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

Elastic fibres are present in lung structures including alveoli, alveolar ducts, airways, vasculature and pleura. The rate of lung elastin synthesis is greatest during fetal and neonatal development, and is minimal in the healthy adult. We have determined that glucocorticoids up-regulate fetal lung tropoelastin expression while concomitantly accelerating terminal airspace maturation. Because there is minimal turnover of elastin in healthy adult lung, the elastin incorporated into the lung early in development supports lung function for the normal lifespan. However, in the adult lung, in pathological circumstances such as emphysema or pulmonary fibrosis there may be reactivation of elastin expression. We have found in silica-induced pulmonary fibrosis that expression of tropoelastin is primarily increased in the walls and the septal tips of the alveolus, with modest increases in other compartments which normally express tropoelastin during development. This finding suggests that the mesenchymal cell of the alveolar wall increases tropoelastin expression during fibrotic disorders. In emphysema and fibrosis, elastin is present in abnormal-appearing, probably non-functional, elastic fibres, suggesting that the adult lung cannot recapitulate the elastic fibre assembly mechanisms operative during normal lung growth.
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PMID:Elastin in lung development and disease. 857 58

The lung volume reduction surgery is now being accepted for patients with lung diffuse emphysema. The basic concept of this procedure is based on reducing overall lung volume by multiple pneumectomy. The lungs are resected on both pathologic and non-pathologic lesions. As a result, elastic pull on the small airway may be achieved and normal underlying compressed lung can be re-expanded. Additionally, the compliance of thoracic wall is ameliorated with normalized thoracic volume. On the other hand, laser ablation of emphysematous lung with bullaectomy under video assisted thoracoscopy (VATS) has been performed recently. This procedure is based on reducing the non-functional air volume due to bullae underlying the pleural surface. Both procedures have still having been under consideration in last three years. In this report, the latest result of both methods in the USA are reported and discussed. Additionally, comparative data with lung transplantation, the other surgical strategy for lung emphysema, is also discussed.
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PMID:[Lung volume reduction surgery (VRS) for lung diffuse emphysema]. 872 58

We report panniculitis revealing alpha-1 antitrypsin deficiency in 3 patients with different Pi phenotypes. The first patient, a 16-year-old woman, had inflammatory skin lesions on the abdomen for 6 months. The lesions regressed spontaneously. Serum alph-1 antitrypsin level was normal but the Pi phenotype was MS. The second case was observed in a 56-year-old man who developed erythematous subcutaneous nodules on the abdomen, legs and buttocks in a trauma context. Serum alpha-1 antitrypsin was very low and the Pi phenotype was ZZ. The third patient was a 40-year-old woman who presented red swelling nodules on the legs. Her serum alpha-1 antitrypsin level was at the lower limit of normal and the Pi phenotype was MZ. Alpha-1 antitrypsin deficiency is an autosomic codominant inherited disorder characterized by inefficient or non-functional serum alpha-1 antitrypsin. The principal clinical manifestations are panlobular emphysema and cirrhoses. About 30 cases of panniculitis have been reported in the literature. In patients presenting panniculitis, we suggest studying the Pi phenotype to determine functional deficiency even if the serum level of alpha-1 antitrypsin is normal.
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PMID:[Panniculitis revealing alpha-1 antitrypsin deficiency. Report of 3 cases]. 1193 84

Alpha1 antitrypsin (AAT) deficiency is an autosomic codominant inherited disorder characterized by inefficient or non-functional serum AAT. The principal clinical manifestations are panlobular emphysema and cirrhoses. Among cutaneous aspects, about 30 cases of panniculitis have been reported in the literature, likewise rare clinical cases: pemphigus herpetiformis, Muir Torre syndrome, urticaria and angioedema, cutis laxa and Marshall syndrome, lupus erythematosus, psoriasis, vasculitis. Probably because of it's high frequency, numerous others diseases have been reported described in association. Acting on several factors of inflammation, AAT deficiency seems product or modify the expression of some, notably cutaneous diseases.
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PMID:[Alpha-1-antitrypsin deficiency. Role in skin disorders]. 1266 63

Lung volume reduction surgery has recently become an option for the management of patients with severe emphysema. The procedure consist of resecting non-functional lung in order to improve respiratory mechanics. Because postoperative prolonged air leakage is a serious complication of the operation, most surgeons now use staplers reinforced with bovine pericardial strips to seal the lung. We report a patient where the migration of an intact pericardium patch caused an obstructive pneumonia and recurrent hemoptysis over several years.
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PMID:Late complication of bovine pericardium patches used for lung volume reduction surgery. 1282 92