Staffing, skill mix, shortage, and survival in the NICU and PICU: pediatrics quo vadis?

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Distended abdomen due to a pseudocyst around a ventriculoperitoneal shunt

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Corrigendum: Assessment of Inadequate Use of Pediatric Emergency Medical Transport Services: The Pediatric Emergency and Ambulance Critical Evaluation (PEACE) Study

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Micro-RNA signatures in monozygotic twins discordant for congenital heart defects

Background: MicroRNAs (miRNAs) are small RNAs regulating gene expression post-transcriptionally. Recent studies demonstrated that miRNAs are involved in the development of congenital heart defects (CHD). In this study, we aimed at identifying the specific patterns of miRNAs in blood of monozygotic twin pairs discordant for CHD and to assess whether miRNAs might be involved in the development or reflect the consequences of CHD. Methods: miRNA microarray analysis and Real-Time Quantitative PCR (RT-qPCR) were employed to determine the miRNA abundance level from 12 monozygotic twins discordant for CHD and their non-CHD co-twins (n = 12). Enrichment analyses of altered miRNAs were performed using bioinformatics tools. Results: Compared with non-CHD co-twins, profiling analysis indicated 34 miRNAs with a significant difference in abundance level (p<0.05, fold change ≥ 1.3), of which 11 miRNAs were up-regulated and 23 miRNAs were down-regulated. Seven miRNAs were validated with RT-qPCR including miR-511-3p, miR-1306-5p, miR-421, miR-4707-3p, miR-4732-3p, miR-5189-3p, and miR-890, and the results were consistent with microarray analysis. Five miRNAs namely miR-511-3p, miR-1306-5p, miR-4732-3p, miR-5189-3p, and miR-890 were found to be significantly up-regulated in twins < 10 years old. Bioinformatics analysis showed that the 7 validated miRNAs were involved in phosphatidylinositol signaling, gap junction signaling, and adrenergic signaling in cardiomyocytes.Conclusions: Our data show deregulated miRNA abundance levels in the peripheral blood of monozygotic twins discordant for CHD, and identify new candidates for further analysis, which may contribute to understanding the development of CHD in the future. Bioinformatics analysis indicated that the target genes of these miRNAs are likely involved in signaling and communication of cardiomyocytes

Syncope in children and adolescents: are the current guidelines being followed?

Hintergrund Synkopen im Kindes‑/Jugendalter sind häufig und meist gutartig. Mögliche kardiale Synkopen müssen durch sorgfältige Basisdiagnostik (Anamnese (I), körperliche Untersuchung (II), Elektrokardiografie (III)) und ggf. weiterführender Diagnostik ausgeschlossen werden. Fragestellung Wurde die Diagnostik bei Vorliegen einer Synkope entsprechend der gültigen S2k-Leitlinie durchgeführt? Material und Methoden Retrospektive Analyse (01/2015–12/2017), Kinderklinik des Universitätsklinikums des Saarlandes, Homburg, Deutschland. Eingeschlossen wurden alle Patienten von 1 bis 18 Jahre, die sich wegen Synkope vorstellten. Ergebnisse Es erlitten 262 Patienten eine Synkope (161 weiblich [61,5 %], 101 männlich [38,5 %], Alter 12,5 ± 3,9 Jahre); davon 183 (69,8 %) Reflexsynkopen, 36 (13,7 %) Präsynkopen, 35 (13,4 %) Synkopen unklarer Genese, 8 (3,1 %) kardiale Synkopen; 43/262 Patienten (16,4 %) erhielten eine vollständiger Basisdiagnostik (I–III) gemäß Leitlinie, 13/43 (30,2 %) wurden korrekt weiterführender Diagnostik zugeführt; 219/262 Patienten (83,6 %) erhielten keine ausreichende Basisdiagnostik (I–III), 135/219 (61,6 %) wurden unnötigen apparativen Untersuchungen zugeführt. Diskussion Die leitlinienkonforme Synkopenabklärung ist wichtig, um unnötige, aber auch nicht ausreichende Diagnostik zu vermeiden und somit Patienten mit Synkope korrekt zu diagnostizieren.Background Syncope in childhood and adolescence is frequent and in most cases benign. A thorough history taking, complete physical examination, electrocardiography and further diagnostic work-up as indicated should rule out possible cardiac syncope. Objective To evaluate whether the diagnosis of syncope was performed according to the currently valid S2k guideline. Material and methods Retrospective study (January 2015–December 2017), University Children’s Hospital of Saarland, Homburg, Germany. All patients aged 1–18 years presenting with the primary complaint of syncope were included. Results In this study 262 patients presented with a history of syncope (161 female (61.5%), 101 male (38.5%), median age 12.5 ± 3.9 years). Of these, 183 (69.8%) were reflex syncopes, 36 (13.7%) presyncopes, 35 (13.4%) undefined and 8 (3.1%) cardiac syncope. Out of 262 patients, 43 (16.4%) were diagnosed in accordance with the published guidelines and 13/43 (30.2%) correctly received further diagnostic work-up. In 219/262 patients (83.6%) basic diagnostic testing was not sufficient and 135/219 (61.6%) were submitted to further unnecessary diagnostic tests. Conclusion Better adherence to the syncope guidelines bears the potential to avoid unnecessary and costly auxiliary medical tests while correctly diagnosing patients with syncope

Standardized Treatment and Diagnostic Approach to Reduce Disease burden in the early postoperative phase in children with congenital heart defects-STANDARD study: a pilot randomized controlled trial

To explore the effect of a daily goal checklist on pediatric cardiac intensive care unit (PCICU) length of stay (LOS) after congenital heart surgery. This study is a prospective randomized single-center study. Group characteristics were as follows: STANDARD group: n=30, 36.7% female, median age 0.9 years; control group: n=33, 36.4% female, median age 1.1 years. Invasive ventilation time, STAT categories, mean vasoactive-inotropic score (VIS)24h, maximal (max.) VIS24h, mean VIS24–48h, max. VIS24–48h, VIS category, number of sedatives, analgesics, diuretics, number of deployed diagnostic modalities, morbidities, and mortality did not differ between both groups. Median PCICU LOS was 96.0 h (STANDARD group) versus 101.5 h (control group) (p=0.63). In the overall cohort, univariate regression analysis identified age at surgery (b=−0.02), STAT category (b=18.3), severity of CHD (b=40.6), mean VIS24h (b=3.5), max. VIS24h (b=2.2), mean VIS24–48h (b=6.5), and VIS category (b=13.8) as significant parameters for prolonged PCICU LOS. In multivariate regression analysis, age at surgery (b=−0.2), severity of CHD (b=44.0), and mean VIS24h (b=6.7) were of significance. Within the STANDARD sub-group, univariate regression analysis determined STAT category (b=32.3), severity of CHD (b=70.0), mean VIS24h (b=5.0), mean VIS24–48h (b=5.9), number of defined goals (b=2.6), number of achieved goals (b=3.3), number of not achieved goals (b=10.8), and number of unevaluated goals (b=7.0) as significant parameters for prolonged PCICU LOS. Multivariate regression analysis identified the number of defined goals (b=2.5) and the number of unevaluated goals (b=−3.0) to be significant parameters. Conclusion: The structured realization and recording of daily goals is of advantage in patients following pediatric cardiac surgery by reducing PCICU LOS

Assessment of the importance of neuropediatric diagnostics in the initial clarification of autism

Hintergrund Die Diagnostik bei Autismus-SpektrumStörungen ist aufgrund fehlender biologischer Marker und zahlreicher Komorbiditäten anspruchsvoll. Ziel dieser Arbeit war es, den Stellenwert der neuropädiatrischen Diagnostik zu beurteilen und eine interne Leitlinie zu erstellen. Methodik Eingeschlossen wurden alle Patienten, die sich zwischen 04/2014 und 12/2017 in der neuropädiatrischen Ambulanz am Universitätsklinikum des Saarlandes mit der Diagnose „tiefgreifende Entwicklungsstörungen“ (ICD-Code F84) vorgestellt haben. Ergebnisse Die Studie umfasste 82 Patienten (männlich 78%, weiblich 22%; Durchschnittsalter 5,9 ± 2,9 Jahre, Spanne 2 bis 16 Jahre). Häufigste Untersuchung war die Elektroenzephalographie (EEG) (74/82; 90,2%); diese war bei 33,8% (25/74) auffällig. Anhand der Anamnese und/oder des EEGs wurde bei 16/82 (19,5%) Kindern die Diagnose „Epilepsie“ gestellt. Eine kranielle Magnetresonanztomographie (cMRT) erhielten 49/82 (59,8%) der Patienten; 22/49 (44,9%) zeigten mindestens einen auffälligen Befund; bei 14/22 (63,6%) ließen sich eindeutige Pathologien feststellen. Eine Stoffwechseldiagnostik wurde bei 44/82 (53,7%) Kindern veranlasst; bei 5/44 (11,4%) resultierte daraus eine Diagnose oder der Verdacht auf eine Stoffwechselerkrankung. Das Ergebnis einer genetischen Diagnostik lag bei 29/82 (35,4%) Kindern vor mit Auffälligkeiten in 41,4% (12/29). Eine motorische Entwicklungsverzögerung war häufiger mit Komorbiditäten, EEG-Auffälligkeiten, Epilepsie und Auffälligkeiten in der Stoffwechsel- sowie genetischen Diagnostik assoziiert. Schlussfolgerung Die neuropädiatrische Mitbeurteilung bei Verdacht auf Autismus sollte bei jedem Kind eine detaillierte Anamnese, eine neurologische Untersuchung sowie ein EEG beinhalten. Die Durchführung einer cMRT, einer Stoffwechsel- sowie einer genetischen Diagnostik wird nur bei klinischer Indikation empfohlen.Background The diagnostics of autism spectrum disorder is complex due to missing biological markers and numerous comorbidities. The aim was to assess the role of neuropediatric diagnostics and to develop a standard operating procedure for a targeted assessment. Method All patients presenting to the neuropediatric outpatient clinic at Saarland University Hospital between April 2014 and December 2017 with ICD code F84 pervasive developmental disorders were included. Results A total of 82 patients were included (male 78%, female 22%; mean age 5.9 ± 2.9 years, range 2–16 years). The most frequent examination was electroencephalography (EEG) (74/82; 90.2%) with pathological findings in 33.8% (25/74). Based on the history and/or EEG epilepsy was diagnosed in 19.5% (16/82). Magnetic resonance imaging (MRI) was performed in 49/82 (59.8%) patients, 22/49 (44.9%) showed at least 1 cerebral abnormality and definite pathologies could be detected in 63.6% (14/22). A metabolic diagnostic work-up was performed in 44/82 (53.7%) cases and in 5/44 (11.4%) it resulted in a diagnosis or suspicion of a metabolic disease. Genetic testing results were available in 29/82 (35.4%) children and 12/29 (41.4%) showed abnormal results. Delay in motor development was more frequently associated with comorbidities, EEG abnormalities, epilepsy and abnormalities in metabolic and genetic testing. Conclusion Neuropediatric examination in cases of suspected autism should include a detailed history, a thorough neurological examination and an EEG. An MRI, comprehensive metabolic and genetic testing are only recommended if clinically indicated

Epileptic Status in a PEDiatric cohort (ESPED) requiring intensive care treatment: A multicenter, national, two-year prospective surveillance study

The aim of this study was to provide seizure etiology, semiology, underlying conditions, and out-of- and in-hospital diagnostics, treatment, and outcome data on children with out-of- or in-hospital-onset status epilepticus (SE) according to the International League Against Epilepsy definition that required admission to the pediatric intensive care unit (PICU) for ≥4 hours.Dr Wolf Epilepsy, Grant/Award Number: N/