首页 | 本学科首页   官方微博 | 高级检索  
相似文献
 共查询到20条相似文献,搜索用时 15 毫秒
1.

Introduction

Mutations of the gene that code bone morphogenic protein type 2 receptor (BMPR2) are involved in the pathogenesis of pulmonary arterial hypertension (PAH), both in its familial (FPAH) and its idiopathic (IPAH) forms.

Method

With the aim of increasing the knowledge of these genetic factors in our area, the BMPR2 gene was studied in 17 patients with PAH, 8 with FPAH and 9 with sporadic IPAH. Additionally, a study was made to see whether the presence of BMPR2 mutations was associated with changes in the CO diffusing CO (DLCO) with the aim of evaluating the interest in this measurement in the pre-clinical diagnosis.

Results

R491Q y R211X mutations were detected in 2 patients with FPAH (prevalence, 25%), and the R332X mutation in one case of IPAH (prevalence, 11%). The familial study of the patient with the R491Q mutation, 14 of the 28 subjects studied had the mutation, and 4 had the diseases (penetration, 36%). A decrease in the DLCO/alveolar volume (KCO) ratio was observed in asymptomatic family members who expressed the mutation, compared to those who did not express it (88±5% and 104±9% of the reference value, respectively; P<0.01).

Conclusion

We conclude that the frequency of mutations in the BMPR2 gene in the patients studied with FPAH is lower than was previously described. The decrease in the KCO observed in asymptomatic carriers of the mutation suggests a certain level of pulmonary vascular changes, therefore its measurement could be useful in the familial study of FPAH.  相似文献   

2.
Nitric oxide (NO) production has been described using a 2-compartment model for the synthesis and movement of NO in both the alveoli and the airways. The alveolar concentration of NO (CaNO), an indirect marker of the inflammatory state of the distal portions of the lung, can be deduced through exhalation at multiple flow rates. Our objective was to determine reference values for CaNO. The fraction of exhaled NO (FeNO) was measured in 33 healthy individuals at a rate of 50 mL/s; the subjects then exhaled at 10, 30, 100, and 200 mL/s to calculate CaNO. A chemiluminescence analyzer (NIOX Aerocrine) was used to perform the measurements. The mean (SD) FeNO was 15 (6) ppb. The mean CaNO was 3.04 (1.30) ppb. These values of CaNO measured in healthy individuals will allow us to analyze alveolar inflammatory behavior in respiratory and systemic processes.  相似文献   

3.
4.

Background

The analysis of exhaled breath condensate (EBC) is a non-invasive technique that enables the determination of several volatile and nonvolatile substances produced in the respiratory tract, whose measurement may be useful for the diagnosis and monitoring of several respiratory diseases.

Objective

The aim of this study was to produce a low-cost reusable device in order to sample exhaled breath condensate in healthy adult volunteers, and to determine the concentration of nitric oxide in the sample collected.

Material and methods

The apparatus was made with a U-shaped tube of borosilicate glass. The tube was placed in a container with ice, and unidirectional respiratory valves were fitted to the distal end. Afterwards, nitric oxide was measured in the exhaled breath condensate (EBC) by chemiluminescence.

Results

The total cost of the device was $120.20. EBC samples were obtained from 116 volunteers of both sexes, aged between 20 and 70. The mean volume of exhaled breath condensate collected during 10 minutes was 1.0 ± 0.6 mL, and the mean level of nitric oxide was 12.99 ± 14.38 μM (median 8.72 μM). There was no correlation between the nitric oxide levels in the exhaled breath condensate and age or gender.

Conclusion

We demonstrate that it is possible to fabricate a low-cost, efficient, reusable device in order to collect and determine nitric oxide levels in EBC. We have identified no correlation between the nitric oxide levels present in the EBC obtained with this method with either age or sex.  相似文献   

5.
The increase in survival that has been achieved with the new treatments in the era of highly active antiretroviral therapy, has enabled clinicians and researchers to analyze issues that emerge in the long term in patients with HIV infection. Although the majority of cardiovascular complications have been widely described, the pathogenesis of pulmonary arterial hypertension is still poorly understood, and is one of the more complex and feared complications as it worsens the prognosis and quality of life of these patients  相似文献   

6.

Background

Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) experience impaired health-related quality of life (HRQL). The objective of this study was to evaluate HRQL in a nation-wide sample.

Patients and methods

This is a prospective, multicenter, non-interventional study of HRQL including 139 (89%) PAH and 17 (11%) CTEPH patients (women 70.5%; mean age, 52.2) recruited from 21 Spanish hospitals. 55% had idiopathic PAH, 34% other PAH and 11% CTEPH. HRQL was measured using the Short Form 36 Health Survey (SF-36) and EuroQoL-5D (baseline and after 6 months).

Results

HRQL in the patients with PAH or CTEPH was impaired. The physical component of SF-36 and the EuroQol-5D correlated with the functional class (FC). Mean EuroQol-5D visual analogical scale (EQ-5D VAS) scores were 73.5 ± 18.4, 62.9 ± 20.7 and 51.3 ± 16.0 (P < .0001) in patients with FC I, II and III, respectively. Every increase of one FC represented a loss of 4.0 on the PCS SF-36 and a loss of 9.5 on the EQ-5D VAS. Eight patients who died or received a transplant during the study period presented poorer initial HRQL compared with the rest of the population. No significant changes in HRQL were observed in survivors after 6 months of follow-up.

Conclusions

HRQL is impaired in this population, especially in PAH/CTEPH patients near death. HRQL measurements could help predict the prognosis in PAH and CTPH and provide additional information in these patients.  相似文献   

7.
8.

Background

Pulmonary hypertension is a serious disease that, in its terminal phase, requires lung transplantation.

Patients and methods

A retrospective study was undertaken of 15 patients with pulmonary hypertension who underwent lung transplantation between 1994 and 2004. Clinical data recorded before the procedure and during follow-up were reviewed.

Results

Pulmonary hypertension was reported as idiopathic in 8 patients (53%) and related to consumption of toxic oil in 2. The remaining causes were documented as chronic peripheral pulmonary embolism, histiocytosis X, venoocclusive disease, scleroderma, and simple corrected congenital heart defect in 1 patient each. The mean values of the hemodynamic variables were 100, 50, and 67 mm Hg for systolic, diastolic, and mean pulmonary artery pressure, respectively; 2.63 L/min for cardiac output; and 20.9 Wood units for total pulmonary resistance. The mean time between diagnosis of pulmonary hypertension and lung transplantation was 5.9 years (range, 0.4–20 y). Seven patients were in functional class III and 8 in functional class IV. The mean 6-minute walk distance was 204 m (range, 0–360 m). Four patients (26%) died during the during the perioperative period and 9 (60%), 7 (46%), and 6 (40%) were still alive at 1, 3, and 5 years, respectively.

Conclusions

Double-lung transplantation is a therapeutic option that, in certain cases, has similar outcomes to those achieved with the most aggressive medical treatment for pulmonary hypertension.  相似文献   

9.

Introduction

An excessive risk for bacteremia has recently been reported in patients with pulmonary arterial hypertension (PAH) treated with intravenous treprostinil. We aimed to assess this association in a cohort of patients from a Spanish referral center.

Patients and methods

We performed a retrospective cohort study that included 55 patients diagnosed with PAH who received a continuous intravenous infusion of a prostanoid (epoprostenol or treprostinil) for ≥ 1 month at our center between January 1991 and December 2011. The risk factors associated with the incidence of bacteremia were analyzed with the log-rank test.

Results

After a total follow-up of 64,453 treatment days, we found 12 episodes of bacteremia: Staphylococcus aureus (5 episodes), non-fermenting gram-negative bacilli (4 episodes), other gram-positive cocci (2 episodes), and Enterobacter cloacae (one episode). The incidence of bacteremia was 0.118 episodes per 1,000 treatment days in patients receiving epoprostenol versus 0.938 episodes per 1,000 treatment-days in patients receiving treprostinil (P = .0037). All episodes of bacteremia due to Gram-negative bacilli were diagnosed in patients on treprostinil. In the univariate analysis the treatment with intravenous treprostinil was associated with the incidence of bacteremia (hazard ratio: 4.09; 95% confidence interval: 1.24-14.53), although the low number of events prevented us from performing a multivariate analysis.

Conclusions

Therapy with intravenous treprostinil is associated with a higher risk for bacteremia, especially due to non-fermenting Gram-negative bacilli. This association should be taken in consideration when choosing empirical antibiotic therapy for patients with PAH and sepsis.  相似文献   

10.
11.

Introduction

The current goal of asthma treatment is to achieve and maintain control. This study aimed to explore the relationship between the ACT (Asthma Control Test) questionnaire and the levels of control according to GINA (Global Initiative for Asthma) to establish the cut-off points for the ACT and evaluate its relationship with lung function and fractionated exhaled nitric oxide level (FeNO).

Patients and methods

A multi-centre prospective study including 441 patients followed up in an outpatient Chest Clinic. A clinical protocol was followed, and FeNO, spirometry and ACT performed. Disease was classified according to levels of control using GINA. The study analysed sensitivity, specificity and area under the curve (ROC), and the ACT cut-off points. We studied the differences between the functional parameters and FeNO between levels of control.

Results

For controlled asthma the cut-off obtained was ACT≥21 (area under the curve 0.791) and for uncontrolled ≤18 (AUC 0.774). We found significant differences in FeNO levels and pulmonary function among ACT≥21 and ACT≤18, although only 26.3% of patients with ACT≤18 had a FEV1 <80% and 40% higher FeNO (≥35 ppb). We found a correlation between baseline FEV1 and ACT (r=0.19, P<0.01) and between ACT and FeNO (r=−0.16, P<0.01).

Conclusions

The cut-off points would be, for controlled asthma ACT≥21, partly controlled asthma ACT=19–20 and uncontrolled asthma ACT≤18. A more complete assessment would require including monitoring operating parameters and FeNO.  相似文献   

12.
Cabergoline is a synthetic dopamine agonist used to treat Parkinson disease. The drug occasionally induces pleuropulmonary adverse effects, which manifest as pleural thickening or effusion, interstitial pneumonitis, pulmonary infiltrates, or fibrosis. We report a rare case of pleural effusion and severe pulmonary hypertension in a 79-year-old man with Parkinson disease who had been treated with cabergoline for 1 year. The symptoms disappeared 10 months after the drug was discontinued.  相似文献   

13.

Introduction

The risk of hepatitis B virus (HBV) transmission through donor organs with HBsAg(−) and anti-HBc(+) serology has not been extensively studied in lung transplantation. The objectives of this study are to ascertain the influence of the anti-HBc(+) on the acceptance of the lung for transplantation, to comment on the published literature and to suggest an algorithm for action.

Methods

A survey conducted in the 7 Spanish lung transplantation teams. The updated search of the literature was performed using medical databases from 1994 to February 2012.

Results

All of the teams vaccinate the lung recipients against HBV, although none quantify the anti-HBs titers. When given an anti-HBc(+) donor, 3 teams change their strategy: one does not accept the offer, one selects the receptor from among patients in emergency status and another adds pharmacological prophylaxis. Only 3 publications refer to the serologic evolution of the receptors. At the moment there have been no reported cases of hepatitis B or HBsAg positivity post-transplant, but 4 out of the 50 anti-HBc(−) receptors changed to anti-HBc(+) in the follow-up.

Conclusions

The presence of anti-HBc in the donor influences the decision to accept a lung donor, although there is little information on its repercussions. To date, there has been no reported case of transmission of HBV, but post-transplant anti-HBc seroconversions have been described, which suggests contact with viral particles. Although rare, an anti-HBc(+) donor can harbor a hidden HBV infection. The risk of infection can be reduced with adequate anti-HBs titers or with appropriate pharmacological measures.  相似文献   

14.

Introduction and objectives

Delayed diagnosis of hypertension may result in inadequate blood pressure control and increased cardiovascular risk. The aim of this study was to estimate the delay in hypertension diagnosis in patients with type 2 diabetes and the likelihood of a diagnosis within a suitable period (first 6 months), and to analyze the patient and physician characteristics associated with delayed diagnosis.

Methods

Retrospective dynamic cohort study, with a 7-year follow-up in primary care, of 8074 adult patients with diabetes who met the diagnostic criteria for hypertension. Two thresholds were considered: 140/90 mmHg and 130/80 mmHg. The time elapsed between meeting these criteria and recording the diagnosis was estimated; the time course of the likelihood of a missed diagnosis and the variables associated with correct diagnosis were assessed by Kaplan-Meier survival analysis and logistic regression analysis, respectively.

Results

The mean diagnostic delay was 8.9 (15.4) months in patients with blood pressure≥140/90 mmHg compared to 15.2 (19.6) months for those with <140/90 mmHg (P<.001). The main variables associated with correct diagnosis were baseline blood pressure ≥140/90 mmHg (odds ratio=2.77; 95% confidence interval, 2.44-3.15), no history of acute myocardial infarction (odds ratio=2.23; 95% confidence interval, 1.67-2.99), obesity (odds ratio=1.70; 95% confidence interval, 1.44-1.99), absence of depression (odds ratio=1.63; 95% confidence interval, 1.27-2.08), female sex (odds ratio=1.29; 95% confidence interval, 1.14-1.46), older age, and taking more intensive antidiabetic therapy. There was an inverse relationship with the age of physicians and a direct relationship with their professional stability.

Conclusions

The mean diagnostic delay in hypertension among diabetic patients was greater than 6 months and varied according to the diagnostic threshold used. Patients with baseline blood pressure≥140/90 mmHg were more likely to receive a timely diagnosis.Full English text available from:www.revespcardiol.org/en  相似文献   

15.

Introduction and objectives

To investigate the relationship between inflammatory and apoptotic parameters and the severity and extent of target organ damage in patients with essential hypertension.

Methods

We studied 159 consecutive patients with treated essential hypertension. An exhaustive evaluation of damage to heart, kidney, and blood vessels was performed and plasma levels of inflammatory (interleukin 6 and soluble receptor of tumor necrosis factor-alpha type 2) and apoptotic markers (soluble receptor of tumor necrosis factor-alpha type 1 and soluble Fas receptor) were determined. Patients were categorized into four groups: a) no organ damage (33 patients); b) 1 organ damaged (52 patients); c) 2 organs damaged (44 patients), and d) 3 organs damaged (30 patients).

Results

Serum levels of interleukin 6, soluble receptor of tumor necrosis factor-alpha type 1 and soluble receptor of tumor necrosis factor-alpha type 2 were higher in patients with target organ damage than in hypertensive patients without organ damage. Increasing levels of these molecules were progressively associated with an increase in the number of organs damaged, and the highest levels were observed in the group with damage to 3 organs (heart, kidney, and blood vessels). There were no differences in soluble Fas receptor levels between groups. Logistic regression analysis showed that age, smoking, diabetes mellitus, abdominal circumference, interleukin 6, and soluble receptor of tumor necrosis factor-alpha type 1 were independently related to the number of target organs damaged.

Conclusions

Extensive hypertensive disease with involvement of more target organs was associated with greater inflammatory and apoptotic activation in these hypertensive patients.Full English text available from:www.revespcardiol.org  相似文献   

16.

Introduction

The six-minute walk test (6MWT) is widely used in evaluating diffuse interstitial lung disease (ILD) and pulmonary hypertension (PH). However, their physiological determining factors have not been well defined.

Objective

To evaluate the physiological changes that occur in ILD and PH during the 6MWT, and compare them with the cardiopulmonary exercise test (CPET).

Material and methods

Thirteen patients with ILD and 14 with PH were studied using the 6MWT and CPET on an ergometer cycle. The respiratory variables were recorded by means of telemetry during the 6MWT.

Results

Oxygen consumption (VO2), respiratory and heart rate reached a plateau from minute 3 of the 6MWT in both diseases. The VO2 did not differ from the peak value in the CPET (14±2 and 15±2 ml/kg/min, respectively, in ILD; 16±6 and 16±6 ml/kg/min, in PH). The arterial oxygen saturation decreased in both diseases, although it was more marked in ILD (−12±5%, p<0,01). The ventilatory equivalent for CO2 (VE/VCO2) in PH during the 6MWT was strongly associated with functional class (FC) (85±14 in FC III-IV, 44±6 in FC I-II; p<0,001).

Conclusions

The 6MWT in ILD and PH behaves like a maximal effort test, with similar VO2 to the CPET, demonstrating a limit in oxygen transport capacity. Monitoring using telemetry during the 6MWT may be useful for the clinical evaluation of patients with ILD or PH.  相似文献   

17.
18.
19.

Objectives

To evaluate whether the location and number of lung biopsies obtained by video-assisted thoracoscopy (VAT) influence the diagnosis of diffuse interstitial lung disease (ILD). To assess the applicability of an Ambulatory Surgery Program (ASP).

Methods

Prospective, multicenter study of VAT lung biopsies due to suspected ILD from January 2007 to December 2009, including 224 patients from 13 Spanish centers (mean age 57.1 years; 52.6% females). Data were prospectively collected in every institution and sent to the coordination center for analysis.

Results

The most affected areas in high resolution chest CT were the lower lobes (55%). Bronchoscopy was performed in 84% and transbronchial biopsy in 49.1%. In 179 cases (79.9%), more than one biopsy was performed, with a diagnostic agreement of 97.2%. A definitive histopathologic diagnosis was obtained in 195 patients (87%). Idiopathic pulmonary fibrosis was the most frequent diagnosis (26%). There were no statistically significant factors that could predict a greater diagnostic yield (neither anatomical location nor number of biopsies). Seventy patients (31.3%) were included in an ASP. After discharge, there were complications in 12 patients (5.4%), similar between patients admitted postoperatively (9/154: 5.8%) and those included in an ASP (3/70: 4.3%).

Conclusions

Anatomical location and number of lung biopsy specimens did not seem to influence the diagnosis. The patients included in an ASP had a complication rate comparable to that of the hospitalized, so this procedure can be included in a surgical outpatient program. Lung biopsy obtained by VAT is a powerful and safe tool for diagnosis of suspected ILD, resulting in a definitive diagnosis for the majority of patients with a low morbidity rate.  相似文献   

20.
设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号