Lung Transplantation in Children. Specific Aspects

Lung transplantation has become in recent years a therapeutic option for infants with terminal lung disease with similar results to transplantation in adults. In Spain, since 1996 114 children lung transplants have been performed; this corresponds to 3.9% of the total transplant number. The most common indication in children is cystic fibrosis, which represents between 70-80% of the transplants performed in adolescents. In infants common indications are interstitial lung disease and pulmonary hypertension. In most children a sequential double lung transplant is performed, generally with the help of extracorpo-real circulation. Lung transplantation in children presents special challenges in monitoring and follow-up, especially in infants, given the difficulty in assessing lung function and performing transbronchial biopsies.There are some more specific complications in children like postransplant lymphoproliferative syndrome or a greater severity of respiratory virus infections. After lung transplantation children usually experiment a very important improvement in their quality of life. Eighty eight per cent of children have no limitations in their activity after 3 years of transplantation. According to the registry of the International Society for Heart & Lung Transplantation (ISHLT) survival at 5 years of transplantation is 54% and at 10 years is around 35%.     

Validation of Global Lung Function Initiative and All Ages Reference Equations for Forced Spirometry in Healthy Spanish Preschoolers

Introduction

Recent publication of multi-ethnic spirometry reference equations for subjects aged from 3 to 95 years aim to avoid age-related discontinuities and provide a worldwide standard for interpreting spirometric test results.

Acute Exacerbation According to GOLD 2017 Categories in Patients with Chronic Obstructive Pulmonary Disease

IntroductionThe association between GOLD categorizations and future exacerbations has not been fully investigated. This study elucidates whether the GOLD 2017 classification is associated with different future exacerbation risk in patients with chronic obstructive pulmonary disease (COPD) compared with the previous GOLD categorization. Another objective was to investigate the impacts of the symptoms and FEV1 on the predicted future exacerbation independently of previous exacerbation history.MethodsWe analyzed patients from three prospective COPD cohorts (SNUH, KOCOSS, and KOLD) and evaluated the risk of moderate to severe exacerbation among different models, including GOLD grade (FEV1), GOLD 2011, and GOLD 2017.ResultsIn total, 611 COPD patients were included (36 from SNUH, 257 from KOCOSS, and 318 from KOLD). GOLD 2017 classification, excluding FEV1% for categorization criteria, showed no differences in future exacerbation risk compared with GOLD grade and GOLD 2011 based on c-statistics. Among those with no frequent exacerbation history and FEV1 ≥50%, the group with more symptoms was significantly associated with future exacerbations than the group with less symptoms. A lower FEV1 (FEV1 <50%) was not associated with a higher future exacerbation risk than a higher FEV1 (FEV1 ≥50%), regardless of prior exacerbation history and symptom group.ConclusionThe GOLD 2017 classification was not different from GOLD grade and GOLD 2011 regarding the association with future exacerbation risk, and there were no significant differences in exacerbation risk according to FEV1%. This suggests that FEV1 might not be an important factor in future exacerbation risk. These results partly support the GOLD 2017 assessment tool.     

Variation in Protein Expression Depending on the Severity of Sleep Apnoea-Hypopnoea Syndrome

ObjectiveA prospective study with a consecutive sample and a control group to determine whether protein expression in patients with sleep apnoea-hypopnoea syndrome (SAHS) is different from that of the control group (IAH ≤ 5).Patients and methodsA total of 32 patients aged between 35 and 60 years who had a polysomnograph performed were included. Patients with an acute or chronic disease were excluded. The first dimension of the proteomic study was carried out on IPG strips (18 cm, pH4–7) and the second on SDS-PAGE gels in triplicate for each group. The gels were stained with SYPRO-Ruby (Bio-Rad®), the images obtained with an FX-Imager laser scanner and the spots were analysed using ProteomWeaver v.4.0 (Bio-Rad®) software. Significant changes between the gels were analysed by replicates and separately, being considered a significant change if the relative intensity of the spots was three times higher or lower than that of the control and if it was observed in 2 of the 3 replicates of each group, with a coefficient of variation of <20%.ResultsThe patients were divided into 8 subjects per group (control, mild, moderate and severe). The comparison of the gels showed significant differences between the control group and the 3 clinical groups, with significant over-expression being observed in 3 spots, and under-expression in 7 spots in the control group.ConclusionThere are significant changes in protein expression between a control group and patients in different stages of disease. The proteomic study can identify biomarkers associated with the diagnosis and severity of the SAHS.     

Chest Wall Reconstruction With Methacrylate Prosthesis in Poland Syndrome

Poland syndrome is a rare congenital malformation. This syndrome was described in 1841 by Alfred Poland at Guy's Hospital in London. It is characterized by hypoplasia of the breast and nipple, subcutaneous tissue shortages, lack of the costosternal portion of the pectoralis major muscle and associated alterations of the fingers on the same side. Corrective treatment of the chest and soft tissue abnormalities in Poland syndrome varies according to different authors. We report the case of a 17-year-old adolescent who underwent chest wall reconstruction with a methyl methacrylate prosthesis. This surgical procedure is recommended for large anterior chest wall defects, and it prevents paradoxical movement. Moreover it provides for individual remodeling of the defect depending on the shape of the patient's chest.     

Home Ventilation Therapy in Obstructive Sleep Apnea–Hypopnea Syndrome

Obstructive sleep apnea–hypopnea is a highly prevalent disease that is often underdiagnosed at present. It has a significant economic and social welfare impact, accounting for a large part of the resources assigned to home respiratory therapies. As part of the 2014 SEPAR Year of the Chronic Patient and Domiciliary Respiratory Care sponsored by the Spanish Society of Pulmonology and Thoracic Surgery, this article reviews the most recent publications on the indications and controversial issues in the treatment of sleep apnea, the latest evidence for indication of various positive pressure devices, and adjustment modes, ranging from the use of empirical formulae or mathematical estimations to modern auto-CPAP equipment, while not forgetting the gold standard of manual titration. Emphasis is placed on the need for monitoring required by patients to ensure treatment adherence and compliance. Finally, other therapies that are not the object of this article are briefly reviewed.     

Serum Levels of Alpha1-antitrypsin and Their Relationship With COPD in the General Spanish Population

BackgroundLow plasma level of alpha1-antitrypsin (AAT) is an established risk factor for early-onset chronic obstructive lung disease (COPD). However, less attention is given to the levels of AAT in the general population.MethodsThis is a part of a multicentre, population-based study conducted at 11 sites throughout Spain. Plasma levels of AAT were available for 837 persons with a mean (SD) age of 58.05 (11.3) years: 328-smokers, 272-ex-smokers and 237 non-smokers. Out of 837, 303 (36.2%) had a diagnosis of COPD, 222 (26.5%) had respiratory symptoms but no COPD, and 312 (37.3%) were healthy controls.ResultsIn the whole cohort, the mean level of plasma AAT was 1.51 (0.47) g/L. Levels were higher in COPD patients [1.55 (0.45) g/L] and individuals with respiratory symptoms [1.57 (0.47) g/L] than in controls [1.43 (0.47) g/L], p < 0.001, a finding which persisted after correction for age and CRP. Plasma AAT levels were negatively associated with FEV1/FVC ratio, after adjustment for age, sex, smoking status, CRP, TNFα, fibrinogen and albumin. The risk for COPD was significantly associated with higher AAT levels in univariate and multivariate models, with odds ratios of 1.8 and 1.5, respectively. In the univariate and multivariate models smoking status, gender, and CRP levels were also associated with COPD probability, demonstrating that they act independently.ConclusionIncreased circulating levels of AAT, similarly to CRP and other markers of systemic inflammation, is an important feature of COPD. Our results highlight a complex interrelationship between levels of AAT and health of respiratory system.     

Development and Validation of the OSA-CPAP Perceived Competence Evaluation Interview

IntroductionContinuous positive airway pressure (CPAP) is one of the most common therapies for Obstructive Sleep Apnea (OSA). We present a brief, patient-reported outcome measure used to assess patients’ levels of adherence with CPAP treatment.MethodsA questionnaire was developed based on academic literature. We qualitatively tested a pool of 18 items. It was tested in a sample of 174 patients from the Hospital La Princesa. Next, 1021 patients from Catalonia were evaluated.Results5 items were removed. Nominal groups referred to three areas: general knowledge about OSA and its risks; CPAP treatment information and expectations; CPAP use, monitoring, and confidence with its use. The 13 retained items maintained the same meaning as the original questionnaire (r = .986; p < .001) and the three proposed dimensions detected a significant increase in general knowledge of OSA (t[173] = 8.097, p < .001); CPAP treatment information (t[173] = 15.170, p < .001); and CPAP use (t[173] = 14.642, p < .001). The final 12-item version was reliable (CRI = .793) and its internal structure was adequate (χ²[51] = 72.073; p = .027, CFI = .967, RMSEA = .020 [.000, .030]). Women had a better general knowledge of OSA (t[1,018] = 2.190, p = .029), CPAP treatment information (t[1,018] = 2.920, p = .004), and higher overall OSA-CPAP scores (t[1,018] = 3.093, p = .002). Scores were positively related to quality of life and motivation, adherence was positively related to CPAP use and monitoring, and the total score was negatively related to daytime sleepiness.ConclusionsThe interview could help clinicians prevent some dropouts by targeting patients with lower adherence. It's a tool for assessing patient adherence to CPAP and to promote strategies through education and external motivational stimuli.     

Influence of Chronic Obstructive Pulmonary Disease on Volatile Organic Compounds in Patients with Non-Small Cell Lung Cancer

IntroductionThe etiology of lung cancer is multifactorial. Exposure to tobacco smoke and the role played by the carcinogenic compounds that it contains would explain the common association between lung cancer and chronic obstructive pulmonary disease (COPD), which is closely linked to tobacco use. In both diseases, sustained inflammation is caused by increased oxidative stress (for example, lipid peroxidation). This generates low molecular weight substances called volatile organic compounds (VOC) that are excreted during breathing. VOC metabolomics provides an indirect measure of oxidative stress.ObjectiveThe aim of this study was to establish the relative influence of COPD on the VOC profile in patients with non-small cell lung cancer (NSCLC), by first studying the possible variation of VOC associated with lung cancer histology.Patients and methodsExhaled air was tested in 107 NSCLC patients, who were divided into 2 groups: NSCLC with COPD and non-COPD with NSCLC. The exhaled air sample was obtained with the BIOVOC® sampler, and transferred to desorption tubes for later analysis by thermal desorption-gas chromatography-mass spectrometry. The VOC analysis showed lineal aldehydes and carboxylic acids.Results and conclusionsNo statistically significant differences were found in VOC associated with histology. NSCLC and COPD patients present a 1.7-fold (1.1–2.7) greater probability of detection of propionic acid (95% CI: 1.22–6.2) than patients without COPD or NSCLC (p = 0.013).