive tract and conjunctivae. This is a case presentation of benign mucous membrane pemphigoid affecting the oral mucosa, pharynx, oesophagus and larynx, leading to cicatricial lesions in the pharynx and larynx, causing dysphagia, hoarseness and stridor. The alternative forms of management for laryngeal scarring due to this disease are explained. The patient was later diagnosed with advanced renal cell carcinoma, raising the possibility of cicatricial pemphigoid manifesting as a paraneoplastic syndrome of underlying renal cell carcinoma.
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ranking = 6
keywords = mucous membrane, membrane
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5/8. A case report of severe tracheal obstruction requiring extracorporeal membrane oxygenation.

    The authors describe the case of a near-fatal airway obstruction requiring extracorporeal membrane oxygenation. The patient presented with severe respiratory distress owing to a bean impacted in the distal trachea. The foreign body could not be removed by bronchoscopy because of instability of the patient. The patient was placed on extracorporeal membrane oxygenation for temporary pulmonary support, and the foreign body was removed using a rigid bronchoscope. The patient had a full recovery and suffered no neurologic sequelae.
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ranking = 0.0189764921812
keywords = membrane
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6/8. Ultrastructure of bronchial submucosal glands in children with chronic respiratory diseases.

    The ultrastructure of bronchial submucosal glands in 4 children with chronic respiratory diseases was studied. Based on their ultrastructure the glandular secretory cells were divided into four categories: 1. mucous cells packed with large electron-lucent coalescing granules with fibrogranular matrix, 2. mucous-like cells with abundant granular endoplasmic reticulum and fewer smaller electron-lucent granules, 3. serous cells containing small to moderately large membrane-bound electron-dense granules with homogenous matrix and prominent granular endoplasmic reticulum, 4. sero-mucous cells similar to the serous ones but containing granules of either serous or mucous morphology. In our patients the mucous and mucous-like cells prevailed, forming 54.6%-100% of the glands' secretory area. Only in 2 children all four types of secretory cells were detected. Signs of pathological alteration were discovered in secretory cells of all patients. The system of intra- and interlobular ducts was lined by tall mucous cells.
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ranking = 0.0031627486968667
keywords = membrane
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7/8. Respiratory complications of mucopolysaccharide storage disorders.

    Twenty-one patients with the diagnosis of mucopolysaccharidosis or mucolipidosis and a history of respiratory complaints or thorough respiratory evaluation were studied retrospectively. Anatomic factors affecting respiratory status included: (i) upper airway narrowing by hypertrophied tongue, tonsils, adenoids, and mucous membranes; (ii) lower airway narrowing by glycosaminoglycan deposition within the tracheobronchial mucosa; (iii) decreased thoracic dimensions due to scoliosis and thoracic hyperkyphosis; and (iv) decreased abdominal dimensions due to lumbar hyperlordosis, gibbus formation and hepatosplenomegaly. Cardiac and neurologic involvement, while present, did not play primary roles in the development of respiratory disease. The functional consequences of these findings included increased risk of developing: (i) respiratory tract infections; (ii) airway compromise during or after anesthesia or sedation; (iii) dyspnea on exertion; (iv) obstructive lung disease; (v) obstructive sleep apnea; and (vi) cor pulmonale. A management approach is presented which can reduce the morbidity and mortality experienced by these patients.
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ranking = 1
keywords = mucous membrane, membrane
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8/8. The trachea in children with respiratory diseases including children presenting as cot deaths.

    The tracheas of 74 children with respiratory disorders were analysed and compared with the tracheas of children with no respiratory symptoms. The test tracheas showed an increase in the size of the lumen and the length of the cartilage and membrane, the increase in the length of membrane being particularly striking. The relation between age at onset and duration of symptoms was uncertain.
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ranking = 0.0063254973937333
keywords = membrane
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