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)

Hemoptyses are common in cystic fibrosis (CF) patients. They range from massive life-threatening (> 240 mL/24 hours) to recurrent minor streaking. Limited pulmonary reserve, potential concurrent chest infection, and the progressive nature of CF pose a high risk to this subgroup. Conservative management and selective bronchial artery embolization (BAE) control most acute episodes, but the recurrence rate is high. The possible need for lung transplantation in future makes an extrapleural approach for bronchial artery ligation desirable. The aim of this study was to assess the role of extrapleural bronchial artery ligation in the treatment of recurrent hemoptysis in CF patients. This is a retrospective analysis of four patients between 1986 and 1999 treated by extrapleural thoracotomy and ligation of bronchial arteries. Indications, surgical experience, and outcome are presented. Three patients underwent unilateral, and one patient bilateral extrapleural thoracotomy (in two separate sessions) for bronchial artery ligation. There were three men and one woman, with a mean age of 26.6 years (range 19-32 years). Indications were failure to stabilize the bronchial arterial catheter for BAE (three cases), recurrence after BAE previously controlled bleeding (one case), and communication with the right costocervical trunk signifying risk to the spinal circulation (one case). The mean follow-up was 68 months (range 3-144 months). There was one death in this series, a patient who was asphyxiated with hemoptysis, requiring ventilation preoperatively. He underwent successful extrapleural thoracotomy for bronchial artery ligation, with no further bleeding but succumbed to severe chest infection and multiorgan failure a few days later. Two patients had recurrent bleeding 12 and 36 months after surgery. Selective bronchial angiography proved the contralateral bronchial arteries to be the culprit. Extrapleural bronchial artery ligation is an effective method of controlling hemoptysis in CF, when BAE has failed. This approach minimizes pleural adhesions and is, therefore, desirable in the future consideration for lung transplantation. In this experience, muscle-sparing thoracotomy and postoperative epidural analgesia significantly improved the postoperative recovery.
 ...
PMID:Extrapleural bronchial artery ligation for life-threatening hemoptysis in cystic fibrosis--a case report. 1099 21

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

We have studied the characteristics of 202 cystic fibrosis adult patients, all with chronic respiratory symptoms, with a median age of 27 yrs (18 to 55 yrs) and a male predominance (56%). At genetic analysis, delta F508 homozygotes were 41%, delta F508 heterozygotes 42% and 17% had no delta F508. The respiratory disease was more severe and complications were more frequent in adults: hemoptysis in 14%, pneumothorax in 15%, lung transplantation in 25 patients. Chronic bronchial colonisation with Pseudomonas aeruginosa, in 76% of patients, contributed to making treatments more severe because of antibiotic i.v. courses and nebulised antibiotics. Respiratory function showed a mean FVC of 62 +/- 22% and a mean FEVI of 48 +/- 94%. External pancreatic insufficiency was found in 83%, diabetes in 14%. Intestinal occlusion syndromes were observed in 11% of patients and hepatic cirrhosis in 8%. In spite of the severity of the respiratory disease, theses patients succeeded in social and occupational insertion; 62% were independent, 18% had children and 77% were working or studying. Analysis of the patients according to age at diagnosis showed that, in 38 patients diagnosed after the age of 18 yrs, the respiratory disease was less severe, pancreatic insufficiency and non-respiratory complications were less frequent (34% had pancreatic insufficiency, 5% had diabetes and none had cirrhosis). This may partly be due to the presence of milder CFTR mutations. In conclusion, cystic fibrosis in adulthood frequently looks like an evolutive form of cystic fibrosis in childhood. Nevertheless, some late diagnosed forms in adults, with better prognosis, have been recently identified.
 ...
PMID:[Characteristics and specificities of cystic fibrosis in adults: evolutive disease of childhood or recently diagnosed disease?]. 1107 85

Respiratory impairment is present in almost all adult cystic fibrosis patients and makes the prognosis. Viscous, infected and abundant secretions, inflammation and bronchial oedema, bronchoconstriction and respiratory muscle fatigue lead to airway obstruction, bronchiectasis and respiratory failure. The disease is preferentially located in the upper lobes. Exacerbations of the disease are due to bronchial infections and are often responsible for drops of the respiratory function. Regular spirometric surveillance is fundamental for the prognosis and the assessment of the effects of the treatment. Among adult patients chronic colonisation with mucoid and often multiresistant strains of Pseudomonas Aeruginosa are common. It is treated with i.v. high doses antibiotic courses and nebulized antibiotics between i.v. courses. Respiratory failure may require long term oxygen and non invasive mechanical ventilation. Systemic hypervascularization around the bronchiectasis may lead to moderate to severe hemoptysis, which may require embolization. Pneumothorax are associated with poor prognosis and are treated by pleural drainage and if failure by thoracoscopy.
 ...
PMID:[Specific aspects and care of lung involvement in adults with cystic fibrosis]. 1107 86

Cystic fibrosis (CF) causes progressive respiratory failure and death in more than 90% of patients. Mechanical ventilation has been discouraged in CF because of poor outcomes, but improved survival and the availability of lung transplantation have increased the indications for care of CF patients in the intensive care unit (ICU). We studied the outcomes of all CF patients admitted to the University of North Carolina Hospitals Medical ICU from January 1990 through December 1998. Seventy-six patients, ranging in ages from 17 to 45 yr (mean: 27 yr), and of whom 53% were female, had 136 admissions for exacerbations of CF with respiratory failure (RF, n = 65), hemoptysis (n = 33), antibiotic desensitization (n = 30), pneumothorax (n = 3), or other reasons (n = 5). Eighty-six percent of the patients with hemoptysis and all of those with desensitization and pneumothorax were alive 1 yr after ICU discharge. Of the 42 patients with RF, 37 (88%) required assisted ventilation. Twenty-three (55%) of the patients with RF survived to ICU discharge and 19 (45%) died. Seventeen (40%) of the patients with RF received lung transplants and 14 were alive 1 yr later. Without transplantation, three (7%) of the patients with RF were alive and three (7%) were dead 1 yr later. Sex, body mass index, and respiratory bacteria did not correlate with survival. We conclude that ICU care for adults with CF who have reversible complications is appropriate and effective. Ventilatory support is appropriate for some transplant candidates.
 ...
PMID:Outcomes of intensive care unit care in adults with cystic fibrosis. 1124 40

Major hemoptysis is a potentially life-threatening complication of cystic fibrosis (CF) lung disease. Bronchial artery embolization (BAE) along with treatment of a CF pulmonary exacerbation has become the most widely used therapeutic approach for major hemoptysis in CF. However, BAE has been associated with severe complications, especially when bronchial artery to spinal artery anastomoses are present. This case study describes the successful treatment of major hemoptysis in CF with tranexamic acid, in an individual in whom 12 previous BAE procedures had been performed and further procedures were contraindicated secondary to bronchial artery to spinal artery collaterals. Recurrence of the hemoptysis occurred after attempts had been made to withdraw the tranexamic acid. Tranexamic acid was resumed with resolution of the hemoptysis, and the therapy has been used continuously for 13 months without any complications.
 ...
PMID:Treatment of recurrent severe hemoptysis in cystic fibrosis with tranexamic acid. 1122 38

The European Epidemiologic Registry of Cystic Fibrosis began collecting longitudinal data on European cystic fibrosis patients in 1994. A cross-sectional analysis was performed to identify the factors associated with low values of % predicted forced expiratory volume in one second (FEV1) upon patient enrollment. Data from 7,010 patients aged > or =6 yrs were included. Clinical conditions, microbiological isolates and medications reported at enrollment or within the following 180 days were analysed for age-specific associations. Factors associated with FEV1 that were lower by >10% of pred values were: lower weight for age percentiles, haemoptysis, pneumothorax, pulmonary symptoms at presentation, Pseudomonas aeruginosa, Burkholderia cepacia, oral corticosteroids, nonsteroid anti-inflammatory drugs, dornase alfa, oxygen and assisted ventilation and, in patients >12 yrs old only, use of airway clearance techniques, inhaled bronchodilators, oral nutritional supplements, pancreatic enzymes and insulin or oral hypoglycaemics. Slightly impaired lung function (5-10%) was associated with: diabetes (> or = 18-yrs-old), gastro-oesophageal reflux, allergic bronchopulmonary aspergillosis, asthma-like symptoms, portal hypertension, Aspergillus spp. and Candida spp. Sex, Haemophilus influenzae and Staphylococcus aureus were not associated with impaired pulmonary status. Regular exercise (especially in older patients) and nasal polyposis were associated with slightly better FEV1. The results confirm those of previous studies and suggest selective prescribing in sicker patients.
 ...
PMID:Factors associated with poor pulmonary function: cross-sectional analysis of data from the ERCF. European Epidemiologic Registry of Cystic Fibrosis. 1152 88

Lung transplantation (Tx) is an optional treatment for cystic fibrosis (CF) patients with end-stage lung disease. The decision to place a patient on the Tx waiting list is frequently complex, difficult, and controversial. This study evaluated the current criteria for lung Tx and assessed additional parameters that may identify CF patients at high risk of death. Data were extracted from the medical records of 392 CF patients. Forty of these patients had a forced expiratory volume in 1 s (FEV(1)) less than 30% predicted, and nine of these 40 patients were transplanted. A comparison was performed between the survival of those transplanted (n = 9) and those not transplanted (n = 31), by means of Kaplan-Meier survival curves. The influence on survival of age, gender, nutritional status, sputum aspergillus, diabetes mellitus, recurrent hemoptysis, oxygen use, and the decline rate of FEV(1), were investigated by means of univariate and multivariate analyses. The rate of decline of FEV(1) was evaluated employing the linear regression model. CF patients with a FEV(1)< 30% and who did not receive a lung transplant had survived longer than CF patients who did receive a lung transplant (median survival 7.33 vs. 3.49 yr, 5-yr survival 73% vs. 29%). Two factors--rate of decline in FEV(1) values and age < 15 yr--were found to influence the mortality rate, while the other parameters examined did not. Our results indicate that the current criterion of FEV(1)< 30% predicted, alone is not sufficiently sensitive to predict the mortality rate in CF patients and time of referral for Tx, as many of these patients survive for long periods of time. Additional criteria to FEV(1)< 30%, should include rapidly declining FEV(1) values and age < 15 yr.
 ...
PMID:Prediction of mortality and timing of referral for lung transplantation in cystic fibrosis patients. 1156 Jul 49

We report a case of massive endobronchial hemorrhage following a fistula formation of the right pulmonary artery to the right mainstem bronchus in a 15-year-old girl. The fistula had occurred 39 days after the patient had undergone bilateral lung transplantation for cystic fibrosis. The post-transplantation course was remarkable for bronchial colonization by Aspergillus at the site of right bronchial anastomosis and an episode of spontaneous, self-limited hemoptysis on postoperative day 17. A massive endobronchial hemorrhage during surveillance bronchoscopy occurred 39 days after transplantation. Immediate intervention, including rigid bronchoscopy followed by surgery, was effective in saving the patient. The pathophysiologic hypothesis to explain the fistula of the right pulmonary artery to the right mainstem bronchus probably involves ischemia of the anastomosis with necrosis of the suture zone complicated by endobronchial infection with Aspergillus. Rigid bronchoscopic intervention associated with an excellent medical surgical collaboration was pivotal in successfully rescuing the patient.
 ...
PMID:Management of an intrabronchial rupture of right main pulmonary artery: a case report. 1188 88

We report the embolization of an aberrant origin of haemoptysis - from the internal branch of the lateral thoracic artery - in a patient affected by cystic fibrosis. The technical implications for embolotheraphy in case of an aberrant origin of a haemorrhage are emphasized. Many different systemic arteries may contribute to the blood supply of the lung and many connections may exist between the systemic, bronchial and pulmonary circulations. The presence of non-bronchial systemic arteries supply should be investigated when inconclusive findings are seen during bronchial artery embolization for haemoptysis.
 ...
PMID:Lateral thoracic artery embolization in cystic fibrosis. 1201 Feb 97


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>