Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bronchiectasis is pathologically defined as an abnormal and permanent dilatation of one or several bronchi. There are localized and generalized types of bronchiectasis. A vicious circle hypothesis, including an initial insult to the lower airways, impaired mucociliary clearance, microbial colonization/infection, bronchial obstruction and a local inflammatory response, has been proposed to explain the damage to the bronchial tree and the adjacent lung parenchyma. The clinical picture is variable and affected individuals might be asymptomatic or suffer from severe respiratory failure. Daily sputum production is the most common, though unspecific symptom of bronchiectasis. Other common symptoms are hemoptysis and recurrent episodes of sputum purulence, fever and pleurisy. Occasionally, major, life-threatening hemoptysis from a ruptured bronchial artery occurs. Infectious complications, e.g. lung abscess, empyema, brain abscess, and secondary amyloidosis are rarely seen today. The chest radiograph reveals changes suggestive of bronchiectasis in the majority of patients with clinically important disease. High resolution computed tomography of the lung has almost completely replaced bronchography for diagnosis, the latter rarely being of value if surgery is contemplated. No etiology is identified in about one- to two-thirds of the patients, although there are many diseases eventually associated with bronchiectasis. Prevention and therapy of underlying diseases are most important. Traditionally, the therapy of symptomatic bronchiectasis is based on antibiotics, antibronchoobstructive medication, and chest physical therapy. Surgical resection is the treatment of choice for localized symptomatic disease. Bilateral lung transplantation should be considered in younger patients with severe, generalized bronchiectasis and respiratory failure. Prospective, randomized, largescale trials supporting any of the different treatment strategies are not available, but antibiotics and surgery probably have improved the long-term outcome of many patients with bronchiectasis. In this review, some recent findings regarding the classification, pathogenesis, pathology, etiology, diagnosis, treatment, and prognosis of bronchiectasis are discussed.
 ...
PMID:[Bronchiectasis--current aspects of an old disease]. 915 28

Allergic bronchopulmonary aspergillosis (ABPA) is a disease resulting from a hypersensitivity response to Aspergillus fumigatus, although the pathogenesis of the disease is unknown and its prevalence in cystic fibrosis (CF) is still poorly defined. Data from the Epidemiologic Registry of Cystic Fibrosis (ERCF) on 12,447 CF patients gathered from 224 CF centres in nine European countries were analysed. The ERCF definition of ABPA diagnosis is a positive skin test and serum precipitins to A. fumigatus, together with serum immunoglobulin (Ig)E levels >1,000 U x mL(-1) and additional clinical or laboratory parameters. The overall prevalence of ABPA in the ERCF population was 7.8% (range: 2.1% in Sweden to 13.6% in Belgium). Prevalence was low <6 yrs of age but was almost constant approximately 10% thereafter. No sex differences were observed. ABPA affected 8.0% of patients with a deltaF508/deltaF508 genotype and 5-6% with deltaF508/G551D, deltaF508/G542X and deltaF508/N1303K genotypes. ABPA patients presented a lower forced expiratory volume in one second (FEV1) than those without ABPA at any age and the prevalence ranged from 6.6% in patients with FEV1 > or =20-12.9% in those with FEV1 <40%. ABPA was associated with higher rates of microbial colonization, pneumothorax and massive haemoptysis, and with higher IgG serum levels and poorer nutritional status. A mixed model regression analysis of lung function showed that FEVI decline during the follow-up period was not substantially different in ABPA patients compared with non-ABPA patients for any subgroups based on age or disease severity at enrollment. To conclude, allergic bronchopulmonary aspergillosis is a frequent complication in cystic fibrosis patients, particularly after the age of 6 yrs, and it is generally associated with a poorer clinical condition. However, any clear independent influence of allergic bronchopulmonary aspergillosis on the rate of lung function decline in the short term was not shown.
 ...
PMID:Allergic bronchopulmonary aspergillosis in cystic fibrosis. A European epidemiological study. Epidemiologic Registry of Cystic Fibrosis. 1148 9