When is Actigraphy Useful for the Diagnosis and Treatment of Sleep Problems?
Actigraphy is an ambulatory study that captures sleep-wake patterns for up to two weeks in the patient ’s natural sleep environment.[1] An actigraph is a wrist-watch sized device that measures movement with an internal accelerometer, and works under the premise that movements are frequent and large when we are awake, but absent or small when we are asleep. What separates actigraphs from consumer we arable devices are the validated algorithms, developed through side-by-side comparisons with polysomnography, the gold standard for sleep assessment. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - July 17, 2018 Category: Respiratory Medicine Authors: Lisa J. Meltzer Source Type: research

Sleep Disordered Breathing in Duchenne Muscular Dystrophy
Symptoms of sleep disordered breathing (SDB) in younger boys with DMD are often poorly perceived and/or articulated by the patients or their families. As a result it is the watchful eye of the care-provider that determines the need for early polysomnographic (PSG) assessments. The use of polysomnography without capnometry should be considered completely inadequate when it comes to diagnosis and management of SDB in these patients. The stabilization of gas exchange with non-invasive ventilation may be achieved by the use of pressure or volume support ventilation. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - July 17, 2018 Category: Respiratory Medicine Authors: Hemant Sawnani Tags: Mini-symposium Source Type: research

Spinal Muscular Atrophy: A modifiable disease emerges
Respiratory muscle failure and death in infancy or early childhood were the hallmarks of the heterogeneous neuromuscular disorder Spinal Muscular Atrophy [SMA] in the absence of intervention with mechanical ventilation and subsequently with the portable and more practical non-invasive respiratory support [NIV] [1]. Many paediatric respiratory centres around the world favoured supportive care without progression to invasive ventilation in the absence of effective therapies for SMA. However, others argued for the use of invasive ventilation for children regardless of the availability of effective treatments [2]. (Source: Pae...
Source: Paediatric Respiratory Reviews - July 12, 2018 Category: Respiratory Medicine Authors: Dominic A. Fitzgerald, Francois Abel, Kristi J. Jones, Michelle A. Farrar Tags: Editorial Source Type: research

Changing respiratory expectations with the new disease trajectory of nusinersen treated spinal muscular atrophy [SMA] type 1
Spinal muscular atrophy [SMA] is the most common genetic cause of childhood mortality, primarily from the most severe form SMA type 1. It is a severe, progressive motor neurone disease, affecting the lower brainstem nuclei and the spinal cord. There is a graded level of severity with SMA children from a practical viewpoint described as “Non-sitters”, “Sitters” and less commonly, “Ambulant” correlating with SMA Type 0/Type 1, Type 2 and Type 3 respectively. Children with SMA Type 0 have a severe neonatal form whilst those with SMA Type 1 develop hypoventilation, pulmonary aspiration, recurrent lower respiratory ...
Source: Paediatric Respiratory Reviews - July 12, 2018 Category: Respiratory Medicine Authors: Dominic A. Fitzgerald, Michael Doumit, Francois Abel Tags: Mini-Symposium: Spinal Muscular Atrophy Source Type: research

A systematic cochrane review of autogenic drainage (Ad) for airway clearance in cystic fibrosis
Cystic fibrosis (CF) is an autosomal recessive genetic condition affecting the gene that codes for the cystic fibrosis transmembrane conductance regulator (CFTR). This in turn interferes with the movement of chloride across the apical membrane of respiratory epithelial cells. Abnormal chloride transport affects the production of airway surface liquid, which disturbs the ability of the cilia to clear the airways[1]. This important physiological process (the mucociliary escalator) protects the airways and disruption of this process makes the airways vulnerable to the infections characterising CF lung disease. (Source: Paedia...
Source: Paediatric Respiratory Reviews - July 6, 2018 Category: Respiratory Medicine Authors: P. McCormack, P. Burnham, K.W. Southern Tags: Editorial Source Type: research

Royal Society of Medicine Cystic Fibrosis Minisymposium Urinary Tract Stones in Cystic Fibrosis
Urinary tract stones are a common problem in a general population but increasingly so in cystic fibrosis (CF) patients as survival improves. Mechanisms of stone formation are discussed, particularly those unique to CF patients. Modalities of treatment and the decision making process in this choice is outlined as well as possible future preventative strategies. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 16, 2018 Category: Respiratory Medicine Authors: J. Graham Young Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

Pertussis: New preventive strategies for an old disease
In the last twenty years, despite high vaccination coverage, epidemics of pertussis are occurring in both developing and developed countries. Many reasons could explain the pertussis resurgence: the increasing awareness of the disease, the availability of new diagnostic tests with higher sensitivity, the emergence of new Bordetella pertussis (B. pertussis) strains different from those contained in the current vaccines, the asymptomatic transmission of B. pertussis in adolescents and adults and the shorter duration of protection given by the acellular pertussis (aP) vaccine. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: Greta Di Mattia, Ambra Nicolai, Antonella Frassanito, Laura Petrarca, Raffaella Nenna, Fabio Midulla Tags: Review Source Type: research

Disease caused by non-tuberculous mycobacteria in children with cystic fibrosis
Non-tuberculous mycobacterial (NTM) (especially M. abscessus complex) infections pose a considerable challenge in the management of lung disease in patients with cystic fibrosis (CF). The apparent increase in prevalence is likely multifactorial. Emergent evidence of patient-to-patient transmission and isolation of highly resistant strains is concerning for all CF centers around the world. Treatment is often long and burdensome with multiple agents. Treatment side effects are frequent and can cause significant morbidity. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: Mimi Lu, Vishal Saddi, Philip N. Britton, Hiran Selvadurai, Paul Robinson, Chetan Pandit, Ben J. Marais, Dominic A. Fitzgerald Source Type: research

Paediatric sarcoidosis
Sarcoidosis, also called Besnier-Boeck-Schaumann disease, was first described in the 19th century as a systemic granulomatous disorder involving predominantly the lungs and the lymphatic system [1]. More than a century after, its pathogenesis remains unknown and its pathophysiology remains controversial [2,3]. The current hypothesis is a multifactorial disease associating a genetic susceptibility and an environmental exposure that triggers or enhances the inflammatory and granulomatous process. Sarcoidosis is mainly an adult disease, children being affected around 10 times less frequently. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: Nadia Nathan, Chiara Sileo, Alain Calender, Yves Pacheco, Dominique Valeyre, Annick Clement, for the French Sarcoidosis Group (GSF), the Silicosis Group Tags: Review Source Type: research

The use of lumacaftor/ivacaftor to treat acute deterioration in paediatric cystic fibrosis
We describe the use of this drug to treat a child with an unusually severe exacerbation of CF lung disease and review the potential of lumacaftor/ivacaftor as a rescue therapy in the paediatric CF population. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: James A. Hammond, Gary J. Connett Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

Tracheal Bronchus Diagnosed In Children Undergoing Flexible Bronchoscopy
This paper describes the clinical features of paediatric patients with tracheal bronchus (TB) identified with flexible bronchoscopy (FB) in a tertiary care hospital.A retrospective review every FB with diagnosis of TB carried out in our centre since 1990 was performed which considered specifically: age at diagnosis, gender, semiology, somatic anomalies, tracheal bronchus type, other bronchoscopic findings and clinical progress. Out of 1665 FB in 1337 patients, TB was found in 26 (1.9%). The median age was 15 months (age range 1 month- 13 years), with no gender differences. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: Estela P érez Ruiz, Pilar Caro Aguilera, Ana Isabel Valdivielso Martínez, Sonia Sanchís Cárdenas, Yazmina Martínez García, Javier Pérez Frías Tags: Clinical usefulness Source Type: research

Age-related ranges of respiratory inductance plethysmography (RIP) reference values for infants and children
The current noninvasive method for respiratory monitoring is respiratory inductance plethysmography (RIP); two bands are connected, one each to the chest and the abdomen, to measure the breathing pattern. RIP requires post hoc analysis to calculate indices such as respiratory rate, phase angle, labored breathing index, and percent of rib cage contribution to breathing. Clinical studies have provided patient RIP values and age-matched normal values, but they lack global evaluation of normative data for a wide age range of pediatric subjects. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: Sona Lakshme Balasubramaniam, Yanping Wang, Lauren Ryan, Jobayer Hossain, Tariq Rahman, Thomas H. Shaffer Tags: Review Source Type: research

Pertussis: new preventive strategies for an old disease.
Authors have no conflict of interest (COI) to declare. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 18, 2018 Category: Respiratory Medicine Authors: Greta Di Mattia, Ambra Nicolai, Antonella Frassanito, Laura Petrarca, Raffaella Nenna, Fabio Midulla Tags: Review Source Type: research

Question 2: What are the effects of inhaled corticosteroids on growth in children?
This review summarizes the current evidence regarding the effects of inhaled corticosteroids (ICS) on growth in children with asthma. The evidence from randomized trials showed a mean reduction of −0.48 cm/year (95% CI −0.65 to −0.30) in linear growth velocity and of −0.61 cm (95% CI −0.83 to −0.38) in height during a one-year treatment with ICS. Some first-generation drugs had a slightly larger suppressive effect on growth than newer drugs, with a mean reduction in linear gro wth velocity of −0.91, −0.59, −0.08 and −0.39 cm/year for beclomethasone, budesonide, ciclesonide and fluticasone, respect...
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Paulo Camargos, Linjie Zhang, Laura Lasmar, Paul Brand Tags: Asthma Frequently Asked Questions Source Type: research

Royal Society of Medicine Cystic Fibrosis Symposium 2017
The articles in this supplement arise from presentations given at the Royal Society of Medicine Cystic Fibrosis (CF) Symposium held in London in November 2017. This highly successful and popular meeting, organised by Dr Iolo Doull, is designed to provide updates in key areas of CF clinical care for members of CF multidisciplinary teams. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Andrew M. Jones Tags: Editorial Source Type: research