European journal of pediatrics
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Multicenter Study
Diagnostic and management practices for phenylketonuria in 19 countries of the South and Eastern European Region: survey results.
To avoid potentially severe outcomes, phenylketonuria (PKU) must be detected as soon as possible after birth and managed with life-long treatment. A questionnaire-based survey was performed to document diagnosis and management practices for PKU in a region of Southern and Eastern Europe. Prevalence and management data were obtained from 37/59 (63 %) centres within 19/22 (86%) contacted countries (N = 8600 patients). The main results' analysis was based on completed questionnaires obtained from 31 centres (53%) within 15 countries (68%). A median of 10 % of patients per centre had been diagnosed after the newborn period. Metabolic dieticians and specialised adult PKU clinics were lacking in 36 and 84% of centres, respectively. In 26% of centres, treatment initiation was delayed until >15 days of life. Blood phenylalanine (Phe) thresholds to start treatment and upper Phe targets were inconsistent across centres. Ten percent of centres reported monitoring Phe every 2 weeks for pregnant women with PKU, which is insufficient to minimise risk of neonatal sequalae. Sapropterin dihydrochloride treatment was available in 48% of centres, with 24-h responsiveness tests most common (36%). Only one centre among the five countries lacking newborn screening provided a completed questionnaire. ⋯ PKU management practices are documented in 19 South and Eastern European countries indicating a heterogeneous situation across the region. Key areas for improvement identified in surveyed centres include a need for comprehensive screening in all countries, increased number of metabolic dietitians and specialised adult PKU clinics, delayed time to treatment initiation, appropriate Phe thresholds, Phe targets and monitoring frequencies, and universal access to currently available treatment options.
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Observational Study
Medicine preparation errors in ten Spanish neonatal intensive care units.
This study assessed the rate of errors in intravenous medicine preparation at the bedside in neonatal intensive care units vs the preparation error rate in a hospital pharmacy service. We conducted a prospective observational study between June and September 2013. Ten Spanish neonatal intensive care units and one hospital pharmacy service participated in the study. Two types of preparation errors were considered: calculation errors and accuracy errors. A total of 522 samples were collected: 238 of vancomycin, 139 of gentamicin, 39 of phenobarbital and 88 of caffeine citrate preparations. Of these, 444 samples were collected by nurses in neonatal intensive care units, and 60 were provided by the hospital pharmacy service. Overall, 18 samples were excluded from the analysis. We detected calculation errors in 6/444 (1.35%) and accuracy errors in 243/444 (54.7%) samples from the neonatal intensive care units. In contrast, in samples from the hospital pharmacy service, no calculation errors were detected, but there were accuracy errors in 23/60 (38.3%) samples. ⋯ Calculation errors are avoided using concentration standard preparation protocols. Accuracy in the preparation process depends mainly on the degree to which commercial drug preparations meet current legal requirements and the syringes and preparation techniques used.
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Practice Guideline
Expert consensus statement 'Neonatologist-performed Echocardiography (NoPE)'-training and accreditation in UK.
Targeted echocardiographic assessments of haemodynamic status are increasingly utilised in many settings. Application in the neonatal intensive care units (NICU) is increasingly demanded but challenging given the risk of underlying structural lesions. This statement follows discussions in UK led by the Neonatologists with an Interest in Cardiology and Haemodynamics (NICHe) group in collaboration with the British Congenital Cardiac Association (BCCA) and the Paediatricians with Expertise in Cardiology Special Interest Group (PECSIG). Clear consensus was agreed on multiple aspects of best practice for neonatologist-performed echocardiogram (NoPE)-rigorous attention to infection control and cardiorespiratory/thermal stability, early referral to paediatric cardiology with suspicion of structural disease, reporting on standardised templates, reliable image storage, regular skills maintenance, collaboration with a designated paediatric cardiologist, and regular scan audit/review. It was agreed that NoPE assessments should confidently exclude structural lesions at first scan. Practitioners would be expected to screen and establish gross normality of structure at first scan and obtain confirmation from paediatric cardiologist if required, and subsequently, functional echocardiography can be performed for haemodynamic assessment to guide management of newborn babies. To achieve training, NICHe group suggested that mandatory placements could be undertaken during core registrar training or neonatal subspecialty grid training with a paediatric cardiology placement for 6 months and a neonatology placement for a minimum of 6 months. In the future, we hope to define a precise curriculum for assessments. Technological advances may provide solutions-improvements in telemedicine may have neonatologists assessing haemodynamic status with paediatric cardiologists excluding structural lesions and neonatal echocardiography simulators could increase exposure to multiple pathologies and allow limitless practice in image acquisition. ⋯ This is the first document of consensus best practice statement for training of neonatologists in neonatologist-performed echocardiogram (NoPE), jointly drafted by Neonatologists with interest in cardiology & haemodynamics (NICHe), paediatric cardiology and paediatricians with expertise in cardiology interest groups in UK. Key elements of a code of practice for neonatologist-performed echocardiogram are suggested.
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To analyse the usefulness of bedside lung ultrasound (LUS) in detecting lung consolidation in a paediatric emergency room (ER) setting, febrile children seen at our ER from 2008 to 2012 with a moderate to severe respiratory distress underwent LUS, chest X-ray (CXR) and laboratory investigations. At first ER assessment, LUS identified a lung consolidation in 207 patients of 222 children enrolled, with a liver-like appearance in 75 (36.2%) and an associated pleural effusion in 36.7% of cases. CXR proved positive in 197 cases, showing a parenchymal consolidation (68.5%) or a focal ground-glass opacity (31.4%). LUS liver-like consolidation was significantly associated with longer duration of fever (p = 0.002), higher neutrophil counts and C-reactive protein (CRP) values (p = 0.015 and p < 0.0001, respectively), and with the discovery of a homogeneous and dense parenchymal consolidation on CXR (p < 0.0001). ⋯ LUS is confirmed to be a reliable imaging technique for the diagnostic work-up of febrile children with respiratory distress, consistent not only with CXR results as previously reported by others but also with clinical and laboratory data. In the hands of trained clinicians, it may represent a valuable supplemental bedside tool for a rapid evaluation in such circumstances.
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This study investigated the association between obesity and obstructive sleep apnea (OSA) in preschool and school-age children. Parents of obese and randomly chosen normal weight children completed a questionnaire on sleep-related symptoms, demography, family, and medical history. All subjects were invited to undergo polysomnography (PSG). OSA cases were defined as obstructive apnea hypopnea index (OAHI) ≥1. A total of 5930 children were studied with 9.5% obese (11.9% boys/6.1% girls), 205/2680 preschool and 360/3250 school children. There were 1030 children (535 obese/495 normal weight) who underwent PSG. OSA was higher in obese children and obese school children had higher OAHI, arousal index, and shorter total sleep time. However, there was no positive correlation between OSA and body mass index (BMI). The main risk factors for OSA in preschool children were adenotonsillar hypertrophy and recurrent respiratory tract infection. The main cause for OSA in school children was a history of parental snoring and obesity. Mallampati scores and sleep-related symptoms were found to be associated with OSA in both preschool and school children. ⋯ A differential prevalence and risk factors for obese preschool and school-age children with OSA has been demonstrated.