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Acute exacerbations of Cystic Fibrosis (AECF) are associated with significant morbidity. Recommendations are to treat for 2-3 weeks despite limited data. Spirometry is a measure of clinical response yet appears to plateau at 7-10 days. While durations9 days have been associated with poorer outcomes, a duration of 10 days may be as effective as 14 days, potentially conferring advantages in terms of cost and adverse events. A 2019 Cochrane review by Abbott et al. did not identify any randomised controlled trials (RCT) comparing durations of treatment. Utilising data from non-randomised studies (NRS), we report a systematic review of intravenous antibiotic treatment, exploring changes in FEVSystematic review of published literature following a search of MEDLINE, Embase, CINAHL and the Cochrane Clinical Trials register. Guidelines from the Preferred Reporting items for Systematic reviews and Meta-Analysis (PRISMA) and reporting Meta-analysis of Observational studies (MOOSE) statement were followed.No randomised controlled trials were identified that specifically examined duration of treatment during AECF. This study included all relevant RCTs and also NRS, grouping according to study characteristics, such as length of treatment, location, year, and also characteristics of the patient population. 52 studies, comprising 79 subgroups, and 1,597 patients, were identified. Mean change (95%CI) in ppFEVThis systematic review provides evidence that shorter durations of treatment may be associated with similar changes in FEV

Background and Aims: Infants born to mothers with gestational diabetes mellitus (GDM) have impaired myocardial performance and are at risk of pulmonary hypertension. We aimed to assess myocardial deformation and left ventricular (LV) rotational mechanics in this population. Methods: We studied 40 infants of mothers with GDM and 40 control infants. Three echocardiograms were carried out over the first 3 days after birth. Results: GDM infants had a lower gestation at birth and a thicker septal wall, a higher LV eccentricity index (indicating septal bowing), and a lower PAATi (indicating higher pulmonary vascular resistance) (all p < 0.05). GDM infants had lower LV strain, systolic and early diastolic strain rates, lower right ventricular (RV) strain, and early diastolic strain rates over the study period (all p < 0.05). By day 3, GDM infants had higher twist, torsion, and higher LV twist and untwist rates (all p < 0.05). GDM status was an independent predictor of LV and RV function and pulmonary vascular resistance (p < 0.01). Conclusion: Infants of mothers with GDM demonstrate important changes in myocardial function in addition to pulmonary vascular resistance that do not resolve by hospital discharge. The observed LV twist increase in GDM infants may be a compensatory mechanism for the lower longitudinal function in this cohort.

Down Syndrome (DS) is the most common chromosomal abnormality of live born babies. Individuals with DS are at increased risk of cardiopulmonary morbidities in the early neonatal period, infancy and childhood that manifest with elevated pulmonary arterial pressures and altered myocardial performance. Pulmonary hypertension (PH) during the early neonatal period remains under-recognised in this population. PH may occur with or without a congenital heart defect in children with DS and is more common than in the general population. Early detection and continued screening of PH throughout infancy and childhood for these at-risk children is crucial for prompt intervention and potential prevention of long-term sequelae on cardiac function. This review summarises the main physiological concepts behind the mechanisms of PH in children with DS and provides a summary of the current available literature on PH and its impact on myocardial performance.

ObjectiveLeft ventricle (LV) rotational mechanics is an emerging tool to characterise LV function, but warrants further evaluation in neonates. The aim of this study was to compare LV rotational mechanics between term and extremely preterm babies over the first week of age.MethodsIn this prospective study, we serially assessed LV rotational parameters in 50 term infants and compared them with a historical dataset of 50 preterm infants born ResultsThere was no change in LV twist, LV torsion, basal rotation or apical rotation in term infants over the study period (all p>0.05). LV twist and torsion were higher in preterm infants, and increased over time. In preterm infants, basal rotation evolved from anticlockwise to clockwise rotation. Apical rotation remained anticlockwise in both groups (all p>0.05). LV twist rate (LVTR) and untwist rate was higher in preterm infants and increased over the three time points (all p>0.05). There was a strong positive correlation between LV torsion and LV untwist rate (LVUTR) in the entire cohort during the third scan.ConclusionTerm infants exhibit minimal LV twist which remains unchanged over the first week of age. This is in contrast to premature infants who demonstrate increasing indices of twist, torsion, LVTR and LVUTR over the first week, likely as a compensatory mechanism for reduced LV compliance.

IntroductionAlthough chronic pulmonary hypertension (cPH) secondary to chronic neonatal lung disease is associated with increased mortality and respiratory and neurodevelopmental morbidities, late diagnosis (typically ≥36 weeks postmenstrual age, PMA) and the use of qualitative echocardiographic diagnostic criterion (flat interventricular septum in systole) remain significant limitations in clinical care. Our objective in this study is to evaluate the utility of relevant quantitative echocardiographic indices to identify cPH in preterm neonates, early in postnatal course and to develop a diagnostic test based on the best combination of markers.Methods and analysisIn this ongoing international prospective multicentre observational diagnostic accuracy study, we aim to recruit 350 neonates born Ethics and disseminationEthics approval has been received by the Mount Sinai Hospital Research Ethics Board (REB) (#16-0111-E), Sunnybrook Health Sciences Centre REB (#228-2016), NHS Health Research Authority (IRAS 266498), University of Iowa Human Subjects Office/Institutional Review Board (201903736), Rotunda Hospital Research and Ethics Committee (REC-2019-008), and UBC Children’s and Women’s REB (H19-02738), and is under review at Boston Children’s Hospital Institutional Review Board. Study results will be disseminated to participating families in lay format, presented to the scientific community at paediatric and critical care conferences and published in relevant peer-reviewed journals.Trail registration numberNCT04402645.

A patent ductus arteriosus (PDA) in preterm infants is associated with increased ventilator dependence and chronic lung disease, necrotizing enterocolitis, intraventricular haemorrhage, and poor neurodevelopmental outcome. Randomised controlled trials of early PDA treatment have not established a drop in the aforementioned morbidities. Those trials did not physiologically categorise PDA severity. Incorporating the specific physiological features of a haemodynamic significant PDA may evolve our understanding of this phenomenon, allowing accurate triaging using echocardiography and targeted treatment. Our group has recently demonstrated that a PDA severity score (PDAsc) derived at 36-48 hours of age can accurately predict the later occurrence of chronic lung disease or death (CLD/Death). Using echocardiography, we assessed PDA characteristics, as well as left ventricular diastolic function and markers of pulmonary overcirculation, and from this formulated a PDAsc. Gestation was also incorporated into the score. We hypothesise that in preterm infants at high risk of developing CLD/Death based on a PDAsc, early treatment with Ibuprofen compared with placebo will result in a reduction in CLD/Death. This is a single centre double-blind two arm randomised controlled trial conducted in the neonatal intensive care unit in the Rotunda Hospital, Dublin. Echocardiogram is carried out in the first 36-48 hours of life to identify preterm infants with a PDAsc ≥ 5.0 and these infants are randomised to Ibuprofen or placebo. Primary outcomes are assessed at 36 weeks post menstrual age. This pilot study’s purpose is to assess the feasibility of performing the trial and to obtain preliminary data to calculate a sample size for a definitive multi-centre trial of early PDA treatment using a PDAsc. We aim to recruit a total of 60 infants with a high risk PDA over three years. Trial Registration: ISRCTN ISRCTN13281214 (26/07/2016) and the European Union Drug Regulating Authorities Clinical Trials Database 2015-004526-33 (03/12/2015).

Background: Down’s syndrome (DS) is the most common chromosomal abnormality globally. Ireland has one of the highest rates of DS in the western world with an incidence of 1:444 live births. Congenital heart disease (CHD) and pulmonary hypertension (PH) are the commonest morbidities affecting the cardiovascular system in DS. PH is associated with significant morbidity and an increase risk of mortality. The impact of the diagnosis of DS, the presence of CHD and the associated PH on myocardial function during transition and over the first 2 years of age in this population is not well defined and warrants further study. In particular, serial measurements of pulmonary pressures in this population over the first week of age are lacking. This study aims to characterise myocardial function and pulmonary haemodynamics in infants with Down syndrome during the transitional period (over the first week of age) and throughout the first two years of age. Methods: A prospective, observational study utilising novel echocardiography techniques to assess myocardial function and pulmonary haemodynamics over the first two years of age in infants with Down Syndrome. A population of healthy infants without CHD or a diagnosis of DS will be recruited as controls. This study will be conducted across the three Dublin maternity units. Discussion: In total, 70 babies with DS have been enrolled into this study with 292 echocardiograms performed to date. Further evaluation of cardiac performance in DS infants with and without CHD may yield more insight into the pathophysiology of cardiac dysfunction and pulmonary hypertension that are recognised features in these patients. This could aid in our ability to monitor and treat patients, as well as improve our ability to predict outcomes.

Objective To evaluate the incidence of direct admission of infants with Down syndrome to the postnatal ward (well newborn nursery) vs the neonatal intensive care unit (NICU), and to describe the incidence of congenital heart disease (CHD) and pulmonary hypertension (PH). Study design This retrospective cohort study of Down syndrome used the maternal/infant database (2011-2016) at the Rotunda Hospital in Dublin, Ireland. Admission location, early neonatal morbidities, outcomes, and duration of stay were evaluated and regression analyses were conducted to identify risk factors associated with morbidity and mortality. Results Of the 121 infants with Down syndrome, 54 (45%) were initially admitted to the postnatal ward, but 38 (70%) were later admitted to the NICU. Low oxygen saturation profile was the most common cause for the initial and subsequent admission to the NICU. Sixty-six percent of the infants (80/121) had CHD, 34% (41/121) had PH, and 6% died. Risk factors independently associated with primary NICU admission included antenatal diagnosis of Down syndrome, presence of CHD, PH, and the need for ventilation. Conclusions Infants with Down syndrome initially admitted to the postnatal ward have a high likelihood of requiring NICU admission. Overall, high rates of neonatal morbidity were noted, including rates of PH that were higher than previously reported. Proper screening of all infants with Down syndrome for CHD and PH is recommended to facilitate timely diagnoses and potentially shorten the duration of the hospital stay.

Objectives The aim of this study was to quantify the rate of incidental findings identified on elective research echocardiography performed on neonates younger than 29 weeks’ gestation. Methods We conducted a retrospective study of echocardiographic examinations performed within the first 24 hours of age on neonates younger than 29 weeks’ gestation over a 3-year period for research purposes. Incidental echocardiographic findings and pertinent clinical data were recorded. Results Echocardiographic examinations performed on 145 neonates were retrospectively reviewed. Forty-three neonates (30%) had a total of 54 unexpected findings (37%). Most comprised malpositioned umbilical venous catheters, where the tip was located in the left atrium. The remainder of the conditions identified included unsuspected congenital heart disease, liver hematomas, and unexpected pulmonary hypertension. Conclusions There is a high rate of incidental findings identified on screening echocardiograms. Routine targeted neonatal echocardiographic screening of preterm neonates may be warranted to identify the considerable likelihood of asymptomatic findings.

BACKGROUND Subjective assessment of right ventricular (RV) function by neonatal echocardiography lacks validation. Incorrect diagnostic assignment in patients with suspected pulmonary hypertension (PH) may lead to unnecessary treatment or missed treatment opportunities. METHODS Six evaluators (experts [n = 3], novice [n = 3]) were asked to independently rate RV characteristics (global function, dilation, and septal flattening) based on standardized echocardiography images. We randomly selected 60 infants, ≥35 weeks gestation at birth, of whom 30 were clinically unwell with acute pulmonary hypertension (aPH) and 30 were healthy controls. aPH was defined by echocardiography presence of right-left shunting across transitional shunts or elevated right ventricular systolic pressure as estimated by the magnitude of the regurgitant jet across the tricuspid valve with impaired oxygenation. Inter-rater comparative evaluation within groups and between groups was performed using Kappa statistics. RESULTS Global agreement between evaluators for subjective assessment of RV function (0.3 [0.03], P

The objective of this study is to test whether myocardial performance is impaired over the first week of age in infants with Down syndrome (DS) without congenital heart disease (CHD). A prospective cohort study of 20 infants with DS without CHD and 17 healthy term infants comparing echocardiographic measures of left (LV) and right (RV) ventricular function and pulmonary hypertension (PH) on days 1, 2, and 5–7. Indices of PH were higher in the DS group over the study period. Infants with DS had larger RV and smaller LV dimensions. Fractional area change and RV longitudinal strain values were lower in the DS group. LV shear strain values were lower in infants with DS driven by a lack of basal rotation. Infants with DS without CHD and echocardiographic evidence of PH during the early neonatal period demonstrate reduced RV systolic function with impaired LV rotational mechanics, reflective of the ventricular interdependence.

Persistent pulmonary hypertension of the newborn (PPHN) is a relatively common condition which results in a mortality of up to 33%. Up to 40% of infants treated with nitric oxide (iNO) either have a transient response or fail to demonstrate an improvement in oxygenation. Milrinone, a selective phosphodiesterase 3 (PDE3) inhibitor with inotropic and lusitropic properties may have potential benefit in PPHN. This pilot study was developed to assess the impact of milrinone administration on time spent on iNO in infants with PPHN. This is a multicentre, randomized, double-blind, two arm pilot study, with a balanced (1:1) allocation of 20 infants. In this pilot study, we hypothesise that infants ≥34 weeks gestation and ≥ 2000 g with a clinical and echocardiography diagnosis of PPHN, intravenous milrinone used in conjunction with iNO will result in a reduction in the time spent on iNO. In addition, we hypothesise that milrinone treatment will lead to an improvement in myocardial performance and global hemodynamics when compared to iNO alone. We will also compare the rate of adverse events associated with the milrinone, and the pre-discharge outcomes of both groups. The purpose of this pilot study is to assess the feasibility of performing the trial and to obtain preliminary data to calculate a sample size for a definitive multi-centre trial of milrinone therapy in PPHN. Trial registration: www.isrctn.com; ISRCTN:12949496; EudraCT Number:2014–002988-16. Electronic supplementary material The online version of this article (10.1186/s40748-018-0093-1) contains supplementary material, which is available to authorized users.

Purpose The impact of Down syndrome on myocardial performance measured using deformation imaging and pulmonary hypertension (PH) in the neonatal period requires exploration. We aimed to assess PH and measure left (LV) and right (RV) ventricular function using deformation imaging in neonates with DS (without structural heart disease) over 5 days of age. Methods Echocardiograms were performed on Days 1, 2 and 5 to measure LV and RV dimensions, LV basal longitudinal strain (LV BLS) and systolic strain rate (LV SR), RV free wall basal longitudinal strain (RV BLS) and systolic SR (RV SR), and LV rotational mechanics. PH was assessed by examining ductal (PDA’s) shunting and tricuspid regurgitation (to estimate RV systolic pressure, RVSp). Values were compared with healthy controls. Results 17 infants with DS with a mean ±SD gestation and birthweight of 38.4±2.1 weeks and 3.1±0.5 Kg respectively were compared with 16 controls (38.3±2.0 weeks and 3.3±0.5 Kg). On Day 1, infants with DS had a higher proportion of bidirectional PDAs (100% vs. 20%, p Conclusion Infants with DS demonstrate elevated pulmonary pressures during the early neonatal period translating into lower RV function measured using deformation imaging. They also demonstrate impaired twist driven by reduced basal rotation. The clinical implications warrant further study.

High-risk neuroblastoma (HR NBL) treatment requires intensive induction chemotherapy. The profoundly emetogenic agents used can compromise nutritional status. Our institution introduced a new antiemetic guideline in 2010 incorporating regular dexamethasone, in addition to ondansetron, for all highly emetogenic protocols.A retrospective comparative review of pediatric patients diagnosed with HR NBL who received rapid COJEC induction chemotherapy as per HR-SIOPEN NBL trial. Prophylactic antiemetics were prescribed regardless of chemotherapy emetogenicity in group A (2004-2010) but for defined time periods considering chemotherapy emetogenicity in group B (2010-2017).Sixty-three children were eligible for inclusion (median age, 31 months; range, 1-88 months). Group A had more episodes of emesis than group B (189 vs. 116, P 0.0001). There was a significant difference in weight-for-age Z score change between the groups by induction end (P = 0.0027). Four children (13%) in group A lost10% body weight versus none in group B. Nutrition support (NS) was utilized by 29 children (94%) in group A and 22 children (69%) in group B. Group A had a median of 3 (range, 1-7) admissions for febrile neutropenia (FN) versus a median of 1.5 (range, 0-4) for group B (P = 0.003) during induction.The review of our guidelines led to reduced emesis frequency for group B. They also required less NS, followed expected growth trajectories more closely and had fewer FN admissions. We propose that this may have occurred due to better emesis control resulting in improved nutritional status and associated enhanced immune function.

Background Although subgaleal hemorrhage can present very soon after delivery with catastrophic consequences, subaponeurotic or subgaleal fluid collections are rare and clinically distinct causes of infant scalp swelling that present weeks to months after birth. Their exact etiology remains uncertain; however, they are frequently associated with instrumental and traumatic delivery. Aim & Methods To characterize 11 subaponeurotic fluid collections that presented to the Temple Street Children's University Hospital Emergency Department (TSCUHED) from July 2013 to July 2015 by a retrospective chart review. Case Report Eleven infants were identified with delayed subaponeurotic fluid collections. Of note, all infants were either successful vacuum delivery or failed vacuum delivery with subsequent forceps delivery or emergency caesarean section. All infants were otherwise well at presentation, and resolution of the scalp swelling occurred within weeks to months. Why Should an Emergency Physician Be Aware of This? This condition follows a benign course and conservative management is the treatment of choice.

Background : Medical students are often considered by anaesthetists to have a lack of knowledge of anaesthesia and the role of the anaesthetist. Aim : The aim of this study was to assess the impact of a clinical clerkship in anaesthesia on Irish medical students understanding of anaesthesia as a specialty, the role of anaesthetists and career choice through comparison of pre-clinical and clinical medical students. Methods : This was a cross sectional study of 80 Irish medical students who completed an anonymous questionnaire consisting of 16 items. Results : Only 47.5% of clinical students were aware that anaesthetists participate in running intensive care units and just 65% knew that anaesthetists were involved in the management of chronic pain. However, 100% of clinical students recognised that anaesthetists administered epidural analgesia to obstetric patients and 30% of clinical students would consider a career in this specialty. Conclusion : This research is providing medical professionals and educators with updated information which may be useful for improving students experience and understanding of anaesthesia.