Cystic Fibrosis Papers of the Year 2016
This is arguably the most exciting era in the treatment of Cystic Fibrosis (CF) with the emergence of potentially disease modifying therapies. The last year has seen fewer landmark papers, with the consolidation of existing knowledge and advances in the understanding of the patho-physiology and management of CF. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - March 15, 2017 Category: Respiratory Medicine Authors: Iolo Doull Tags: Review Source Type: research
Disease-modifying drug therapy in CF
This article reviews progress and highlights some of the current and future challenges in CFTR modulator therapies. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - March 15, 2017 Category: Respiratory Medicine Authors: Katharine Harman, Rebecca Dobra, Jane C. Davies Tags: Review Source Type: research
The impact of co-morbidity in childhood Cystic Fibrosis
A number of risk factors have been identified for deterioration of lung disease in children with Cystic Fibrosis (CF), and current management strategies are based on the prevention and treatment of such elements. Further challenge ensues when a patient has co-morbid disease in addition to CF, particularly when faced with rapidly deteriorating pulmonary status. It is difficult to measure the contribution of other pathologies to this decline and optimisation of both CF care and co-morbidity is paramount. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - March 15, 2017 Category: Respiratory Medicine Authors: Julie A. Duncan, Sarah M.N. Brown Tags: Review Source Type: research
Highlights from the 30th North American Cystic Fibrosis Conference, Orlando 2016
This is a selection of papers presented at the 30th North American Cystic Fibrosis Conference held in Orlando in October 2016. The papers discussed are thought to be of particular interest to CF caregivers in the UK. We highlight the major themes covered in the conference including novel therapies, recently published and proposed guidelines and insights from registry studies. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - March 15, 2017 Category: Respiratory Medicine Authors: Anna Shawcross, Peter J. Barry Tags: Review Source Type: research
Cystic fibrosis in the era of precision medicine
The treatment of people with cystic fibrosis (CF) has been transformed by the availability of drugs that target the basic chloride defect in the disease. The use of drugs that target specific molecular defects embodies the goals of precision medicine, which incorporate preventive and therapeutic strategies and takes into account differences among individuals. However, the entirety of CF care, from diagnosis to understanding the clinical phenotype and developing a therapeutic strategy depends on taking into account individual characteristics to achieve optimal outcomes. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - March 8, 2017 Category: Respiratory Medicine Authors: Shruti M. Paranjape, Peter J. Mogayzel Tags: Review Source Type: research
Lies, damned lies and statistics: Clinical importance versus statistical significance in research
(Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - February 27, 2017 Category: Respiratory Medicine Authors: Craig Mellis Tags: Mini-Symposium: Interpreting Clinical Research Source Type: research
Obstructive sleep apnoea and the role of tongue reduction surgery in children with Beckwith-Wiedemann syndrome
Beckwith-Wiedemann syndrome (BWS) is a rare paediatric overgrowth disorder. Associated macroglossia is a feature of many children with BWS and is felt to be a risk factor for obstructive sleep apnoea (OSA). Sleep-disordered breathing is highly variable in this population. The relationship between degree of macroglossia or other genotypic or phenotypic factors and OSA severity has not been established. The natural history of OSA in this population is unknown; a variety of conservative and surgical therapies have been used to treat OSA in children with BWS but none have been studied systematically. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - February 22, 2017 Category: Respiratory Medicine Authors: Christopher M. Cielo, Kelly A. Duffy, Aesha Vyas, Jesse A. Taylor, Jennifer M. Kalish Tags: Review Source Type: research
Indications for lung resection surgery and lung transplant in South American children with cystic fibrosis
The current available literature evaluating lung resection surgery and lung transplantation in children with cystic fibrosis (CF) was reviewed through a PubMed search and references from selected studies were additionally included. Pulmonary resections, i.e. lobectomy, segmentectomy, and pneumonectomy, are seldom performed in CF. The main indications, in patients with a forced expiratory volume in 1second (FEV1) that is greater than 30% predicted, are localized bronchiectasis/atelectasis, severe hemoptysis, and bronchopleural fistula refractory to medical management. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - February 13, 2017 Category: Respiratory Medicine Authors: Fab íola Villac Adde, Sílvia Vidal Campos, Ricardo Henrique de Oliveira Braga Teixeira, Joaquim Carlos Rodrigues Tags: Review Source Type: research
Systematic review of instruments aimed at evaluating the severity of bronchiolitis
The objective of the present study was to perform a systematic review of instruments aimed at evaluating the severity of bronchiolitis and to evaluate their measurement properties. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - February 13, 2017 Category: Respiratory Medicine Authors: Carlos E. Rodr íguez-Martínez, Monica P. Sossa-Briceño, Gustavo Nino Tags: Review Source Type: research
Can we trust what parents tell us? A systematic review
(Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - February 7, 2017 Category: Respiratory Medicine Authors: Paul L.P. Brand, Sandra van Dulmen Tags: Review Source Type: research
Daytime sleepiness in children: When a quiet child is not necessarily a good thing
Excessive daytime sleepiness (EDS) in children is a common but under-recognized symptom. A quiet child is a “good” child who does not cause problems and may be ignored by a busy teacher in an overcrowded classroom, or a harried parent juggling household and work responsibilities. EDS is common; in a community-based sample of school-aged children, the prevalence of parent or teacher-reported EDS was es timated at 15% [1]. However, few of these children will come to medical attention, and it thus behooves the pediatrician to ask questions regarding sleep during regular health maintenance visits. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - January 18, 2017 Category: Respiratory Medicine Authors: Carole L. Marcus Tags: Mini-Symposium: Pediatric Hypersomnolence Symposium Source Type: research
Mini-symposium Daytime sleepiness in children: When a quiet child is not necessarily a good thing
(Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - January 18, 2017 Category: Respiratory Medicine Authors: Carole L. Marcus Tags: Editorial Source Type: research
Immune and inflammatory response in bronchiolitis due to respiratory syncytial virus and rhinovirus infections in infants
Bronchiolitis is a common disease in infancy, mostly due to Respiratory Syncytial Virus and Rhinovirus. In addition to acute infection, viral bronchiolitis is responsible for sequelae including recurrent wheezing and asthma. The analysis of the viral characteristics and of the pathogenesis of the infection shows differences between the two viruses that may be helpful for the development of therapies and preventive strategies. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - January 13, 2017 Category: Respiratory Medicine Authors: Silvia Vandini, Elisabetta Calamelli, Giacomo Faldella, Marcello Lanari Tags: Review Source Type: research
Obstructive Sleep Apnea and Pulmonary Hypertension in Children
Obstructive sleep apnea (OSA) is a common pediatric breathing disorder, affecting 1-5% of all children. Pulmonary hypertension (PH), a severe complication of OSA, is associated with significant morbidity and mortality. Despite this important relationship between OSA and PH, there is sparse literature addressing this subject in children. This review will examine the putative relationship between OSA and PH, synthesize the available literature in children, and suggest a reasonable approach, despite limited data, for clinicians. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - January 12, 2017 Category: Respiratory Medicine Authors: David G. Ingram, Alvin V. Singh, Zarmina Ehsan, Brian F. Birnbaum Tags: Mini-Symposium: Pulmonary Hypertension Source Type: research
Stem cells and Bronchopulmonary Dysplasia - The five questions: Which cells, when, in which dose, to which patients via which route?
Preterm birth is the leading cause of death in newborns and children. Despite advances in perinatology, immature infants continue to face serious risks such chronic respiratory impairment from bronchopulmonary dysplasia (BPD). Current treatment options are insufficient and novel approaches are desperately needed. In recent years stem cells have emerged as potential candidates to treat BPD with mesenchymal stem/stromal cells (MSCs) being particularly promising. MSCs originate from several stem cell niches including bone marrow, skin, or adipose, umbilical cord, and placental tissues. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - December 23, 2016 Category: Respiratory Medicine Authors: Martin Mueller, Boris W. Kramer Source Type: research
