La prueba de la marcha de 6 minutos como factor pronóstico y de capacidad de ejercicio en pacientes con silicosis

Objective

To evaluate exercise capacity using the 6-minute walk test (6MWT) in patients with complicated silicosis, and to determine how results shape the prognostic value of 6MWT for hospitalization and mortality.

Derrame pleural e hipertensión pulmonar en un paciente con enfermedad de Parkinson en tratamiento con cabergolina

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.     

El factor de crecimiento de hígado mejora la fibrosis pulmonar inducida tras la administración de cadmio en ratas

Introduction

Liver growth factor (LGF) is a liver mitogen with regenerating and anti-fibrotic activity even at extrahepatic sites. We used LGF in a lung fibrosis model induced by cadmium chloride (CdCl₂), to study its antifibrotic capacity.

Methods

Forty-two male Wistar rats were administered a single dose of 0.5 ml/rat of CdCl2 0.025% (n=21) or the same volume of saline (control group, n=21). After 35 days, once a lesion was established, we started a 3 week treatment with LGF, after which we determined lung function —inspiratory capacity (IC), lung compliance (LC), forced vital capacity (FVC) and expiratory flow at 75% (FEF75%)—, lung morphometry —alveolar internal area (AIA), mean linear intersection (LM)—, and collagen (both by Sirius red and hydroxyproline residues) and elastin contents.

Results

Pulmonary fibrosis in CdCl₂ rats was characterized by a marked decrease in pulmonary function with respect to healthy controls —reductions of 28% in IC, 38% in CL, 31% in FVC, and 54% in FEF75%— which was partially recovered after LGF injection —18% IC, 27% CL, 19% FVC and 35% FEF75%—; increase in collagen and elastin contents —165% and 76%, respectively, in CdCl2 rats, versus 110% and 34% after LGF injection—; and increases in AIA and LM, partially reverted by LGF. Conclusions: Together, these data seem to demonstrate that LGF is able to improve lung function and partially reverts the increase in lung matrix proteins produced by CdCl₂ instillation.     

Normativa sobre el diagnóstico y tratamiento de la fibrosis pulmonar idiopática

Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options.     

Trasplante bipulmonar en hipertensión pulmonar. Una serie de 15 pacientes

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.     

Calidad de vida relacionada con la salud en una cohorte nacional de pacientes con hipertensión arterial pulmonar o hipertensión pulmonar tromboembólica crónica

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.     

Biopsia pulmonar por videotoracoscopia en el diagnóstico de la enfermedad pulmonar intersticial. Estudio prospectivo y multicéntrico en 224 pacientes

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.     

Hipertensión arterial pulmonar en pacientes con infección por el virus de la inmunodeficiencia humana: situación actual

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     

Estudio comparativo entre azatioprina oral y pulsos intravenosos de ciclofosfamida en el tratamiento de la fibrosis pulmonar idiopática

Introduction

The purpose of the study was to establish the efficacy of treatment with intravenous cyclophosphamide pulses and oral corticoids in idiopathic pulmonary fibrosis, compared with the standard treatment with corticoids and azathioprine.

Patients and method

A prospective, non-controlled study with 2 parallel groups. One group received prednisone plus oral azathioprine for 24 months (AZA group). The second one (CIC group) received prednisone plus intravenous cyclophosphamide pulses (6 monthly and 6 three-monthly). The primary outcome was survival or period without need for a transplant at 36 months. The secondary outcomes were the forced vital capacity, the carbon monoxide diffusing capacity, and baseline arterial oxygen pressure at 3, 6, 12, 18, 24, 30, and 36 months.

Results

A total of 46 patients were included in the study, of whom 25 were assigned to the AZA group and 21 to the CIC group. The survival or absence of lung transplant at 36 months was 44% in the AZA group and 76% in the CIC group (P=0.028). The forced vital capacity was worse in 8.8% of the AZA group compared to 6.7% in the CIC group (P=0.16). The carbon monoxide diffusing capacity worsened in 11.81% of patients of the AZA group and in 4.6% of those in the CIC group (P=0.0569). No significant differences were observed in the arterial oxygen pressure. There was one dropout of treatment with prednisone.

Conclusions

Treatment with intravenous cyclophosphamide pulses produced a significant improvement in survival. There were no significant differences in the lung function parameters or gas exchange. Neither of the cytostatics had serious side effects.     

Asincronía e hiperinsuflación en pacientes con enfermedad pulmonar obstructiva crónica durante 2 tipos de ejercicio de las extremidades superiores

Introduction

Occurrence of dynamic hyperinflation during upper-limbs exercises in chronic obstructive pulmonary disease (COPD) patients it is not well established. We hypothesized that dynamic hyperinflation and thoracoabdominal asynchrony occurs in COPD patients accomplishing arms exercises. We assessed the occurrence and association of dynamic hyperinflation and thoracoabdominal asynchrony in COPD patients during the accomplishment of two arm exercises.

Patients and methods

This was a prospective study with 25 COPD patients. A maximal and a sub-maximal upper limbs exercise test with 50% load were performed with the diagonal technique and the arm cycle ergometer technique. Respiratory pattern, thoracoabdominal configuration and dynamic hyperinflation were assessed in the exercise tests.

Results

Thirty per cent and 60% of patients hyperinflated at the end of the sub-maximum exercise tests with the diagonal and cycle ergometer techniques, respectively. Thoracoabdominal asynchrony occurred in 80% and 100% of patients who hyperinflated with the diagonal and cycle ergometer techniques, respectively. For both exercises we found enhancement of pulmonary ventilation, dyspnea, central respiratory drive and shortening of expiratory time (P < .05). Upper-limbs exercises with the diagonal technique presented less number of patients with these alterations.

Conclusions

Dynamic pulmonary hyperinflation and thoracoabdominal asynchrony association occurred in both upper-limbs exercises; however, the diagonal technique developed less dynamic hyperinflation and thoracoabdominal asynchrony in COPD patients than the arm cycle ergometer.     

Estudio de la repetición del pentanucleótido CCTTT en el gen de la sintasa inducible del óxido nítrico en pacientes con hipertensión arterial pulmonar

Introduction

One of the pathways involved in pulmonary arterial hypertension (PAH) is the nitric oxide (NO) pathway. A polymorphism in the inducible NO synthase (NOS2) gene has been described, consisting of the CCTTT pentanucleotide repeat, which causes a reduction in NO production. The aim of this study was to determine if this polymorphism increases susceptibility to developing PAH.

Methods

Sixty four patients with a diagnosis of PAH groups i and iv and 50 healthy controls were compared. DNA genotyping of the samples for this polymorphism was performed using PCR. The distribution between both groups was compared and correlated with clinical and haemodynamic parameters and therapeutic response.

Results

A significantly different distribution was observed in the number of repeats between patients and controls (P < .0001). When the samples were categorised by short forms (both alleles with less than 12 repeats) and long forms (≥ 12 repeats), it was observed that the former had an almost 4-fold risk of developing PAH (odds ratio: 3.83; 95% CI: 1.19-12.32, P = .024). There were no differences between the most common types of PAH, either in therapeutic response or survival. There was no correlation between haemodynamic parameters and the number of repeats in the patients, and only a weak correlation with systolic PAH.

Conclusions

There are significant differences in the distribution of the NOS2 promotor CCTTT polymorphism between patients with PAH and the healthy population. A minor CCTTT pentanucleotide repeat in the NOS2 gene may increase the risk of developing PAH.     

Intervención domiciliaria y variables predictoras para reingreso hospitalario en la enfermedad pulmonar obstructiva crónica agudizada

Background

Chronic obstructive pulmonary disease (COPD) exacerbation increases mortality and resources used associated with hospitalization. We studied whether early home monitoring reduces the rate of readmission and if there are any predictor variables.

Patients and methods

We performed a prospective, controlled, parallel-group study in patients who were hospitalized for COPD. Patients whose residence was within less than 15 km from the hospital were assigned to an interventional group (home visits by nurses about 48-72 hours after discharge), the remainder were assigned to a conventional care group. The rate of rehospitalization within the first month was compared between the two groups, as well as those variables that showed a predictive capability.

Results

Seventy one patients were included: 35 in the conventional care group and 36 in the interventional group. In the latter, the treatment was modified in 13 patients (36%). The hospital readmission rate was 17%, which was similar in both groups (P = .50). For every 5-year increase in age, the risk for readmission was 2.54 (95% CI, 1.06-5.07) and for each increase of 10 mmHg in PaCO₂, the risk of readmission was 8.34 (95% CI, 2.43-18.55).

Conclusions

Early home monitoring did not decrease the readmission rate during the first month. Older age and high PaCO₂ are factors that identify the group with a high risk for rehospitalization.     

Comparación de la respiración con presión positiva intermitente y la presión espiratoria positiva temporal en pacientes con enfermedad pulmonar obstructiva crónica grave

Background

Results supporting the use and the effectiveness of positive expiratory, pressure devices in chronic obstructive pulmonary disease (COPD) patients are still controversial, We have tested the hypothesis that adding TPEP or IPPB to standard pharmacological therapy may provide additional clinical benefit over, pharmacological therapy only in patients with severe COPD.

Methods

Fourty-five patients were randomized in three groups: a group was treated; with IPPB,a group was treated with TPEP and a group with pharmacological; therapy alone (control group).Primary outcome measures included the measurement of scale or, questionnaire concerning dyspnea (MRC scale),dyspnea,cough, and, sputum (BCSS) and quality of life (COPD assessment test) (CAT). Secondary, outcome measures were respiratory function testing,arterial blood gas,analysis,and hematological examinations.

Results

Both patients in the IPPB group and in the TPEP group showed a significant, improvement in two of three tests (MRC,CAT) compared to the control, group.However,in the group comparison analysis for, the same variables between IPPB group and TPEP group we observed a, significant improvement in the IPPB group (P ≤ .05 for MRC and P ≤ .01 for, CAT).The difference of action of the two techniques are evident in the results of, pulmonary function testing: IPPB increases FVC, FEV1, and MIP; this reflects, its capacity to increase lung volume. Also TPEP increases FVC and FEV1 (less, than IPPB), but increases MEP, while decreasing total lung capacity and, residual volume.

Conclusions

The two techniques (IPPB and TPEP) improves significantly dyspnea; quality of; life tools and lung function in patients with severe COPD. IPPB demonstrated a greater effectiveness to improve dyspnea and quality of life tools (MRC, CAT) than TPEP.     

Validación de la versión española del cuestionario revisado de calidad de vida para fibrosis quística en adolescentes y adultos (CFQR 14+ Spain)

Background

The aim of this study was to assess the validity and reliability of the Spanish version of the revised disease-specific health related quality of life questionnaire for adolescents and adults with cystic fibrosis (CFQR 14+ Spain).

Methods

A total of 43 cystic fibrosis (CF) patients completed the CFQR 14+ Spain. Forced expiratory volume in 1 second, in percentage of predicted - FEV₁ (%)-, number of respiratory exacerbations, 6-minute walk test, Bhalla score (based on computerized tomography of the chest), fat-free mass index, body mass index (BMI), faecal fat and St George's Respiratory Questionnaire were included as measurements of health status.

Results

Ten out of the twelve scales had alpha coefficients above 0.70. Test–retest correlations (Spearman) ranged from 0.49 to 0.95 and they were significant in all scales. Intraclass correlations ranged from 0.47 to 0.95 (ten out of the twelve scales were >0.70) forty out of the fifty ítems have correlations between items and scale above 0.70. All the CFQR+14 scales, except the digestive symptoms scale, discriminated significantly between patients with mild, moderate and severe disease (according to FEV₁ (%)). Other respiratory parameters also discriminated significantly between patients with mild-moderate and severe disease. Only some scales discriminated significantly between nourished and malnourished patients. All of the scales met standards for floor effects (<15% of the responders with the lowest score) but not for ceiling effects (only five out of the twelve).

Conclusion

The Spanish CFQR14+ (Spain) is a reliable and valid instrument for measuring the health-related quality of life in Spanish adolescents and adults with CF, though with the exception of a few of its subscales.