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1.
Bol Med Hosp Infant Mex ; 78(1): 29-33, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33661877

RESUMEN

Background: Cystic fibrosis (CF) is a potentially mortal disease characterized by a chronic pulmonary disease with persistent airway infection. Children with this disease are more susceptible to respiratory infections due to the limitation in mucociliary transport and anatomical disruption of the bronchial tree. SARS-CoV-2 causes COVID-19, a respiratory illness related to exacerbations of chronic pulmonary pathologies in children, such as CF and asthma. There are not enough case reports on pediatric patients with SARS-CoV-2 infection and CF, for which we share our experience. Case report: A 22-month-old male patient diagnosed with CF presented in the hospital with cough, fever, and increased respiratory work. The patient received supplemental oxygen and antibiotic and antiviral therapy. Positive results for type B influenza and RT-PCR (reverse transcription-polymerase chain reaction) for SARS-CoV-2 were obtained. Due to the persistence of respiratory difficulty, high-flow therapy was initiated, with a good response. After an episode of hypoxemia, bradycardia, and increased respiratory work secondary to accumulated secretions, orotracheal intubation and invasive mechanical ventilation were performed. The patient evolved with clinical and gasometric improvement. After 10 days of in-hospital antibiotic management with adequate clinical evolution, the patient was discharged to complete oral treatment and home isolation. Conclusions: We present a case of chronic respiratory disease and SARS-CoV-2 infection with severity criteria in a pediatric patient. The evolution was favorable with timely support management and antibiotic therapy in a third-level hospital.


Asunto(s)
/fisiopatología , Fibrosis Quística/complicaciones , /diagnóstico , Tos/virología , Fibrosis Quística/fisiopatología , Fiebre/virología , Humanos , Lactante , Masculino , Respiración Artificial , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Factores de Riesgo , Índice de Severidad de la Enfermedad
2.
Arch Biochem Biophys ; 699: 108763, 2021 03 15.
Artículo en Inglés | MEDLINE | ID: mdl-33460581

RESUMEN

MicroRNAs (miRNAs) are small non-coding highly conserved RNA molecules that can act as master regulators of gene expression in a sequence-specific manner either by translation repression or mRNA degradation, influencing a wide range of biologic processes that are essential for the maintenance of cellular homeostasis. Chronic pediatric diseases are the leading cause of death worldwide among children and the recent evidence indicates that aberrant miRNA expression significantly contributes to the development of chronic pediatric diseases. This review focuses on the role of miRNAs in five major chronic pediatric diseases including bronchial asthma, congenital heart diseases, cystic fibrosis, type 1 diabetes mellitus, and epilepsy, and their potential use as novel biomarkers for the diagnosis and prognosis of these disorders.


Asunto(s)
Asma/fisiopatología , Fibrosis Quística/fisiopatología , Diabetes Mellitus Tipo 1/fisiopatología , Epilepsia/fisiopatología , Cardiopatías Congénitas/fisiopatología , MicroARNs/fisiología , Asma/diagnóstico , Asma/metabolismo , Biomarcadores/sangre , Biomarcadores/metabolismo , Enfermedad Crónica , Fibrosis Quística/diagnóstico , Fibrosis Quística/metabolismo , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/metabolismo , Epilepsia/diagnóstico , Epilepsia/metabolismo , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/metabolismo , Humanos , MicroARNs/sangre , MicroARNs/metabolismo , Pediatría , Pronóstico
4.
Life Sci ; 268: 118959, 2021 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-33383045

RESUMEN

Cystic fibrosis (CF) is an autosomal recessive disease which involves the mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CF involves in the inflammatory processes and is considered as a multisystem disorder that is not confined to lungs, but it also affects other vital organs that leads to numerous co-morbidities. The respiratory disorder in the CF results in mortality and morbidity which is characterized by series of serious events involving mucus hypersecretion, microbial infections, airways obstruction, inflammation, destruction of epithelium, tissue remodeling and terminal lung diseases. Mucins are the high molecular weight glycoproteins important for the viscoelastic properties of the mucus, play a significant role in the disease mechanisms. Determining the functional association between the CFTR and mucins might help to identify the putative target for specific therapeutic approach. In fact, furin enzyme which helps in the entry of novel COVID-19 virus into the cell, is upregulated in CF and this can also serve as a potential target for CF treatment. Moreover, the use of nano-formulations for CF treatment is an area of research being widely studied as they have also demonstrated promising outcomes. The in-depth knowledge of non-coding RNAs like miRNAs and lncRNAs and their functional association with CFTR gene expression and mutation can provide a different range of opportunity to identify the promising therapeutic approaches for CF.


Asunto(s)
/virología , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Fibrosis Quística/fisiopatología , Animales , Fibrosis Quística/genética , Fibrosis Quística/terapia , Regulación de la Expresión Génica , Humanos , MicroARNs/genética , Mucinas/metabolismo , Mutación , ARN Largo no Codificante/genética , /patogenicidad
5.
Isr Med Assoc J ; 22(12): 770-774, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33381950

RESUMEN

BACKGROUND: The single-breath diffusing capacity of the lungs (DLCOSB) test measures the extent to which carbon monoxide (CO) passes from the lung air sacs into the blood. The accessible alveolar volume (VASB) is measured by inert gas during a 10-second period. The single-breath transfer coefficient of the lung for carbon monoxide (KCOSB) is the DLCOSB divided by VASB. Cystic fibrosis (CF) disease comprises progressive airway obstruction with bronchiectasis and parenchyma fibrosis. Yet, the KCOSB appears insignificant in the assessment of pulmonary function in CF. OBJECTIVES: To challenge the precision of normal KCOSB in CF. METHODS: The authors collected pulmonary function tests (PFT) data from 74 confirmed CF patients (mean age 26 ± 10 years) with various levels of pulmonary disease severity. Tests included spirometry, DLCOBP, and body plethysmography (BP). Anatomical dead space was calculated by deducting anatomical dead space from total lung capacity TLC(BP) to establish alveolar volume (VABP) and to determine KCOBP. We also included individual data of arterial pCO2 blood-gas level. RESULTS: KCOSB values were normal or higher in most patients, regardless of patient FEV1 value (R2 = 0.2204; P < 0.02). In contrast, the measurements of KCOBP were low corresponding with low FEV1 values, and negatively correlated with the elevation of trapped air and pCO2 levels (R2 = 0.1383; P = 0.0133, P > 0.05, respectively). CONCLUSIONS: The 10- second perfusion time of the inert gas during DLCOSB represent the communicative alveolar volume in CF patients with advanced pulmonary disease. The findings justify the use of DLCOSB with the deterioration of FEV1 and elevation of pCO2 levels.


Asunto(s)
Fibrosis Quística/complicaciones , Enfermedades Pulmonares/etiología , Capacidad de Difusión Pulmonar , Adolescente , Adulto , Niño , Fibrosis Quística/fisiopatología , Femenino , Humanos , Enfermedades Pulmonares/fisiopatología , Mediciones del Volumen Pulmonar , Masculino , Persona de Mediana Edad , Pletismografía Total , Pruebas de Función Respiratoria , Espirometría , Adulto Joven
6.
Monaldi Arch Chest Dis ; 90(4)2020 Dec 23.
Artículo en Inglés | MEDLINE | ID: mdl-33372741

RESUMEN

We report the case of a man affected by cystic fibrosis who developed a severe SARS-CoV-2 related pneumonia in March 2020. In addition to lopinavir/ritonavir and hydroxychloroquine, he was treated with two doses of tocilizumab, displaying a significant clinical improvement. This is the first case described in the literature of an adult patient affected by cystic fibrosis who received tocilizumab for COVID-19, with documented total recovery, also assessed by a spirometry.


Asunto(s)
Anticuerpos Monoclonales Humanizados/administración & dosificación , Fibrosis Quística , Hidroxicloroquina/administración & dosificación , Lopinavir/administración & dosificación , Neumonía Viral , Infecciones del Sistema Respiratorio/microbiología , Ritonavir/administración & dosificación , /aislamiento & purificación , Adulto , Antivirales/administración & dosificación , /diagnóstico , /fisiopatología , Fibrosis Quística/complicaciones , Fibrosis Quística/inmunología , Fibrosis Quística/fisiopatología , Combinación de Medicamentos , Humanos , Pulmón/diagnóstico por imagen , Pulmón/fisiopatología , Masculino , Terapia por Inhalación de Oxígeno/métodos , Neumonía Viral/diagnóstico , Neumonía Viral/tratamiento farmacológico , Neumonía Viral/etiología , Receptores de Interleucina-6/antagonistas & inhibidores , Pruebas de Función Respiratoria/métodos , Infecciones del Sistema Respiratorio/tratamiento farmacológico , Infecciones del Sistema Respiratorio/etiología , Tomografía Computarizada por Rayos X/métodos , Resultado del Tratamiento
8.
PLoS One ; 15(9): e0238524, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32915806

RESUMEN

BACKGROUND: Markers of lung inflammation measured directly in expectorated sputum have the potential of improving the timing of antibiotic treatment in cystic fibrosis (CF). L-Lactate might be a marker of inflammation, as it is produced from glucose by polymorphonuclear neutrophils (PMNs) in CF lungs. We aimed to investigate changes in and associations between PMNs, glucose and L-lactate in sputum during antibiotic treatment. In addition, the effect of hemoglobin A1c and plasma glucose on these biomarkers were investigated. METHODS: We sampled non-induced sputum at day 0, 7, 14 and 42 in 27 chronically infected CF patients electively treated with 14 days of intravenous antibiotic. To analyze sputum samples, we used flowcytometry to count PMNs and colorimetric assays to estimate lactate and glucose. RESULTS: No changes in levels of PMNs, glucose and lactate were detected in sputum during the antibiotic treatment. Sputum PMNs were positively associated with both glucose (log coefficient = 0.20, p = 0.01) and L-lactate (log coefficient = 0.34, p<0.001). In multivariate analyses, hemoglobin A1c was negatively associated with sputum PMNs (log coefficient = -1.68, p<0.001) and plasma glucose was negatively associated with sputum glucose (log coefficient = -0.09, p = 0.02). CONCLUSIONS: In CF sputum PMNs, glucose and lactate were unchanged during elective antibiotic treatment. However, sputum PMNs were associated with both sputum glucose and sputum lactate. Surprisingly, hyperglycemia seemed to be associated with less PMNs infiltration and less glucose in CF sputum.


Asunto(s)
Fibrosis Quística/sangre , Glucosa/metabolismo , Ácido Láctico/metabolismo , Neutrófilos/metabolismo , Esputo/metabolismo , Adulto , Anciano , Antibacterianos/administración & dosificación , Antibacterianos/uso terapéutico , Biomarcadores/metabolismo , Glucemia/metabolismo , Fibrosis Quística/fisiopatología , Femenino , Hemoglobina A Glucada/metabolismo , Humanos , Inyecciones Intravenosas , Recuento de Leucocitos , Masculino , Análisis Multivariante , Pruebas de Función Respiratoria , Adulto Joven
9.
Pediatr Pulmonol ; 55(12): 3579-3586, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32946202

RESUMEN

BACKGROUND: We aim to assess the anxiety and depressive symptoms related to the COVID-19 pandemic in children with chronic lung disease and their parents and also to evaluate parents' coping strategies. METHODS: Parents of children aged 4-18 years, with chronic lung disease (study group n = 113) and healthy control (n = 108) were enrolled in the study. General Health Questionnaire-12, specific COVID-19 related anxiety questions, The Coping Orientation to Problems Experienced inventory, coronavirus-related psychiatric symptom scale in children-parental form were used to analyze the psychiatric effects of COVID-19. Parents were also asked about how online education affected their family life and children. All data were compared between children/parents in the study and control groups. Risk factors related with anxiety scores of children were also analyzed. RESULTS: Talking about the pandemic, concern about coronavirus transmission, taking precaution to prevent coronavirus transmission, making pressure to protect from COVID-19 were significantly higher in parents within the study group (p < .05). Parents in the study group used more problem-focused coping than parents in the control group (p = .003). Anxiety symptoms score was higher in children of the study group (p = .007). Parents in the study group found online education more useful than parents in the control group. CONCLUSION: Children with chronic lung diseases and their parents have more anxiety due to COVID-19 pandemic and these parents use more mature coping strategies to manage the stress of the pandemic. Longitudinal and larger studies should be done in all aspects of online education in children with chronic lung diseases.


Asunto(s)
Ansiedad/psicología , Trastornos de la Motilidad Ciliar/psicología , Infecciones por Coronavirus , Fibrosis Quística/psicología , Enfermedades Pulmonares Intersticiales/psicología , Pandemias , Padres/psicología , Neumonía Viral , Estrés Psicológico/psicología , Adaptación Psicológica , Adolescente , Adulto , Betacoronavirus , Estudios de Casos y Controles , Niño , Preescolar , Trastornos de la Motilidad Ciliar/fisiopatología , Fibrosis Quística/fisiopatología , Femenino , Estado de Salud , Humanos , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Persona de Mediana Edad , Factores de Riesgo
10.
Artículo en Inglés | MEDLINE | ID: mdl-32750662

RESUMEN

Cystic fibrosis (CF) is a recessively inherited fatal disease that is the subject of extensive research and ongoing development of therapeutics targeting the defective protein, cystic fibrosis transmembrane conductance regulator (CFTR). Despite progress, the link between CFTR and clinical symptoms is incomplete. The severe CF phenotypes are associated with a deficiency of linoleic acid, which is the precursor of arachidonic acid. The release of arachidonic acid from membranes via phospholipase A2 is the rate-limiting step for eicosanoid synthesis and is increased in CF, which contributes to the observed inflammation. A potential deficiency of docosahexaenoic acid may lead to decreased levels of specialized pro-resolving mediators. This pathophysiology may contribute to an early and sterile inflammation, mucus production, and to bacterial colonization, which further increases inflammation and potentiates the clinical symptoms. Advances in lipid technology will assist in elucidating the role of lipid metabolism in CF, and stimulate therapeutic modulations of inflammation.


Asunto(s)
Ácido Araquidónico/metabolismo , Regulador de Conductancia de Transmembrana de Fibrosis Quística/metabolismo , Fibrosis Quística/metabolismo , Ácidos Docosahexaenoicos/deficiencia , Ácido Linoleico/deficiencia , Ácido Araquidónico/deficiencia , Fibrosis Quística/fisiopatología , Humanos , Inflamación/metabolismo , Ácido Linoleico/metabolismo , Metabolismo de los Lípidos , Pulmón/metabolismo , Pulmón/microbiología , Pulmón/fisiopatología , Moco/metabolismo
12.
Am J Respir Crit Care Med ; 202(10): 1419-1429, 2020 11 15.
Artículo en Inglés | MEDLINE | ID: mdl-32603604

RESUMEN

Rationale: Cystic fibrosis (CF) is a life-shortening, multisystem hereditary disease caused by abnormal chloride transport. CF lung disease is driven by innate immune dysfunction and exaggerated inflammatory responses that contribute to tissue injury. To define the transcriptional profile of this airway immune dysfunction, we performed the first single-cell transcriptome characterization of CF sputum.Objectives: To define the transcriptional profile of sputum cells and its implication in the pathogenesis of immune function and the development of CF lung disease.Methods: We performed single-cell RNA sequencing of sputum cells from nine subjects with CF and five healthy control subjects. We applied novel computational approaches to define expression-based cell function and maturity profiles, herein called transcriptional archetypes.Measurements and Main Results: The airway immune cell repertoire shifted from alveolar macrophages in healthy control subjects to a predominance of recruited monocytes and neutrophils in CF. Recruited lung mononuclear phagocytes were abundant in CF and were separated into the following three archetypes: activated monocytes, monocyte-derived macrophages, and heat shock-activated monocytes. Neutrophils were the most prevalent in CF, with a dominant immature proinflammatory archetype. Although CF monocytes exhibited proinflammatory features, both monocytes and neutrophils showed transcriptional evidence of abnormal phagocytic and cell-survival programs.Conclusions: Our findings offer an opportunity to understand subject-specific immune dysfunction and its contribution to divergent clinical courses in CF. As we progress toward personalized applications of therapeutic and genomic developments, we hope this inflammation-profiling approach will enable further discoveries that change the natural history of CF lung disease.


Asunto(s)
Resistencia de las Vías Respiratorias/genética , Fibrosis Quística/genética , Fibrosis Quística/fisiopatología , Inflamación/genética , Inflamación/fisiopatología , Activación Transcripcional/genética , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis de la Célula Individual
13.
Cochrane Database Syst Rev ; 4: CD006842, 2020 04 30.
Artículo en Inglés | MEDLINE | ID: mdl-32352564

RESUMEN

BACKGROUND: Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis. Oscillating devices generate intra- or extra-thoracic oscillations orally or external to the chest wall. Internally they create variable resistances within the airways, generating controlled oscillating positive pressure which mobilises mucus. Extra-thoracic oscillations are generated by forces outside the respiratory system, e.g. high frequency chest wall oscillation. This is an update of a previously published review. OBJECTIVES: To identify whether oscillatory devices, oral or chest wall, are effective for mucociliary clearance and whether they are equivalent or superior to other forms of airway clearance in the successful management of secretions in people with cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and hand searches of relevant journals and abstract books of conference proceedings. Latest search of the Cystic Fibrosis Trials Register: 29 July 2019. In addition we searched the trials databases ClinicalTrials.gov and the WHO International Clinical Trials Registry Platform. Latest search of trials databases: 15 August 2019. SELECTION CRITERIA: Randomised controlled studies and controlled clinical studies of oscillating devices compared with any other form of physiotherapy in people with cystic fibrosis. Single-treatment interventions (therapy technique used only once in the comparison) were excluded. DATA COLLECTION AND ANALYSIS: Two authors independently applied the inclusion criteria to publications, assessed the quality of the included studies and assessed the evidence using GRADE. MAIN RESULTS: The searches identified 82 studies (330 references); 39 studies (total of 1114 participants) met the inclusion criteria. Studies varied in duration from up to one week to one year; 20 of the studies were cross-over in design. The studies also varied in type of intervention and the outcomes measured, data were not published in sufficient detail in most of these studies, so meta-analysis was limited. Few studies were considered to have a low risk of bias in any domain. It is not possible to blind participants and clinicians to physiotherapy interventions, but 13 studies did blind the outcome assessors. The quality of the evidence across all comparisons ranged from low to very low. Forced expiratory volume in one second was the most frequently measured outcome and while many of the studies reported an improvement in those people using a vibrating device compared to before the study, there were few differences when comparing the different devices to each other or to other airway clearance techniques. One study identified an increase in frequency of exacerbations requiring antibiotics whilst using high frequency chest wall oscillation when compared to positive expiratory pressure (low-quality evidence). There were some small but significant changes in secondary outcome variables such as sputum volume or weight, but not wholly in favour of oscillating devices and due to the low- or very low-quality evidence, it is not clear whether these were due to the particular intervention. Participant satisfaction was reported in 13 studies but again with low- or very low-quality evidence and not consistently in favour of an oscillating device, as some participants preferred breathing techniques or techniques used prior to the study interventions. The results for the remaining outcome measures were not examined or reported in sufficient detail to provide any high-level evidence. AUTHORS' CONCLUSIONS: There was no clear evidence that oscillation was a more or less effective intervention overall than other forms of physiotherapy; furthermore there was no evidence that one device is superior to another. The findings from one study showing an increase in frequency of exacerbations requiring antibiotics whilst using an oscillating device compared to positive expiratory pressure may have significant resource implications. More adequately-powered long-term randomised controlled trials are necessary and outcomes measured should include frequency of exacerbations, individual preference, adherence to therapy and general satisfaction with treatment. Increased adherence to therapy may then lead to improvements in other parameters, such as exercise tolerance and respiratory function. Additional evidence is needed to evaluate whether oscillating devices combined with other forms of airway clearance is efficacious in people with cystic fibrosis.There may also be a requirement to consider the cost implication of devices over other forms of equally advantageous airway clearance techniques. Using the GRADE method to assess the quality of the evidence, we judged this to be low or very low quality, which suggests that further research is very likely to have an impact on confidence in any estimate of effect generated by future interventions.


Asunto(s)
Oscilación de la Pared Torácica/instrumentación , Fibrosis Quística/complicaciones , Enfermedades Pulmonares Obstructivas/terapia , Depuración Mucociliar , Moco/metabolismo , Vibración/uso terapéutico , Adolescente , Adulto , Ejercicios Respiratorios , Niño , Fibrosis Quística/fisiopatología , Progresión de la Enfermedad , Flujo Espiratorio Forzado , Volumen Espiratorio Forzado , Humanos , Enfermedades Pulmonares Obstructivas/etiología , Persona de Mediana Edad , Satisfacción del Paciente , Ensayos Clínicos Controlados Aleatorios como Asunto , Esputo/metabolismo
14.
Cochrane Database Syst Rev ; 5: CD008649, 2020 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-32358807

RESUMEN

BACKGROUND: Several agents are used to clear secretions from the airways of people with cystic fibrosis. Mannitol increases mucociliary clearance, but its exact mechanism of action is unknown. The dry powder formulation of mannitol may be more convenient and easier to use compared with established agents which require delivery via a nebuliser. Phase III trials of inhaled dry powder mannitol for the treatment of cystic fibrosis have been completed and it is now available in Australia and some countries in Europe. This is an update of a previous review. OBJECTIVES: To assess whether inhaled dry powder mannitol is well tolerated, whether it improves the quality of life and respiratory function in people with cystic fibrosis and which adverse events are associated with the treatment. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic databases, handsearching relevant journals and abstracts from conferences. Date of last search: 12 December 2019. SELECTION CRITERIA: All randomised controlled studies comparing mannitol with placebo, active inhaled comparators (for example, hypertonic saline or dornase alfa) or with no treatment. DATA COLLECTION AND ANALYSIS: Authors independently assessed studies for inclusion, carried out data extraction and assessed the risk of bias in included studies. The quality of the evidence was assessed using GRADE. MAIN RESULTS: Six studies (reported in 36 unique publications) were included with a total of 784 participants. Duration of treatment in the included studies ranged from 12 days to six months, with open-label treatment for an additional six months in two of the studies. Five studies compared mannitol with control (a very low dose of mannitol or non-respirable mannitol) and the final study compared mannitol to dornase alfa alone and to mannitol plus dornase alfa. Two large studies had a similar parallel design and provided data for 600 participants, which could be pooled where data for a particular outcome and time point were available. The remaining studies had much smaller sample sizes (ranging from 22 to 95) and data could not be pooled due to differences in design, interventions and population. Pooled evidence from the two large parallel studies was judged to be of low to moderate quality and from the smaller studies was judged to be of low to very low quality. In all studies, there was an initial test to see if participants tolerated mannitol, with only those who could tolerate the drug being randomised; therefore, the study results are not applicable to the cystic fibrosis population as a whole. While the published papers did not provide all the data required for our analysis, additional unpublished data were provided by the drug's manufacturer and the author of one of the studies. Pooling the large parallel studies comparing mannitol to control, up to and including six months, lung function (forced expiratory volume at one second) measured in both mL and % predicted was significantly improved in the mannitol group compared to the control group (moderate-quality evidence). Beneficial results were observed in these studies in adults and in both concomitant dornase alfa users and non-users in these studies. In the smaller studies, statistically significant improvements in lung function were also observed in the mannitol groups compared to the non-respirable mannitol groups; however, we judged this evidence to be of low to very low quality. For the comparisons of mannitol and control, we found no consistent differences in health-related quality of life in any of the domains except for burden of treatment, which was less for mannitol up to four months in the two pooled studies of a similar design; this difference was not maintained at six months. It should be noted that the tool used to measure health-related quality of life was not designed to assess mucolytics and pooling of the age-appropriate tools (as done in some of the included studies) may not be valid so results were judged to be low to very low quality and should be interpreted with caution. Cough, haemoptysis, bronchospasm, pharyngolaryngeal pain and post-tussive vomiting were the most commonly reported side effects in both treatment groups. Where rates of adverse events could be compared, statistically no significant differences were found between mannitol and control groups; although some of these events may have clinical relevance for people with CF. For the comparisons of mannitol to dornase alfa alone and to mannitol plus dornase alfa, very low-quality evidence from a 12-week cross-over study of 28 participants showed no statistically significant differences in the recorded domains of health-related quality of life or measures of lung function. Cough was the most common side effect in the mannitol alone arm but there was no occurrence of cough in the dornase alfa alone arm and the most commonly reported reason of withdrawal from the mannitol plus dornase alfa arm was pulmonary exacerbations. In terms of secondary outcomes of the review (pulmonary exacerbations, hospitalisations, symptoms, sputum microbiology), evidence provided by the included studies was more limited. For all comparisons, no consistent statistically significant and clinically meaningful differences were observed between mannitol and control treatments (including dornase alfa). AUTHORS' CONCLUSIONS: There is moderate-quality evidence to show that treatment with mannitol over a six-month period is associated with an improvement in some measures of lung function in people with cystic fibrosis compared to control. There is low to very low-quality evidence suggesting no difference in quality of life for participants taking mannitol compared to control. This review provides very low-quality evidence suggesting no difference in lung function or quality of life comparing mannitol to dornase alfa alone and to mannitol plus dornase alfa. The clinical implications from this review suggest that mannitol could be considered as a treatment in cystic fibrosis; but further research is required in order to establish who may benefit most and whether this benefit is sustained in the longer term. Furthermore, studies comparing its efficacy against other (established) mucolytic therapies need to be undertaken before it can be considered for mainstream practice.


Asunto(s)
Fibrosis Quística/tratamiento farmacológico , Desoxirribonucleasa I/administración & dosificación , Manitol/administración & dosificación , Administración por Inhalación , Adulto , Niño , Fibrosis Quística/fisiopatología , Volumen Espiratorio Forzado , Humanos , Manitol/efectos adversos , Depuración Mucociliar , Polvos , Ensayos Clínicos Controlados Aleatorios como Asunto , Proteínas Recombinantes/administración & dosificación , Pruebas de Función Respiratoria , Capacidad Vital
15.
Pediatr Pulmonol ; 55(8): 1974-1983, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32364318

RESUMEN

BACKGROUND: Studies have shown that sleep disorders occur in cystic fibrosis (CF) patients and may be present before daytime clinical manifestations. OBJECTIVES: To evaluate the presence of sleep disorders among children and adolescents with CF, attempting to identify associations with pulmonary function, nutritional status, days in hospital, and days taking antibiotics. METHODS: Individuals with a diagnosis of CF aged between 6 and 18 years were included. Information on sociodemographic, clinical profile, history of hospitalizations, and use of antibiotics in the last year were collected. Spirometry, bioimpedance, and polysomnography were performed. The presence of nocturnal hypoxemia and obstructive sleep apnea syndrome (OSAS) were evaluated and participants divided according to their presence. RESULTS: Thirty-one patients were included. The prevalence of OSAS was 32.3% and nocturnal hypoxemia was 29.0%. Average nocturnal peripheral oxyhemoglobin saturation (SpO2 ) correlated (P < .001) with forced vital capacity (r = .55) and forced expiratory volume in the first second (r = .62). The higher the percentage of total sleep time (TST) with SpO2 less than 90%, the lower the pulmonary function. Individuals with OSAS and nocturnal hypoxemia had lower spirometric values compared to patients without these disorders, but the nocturnal hypoxemia group also had lower Shwachman-Kulczycki score, longer hospitalization time and antibiotic use. TST with SpO2 less than 90% was associated with length of hospitalization (r2 = .53). CONCLUSION: Children and adolescents with CF have sleep disorders, including OSAS (32.3%) and nocturnal hypoxemia (29%). Individuals with nocturnal hypoxemia presented lower lung function, worse clinical score, and higher morbidity. TST with SpO2 less than 90% was associated with length of hospitalization.


Asunto(s)
Fibrosis Quística/fisiopatología , Hipoxia/fisiopatología , Síndromes de la Apnea del Sueño/fisiopatología , Adolescente , Biomarcadores , Niño , Fibrosis Quística/epidemiología , Femenino , Volumen Espiratorio Forzado , Humanos , Hipoxia/epidemiología , Masculino , Morbilidad , Estado Nutricional , Polisomnografía , Prevalencia , Síndromes de la Apnea del Sueño/epidemiología , Trastornos del Sueño-Vigilia , Espirometría , Capacidad Vital
16.
Pediatr Pulmonol ; 55(8): 2108-2114, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32437013

RESUMEN

INTRODUCTION: The multiple breath nitrogen washout (MBW) test offers a sensitive measure of airway function. In this study we aim to (a) assess the validity of the EasyOne Pro LAB (MBWndd ) in an in vitro lung model, (b) assess the feasibility, repeatability, and reproducibility of MBWndd and (c) compare outcomes with the Exhalyzer D (MBWEM ) and body plethysmography. METHODS: In vitro, functional residual capacity (FRC) measurements were assessed using a lung model under quasi-physiological conditions and compared to measured FRC. In vivo plethysmography and MBW were performed in a prospective study of children at two visits (n = 45 healthy; n = 41 cystic fibrosis [CF]). Bland-Altman plots were used to compare agreement between FRC and lung clearance index (LCI) measurements. RESULTS: In vitro FRCndd measurements were repeatable but lung volumes were underestimated (mean relative difference -5.4% (limits of agreement [LA] -9.6%; -1.1%), 95% confidence interval (CI) -6.27; -4.45). In vivo, compared to plethysmography, FRCndd was consistently lower (-19.3% [-40.5; 1.9], 95% CI [-23.9; -14.7]), and showed a volume dependency. LCIndd values were also higher in children with smaller lung volumes. The within-test coefficient of variation of the FRCndd and LCIndd were 4.9% in health, and 5.6% and 6.9% in CF respectively. LCIndd was reproducible between-visits (mean relative difference [LA] -3.7% [-14.8, -7.5; 95% CI -6.6; -0.73] in health [n = 17] and 0.34% [-13.2, 22.8; 95% CI -5.0; 5.69] in CF [n = 23]). When calculated using the same algorithm, LCIndd was similar to LCIEM in health. CONCLUSIONS: MBWndd measurements are feasible, repeatable, and reproducible, however, MBW-derived outcomes are not interchangeable with MBWEM .


Asunto(s)
Pruebas Respiratorias , Fibrosis Quística/fisiopatología , Nitrógeno/análisis , Adolescente , Niño , Femenino , Humanos , Pulmón/fisiopatología , Masculino , Pletismografía Total , Reproducibilidad de los Resultados , Pruebas de Función Respiratoria
17.
Int J Infect Dis ; 96: 663-670, 2020 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-32450290

RESUMEN

OBJECTIVES: To study the prevalence of fungal species in cystic fibrosis (CF) patients over a 16 years period. To examine the impact of Candida albicans (C. albicans), Candida dubliniensis (C. dubliniensis) and Aspergillus fumigatus (A. fumigatus) on lung function. METHODS: Observational single-center cohort study (2000-2015) including 133 CF patients (ages 6-66 years). Linear mixed models with autoregressive covariance matrix were used. RESULTS: The most common fungus was C. albicans (prevalence 62%) followed by A. fumigatus (22%) and C. dubliniensis (11%). In the initial year of detection, there was no impact of C. albicans, C. dubliniensis or A. fumigatus on lung function. However, one and two years after detection of C. dubliniensis a reduction in percent predicted forced expiratory volume in the first second (ppFEV1) was observed of 3.8% (p = 0.022) and 4.1% (p = 0.017), respectively, compared with CF patients without these findings. Furthermore, patients with positive cultures for any of these fungal species for three consecutive years exhibited a decline in lung function: C. dubliniensis, 7.6% reduction in ppFEV1 (p = 0.001); A. fumigatus, 4.9% (p = 0.007); C. albicans, 2.6% (p = 0.014). The results were adjusted for age, CFTR genotype, chronic and intermittent P. aeruginosa colonization, and numbers of intravenous antibiotic treatments per year. Persistence of C. dubliniensis for three consecutive years was positively correlated to age and erythrocyte sedimentation rate (ESR) (both p = 0.001). CONCLUSIONS: Cystic fibrosis patients who were cultured positive for C. dubliniensis, C. albicans or A. fumigatus in sputum exhibited a decline in ppFEV1 over time. The effect was most pronounced for C. dubliniensis.


Asunto(s)
Candida/aislamiento & purificación , Fibrosis Quística/microbiología , Fibrosis Quística/fisiopatología , Pulmón/microbiología , Adolescente , Adulto , Anciano , Biodiversidad , Candida/clasificación , Candida/genética , Candida/fisiología , Niño , Fibrosis Quística/complicaciones , Femenino , Volumen Espiratorio Forzado , Hongos/clasificación , Hongos/genética , Hongos/aislamiento & purificación , Humanos , Pulmón/fisiopatología , Masculino , Persona de Mediana Edad , Prevalencia , Pruebas de Función Respiratoria , Estudios Retrospectivos , Esputo/microbiología , Adulto Joven
18.
Pediatr Pulmonol ; 55(8): 1996-2010, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32453897

RESUMEN

BACKGROUND: Exercise testing is routinely used to measure exercise capacity in children with cystic fibrosis (CF). Various tests are available, however the psychometric properties of these measures have not been systematically reviewed for this population. METHOD: A systematic search of electronic databases (PubMed, Web of Science, Medline, CINHAL, Cochrane, and PEDro) was performed to identify papers that: (a) reported original psychometric data, (b) examined a measure of exercise capacity, (c) examined children with CF aged eight to 18 years; and (d) were published in English after 1950. The level of psychometric evidence was evaluated using the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist. RESULTS: Searches identified 1025 papers. Forty-six papers were included, covering 15 tests: incremental cardiopulmonary exercise test using a cycle ergometer (CPET(cycle)) or treadmill (CPET(treadmill)), 6 minute walk test (6MWT), modified shuttle test (MST), 3-minute step test (3MST), 2 minute walk test (2MWT), Bratteby walk test, intermittent sprint test, speed ramp test, incremental step test, forward-backwards jump test (FBJT), astride jump test (AJT), motor quotient test, Munich fitness test, and Glittre ADL test. CONCLUSION: There is a plethora of exercise tests available with varying psychometric robustness. The CPET, 6MWT, and MST have fair to good psychometric properties, but each with their clinical advantages and limitations. Thus, a Selection Guide was developed to assist clinicians and researchers in selecting the most appropriate exercise test for various situations.


Asunto(s)
Fibrosis Quística/fisiopatología , Prueba de Esfuerzo , Niño , Tolerancia al Ejercicio , Humanos
19.
Pediatr Pulmonol ; 55(8): 1984-1992, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32320537

RESUMEN

INTRODUCTION: High-frequency chest wall oscillation (HFCWO) is a commonly prescribed airway clearance technique (ACT) for patients whose ability to expectorate sputum is compromised. This study aimed to assess the effectiveness of a newly developed mobile ACT device (mHFCWO-The Monarch Airway Clearance System) in patients with cystic fibrosis (CF). A standard nonmobile HFCWO device (sHFCWO) was used as a comparator. METHODOLOGY: This was a randomized, open-label, crossover pilot study. CF patients were treated with each device. Sputum was collected during and after each therapy session, while spirometry tests, Brody score assessment and functional respiratory imaging were performed before and after treatments. RESULTS: Wet weight of sputum collected during and after treatment was similar for mHFCWO and sHFCWO (6.53 ± 8.55 vs 5.80 ± 5.82; P = .777). Interestingly, the mHFCWO treatment led to a significant decrease in specific airway volume (9.55 ± 9.96 vs 8.74 ± 9.70 mL/L; P < .001), while increasing specific airway resistance (0.10 ± 0.16 vs 0.16 ± 0.23 KPA*S; P < .001). These changes were heterogeneously-distributed throughout the lung tissue and were greater in the distal areas, suggesting a shift of mucus. Changes were accompanied by an overall improvement in the Brody index (57.71 ± 16.55 vs 55.20 ± 16.98; P = .001). CONCLUSION: The newly developed mobile device provides airway clearance for CF patients comparable to a nonmobile sHFCWO device, yielding a change in airway geometry and patency by the shift of mucus from the more peripheral regions to the central airways.


Asunto(s)
Oscilación de la Pared Torácica/instrumentación , Fibrosis Quística/terapia , Adulto , Oscilación de la Pared Torácica/métodos , Estudios Cruzados , Fibrosis Quística/fisiopatología , Femenino , Humanos , Pulmón/fisiopatología , Masculino , Moco , Proyectos Piloto , Espirometría , Esputo , Volumen de Ventilación Pulmonar , Adulto Joven
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