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Introduction: Our hospital has recently launched a dedicated inpatient setup bed (ISUB) with staff available to train and support out of our hours in order to admit children for management of their non-invasive ventilation (NIV). The patients are admitted for up to 1 week enabling intensive support and ability to adjust management swiftly in response to observations. Aims: 1. To identify patient and parents perceptions of ISUB 2. To identify any trends in patients utilising the ISUB. Methods: We conducted a prospective patient/parent survey as part of a service evaluation, gathering feedback on patient experiences of using ISUB to commence or adjust NIV. Patients were given the survey following their inpatient stay. Results of progress at discharge and 3 months were recorded, along with any comments from families. Results: The survey was given to 10 patients, with 4 responses. 2 of these patients (50%) were previously established and required monitoring during titration. 4 patients (100%) left fully established (over 4 hours use) on nocturnal NIV. 3 patients (75%) found the overall experience either good or extremely good (Figure 1). Conclusions: This preliminary data supports the use of a dedicated NIV setup bed for selected patients in order to establish optimal NIV. We were unable to identify any trends in usage/success due to the small numbers, however we hope to gather further data allowing this analysis in the future.

Background The mechanisms regulating antibody expression within the human lung during airway infection are largely unknown. In this study, our objectives were to determine if infection with respiratory syncytial virus (RSV) upregulates expression of the B cell differentiation factors A proliferation inducing ligand (APRIL) and B cell activating factor of the TNF family (BAFF), if this is a common feature of viral airway infection, and how this is regulated in human airway epithelial cells. Methods We measured BAFF and APRIL protein expression in bronchoalveolar lavage (BAL) fluid from infants with severe RSV disease, and healthy control children, and in nasopharyngeal aspirates from preschool children with other single respiratory viral infections. We also measured mRNA expression in bronchial brushings from RSV-infected infants, and in RSV-infected paediatric primary airway epithelial cell cultures (pAEC). Beas-2B cell cultures were used to examine mechanisms regulating BAFF expression. Results BAFF protein and mRNA were elevated (in marked contrast with APRIL) in BAL and bronchial brushings, respectively, from RSV-infected infants. BAFF protein was also found in upper airway secretions from children with human metapneumovirus, H1N1, bocavirus, rhinovirus, RSV and Mycoplasma pneumoniae infection. BAFF mRNA and protein were expressed following in vitro RSV infection of both pAEC and Beas-2B cultures, with mRNA expression peaking 12-h postinfection. BAFF induction was blocked by addition of a neutralising anti-interferon-β antibody or palivizumab. Conclusions BAFF, produced through an interferon-β-dependant process, is a consistent feature of airway infection, and suggests a role for the airway epithelia in supporting protective antibody and B cell responses in the lung.

Monitoring respiratory status in cystic fibrosis (CF) is challenging, particularly in young children. We aimed to test whether the Liverpool Respiratory Symptom Questionnaire (LRSQ) could distinguish well, pre-school and older children with and without CF, whether it could distinguish well and unwell children with CF and, finally, whether LRSQ scores in older children with CF correlated with established measures of disease severity. 20 stable pre-school children with CF had significantly higher total LRSQ scores than 51 pre-school controls, and higher scores in two out of eight domains. Similarly, 21 stable 6- to 12-yr-old children with CF had higher total scores than 97 6- to 12-yr-old controls, and higher scores in seven out of eight domains. In older children with CF, LRSQ scores correlated negatively with Shwachman score and forced expiratory volume in 1 s (r = -0.58, p < 0.001, n = 31; and r = -0.46, p < 0.010, n = 34, respectively). Within the CF group, patients who cultured Pseudomonas aeruginosa, who used more "back-up" antibiotics or whose school attendance was lower also had higher LRSQ scores. The LRSQ differentiates well children from those with CF in both pre-school and the 6- to 12-yr-old age group, even at a point of stability. It also differentiates stable from unwell children with CF, and scores correlate with other measures of respiratory disease, highlighting its potential as a clinical monitoring tool in paediatric CF.

We describe three cousins who presented with agitation, dysphasia and/or coma, and developed hemiplegia following initial onset of symptoms. Two cases followed minor head injuries, two were pyrexial and two were associated with neutrophilia. Two cases required ventilatory support on the intensive care unit. Magnetic resonance imaging in all three cases showed cortical swelling, and one had evidence of restricted water diffusion on diffusion-weighted imaging, suggestive of ischaemia/infarction. A complete family history at the time of presentation would have led to an earlier diagnosis of profound encephalopathy in familial hemiplegic migraine, which would have enabled better prognostication of their clinical course and caused less distress for the families.

Recurrent respiratory problems are common in children with severe neurodisability, and respiratory deterioration is a leading cause of premature death in this group. Although the etiology is multifactorial, recurrent pulmonary aspiration is thought to play a significant role. Gastroesophageal reflux is known to be common, as is oral-motor discoordination. Differentiating direct aspiration of food and saliva and gastric reflux aspiration is difficult and presents a challenge in managing patients and assessing their suitability for surgical antireflux procedures. This is particularly the case when children present with predominantly respiratory symptoms, where there may be direct aspiration, reflux aspiration, neither, or both. A clinical biomarker to identify and quantify reflux aspiration would therefore be useful in surgical assessment and may also be applicable as an outcome measure for clinical trials of antireflux surgery. In this review, we discuss the evidence base behind existing and potentially novel biomarkers of aspiration in bronchoalveolar lavage fluid. We highlight the limitations of the lipid-laden macrophage index, particularly with regard to its specificity and interrater/intrarater reliability. We discuss the laboratory methods available to measure promising new biomarkers (pepsin and bile acids) and highlight their potential advantages and disadvantages. Finally, to understand how aspiration causes clinical signs and symptoms in our patients, we need to study the effect of aspirated substances on the lung, and here we review the available in vivo and in vitro literature.

BackgroundChildren with severe neurodisability (ND) commonly suffer from chronic respiratory symptoms that impact greatly on quality of life, and lead to recurrent hospital admissions. This morbidity (and its causes) is poorly described, despite being well recognised by paediatricians. In this study, we characterised respiratory symptoms in children with ND at times of stability and deterioration. We also assessed the relationship between respiratory symptoms, lower airway inflammatory markers and levels of infection/colonisation.MethodsND children were recruited upon admission for elective surgery (Elective-ND [n = 16]), or acutely upon admission to Intensive Care (PICU-ND [n = 19]), and compared to healthy control children [n = 12]. Parents completed a validated respiratory symptom questionnaire in which symptoms associated with activity were removed (total maximal score of 108). Bronchoalveolar lavage (BAL) was collected, and BAL neutrophil counts, IL-8 and TGFβ-1 levels measured. BAL microbial analysis was performed using a 16S/18S rRNA gene based assay and Pseudomonas aeruginosa PCR.ResultsAll ND children had high levels of respiratory symptoms (median [IQR] symptom score PICU-ND, 55[38-64]; Elective-ND, 26[7-45]; Control, 4[0-7]: p20 invariably had BAL neutrophilia. Elective patients with 16S/18S microbial rDNA positive BAL had higher neutrophil counts (positive, 33[18-70]%; negative, 8[4-38]%: pConclusionsChildren with severe ND often have high levels of chronic respiratory symptoms, which may relate to lower airway inflammation. Bacterial airway colonisation, particularly with oral commensals, may play a role in both symptom generation and inflammation.