Aplasia cutis congenita in surviving co-twin after propylthiouracil exposure in utero

Aim: Aplasia cutis congenita (ACC) has been observed after fetal exposure to the antithyroid drug methimazole (MMI), but not reported after propylthiouracil (PTU), the current antithyroid drug of choice during pregnancy. This occurrence has implications for patient information and causal research. Case report: We describe a surviving term co-twin to a mother with hyperthyroidism exposed to PTU from conception to 34 weeks of gestation presenting with ACC at birth. Discussion: The association between PTU exposure and ACC is clinically relevant and allows speculation on the etiology. A similar mechanism to the classical MMI-induced ACC is postulated, unless a vascular etiology suggested by a vanishing twin or maternal hyperthyroidism itself is causal. Coincidence of PTU exposure and ACC seems unlikely. Conclusion: ACC in a newborn after PTU exposure during pregnancy hitherto observed only after MMI strongly encourages further reports of similar cases that may remain clinically underdiagnosed or unreported. Such confirmation could have significant implications for maternal treatment of hyperthyroidism, common in women of childbearing ag

Nurses' and physicians' reported difficulties and enablers to recognising and reporting child abuse in Swiss paediatric emergency and paediatric surgery departments - an observational study.

BACKGROUND Under-detection and under-reporting of child abuse remains a considerable challenge in paediatric care, with a high number of cases missed each year in Switzerland and abroad. Published data regarding the obstacles and facilitators of detecting and reporting child maltreatment among paediatric nursing and medical staff in the paediatric emergency department (PED) are scarce. Despite the existence of international guidelines, the measures taken to counteract the incomplete detection of harm done to children in paediatric care are insufficient. AIM We sought to examine up-to-date obstacles and enablers for detecting and reporting child abuse among nursing and medical staff in PED and paediatric surgery departments in Switzerland. METHODS We surveyed 421 nurses and physicians working in PEDs and on paediatric surgical wards in six large Swiss paediatric hospitals using an online questionnaire between February 1, 2017, and August 31, 2017. RESULTS The survey was returned by 261/421 (62.0%) respondents (complete n = 200, 76.6%; incomplete n = 61, 23.3%) with a preponderance of nurses (n = 150/261; 57.5%), 106/261 (40.6%) physicians, and 1/261 (0.4%) psychologists (n = 4/261; 1.5% missing profession). The stated obstacles to reporting child abuse were uncertainty about the diagnosis (n = 58/80; 72.5%), feeling unaccountable for notification (n = 28/80; 35%), uncertainty of whether reporting has any consequences (n = 5/80; 6.25%), lack of time (n = 4/80; 5%), forgetting to report (n = 2/80; 2.5%), and parental protection (n = 2/80; 2.5%) (unspecific answer, n = 4/80; 5%, multiple answers were possible, therefore items don not sum up to 100%). Even though most (n = 249/261 95.4%) respondents had previously been confronted with child abuse at/outside work, only 185/245 (75.5%) reported cases; significantly fewer nursing (n = 100/143, 69.9%) than medical staff (n = 83/99, 83.8%) (p = 0.013). Furthermore, significantly more nursing (n = 27/33; 81.8%) than medical staff (n = 6/33; 18.2%) (p = 0.005) reported a discrepancy between the number of suspected and reported cases (total 33/245 (13.5%). An overwhelming amount of participants were strongly interested in mandatory child abuse training (n= 226/242, 93.4%) and in the availability of standardised patient questionnaires and documentation forms (n = 185/243, 76.1%). CONCLUSION In line with previous studies, insufficient knowledge about and lack of confidence in detecting the signs and symptoms of child abuse were the principal obstacles to reporting maltreatment. To finally address this unacceptable gap in child abuse detection, we recommend the implementation of mandatory child protection education in all countries where no such education has been implemented in addition to the introduction of cognitive aid tools and validated screening tools to increase child abuse detection rates and ultimately prevent further harm to children

Avatar and distance simulation as a learning tool - virtual simulation technology as a facilitator or barrier? A questionnaire-based study on behalf of Netzwerk Kindersimulation e.V.

Background Virtual simulation modalities have been implemented widely since the onset of the severe acute respiratory syndrome coronavirus 2 pandemic restrictions in March 2020, as educators face persistent restrictions to face-to-face education of medical students and healthcare professionals.There is paucity of published data regarding the benefits and barriers of distance and avatar simulation training modalities. Methods Following a 2-day virtual pediatric simulation competition facilitated by Netzwerk Kindersimulation e.V., using remote human avatars and distance simulation, we conducted a multicenter survey to explore the advantages and challenges of avatar and distance simulation among participants. We used a modified Delphi approach to draft and develop the 32-item online questionnaire with 7-point Likert-like scales (7 being the highest rating). Results Twenty participants answered our questionnaire. Respondents indicated both a high overall satisfaction (median of 5.0 [Q25-Q75: 4.0-6.0] ) for avatar and distance simulation 6.0 (5.0-6.0), respectively, as well as a high achieved psychological safety with both simulation types (5.0 [4.0-6.0] vs. 5.0 [4.0-6.0]). The most frequently reported profits of avatar and distance simulation included the elimination of travel distances, associated lower costs, less time spent attending the education activity, and effective communication and leadership training, especially with avatar simulation. Most often named challenges were technical problems, limited reception of non-verbal cues and a spatial distance from the team/educator. Discussion Based on the results of this pilot study, avatar and distance simulation can be employed successfully and appear to be good supplements to face-to-face simulation. Other studies are warranted to further explore the effectiveness of various types of virtual simulation compared to conventional presential simulation. We suggest using avatar-based simulation for targeted communication and leadership skills training and the application of distance simulation to bring simulation experts virtually to remote places where educator resources are lacking

Readiness for and Response to Coronavirus Disease 2019 Among Pediatric Healthcare Providers: The Role of Simulation for Pandemics and Other Disasters

Objectives: Early preparation for the training and education of healthcare providers, as well as the continuation or modification of routine medical education programs, is of great importance in times of the coronavirus disease 2019 pandemic or other public health emergencies. The goal of this study was to characterize these self-reported efforts by the pediatric simulation community. Design: This was a global, multicenter survey developed via a Delphi process. Setting: International survey study. SUBJECTS: The survey was sent to 555 individual members of the three largest international pediatric simulation societies (The International Pediatric Simulation Society, International Network for Simulation-based Pediatric Innovation, Research & Education, and Netzwerk Kindersimulation e.V.) between April 27, 2020, and May 18, 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Description of coronavirus disease 2019 pandemic simulation-based preparation activities of pediatric acute and critical care healthcare providers. The Delphi process included 20 content experts and required three rounds to reach consensus. The survey was completed by 234 participants (42.2%) from 19 countries. Preparation differed significantly between the geographic regions, with 79.3% of Anglo-American/Anglo-Saxon, 82.6% of Indian, and 47.1% of European participants initiating specifically coronavirus disease 2019-related simulation activities. Frequent modifications to existing simulation programs included the use of telesimulation and virtual reality training. Forty-nine percent of institutions discontinued noncoronavirus disease 2019-related simulation training. Conclusions: The swift incorporation of disease-specific sessions and the transition of standard education to virtual or hybrid simulation training modes occurred frequently. The approach used, however, depended heavily on local requirements, limitations, and circumstances. In particular, the use of telesimulation allowed education to continue while maintaining social distancing requirements

Genome-wide screening of loss of heterozygosity in human midgut carcinoid tumors with fluorescent technique

Hintergrund: Karzinoid-Tumoren des embryonalen Mitteldarms sind seltene intestinale neuroendokrine Tumoren, bei denen zum Zeitpunkt der Diagnose häufig Metastasen vorliegen. Im Gegensatz zu Karzinoiden des Vorderdarms und Respirationstraktes sind sie nicht mit der Multiplen Endokrinen Neoplasie Typ 1 (MEN1) vergesellschaftet. Die Mechanismen ihrer Tumorigenesis sind weitgehend unbekannt. Methoden: Tumorgewebe acht sporadischer, maligner Dünndarm-Karzinoide war Objekt dieser Studie über Verlust der Heterozygotie ("Loss Of Heterozygosity" (LOH)) mit 131 fluoreszierenden Mikrosatelliten. DNA Sequenz-Analyse mit Oligonucleotid Primern, die Exon 8-11 des SMAD4/DPC4 Gens flankieren sowie immunhistochemische Färbung mit Smad4/DPC4 antikörpern wurde durchgeführt. Ergebnis: Chromosom 18 wies Deletionen in 88% der Tumoren auf. Alle außer einem Tumor hatten sowohl 18p als auch 18q verloren, in einem der Tumoren war eine kleine Region telomer zu den SMAD4/DPC4/DCC Genen auf 18q21 verloren. Andere Chromosomen waren nur in drei Tumoren betroffen. LOH auf Chromosom 11q13, dem MEN1 Lokus, wurde nicht gefunden.Sequenzierung der DNA und immunhistochemische Färbung für das SMAD4/DPC4 Gen zeigten keine Aberrationen. Diskussion: Die Funde der Chromosom 18 Deletionen weisen eindeutig auf ein entscheidendes Ereignis in der Tumorigenese von Karzinoiden des Mitteldarms hin. An der Entstehung dieser Tumoren könnte ein mutmaßliches Tumor Suppressor Gen beteiligt sein, welches auf Chromosom 18 lokalisiert ist. Dahingegen ist SMAD4/DPC4 wahrscheinlich nicht in die Tumorneogenese von Carcinois Tumoren involviert.Background: Midgut carcinoid tumors are rare malignant tumors with origin in the neuroendocrine cells of the small intestine. Due to secretion of a variety of peptide hormones and biogenic amines they cause the carcinoid syndrome. Metastases are often present at first diagnosis. Despite this, patients have a realistic chance to survive for a prolonged period (30% (unresectable/metastatic disease) -79% (non-metastatic disease) 5-year survival rate) if treated by a combination of surgery and medication. Unlike their foregut counterparts, midgut carcinoid tumors are not or rarely associated with the multiple endocrine neoplasia type 1 (MEN1) syndrome. The genetic back-ground to tumorigenesis of these neoplasms is unknown. In contrast, the events involved in tumorigenesis of gastroenteropancreatic adenocarcinomas are better characterized with frequent mutations e.g. of the Smad4/DPC4, Smad2/MADR2/JV18-1 and DCC genes on chromosome 18. Methods: Eight metastatic midgut carcinoids were analysed by a genome-wide screening for loss of heterozygosity using 131 PCR-amplified fluorescent-labelled microsatellite markers. DNA sequence analysis using oligonucleotide primers flanking exons 8-11 of the Smad4/DPC4 gene and immunohistochemical staining with Smad4/DPC4 antibodies was performed. Results: Chromosome 18 was deleted in seven out of eight tumors (88%). All but one of these tumors had lost both 18p and 18q, the remaining tumor had lost the long arm but retained the short arm. Several other chromosomal alleles were lost in a subset of the tumors. Loss of heterozygosity (LOH) on chromosome 11q13, the MEN 1 locus, was not found. Smad4/DPC4 wild-type sequence and normal immunohistochemical staining for Smad4/DPC4 protein was found for all analysed tumors. Conclusions: Our finding of a high frequency of chromosome 18 deletions in 88% of the tumors strongly suggests that midgut carcinoid tumorigenesis might involve inactivation of a candidate tumor suppressor gene located in that region while Smad4/DPC4 is unlikely to be involved in that process. A more detailed analysis of the genetic events in midgut carcinoid tumors is warranted to clarify their neogenetic origin

Balanced Electrolyte Solutions or Normal Saline? Resuscitative Fluid Administration Practice in Swiss Pediatric Acute Care

INTRODUCTION: The ideal asanguineous intravenous fluid for volume resuscitation in children is controversially debated and clinical practice guidelines are scarce. Administration of large amounts of normal saline has been associated with complications including hyperchloremic acidosis, dysnatremia, neurologic damage, and fatality. AIM: We examined the current practice of intravenous fluid and blood product administration in acutely ill and injured children among pediatric acute care physicians in Switzerland. METHODS: For this descriptive, cross-sectional study, pediatric emergency departments, pediatric and neonatal intensive care units were surveyed by means of an online questionnaire. RESULTS: Sixty of 66 departments and 47 of 87 participants returned the survey. Normal saline (NS) was most commonly administered (n = 42/46, 91.3%) and twice as many times as balanced electrolyte solutions (n = 20/46, 43.5%). The mean fluid volumes ranged from 7.9 to 19.1 mL/kg. Hypertonic saline/NS were selected most often for shock with severe head injury. Half of participants administered colloids (48.9%). Packed red blood cells (97.7%) and fresh frozen plasma (88.4%) were most frequently given blood products. CONCLUSION: There is a distinct practice variation in intravenous fluid and blood product administration in children in Switzerland. Although NS is most frequently given, we observed a trend toward the use of balanced electrolyte solutions. Prospective studies are warranted to compare NS with balanced electrolyte solution (BES) in the pediatric acute care setting. We suggest that pediatric fluid administration guidelines and mass transfusion protocols are implemented to standardize this frequent intervention and minimize complications