One Year of Recombinant Human Growth Hormone Treatment in Adults with Prader-Willi Syndrome Improves Body Composition, Motor Skills and Brain Functional Activity in the Cerebellum.

We compared body composition, biochemical parameters, motor function, and brain neural activation in 27 adults with Prader-Willi syndrome and growth-hormone deficiency versus age-and sex-matched controls and baseline versus posttreatment values of these parameters after one year of recombinant human growth hormone (rhGH) treatment. To study body composition, we analyzed percentage of fat mass, percentage of lean mass, and muscle-mass surrogate variables from dual X-ray absorptiometry. Biochemical parameters analyzed included IGF-I, glucose metabolism, and myokines (myostatin, irisin, and IL6). To explore muscle function, we used dynamometer-measured handgrip strength, the Timed Up and Go (TUG) test, and the Berg Balance Scale (BBS). To study brain activation, we acquired functional magnetic resonance images during three motor tasks of varying complexity. After one year of treatment, we observed an increase in lean mass and its surrogates, a decrease in fat mass, improvements in TUG test and BBS scores, and increased neural activation in certain cerebellar areas. The treatment did not significantly worsen glucose metabolism, and no side-effects were reported. Our findings support the benefits of rhGH treatment in adults with Prader-Willi syndrome and growth-hormone deficiency on body composition and suggest that it may also improve balance and brain neural activation.

Phenotypic characterisation of early COPD: a prospective case-control study.

The phenotypic characteristics of chronic obstructive pulmonary disease (COPD) in individuals younger than 50 years of age (early COPD) are not well defined. This prospective, multicentre, case-control study sought to describe these characteristics and compare them with those of smokers (≥10 pack-years) of similar age with normal spirometry (controls). We studied 92 cases (post-bronchodilator forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) <0.7) and 197 controls. Results were contrasted with participants with similar inclusion criteria recruited into the ECLIPSE and COPDGene cohorts. Cases had moderate airflow limitation (FEV1 71.3±20.8%) but were often symptomatic, used healthcare resources frequently, had air trapping (residual volume 150.6±55.5% ref.), had reduced diffusing capacity (84.2±20.7% ref.) and had frequent evidence of computed tomography (CT) emphysema (61%). Of note, less than half of cases (46%) had been previously diagnosed with COPD. Interestingly, they also often reported a family history of respiratory diseases and had been hospitalised because of respiratory problems before the age of 5 years more frequently than controls (12% versus 3%, p=0.009). By and large, these observations were reproduced when available in the ECLIPSE and COPDGene cohorts. These results show that early COPD is associated with substantial health impact and significant structural and functional abnormalities, albeit it is often not diagnosed (hence, treated). The fact that a sizeable proportion of patients with early COPD report a family history of respiratory diseases and/or early-life events (including hospitalisations before the age of 5 years) renders further support to the possibility of early-life origin of COPD.

Carcinoma epidermoide de pared torácica en una toracostomía abierta / Squamous Cell Carcinoma of the Chest Wall in Open Thoracostomy

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Tumor de Krukenberg secundario a un adenocarcinoma de pulmón / Krukenberg Tumor Secondary to Lung Adenocarcinoma

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Traqueobroncopatía osteocondroplásica, un hallazgo casual / Tracheobronchopathia Osteochondroplastica. A Chance Finding

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Hipoplasia pulmonar en un adulto joven / Pulmonary Hypoplasia in a Young Adult

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Impact of Bariatric Surgery on Heme Oxygenase-1, Inflammation, and Insulin Resistance in Morbid Obesity with Obstructive Sleep Apnea.

INTRODUCTION: Morbid obesity and obstructive sleep apnea (OSA) interact at an inflammatory level. Bariatric surgery reduces inflammatory responses associated with obesity. Heme oxygenase-1 (HO-1) is an enzyme with anti-inflammatory properties, which might be increased in morbid obesity or OSA. We studied morbidly obese patients with OSA to determine: (a) HO-1 plasma concentrations according to OSA severity and their relationship with insulin resistance and inflammation and (b) the impact of bariatric surgery on HO-1 and parameters of insulin resistance and inflammation. MATERIAL AND METHODS: We analyzed the homeostasis model insulin resistance index (HOMA) and plasma concentrations of HO-1, tumor necrosis factor alpha, interleukin-6, interleukin-1-beta, C reactive protein (CRP), and adiponectin according to polysomnography findings in 66 morbidly obese patients before bariatric surgery and 12 months after surgery. RESULTS: Before surgery, HO-1 plasma concentrations were similar in three groups of patients with mild, moderate, and severe OSA, and correlated with HOMA (r = 0.27, p = 0.02). Twelve months after surgery, low-grade inflammation and insulin resistance had decreased in all the groups, but HO-1 plasma concentration had decreased only in the severe OSA group (p = 0.02). In this group, the reduction in HO-1 correlated with a reduction in CRP concentrations (r = 0.43, p = 0.04) and with improved HOMA score (r = 0.37, p = 0.03). CONCLUSIONS: Bariatric surgery decreases HO-1 concentrations in morbid obesity with severe OSA, and this decrease is associated with decreases in insulin resistance and in inflammation.

Derrame pleural secundario a un hemangioma capilar de localización pleural / Pleural effusion caused by a capillary hemangioma in the pleural cavity

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Obstructive sleep apnoea in the elderly: role of continuous positive airway pressure treatment.

Almost all the information about the effect of continuous positive airway pressure (CPAP) in patients with obstructive sleep apnoea (OSA) comes from clinical trials involving only middle-aged patients. The objective of this study was to assess the effect of CPAP treatment in elderly patients with severe OSA on clinical, quality-of-life and neurocognitive spheres. We performed an open-label, randomised, multicentre clinical trial in a consecutive clinical cohort of 224 elderly (≥70 years old) patients with confirmed severe OSA (apnoea-hypopnea index ≥30) randomised to receive CPAP (n=115) or no CPAP (n=109) for 3 months. A sleep study was performed by either full polysomnography or respiratory polygraphy. CPAP titration was performed by an autoCPAP device. The primary endpoint was quality of life (Quebec Sleep Questionnaire) and secondary endpoints included sleep-related symptoms, presence of anxiety/depression, office-based blood pressure and some neurocognitive tests. The mean±sd age was 75.5±3.9 years. The CPAP group achieved a greater improvement in all quality-of-life domains (p<0.001; effect size: 0.41-0.98), sleep-related symptoms (p<0.001; effect size 0.31-0.91) as well as anxiety (p=0.016; effect size 0.51) and depression (p<0.001; effect size: 0.28) indexes and some neurocognitive tests (digit symbol test (p=0.047; effect size: 0.20) and Trail Making Test A (p=0.029; effect size: 0.44)) in an intention-to-treat analysis. In conclusion, CPAP treatment resulted in an improvement in quality of life, sleep-related symptoms, anxiety and depression indexes and some neurocognitive aspects in elderly people with severe OSA.

Effectiveness of home single-channel nasal pressure for sleep apnea diagnosis.

INTRODUCTION: Home single-channel nasal pressure (HNP) may be an alternative to polysomnography (PSG) for obstructive sleep apnea (OSA) diagnosis, but no cost studies have yet been carried out. Automatic scoring is simpler but generally less effective than manual scoring. OBJECTIVES: To determine the diagnostic efficacy and cost of both scorings (automatic and manual) compared with PSG, taking as a polysomnographic OSA diagnosis several apnea-hypopnea index (AHI) cutoff points. METHODS: We included suspected OSA patients in a multicenter study. They were randomized to home and hospital protocols. We constructed receiver operating characteristic (ROC) curves for both scorings. Diagnostic efficacy was explored for several HNP AHI cutoff points, and costs were calculated for equally effective alternatives. RESULTS: Of 787 randomized patients, 752 underwent HNP. Manual scoring produced better ROC curves than automatic for AHI < 15; similar curves were obtained for AHI ≥ 15. A valid HNP with manual scoring would determine the presence of OSA (or otherwise) in 90% of patients with a polysomnographic AHI ≥ 5 cutoff point, in 74% of patients with a polysomnographic AHI ≥ 10 cutoff point, and in 61% of patients with a polysomnographic AHI ≥ 15 cutoff point. In the same way, a valid HNP with automatic scoring would determine the presence of OSA (or otherwise) in 73% of patients with a polysomnographic AHI ≥ 5 cutoff point, in 64% of patients with a polysomnographic AHI ≥ 10 cutoff point, and in 57% of patients with a polysomnographic AHI ≥ 15 cutoff point. The costs of either HNP approaches were 40% to 70% lower than those of PSG at the same level of diagnostic efficacy. Manual HNP had the lowest cost for low polysomnographic AHI levels (≥ 5 and ≥ 10), and manual and automatic scorings had similar costs for higher polysomnographic cutoff points (AHI ≥ 15) of diagnosis. CONCLUSION: Home single-channel nasal pressure (HNP) is a cheaper alternative than polysomnography for obstructive sleep apnea diagnosis. HNP with manual scoring seems to have better diagnostic accuracy and a lower cost than automatic scoring for patients with low apnea-hypopnea index (AHI) levels, although automatic scoring has similar diagnostic accuracy and cost as manual scoring for intermediate and high AHI levels. Therefore, automatic scoring can be appropriately used, although diagnostic efficacy could improve if we carried out manual scoring on patients with AHI < 15. CLINICAL TRIALS INFORMATION: Clinicaltrials.gov identifier: NCT01347398.

Maximal oxygen uptake cannot be estimated from resting lung function and submaximal exercise in patients with chronic obstructive pulmonary disease.

BACKGROUND: Maximal oxygen uptake ((V)O(2max)) obtained from incremental exercise testing is a useful indicator of limited exercise capacity. Several prediction equations have been developed to estimate (V)O(2max) in patients with chronic obstructive pulmonary disease (COPD), but agreement studies between estimated and measured (V)O(2max) are lacking. This study aims to assess agreement between the 6 estimated (V)O(2max) evaluated during maximal incremental exercise testing in male COPD patients. METHODS: Patients with stable COPD, in accordance with GOLD guidelines, were included in the study. Agreement between (V)O(2max) obtained during incremental exercise testing and (V)O(2max) obtained from 6 prediction equations were studied. To estimate (V)O(2max) from anthropometric prediction equations, lung function variables and submaximal exercise testing were used. RESULTS: Of the 60 male patients in the study, 12 were GOLD stage II, 24 GOLD stage III, and 24 GOLD stage IV. Five prediction equations underestimated the value of (V)O(2max) in relation to measured (V)O(2max) : equations 1, 2, 3, 4, and 6, by 14%, 66%, 42.2%, 35%, and 23.3%, respectively. Conversely, prediction equation 5 overestimated measured (V)O(2max) by 76.9%. Agreement between all (V)O(2max) prediction equations and measured (V)O(2max) was poor. Discrepancy between (V)O(2max) prediction equations and measured (V)O(2max) varied from 20.857 to 0.736 L/min. CONCLUSIONS: The use of lung function at rest and submaximal exercise testing is inaccurate for determining (V)O(2max) , which cannot be estimated by prediction equations in patients with stable COPD.

Validez y sensibilidad al cambio de la versión española autoadministrada del cuestionario de la enfermedad respiratoria crónica (CRQ-SAS) / The Validity and Sensitivity to Change of the Spanish Self-Administered Version of the Chronic Respiratory Questionnaire (CRQ-SAS)

Introducción: El cuestionario de la enfermedad respiratoria crónica con entrevistador (CRQ-IA) ha demostradoexcelentes propiedades de medida de la calidad de vida relacionada con la salud (CVRS) en pacientescon enfermedad pulmonar obstructiva crónica (EPOC). La validación en español de la versión estandarizadaautoadministrada (CRQ-SAS) no se ha realizado. El objetivo de este estudio fue evaluar la validez yla sensibilidad a los cambios, de la versión española del CRQ-SAS en pacientes con EPOC.Pacientes y métodos: Aleatorizamos a 40 pacientes con EPOC (33 en tratamiento con rehabilitación respiratoriay 7 con oxígeno líquido) a uno de los dos métodos de administración del CRQ (SAS vs. IA) antesy 8 semanas después de la intervención. Los pacientes completaban el cuestionario SF-36, pruebas defunción pulmonar y prueba de los 6 min de marcha.Resultados: El CRQ-SAS ha demostrado una buena validez de constructo longitudinal en todas las áreascon un rango de correlaciones, para las puntuaciones del cambio, entre 0,46 (p = 0,05) y 0,71 (p = 0,01).En cuanto a la sensibilidad a los cambios, se observa un cambio mínimo clínicamente significativo en lamayoría de áreas (fatiga 0,71 [p = 0,02], factor emocional 0,62 [p = 0,04], control de la enfermedad 0,83[p = 0,06]).Conclusiones: La versión española del CRQ-SAS resulta ser válida para evaluar la CVRS de los pacientescon EPOC. Las correlaciones con otros instrumentos aportan validez de constructo y se demuestra unabuena sensibilidad a los cambios(AU)

Introduction: The interviewer-administered chronic respiratory questionnaire (CRQ-IA) is widely usedand has demonstrated excellent properties for measuring health-related quality of life (HRQL) in patientswith chronic obstructive pulmonary disease (COPD). However, the self-administered version (CRQ-SAS)in Spanish has not been validated.The aim of this trial was to evaluate the validity and the sensitivity of the Spanish version of the CRQ-SASin patients with COPD.Material and methods: We randomized 40 patients with COPD (33 treated with pulmonary rehabilitation and 7 with liquid oxygen therapy) to one of the two methods of administration of CRQ (SAS vs. IA) both before and 8 weeks after the treatment. In addition, patients completed the SF-36 questionnaire, pulmonary function tests, and six-minute walk test. Results: The CRQ-SAS demonstrated good longitudinal construct validity on all domains with a rangeof correlations, for the change scores, between 0.46 (P=.05) and 0.71 (P=.01). Regarding sensitivityto change, we observed a minimal clinically significant change in most domains (fatigue 0.71 [P = .02],emotional factor 0.62 [P=.04], control of the disease 0.83 [P=.06]).Conclusions: The Spanish version of CRQ-SAS is valid for evaluating HRQL in COPD patients. Thecorrelations of the CRQ-SAS with other tools provide construct validity and show good sensitivity tochange(AU)

[The validity and sensitivity to change of the Spanish self-administered version of the chronic respiratory questionnaire (CRQ-SAS)]. / Validez y sensibilidad al cambio de la versión española autoadministrada del cuestionario de la enfermedad respiratoria crónica (CRQ-SAS).

INTRODUCTION: The interviewer-administered chronic respiratory questionnaire (CRQ-IA) is widely used and has demonstrated excellent properties for measuring health-related quality of life (HRQL) in patients with chronic obstructive pulmonary disease (COPD). However, the self-administered version (CRQ-SAS) in Spanish has not been validated. The aim of this trial was to evaluate the validity and the sensitivity of the Spanish version of the CRQ-SAS in patients with COPD. MATERIAL AND METHODS: We randomized 40 patients with COPD (33 treated with pulmonary rehabilitation and 7 with liquid oxygen therapy) to one of the two methods of administration of CRQ (SAS vs. IA) both before and 8 weeks after the treatment. In addition, patients completed the SF-36 questionnaire, pulmonary function tests, and six-minute walk test. RESULTS: The CRQ-SAS demonstrated good longitudinal construct validity on all domains with a range of correlations, for the change scores, between 0.46 (P=.05) and 0.71 (P=.01). Regarding sensitivity to change, we observed a minimal clinically significant change in most domains (fatigue 0.71 [P=.02], emotional factor 0.62 [P=.04], control of the disease 0.83 [P=.06]). CONCLUSIONS: The Spanish version of CRQ-SAS is valid for evaluating HRQL in COPD patients. The correlations of the CRQ-SAS with other tools provide construct validity and show good sensitivity to change.

Biot's breathing in a woman with fatal familial insomnia: is there a role for noninvasive ventilation?

We present the case of a 48-year-old woman suffering from fatal familial insomnia (FFI)--a rare prion disease--who developed Biot's breathing and secondary respiratory failure during the early stages of the illness. Once hypercapnia was detected a trial of nocturnal noninvasive ventilation (NIV) was offered with important improvement of arterial blood gases (ABG), and subjective good quality of sleep. To our knowledge, this is the first report in the medical literature of the use of NIV in the management approach of this devastating disease. Its impact on the prognosis and survival of these patients, however, is yet to be elucidated.

Effectiveness of unattended ambulatory sleep studies for the diagnosis and treatment of OSAS.

BACKGROUND: Unattended home sleep studies have been implemented to minimize the cost and delay in establishing obstructive sleep apnoea syndrome (OSAS) diagnosis and therapy. However, their accuracy and validity remain disputed. The purpose of our study is to assess their effectiveness (i.e. their technical reliability in real life conditions). SETTING: Private sleep unit attending to private and social security patients. POPULATION: Consecutively admitted patients, aged over 18 years, referred to our clinic for confirmation or treatment of OSAS without co-morbidities. METHODS: A clinical history was obtained for each patient with special attention to sleep, smoking habit, educational level and type of insurance. Chest X-ray and forced spirometry were performed. For the portable respiratory recording device an eight-channel polygraph was used and for continuous positive airway pressure (CPAP) titration an automatic positive airway pressure device was applied; both instruments were previously validated. Patients were instructed how to use the devices at the clinic and then applied them at home by themselves. RESULTS: For diagnostic sleep studies, 145 out of 150 (96.7%: CI: 92.4-98.9) and for CPAP titration 42 out of the 61 patients (69%: CI: 56.4-81.3) performed the sleep study correctly according to the pre-established criteria. No statistically significant differences were found between correct performance and educational level, sex, smoking habit or type of health insurance. CONCLUSIONS: (1) Unattended home sleep studies appear as effective as in-hospital studies for diagnosis of OSAS without co-morbidities; (2) for CPAP titration, the effectiveness was slightly lower; and (3) factors such as patients' educational level or type of health insurance do not appear to influence outcome.