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1.
Medicine (Baltimore) ; 99(10): e19301, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32150064

RESUMEN

We treated skin sclerosis with triple therapy consisting of a glucocorticoid, intravenous cyclophosphamide, and double-filtration plasmapheresis. The objective of this study was to analyze its effectiveness in a case series of patients who received triple therapy.We enrolled 8 patients with diffuse cutaneous systemic sclerosis (dcSSc) who received triple therapy at our hospital from 2008 to 2016. We analyzed the mean change in the modified Rodnan skin score (mRSS), percentage of the predicted forced vital capacity (%FVC), percentage of the predicted carbon monoxide diffusing capacity (%DLCO), and serum KL-6 levels from baseline to follow-up.All patients were treated with an intermediate dose of oral prednisolone (30.6 ±â€Š2.1 mg/day) initially. The mean cumulative dose of intravenous cyclophosphamide was 1.4 ±â€Š0.2 g. The mean mRSS decreased significantly at follow-up compared with that at baseline (27.0 ±â€Š3.3 vs 15.8 ±â€Š3.5; P = .03). At the end of the treatment, the mean %FVC and %DLCO were improved moderately, although the differences were not significant. The serum KL-6 levels decreased from 578.9 ±â€Š146.5 to 205.3 ±â€Š43.1 U/ml (P = .02). No significant correlation was found between the change in mRSS or disease duration and the initial skin score severity.Triple therapy may improve skin sclerosis, with effectiveness equal or superior to other reported treatments. This preliminary case series demonstrates the potential of triple therapy for treating dcSSc. However, prospective studies with long-term follow-up should be performed to assess its role.


Asunto(s)
Ciclofosfamida/uso terapéutico , Glucocorticoides/uso terapéutico , Inmunosupresores/uso terapéutico , Plasmaféresis , Prednisolona/uso terapéutico , Esclerodermia Difusa/terapia , Administración Oral , Anciano , Terapia Combinada , Femenino , Humanos , Infusiones Intravenosas , Masculino , Persona de Mediana Edad , Mucina-1/sangre , Capacidad de Difusión Pulmonar , Estudios Retrospectivos , Esclerodermia Difusa/tratamiento farmacológico , Esclerodermia Difusa/fisiopatología , Resultado del Tratamiento , Capacidad Vital
2.
Zhonghua Er Ke Za Zhi ; 58(2): 140-144, 2020 Feb 02.
Artículo en Chino | MEDLINE | ID: mdl-32102152

RESUMEN

Objective: To explore the cutoff value of FEV(1)/FVC for evaluating obstructive ventilation dysfunction in children. Methods: Three hundred and eighty-three healthy children (190 boys and 193 girls) aged 6-14 years in primary and middle school in Beijing, who were enrolled from May 2010 to June 2011, had their spirometry done with the Jaeger lung function instrument, and the lower limit of normal (LLN) of FEV(1)/FVC was calculated. Two hundred and thirteen asthmatic children (151 boys and 62 girls) aged 6-14 years from the outpatient Department of Allergy, Capital Institute of Pediatrics were enrolled consecutively from June to July 2018, whose clinical history and pulmonary function parameters were collected. The expected value of spirometric parameters of asthmatic children was calculated according to Zapletal prediction equations. And the LLN of FEV(1)/FVC in healthy children was used as the gold standard to calculate the sensitivity, specificity, Youden index and Kappa value of FEV(1)/FVC<80% and FEV(1)/FVC< 92% predicted. Results: In healthy children, the FEV(1)/FVC and the LLN were 91%±5% and 82% respectively, while 90%±6% and 81% in boys, and 92%±5% and 84% in girls. There were 27 (12.7%) asthmatic children whose FEV(1)/FVC<80% but FEV(1)/FVC≥92% predicted (χ(2)=123.7, P<0.01). The sensitivity, specificity, Youden index and Kappa value of FEV(1)/FVC<80% were 80.3%, 100%, 0.803 and 0.787 respectively when FEV(1)/FVC<80% was used as the criteria to assess lung function, while they were 57.3%, 100%, 0.573 and 0.547, respectively, when FEV(1)/FVC<92% predicted was used as criteria. Conclusions: The 80% of FEV(1)/FVC has a better consistency with the LLN of FEV(1)/FVC than 92% of FEV(1)/FVC% predicted. It is more accarate to use FEV(1)/FVC<80% as the diagnostic criteria for airway obstruction.


Asunto(s)
Obstrucción de las Vías Aéreas/diagnóstico , Volumen Espiratorio Forzado/fisiología , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Capacidad Vital , Adolescente , Obstrucción de las Vías Aéreas/fisiopatología , Beijing , Estudios de Casos y Controles , Niño , Femenino , Humanos , Masculino , Guías de Práctica Clínica como Asunto , Espirometría
3.
Expert Opin Pharmacother ; 21(2): 213-231, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31955671

RESUMEN

Introduction: Asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS) is a disease phenotype that shares T helper lymphocyte cell Th1/neutrophilic/non-Type-2 Inflammation pathways thought to be key in COPD and Th2/eosinophilic/Type-2 inflammatory pathways of asthma. The pharmacology of treating ACOS is challenging in severe circumstances.Areas covered: This review evaluates the stepwise treatment of ACOS using pharmacological treatments used in both COPD and asthma. The most common medications involve the same inhalers used to treat COPD and asthma patients. Advanced stepwise therapies for ACOS patients are based on patient characteristics and biomarkers. Very few clinical trials exist that focus specifically on ACOS patients.Expert opinion: After inhalers, advanced therapies including phosphodiesterase inhibitors, macrolides, N-acetylcysteine and statin therapy for those ACOS patients with a COPD appearance and exacerbations are available. In atopic ACOS patients with exacerbations, advanced asthma therapies (leukotriene receptor antagonists and synthesis blocking agents.) are used. ACOS patients with elevated blood eosinophil/IgE levels are considered for immunotherapy or therapeutic monoclonal antibodies blocking specific Th2/Type-2 interleukins or IgE. Symptom control, stabilization/improvement in pulmonary function and reduced exacerbations are the metrics of success. More pharmacological trials of ACOS patients are needed to better understand which patients benefit from specific treatments.Abbreviations: 5-LOi: 5-lipoxygenase inhibitor; ACOS: asthma - COPD overlap syndrome; B2AR: Beta2 adrenergic receptors; cAMP: cyclic adenosine monophosphate; cGMP: cyclic guanosine monophosphate; CI: confidence interval; COPD: chronic obstructive pulmonary disease; CRS : chronic rhinosinusitis; cys-LT: cysteinyl leukotrienes; DPI: dry powder inhaler; EMA: European Medicines Agency; FDA: US Food and Drug Administration; FDC: fixed-dose combination; FeNO: exhaled nitric oxide; FEV1: forced expiratory volume in one second; FVC: forced vital capacity; GM-CSF: granulocyte-macrophage colony-stimulating factor; ICS : inhaled corticosteroids; IL: interleukin; ILC2: Type 2 innate lymphoid cells; IP3: Inositol triphosphate; IRR: incidence rate ratio; KOLD: Korean Obstructive Lung Disease; LABA: long-acting B2 adrenergic receptor agonist; LAMA: long-acting muscarinic receptor antagonist; LRA: leukotriene receptor antagonist; LT: leukotrienes; MDI: metered-dose inhalers; MN: M-subtype muscarinic receptors; MRA: muscarinic receptor antagonist; NAC: N-acetylcysteine; NEB: nebulization; OR: odds ratio; PDE: phosphodiesterase; PEFR: peak expiratory flow rate; PGD2: prostaglandin D2; PRN: as needed; RR: risk ratio; SABA: short-acting B2 adrenergic receptor agonist; SAMA: short-acting muscarinic receptor antagonist; SDMI: spring-driven mist inhaler; Th1: T helper cell 1 lymphocyte; Th2: T helper cell 2 lymphocytes; TNF-α: tumor necrosis factor alpha; US : United States.


Asunto(s)
/tratamiento farmacológico , Broncodilatadores/uso terapéutico , Administración por Inhalación , Corticoesteroides/uso terapéutico , Agonistas de Receptores Adrenérgicos beta 2/uso terapéutico , Volumen Espiratorio Forzado , Humanos , Antagonistas Muscarínicos/uso terapéutico , Nebulizadores y Vaporizadores , Capacidad Vital
4.
BMC Public Health ; 20(1): 6, 2020 Jan 06.
Artículo en Inglés | MEDLINE | ID: mdl-31906909

RESUMEN

BACKGROUND: Although previous studies have reported the benefits of physical activity (PA) to lung function in middle-aged and older adults, the biological mechanisms are still unclear. This study aimed to assess the extent to which C-reactive protein (CRP) mediates the association between leisure-time PA and lung function. METHODS: A population-based sample was recruited from English Longitudinal Study of Ageing (ELSA), Wave 6 (2012-2013). PA was self-reported by questionnaires. CRP was analyzed from peripheral blood. Lung function parameters including forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were measured by using a spirometer. Baron and Kenny's causal steps method and multiple linear regression models based on the Karlson/Holm/Bree (KHB) method were used to assess the mediating effect. RESULTS: Among 6875 participants, 28.4% were classified into low PA, 49.8% into moderate PA, and 21.8% into high PA. Multiple linear regression models suggested that higher PA was associated with lower levels of CRP (ß = - 0.048, P = 0.002 for moderate PA; ß = - 0.108, P < 0.001 for high PA). CRP negatively correlated with FEV1 (ß = - 0.180, P < 0.001) and FVC (ß = - 0.181, P < 0.001). Higher levels of PA were associated with better FEV1 (ß = 0.085, P < 0.001 for moderate PA; ß = 0.150, P < 0.001 for high PA) and FVC (ß = 0.131, P < 0.001 for moderate PA; ß = 0.211, P < 0.001 for high PA). After introducing the CRP into the models, regression coefficients of PA with FEV1 (ß = 0.077, P < 0.001 for moderated PA; ß = 0.130, P < 0.001 for high PA) and FVC (ß = 0.123, P < 0.001 for moderated PA; ß = 0.188, P < 0.001 for high PA) decreased. The indirect effect of high PA on lung function via CRP was significant, with 9.42-12.99% of the total effect being mediated. CONCLUSIONS: The association between PA and lung function is mediated by CRP, suggesting that this association may be partially explained by an inflammation-related biological mechanism. This finding highlights the possible importance of PA in systemic inflammation and lung function, thus, middle-aged and older adults should be encouraged to enhance PA levels.


Asunto(s)
Proteína C-Reactiva/análisis , Ejercicio/fisiología , Volumen Espiratorio Forzado/fisiología , Actividades Recreativas , Capacidad Vital/fisiología , Anciano , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria , Autoinforme
5.
J Pediatr Orthop ; 40(1): 42-47, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31815861

RESUMEN

BACKGROUND: Several authors mentioned surgical outcome for scoliosis associated with neurofibromatosis type 1 (NF-1). However, no studies have summarized long-term surgical outcomes after surgical treatment of early-onset scoliosis (EOS) with NF-1. The purpose of this study is to evaluate the long-term surgical outcome after early definitive spinal fusion for EOS in NF-1 patients. METHODS: We performed a retrospective review on a cohort of 11 patients diagnosed with EOS associated with NF-1 between 1990 and 2009 in our hospital. The study included 7 male and 4 female NF-1 patients with a mean age at fusion surgery of 8 years 4 months who underwent early definitive spinal fusion for EOS. We reviewed radiographs, computed tomography and magnetic resonance images for each patient. The data collected included type of NF-1, Cobb angles, kyphotic angle, spinal length (T1-S1), and pulmonary function. We also evaluated progression of the dystrophic change through radiographs and computed tomography during the follow-up period. RESULTS: The mean follow-up duration was 14 years (5 y 10 mo to 25 y). All patients underwent anterior and posterior fusion surgeries in either 1 or 2 stages. Seven cases had subtotal tumor resection from the concave side followed by rib strut grafting. The mean preoperative Cobb angle of the primary curve was corrected from 71.2 to 24.1 degrees and was maintained at the final follow-up at 23.5 degrees. Mean values for preoperative and final follow-up T1-S1 heights were 30.7 and 36.2 cm, respectively. Mean values for preoperative and final follow-up % forced vital capacity (the present/the predicted) were 75.0% and 74.4%, respectively. The dystrophic change progressed in all cases during the long-term follow-up. CONCLUSIONS: Our goals for NF-1 scoliosis are to obtain deformity correction, to achieve stable instrumentation by using longer fusion levels and to attain a solid fusion mass that can tolerate continual erosion during the long-term follow-up. We achieve this by using a circumferential approach for EOS associated with NF-1. LEVEL OF EVIDENCE: Level IV-case series.


Asunto(s)
Neurofibromatosis 1/complicaciones , Escoliosis/complicaciones , Escoliosis/cirugía , Fusión Vertebral , Adolescente , Adulto , Niño , Preescolar , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Cifosis/diagnóstico por imagen , Cifosis/cirugía , Imagen por Resonancia Magnética , Masculino , Neurofibromatosis 1/cirugía , Estudios Retrospectivos , Escoliosis/diagnóstico por imagen , Escoliosis/fisiopatología , Fusión Vertebral/métodos , Factores de Tiempo , Tomografía por Rayos X , Resultado del Tratamiento , Capacidad Vital , Adulto Joven
6.
J Surg Res ; 245: 168-178, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31421359

RESUMEN

BACKGROUND: Esophagectomy for esophageal cancer is known to lead to deterioration in respiratory function (RF). The aim of this study was to assess long-term trends in RF after esophagectomy and the impact of different operative procedures. METHODS: A total of 52 patients with thoracic esophageal cancer who were scheduled for esophagectomy from 2003 to 2012 were enrolled. We prospectively evaluated patients for vital capacity (VC), forced expiratory volume in 1 s (FEV1.0), and 6-min walk distance (6MWD) before and after esophagectomy at 3, 6, 12, 24, and 60 mo. RESULTS: Patients had mostly recovered their VC and FEV1.0 after 12 mo. After that point, VC and FEV1.0 declined again, reaching levels lower than baseline at 60 mo, with a median change ratio of 0.85 and 0.86, respectively. Although the 6MWD after open esophagectomy declined, patients treated with transhiatal esophagectomy and minimally invasive esophagectomy maintained above baseline levels throughout the follow-up period. Furthermore, we identified transhiatal esophagectomy (odds ratio [OR] = 0.03, 95% confidence interval [CI] 0.002-0.43, P = 0.01) and minimally invasive esophagectomy (OR = 0.14, 95% CI 0.02-0.94, P = 0.04) as favorable factors and postoperative pulmonary complication (OR = 9.14, 95% CI 1.22-68.6, P = 0.03) as an unfavorable factor for RF after 12 mo. Operative procedures had no significant impact on RF after 60 mo. CONCLUSIONS: Our results support the notion that RF does not recover to the baseline level, and operative procedures have no significant impact on RF at late phase after esophagectomy.


Asunto(s)
Neoplasias Esofágicas/cirugía , Esofagectomía/efectos adversos , Volumen Espiratorio Forzado/fisiología , Complicaciones Posoperatorias/diagnóstico , Capacidad Vital/fisiología , Anciano , Esofagectomía/métodos , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/fisiopatología , Periodo Posoperatorio , Estudios Prospectivos , Factores de Tiempo , Resultado del Tratamiento
7.
Sci Total Environ ; 699: 134397, 2020 Jan 10.
Artículo en Inglés | MEDLINE | ID: mdl-31677469

RESUMEN

Children are vulnerable to air pollution-induced lung function deficits, and the prevalence of obesity has been increasing in children. To evaluate the joint effects of long-term PM1 (particulate matter with an aerodynamic diameter ≤ 1.0 µm) exposure and obesity on children's lung function, a cross-sectional sample of 6740 children (aged 7-14 years) was enrolled across seven northeastern Chinese cities from 2012 to 2013. Weight and lung function, including forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), peak expiratory flow (PEF), and maximal mid-expiratory flow (MMEF), were measured according to standardized protocols. Average PM1, PM2.5, PM10 and nitrogen dioxide (NO2) exposure levels were estimated using a spatiotemporal model, and sulphur dioxide (SO2) and ozone (O3) exposure were estimated using data from municipal air monitoring stations. Two-level logistic regression and general linear models were used to analyze the joint effects of body mass index (BMI) and air pollutants. The results showed that long-term air pollution exposure was associated with lung function impairment and there were significant interactions with BMI. Associations were stronger among obese and overweight than normal weight participants (the adjusted odds ratios (95% confidence intervals) for PM1 and lung function impairments in three increasing BMI categories were 1.50 (1.07-2.11) to 2.55 (1.59-4.07) for FVC < 85% predicted, 1.44 (1.03-2.01) to 2.51 (1.53-4.11) for FEV1 < 85% predicted, 1.34 (0.97-1.84) to 2.04 (1.24-3.35) for PEF < 75% predicted, and 1.34 (1.01-1.78) to 1.93 (1.26-2.95) for MMEF < 75% predicted). Consistent results were detected in linear regression models for PM1, PM2.5 and SO2 on FVC and FEV1 impairments (PInteraction < 0.05). These modification effects were stronger among females and older participants. These results can provide policy makers with more comprehensive information for to develop strategies for preventing air pollution induced children's lung function deficits among children.


Asunto(s)
Contaminación del Aire/estadística & datos numéricos , Exposición a Riesgos Ambientales/estadística & datos numéricos , Obesidad/epidemiología , Adolescente , Contaminantes Atmosféricos/análisis , Niño , China/epidemiología , Ciudades , Femenino , Volumen Espiratorio Forzado , Humanos , Pulmón/efectos de los fármacos , Masculino , Dióxido de Nitrógeno/análisis , Sobrepeso , Ozono/análisis , Material Particulado/análisis , Pruebas de Función Respiratoria , Dióxido de Azufre , Capacidad Vital
8.
J Bras Pneumol ; 45(6): e20180355, 2019.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-31851214

RESUMEN

OBJECTIVE: A resolution passed by the government of the Brazilian state of São Paulo established a protocol for requesting free COPD medications, including tiotropium bromide, creating regional authorization centers to evaluate and approve such requests, given the high cost of those medications. Our objective was to analyze the requests received by an authorization center that serves cities in the greater metropolitan area of (the city of) São Paulo between 2011 and 2016. METHODS: Data regarding the authorization, return, or rejection of the requests were compiled and analyzed in order to explain those outcomes. Subsequently, the clinical and functional data related to the patients were evaluated. RESULTS: A total of 7,762 requests for dispensing COPD medication were analyzed. Requests related to male patients predominated. Among the corresponding patients, the mean age was 66 years, 12% were smokers, 88% had frequent exacerbations, and 84% had severe/very severe dyspnea. The mean FEV1 was 37.2% of the predicted value. The total number of requests decreased by 24.5% from 2012 to 2013 and was lowest in 2015. Most (65%) of the requests were accepted. The main reasons for the rejection/return of a request were a post-bronchodilator FEV1/FVC ratio > 0.7, a post-bronchodilator FEV1 > 50% of the predicted value, and failure to provide information regarding previous use of a long-acting ß2 agonist. During the study period, the total number of requests returned/rejected decreased slightly, and there was improvement in the quality of the data included on the forms. CONCLUSIONS: Here, we have identified the characteristics of the requests for COPD medications and of the corresponding patients per region served by the authorization center analyzed, thus contributing to the improvement of local public health care measures.


Asunto(s)
Broncodilatadores/economía , Broncodilatadores/provisión & distribución , Enfermedad Pulmonar Obstructiva Crónica/economía , Bromuro de Tiotropio/economía , Bromuro de Tiotropio/provisión & distribución , Anciano , Brasil , Costos de los Medicamentos/estadística & datos numéricos , Femenino , Volumen Espiratorio Forzado , Accesibilidad a los Servicios de Salud/economía , Humanos , Masculino , Persona de Mediana Edad , Enfermedad Pulmonar Obstructiva Crónica/tratamiento farmacológico , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Factores de Tiempo , Capacidad Vital/fisiología
9.
Environ Health Perspect ; 127(12): 127008, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-31873044

RESUMEN

BACKGROUND: The association between long-term exposure to ambient fine particulate matter with aerodynamic diameter ≤2.5µm (PM2.5) and lung function in young people remains uncertain, particularly in Asia, where air pollution is generally a serious problem. OBJECTIVES: This study investigated the association between long-term exposure to ambient PM2.5 and lung function in Taiwanese children, adolescents, and young adults. METHODS: This study comprised 24,544 participants 6-24 years of age, with 33,506 medical observations made between 2000 and 2014. We used a spatiotemporal model to estimate PM2.5 concentrations at participants' addresses. Spirometry parameters, i.e., forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and maximum midexpiratory flow (MMEF), were determined. A generalized linear mixed model was used to examine the associations between long-term exposure to ambient PM2.5 and lung function. The odds ratios (ORs) of poor lung function were also calculated after adjusting for a range of covariates. RESULTS: Every 10-µg/m3 increase in the 2-y average PM2.5 concentration was associated with decreases of 2.22% [95% confidence interval (CI): -2.60, -1.85], 2.94 (95% CI: -3.36, -2.51), and 2.79% (95% CI: -3.15, -2.41) in the FVC, FEV1, and MMEF, respectively. Furthermore, it was associated with a 20% increase in the prevalence of poor lung function (OR: 1.20; 95% CI: 1.12, 1.29). CONCLUSIONS: Two-year ambient PM2.5 concentrations were inversely associated with lung function and positively associated with the prevalence of poor lung function in children, adolescents, and young adults in Taiwan. https://doi.org/10.1289/EHP5220.


Asunto(s)
Contaminantes Atmosféricos , Exposición a Riesgos Ambientales/estadística & datos numéricos , Material Particulado , Adolescente , Contaminación del Aire , Niño , Femenino , Volumen Espiratorio Forzado , Humanos , Masculino , Pruebas de Función Respiratoria , Taiwán/epidemiología , Capacidad Vital , Adulto Joven
10.
Neurology ; 93(21): e1932-e1943, 2019 11 19.
Artículo en Inglés | MEDLINE | ID: mdl-31653707

RESUMEN

OBJECTIVE: To identify the rate of change of clinical outcome measures in children with 2 types of congenital muscular dystrophy (CMD), COL6-related dystrophies (COL6-RDs) and LAMA2-related dystrophies (LAMA2-RDs). METHODS: Over the course of 4 years, 47 individuals (23 with COL6-RD and 24 with LAMA2-RD) 4 to 22 years of age were evaluated. Assessments included the Motor Function Measure 32 (MFM32), myometry (knee flexors and extensors, elbow flexors and extensors), goniometry (knee and elbow extension), pulmonary function tests, and quality-of-life measures. Separate linear mixed-effects models were fitted for each outcome measurement, with subject-specific random intercepts. RESULTS: Total MFM32 scores for COL6-RDs and LAMA2-RDs decreased at a rate of 4.01 and 2.60 points, respectively, each year (p < 0.01). All muscle groups except elbow flexors for individuals with COL6-RDs decreased in strength between 1.70% (p < 0.05) and 2.55% (p < 0.01). Range-of-motion measurements decreased by 3.21° (p < 0.05) at the left elbow each year in individuals with LAMA2-RDs and 2.35° (p < 0.01) in right knee extension each year in individuals with COL6-RDs. Pulmonary function demonstrated a yearly decline in sitting forced vital capacity percent predicted of 3.03% (p < 0.01) in individuals with COL6-RDs. There was no significant change in quality-of-life measures analyzed. CONCLUSION: Results of this study describe the rate of change of motor function as measured by the MFM32, muscle strength, range of motion, and pulmonary function in individuals with COL6-RDs and LAMA2-RDs.


Asunto(s)
Distrofias Musculares/fisiopatología , Esclerosis/fisiopatología , Adolescente , Artrometría Articular , Niño , Preescolar , Progresión de la Enfermedad , Nutrición Enteral , Femenino , Humanos , Modelos Lineales , Estudios Longitudinales , Masculino , Limitación de la Movilidad , Fuerza Muscular , Dinamómetro de Fuerza Muscular , Calidad de Vida , Pruebas de Función Respiratoria , Capacidad Vital , Adulto Joven
12.
N Engl J Med ; 381(18): 1718-1727, 2019 10 31.
Artículo en Inglés | MEDLINE | ID: mdl-31566307

RESUMEN

BACKGROUND: Preclinical data have suggested that nintedanib, an intracellular inhibitor of tyrosine kinases, inhibits processes involved in the progression of lung fibrosis. Although the efficacy of nintedanib has been shown in idiopathic pulmonary fibrosis, its efficacy across a broad range of fibrosing lung diseases is unknown. METHODS: In this double-blind, placebo-controlled, phase 3 trial conducted in 15 countries, we randomly assigned patients with fibrosing lung disease affecting more than 10% of lung volume on high-resolution computed tomography (CT) to receive nintedanib at a dose of 150 mg twice daily or placebo. All the patients met criteria for progression of interstitial lung disease in the past 24 months despite treatment and had a forced vital capacity (FVC) of at least 45% of the predicted value and a diffusing capacity of the lung for carbon monoxide ranging from 30 to less than 80% of the predicted value. Randomization was stratified according to the fibrotic pattern (a pattern of usual interstitial pneumonia [UIP] or other fibrotic patterns) on high-resolution CT. The primary end point was the annual rate of decline in the FVC, as assessed over a 52-week period. The two primary populations for analysis were the overall population and patients with a UIP-like fibrotic pattern. RESULTS: A total of 663 patients were treated. In the overall population, the adjusted rate of decline in the FVC was -80.8 ml per year with nintedanib and -187.8 ml per year with placebo, for a between-group difference of 107.0 ml per year (95% confidence interval [CI], 65.4 to 148.5; P<0.001). In patients with a UIP-like fibrotic pattern, the adjusted rate of decline in the FVC was -82.9 ml per year with nintedanib and -211.1 ml per year with placebo, for a difference of 128.2 ml (95% CI, 70.8 to 185.6; P<0.001). Diarrhea was the most common adverse event, as reported in 66.9% and 23.9% of patients treated with nintedanib and placebo, respectively. Abnormalities on liver-function testing were more common in the nintedanib group than in the placebo group. CONCLUSIONS: In patients with progressive fibrosing interstitial lung diseases, the annual rate of decline in the FVC was significantly lower among patients who received nintedanib than among those who received placebo. Diarrhea was a common adverse event. (Funded by Boehringer Ingelheim; INBUILD ClinicalTrials.gov number, NCT02999178.).


Asunto(s)
Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Indoles/uso terapéutico , Inhibidores de Proteínas Quinasas/uso terapéutico , Anciano , Diarrea/inducido químicamente , Progresión de la Enfermedad , Método Doble Ciego , Femenino , Humanos , Fibrosis Pulmonar Idiopática/fisiopatología , Indoles/efectos adversos , Masculino , Persona de Mediana Edad , Inhibidores de Proteínas Quinasas/efectos adversos , Capacidad Vital/efectos de los fármacos
13.
Muscle Nerve ; 60(6): 679-686, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-31566774

RESUMEN

INTRODUCTION: The purpose of this study was to comprehensively evaluate respiratory muscle function in adults with facioscapulohumeral muscular dystrophy (FSHD). METHODS: Fourteen patients with FSHD (9 men, 53 ± 16 years of age) and 14 matched controls underwent spirometry, diaphragm ultrasound, and measurement of twitch gastric and transdiaphragmatic pressures (twPgas and twPdi; n = 10) after magnetic stimulation of the lower thoracic nerve roots and the phrenic nerves. The latter was combined with recording of diaphragm compound muscle action potentials (CMAPs; n = 14). RESULTS: The following parameters were significantly lower in patients vs controls: forced vital capacity (FVC); maximum inspiratory and expiratory pressure; peak cough flow; diaphragm excursion amplitude; and thickening ratio on ultrasound, twPdi (11 ± 5 vs 20 ± 6 cmH2 O) and twPgas (7 ± 3 vs 25 ± 20 cmH2 O). Diaphragm CMAP showed no group differences. FVC correlated inversely with the clinical severity scale score (r = -0.63, P = .02). DISCUSSION: In FSHD, respiratory muscle weakness involves both the diaphragm and the expiratory abdominal muscles.


Asunto(s)
Diafragma/fisiopatología , Debilidad Muscular/fisiopatología , Distrofia Muscular Facioescapulohumeral/fisiopatología , Músculos Respiratorios/fisiopatología , Potenciales de Acción/fisiología , Adulto , Anciano , Estudios de Casos y Controles , Estudios Transversales , Diafragma/diagnóstico por imagen , Femenino , Humanos , Masculino , Presiones Respiratorias Máximas , Persona de Mediana Edad , Debilidad Muscular/etiología , Distrofia Muscular Facioescapulohumeral/complicaciones , Conducción Nerviosa , Nervio Frénico , Raíces Nerviosas Espinales , Espirometría , Vértebras Torácicas , Ultrasonografía , Capacidad Vital
14.
Respir Res ; 20(1): 161, 2019 Oct 08.
Artículo en Inglés | MEDLINE | ID: mdl-31590675

RESUMEN

BACKGROUND: There is growing literature suggesting a link between vitamin D and asthma lung function, but the results from systematic reviews are conflicting. We conducted this meta-analysis to investigate the relation between serum vitamin D and lung function in asthma patients. METHODS: Major databases, including OVID, MEDLINE, Web of Science and PUBMED, were searched until 10th October 2018. All published observational studies related to vitamin D and asthma were extracted. All meta-analyses were performed using Review Manager 5.3.5. RESULTS: This quantitative synthesis found that asthma patients with low vitamin D levels had lower forced expiratory volume In 1 s (FEV1) (mean difference (MD) = - 0.1, 95% CI = - 0.11 to - 0.08,p < 0.01;I2 = 49%, p = 0.12) and FEV1% (MD = - 10.02, 95% CI = - 11 to - 9.04, p < 0.01; I2 = 0%, p = 0.82) than those with sufficient vitamin D levels. A positive relation was found between vitamin D and FEV1 (r = 0.12, 95% CI = 0.04 to 0.2, p = 0.003; I2 = 59%,p = 0.01), FEV1% (r = 0.19, 95% CI = 0.13 to 0.26, p < 0.001; I2 = 42%, p = 0.11), forced vital capacity (FVC) (r = 0.17, 95% CI = 0.00 to 0.34, p = 0.05; I2 = 60%, p = 0.04), FEV1/FVC (r = 0.4, 95% CI = 0.3 to 0.51, p < 0.001; I2 = 48%, p = 0.07), and the asthma control test (ACT) (r = 0.33, 95% CI = 0.2 to 0.47, p < 0.001; I2 = 0%, p = 0.7). Subgroup analysis indicated that the positive correlation between vitamin D and lung function remained significant in both children and adults. CONCLUSIONS: Our meta-analysis suggested that serum vitamin D levels may be positively correlated with lung function in asthma patients. Future comprehensive studies are required to confirm these relations and to elucidate potential mechanisms.


Asunto(s)
Asma/fisiopatología , Pulmón/fisiopatología , Deficiencia de Vitamina D/fisiopatología , Adolescente , Adulto , Niño , Preescolar , Femenino , Volumen Espiratorio Forzado , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Estudios Observacionales como Asunto , Pruebas de Función Respiratoria , Capacidad Vital , Adulto Joven
15.
J Bras Pneumol ; 45(6): e20180252, 2019.
Artículo en Portugués, Inglés | MEDLINE | ID: mdl-31644702

RESUMEN

OBJECTIVE: To establish a cut-off point for clinical and functional variables to determinate sarcopenia and dynapenia in COPD patients, and to analyze the impact of skeletal muscle dysfunction (SMD) on these variables. METHODS: Cross-sectional study, screened COPD patients for sarcopenia or dynapenia through low muscle mass and hand grip strength (HGS). Clinical variables: pulmonary function, respiratory muscle strength and functional capacity (FC). The precision of the variables in determining points of predictive cut-off for sarcopenia or dynapenia were performed using the Receiver Operating Characteristic curve and two-way analysis of variance. RESULTS: 20 COPD patients stratified for sarcopenia (n = 11) and dynapenia (n = 07). Sarcopenia group presented lower lean mass and lower maximal inspiratory pressure (MIP), decreased HGS, reduced FC (p<0.050). Dynapenia group presented reduced MIP, lower HGS and walked a shorter distance at Incremental shuttle walk test (ISWT) (p<0.050). We found cut-off points of forced expiratory volume in one second (FEV1), MIP and maximal expiratory pressure (MEP) and ISWT. It is possible to identify sarcopenia or dynapenia in these patients. We found the coexistence of the conditions (SMD effect) in COPD - reduction in the distance in the ISWT (p = 0.002) and %ISWT (p = 0.017). CONCLUSION: In moderate to very severe COPD patients the sarcopenia could be predicted by FEV1 (%predicted) < 52, MIP < 73 cmH2O, MEP < 126 cmH2O and distance traveled of < 295 m in ISWT. Whereas dynapenia could be predicted by FEV1 < 40%, MIP < 71 cmH2O, MEP < 110 cmH2O and distance of < 230 m traveled in ISWT.


Asunto(s)
Pulmón/fisiopatología , Debilidad Muscular/fisiopatología , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Músculos Respiratorios/fisiopatología , Sarcopenia/fisiopatología , Anciano , Análisis de Varianza , Estudios Transversales , Femenino , Volumen Espiratorio Forzado/fisiología , Fuerza de la Mano/fisiología , Humanos , Masculino , Presiones Respiratorias Máximas , Persona de Mediana Edad , Fuerza Muscular/fisiología , Curva ROC , Valores de Referencia , Índice de Severidad de la Enfermedad , Estadísticas no Paramétricas , Capacidad Vital/fisiología , Prueba de Paso
16.
Neurology ; 93(19): e1756-e1767, 2019 11 05.
Artículo en Inglés | MEDLINE | ID: mdl-31619483

RESUMEN

OBJECTIVE: To determine the effects of 10 years of enzyme replacement therapy (ERT) in adult patients with Pompe disease, focusing on individual variability in treatment response. METHODS: In this prospective, multicenter cohort study, we studied 30 patients from the Netherlands and France who had started ERT during the only randomized placebo-controlled clinical trial with ERT in late-onset Pompe disease (NCT00158600) or its extension (NCT00455195) in 2005 to 2008. Main outcomes were walking ability (6-minute walk test [6MWT]), muscle strength (manual muscle testing using Medical Research Council [MRC] grading), and pulmonary function (forced vital capacity [FVC] in the upright and supine positions), assessed at 3- to 6-month intervals before and after the start of ERT. Data were analyzed with linear mixed-effects models for repeated measurements. RESULTS: Median follow-up duration on ERT was 9.8 years (interquartile range [IQR] 8.3-10.2 years). At the group level, baseline 6MWT was 49% of predicted (IQR 41%-60%) and had deteriorated by 22.2 percentage points (pp) at the 10-year treatment point (p < 0.001). Baseline FVC upright was 54% of predicted (IQR 47%-68%) and decreased by 11 pp over 10 years (p < 0.001). Effects of ERT on MRC sum score and FVC supine were similar. At the individual level, 93% of patients had initial benefit of ERT. Depending on the outcome measured, 35% to 63% of patients had a secondary decline after ≈3 to 5 years. Still, at 10 years of ERT, 52% had equal or better 6MWT and/or FVC upright compared to baseline. CONCLUSIONS: The majority of patients with Pompe disease benefit from long-term ERT, but many patients experience some secondary decline after ≈3 to 5 years. Individual variation, however, is considerable. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for the majority of adults with Pompe disease, long-term ERT positively affects, or slows deterioration in, muscle strength, walking ability, and/or pulmonary function.


Asunto(s)
Terapia de Reemplazo Enzimático , Enfermedad del Almacenamiento de Glucógeno Tipo II/tratamiento farmacológico , alfa-Glucosidasas/uso terapéutico , Adulto , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Francia , Enfermedad del Almacenamiento de Glucógeno Tipo II/complicaciones , Enfermedad del Almacenamiento de Glucógeno Tipo II/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Limitación de la Movilidad , Fuerza Muscular , Debilidad Muscular/etiología , Debilidad Muscular/fisiopatología , Países Bajos , Ventilación no Invasiva/estadística & datos numéricos , Estudios Prospectivos , Ensayos Clínicos Controlados Aleatorios como Asunto , Insuficiencia Respiratoria/etiología , Insuficiencia Respiratoria/fisiopatología , Insuficiencia Respiratoria/terapia , Resultado del Tratamiento , Capacidad Vital , Prueba de Paso , Silla de Ruedas
17.
Am J Case Rep ; 20: 1530-1535, 2019 Oct 18.
Artículo en Inglés | MEDLINE | ID: mdl-31624225

RESUMEN

BACKGROUND Upside-down stomach (UDS) is the rarest type of hiatal hernia (HH), with organoaxial gastric volvulus. A large HH sometimes causes cardiopulmonary impairments owing to multiple factors. CASE REPORT We herein report a case of a large HH with UDS that had induced weight loss and severe cardiopulmonary dysfunction in a 74-year-old female patient who presented with shortness of breath, chest pain, severe anorexia, and weight loss of 5 kg over the 3 previous months. Chest X-ray and CT examination revealed that her heart was retracted on the right side, and the hernia contents had induced physical compression of the left lung on the cranial side. Spirometry revealed that the patient's vital capacity (VC), percentage VC, and percentage forced expiratory volume (% FEV) at 1 s were 1.32 L, 60.2%, and 67.5%, respectively. A barium swallow test confirmed a diagnosis of HH with UDS. On the basis of these findings, we performed a laparoscopic Nissen procedure, which resulted in the patient's dramatic recovery. Postoperative examinations showed that the stomach and heart were once again normally located, and the left lung had re-inflated. Postoperative spirometry dramatically improved. CONCLUSIONS A large HH causes cardiac and pulmonary compression due to mass effects and leads to cardiopulmonary dysfunction. For cases that have both a complicated HH and cardiopulmonary dysfunction owing to the mass effects of hernia contents, laparoscopic HH repair can be a good alternative procedure.


Asunto(s)
Corazón/fisiopatología , Hernia Hiatal/diagnóstico por imagen , Hernia Hiatal/cirugía , Pulmón/fisiopatología , Vólvulo Gástrico/diagnóstico por imagen , Vólvulo Gástrico/cirugía , Anciano , Anorexia/etiología , Dolor en el Pecho/etiología , Disnea/etiología , Femenino , Fundoplicación , Humanos , Laparoscopía , Espirometría , Capacidad Vital
18.
Zhongguo Zhong Yao Za Zhi ; 44(17): 3806-3815, 2019 Sep.
Artículo en Chino | MEDLINE | ID: mdl-31602957

RESUMEN

The randomized controlled trials about modified Sangbaipi Decoction in the treatment of acute exacerbation of chronic obstructive pulmonary disease( AECOPD) patients were collected from 7 databases( PubMed,CNKI,et al) from the establishment to December 5,2018. All the studies searched were strictly evaluated. Literatures were independently screened by two researchers according to the inclusion and exclusion criteria,and the methodological quality of included studies was evaluated. To systematically review the efficacy of modified Sangbaipi Decoction in treating AECOPD,the Meta-analysis and trial sequential analysis were conducted by using Stata/SE 14. 0 and TSA 0. 9. 5. 10 Beta,respectively. A total of 25 RCTs involving 1 784 patients were included. According to the results of Meta-analysis,compared with the control groups,the trial group had a higher clinical efficacy in AECOPD patients( RR =1. 18,95%CI[1. 13,1. 22],P = 0),improved pulmonary functions including forced expiratory volume in one second( FEV1,WMD =0. 44,95%CI[0. 01,0. 87],P = 0. 046),and the forced vital capacity( FVC,WMD = 0. 42,95%CI[0. 07,0. 22],P = 0),but no statistical significance in the percentage of forced expiratory volume in one second( FEV1%,P = 0. 067) and the first seconds breathing volume percentage of forced vital capacity( FEV1/FVC,P = 0. 238); it improved the arterial oxygen partial pressure( PaO2,SMD =0. 85,95%CI[0. 41,1. 30],P = 0) and decreased the arterial partial pressure of carbon dioxide( PaCO2,SMD =-0. 94,95% CI[-1. 70,-0. 18],P= 0. 016); and in terms of inflammatory markers,it improved the white blood cell count( WBC,WMD=-0. 94,95%CI[-1. 17,-0. 70],P = 0). The trial sequential analysis showed that the studies included with the improvement of clinical efficacy had passed the conventional and TSA threshold,so as to further confirm the evidence. According to the findings,in addition to conventional Western medicine treatment,modified Sangbaipi Decoction could improve the efficiency in treating acute exacerbation patients with chronic obstructive pulmonary disease,increase PaO2,and decrease PaCO2,with a high safety but no effect on pulmonary function. However,restricted by the low quality of studies included,this conclusion shall be further verified by more high-quality clinical trials.


Asunto(s)
Medicamentos Herbarios Chinos/uso terapéutico , Enfermedad Pulmonar Obstructiva Crónica/tratamiento farmacológico , Presión Arterial , Volumen Espiratorio Forzado , Humanos , Pulmón , Presión Parcial , Ensayos Clínicos Controlados Aleatorios como Asunto , Capacidad Vital
19.
Med Sci Monit ; 25: 7763-7769, 2019 Oct 16.
Artículo en Inglés | MEDLINE | ID: mdl-31618191

RESUMEN

BACKGROUND Pulmonary hypertension is a common complication of interstitial lung disease. This study was conducted to retrospectively analyze the incidence of pulmonary hypertension among interstitial lung disease patients and the correlation between systolic pulmonary artery pressure (PASP) and pulmonary functions. We also intended to investigate whether antinuclear antibody (ANA) could be an effective indicator of pulmonary hypertension. MATERIAL AND METHODS There were 182 patients diagnosed with interstitial lung disease through high-resolution computed tomography (HRCT). Pulmonary hypertension was defined as an increase of mean pulmonary arterial pressure (PAPm) ≥25 mmHg (~PASP ≥40 mmHg) at rest. Severe pulmonary hypertension was defined as PAPm ≥35 mmHg. There were 104 cases including in this study. There were 67 cases from the ANA positive (ANA+) group and 37 cases from the ANA negative (ANA-) group. All study patients had pulmonary function tests, which included the measurements of maximal voluntary ventilation (MVV), residual volume (RV), total lung capacity (TLC), forced expiratory volume (FVC), vital capacity (VC), and diffusing capacity of the lungs for carbon monoxide (DLCO). RESULTS The pulmonary hypertension incidence in the study cohort was 25%, and the severe pulmonary hypertension incidence was 6.48%. The incidence of pulmonary hypertension in ANA+ cases was 22.22%. The incidence of pulmonary hypertension in the ANA- cases was 32.14%. The lung function test results showed moderate relationships between DLCO, FVC%, VC%, and PASP; no relationship between MVV, FEV1/FVC%, RV/TLC, and PASP; minimum relationship between FVC%, VC%, and PASP in the ANA+ group; and moderate relationship between FVC%, VC%, and PASP in the ANA- group. CONCLUSIONS Pulmonary hypertension occurred in 25% of the 182 interstitial lung disease patients and was negatively associated with deteriorated lung functions (specifically VC%, FVC%, and DLCO parameters). ANA level was not associated with the prognosis of pulmonary hypertension of patients with interstitial lung disease, and it did not significantly affect the correlation between PASP and pulmonary functions. Thus, ANA level did not seem to be a necessary indicator of pulmonary hypertension, and a more effective treatment method for pulmonary hypertension of patients with interstitial lung disease is urgently needed.


Asunto(s)
Hipertensión Pulmonar/epidemiología , Enfermedades Pulmonares Intersticiales/epidemiología , Anciano , Anticuerpos Antinucleares/análisis , Biomarcadores/sangre , Presión Sanguínea/fisiología , China/epidemiología , Estudios de Cohortes , Femenino , Volumen Espiratorio Forzado , Humanos , Hipertensión Pulmonar/sangre , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares Intersticiales/sangre , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria/métodos , Estudios Retrospectivos , Capacidad Vital
20.
J Bras Pneumol ; 45(6): e20180132, 2019.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-31618297

RESUMEN

OBJECTIVE: Preoperative functional evaluation is central to optimizing the identification of patients with non-small cell lung cancer (NSCLC) who are candidates for surgery. The minute ventilation/carbon dioxide output (VE/VCO2) slope has proven to be a predictor of surgical complications and mortality. Pulmonary rehabilitation programs (PRPs) could influence short-term outcomes in patients with COPD undergoing lung resection. Our objective was to evaluate the effects of a PRP on the VE/VCO2 slope in a cohort of patients with COPD undergoing lung resection for NSCLC. METHODS: We retrospectively evaluated 25 consecutive patients with COPD participating in a three-week high-intensity PRP prior to undergoing lung surgery for NSCLC, between December of 2015 and January of 2017. Patients underwent complete functional assessment, including spirometry, DLCO measurement, and cardiopulmonary exercise testing. RESULTS: There were no significant differences between the mean pre- and post-PRP values (% of predicted) for FEV1 (61.5 ± 22.0% vs. 62.0 ± 21.1%) and DLCO (67.2 ± 18.1% vs. 67.5 ± 13.2%). Conversely, there were significant improvements in the mean peak oxygen uptake (from 14.7 ± 2.5 to 18.2 ± 2.7 mL/kg per min; p < 0.001) and VE/VCO2 slope (from 32.0 ± 2.8 to 30.1 ± 4.0; p < 0.01). CONCLUSIONS: Our results indicate that a high-intensity PRP can improve ventilatory efficiency in patients with COPD undergoing lung resection for NSCLC. Further comprehensive prospective studies are required to corroborate these preliminary results.


Asunto(s)
Carcinoma de Pulmón de Células no Pequeñas/rehabilitación , Terapia por Ejercicio/métodos , Neoplasias Pulmonares/rehabilitación , Enfermedad Pulmonar Obstructiva Crónica/rehabilitación , Anciano , Dióxido de Carbono/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/fisiopatología , Carcinoma de Pulmón de Células no Pequeñas/cirugía , Prueba de Esfuerzo , Femenino , Volumen Espiratorio Forzado/fisiología , Humanos , Neoplasias Pulmonares/fisiopatología , Neoplasias Pulmonares/cirugía , Masculino , Persona de Mediana Edad , Consumo de Oxígeno/fisiología , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Valores de Referencia , Estudios Retrospectivos , Espirometría , Factores de Tiempo , Resultado del Tratamiento , Capacidad Vital/fisiología
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