De Europoort voor aangeboren anatomische afwijkingen

Rede, Uitgesproken ter gelegenheid van het aanvaarden van het ambt van bijzonder hoogleraar met als leeropdracht kinderchirurgie aan het Erasmus MC, faculteit van de Erasmus Universiteit Rotterdam op 12 mei 201

Esophageal atresia: Long-term morbidities in adolescence and adulthood

Survival rates in esophageal atresia (EA) patients have reached 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We evaluated the long-term morbidity in adolescent and adult EA patients and discussed mainly nonsurgical issues. Dysphagia is common and reported in up to 85% of patients. In young adults gastroesophageal reflux disease occurs frequently with development of Barrett esophagus in 6% reported in different series. It is difficult to estimate respiratory morbidity from the literature because many different definitions, questionnaires, and study designs have been used. However, many patients seem to suffer from respiratory problems even into adulthood. In conclusion, morbidity is not only restricted to surgical problems but many different domains are involved. These are all related and together determine to a large extent the quality of life of EA patients and also of their families. We assume that a multidisciplinary care approach seems best to address their special needs

Assessment and significance of long-term outcomes in pediatric surgery

Treatment modalities for newborns with anatomical congenital anomalies have greatly improved over the past decades, with a concomitant increase in survival. This review will briefly discuss specific long-term outcomes to illustrate, which domains deserve to be considered in long-term follow-up of patients with anatomical congenital anomalies. Apart from having disease-specific morbidities these children are at risk for impaired neurodevelopmental problems and school failure, which may affect participation in society in later life. There is every reason to offer them long-term multidisciplinary follow-up programs. We further provide an overview of the methodology of long-term follow-up, its significance and discuss ways to improve care for newborns with anatomical congenital anomalies from childhood into adulthood. Future initiatives should focus on transition of care, risk stratification, and multicenter collaboration

The Role of Prosocial and Aggressive Popularity Norm Combinations in Prosocial and Aggressive Friendship Processes

Prior work has shown that popular peers can set a powerful norm for the valence and salience of aggression in adolescent classrooms, which enhances aggressive friendship processes (selection, maintenance, influence). It is unknown, however, whether popular peers also set a norm for prosocial behavior that can buffer against aggressive friendship processes and stimulate prosocial friendship processes. This study examined the role of prosocial and aggressive popularity norm combinations in prosocial and aggressive friendship processes. Three waves of peer-nominated data were collected in the first- and second year of secondary school (N = 1816 students; 81 classrooms; M-age = 13.06; 50.5% girl). Longitudinal social network analyses indicate that prosocial popularity norms have most power to affect both prosocial and aggressive friendship processes when aggressive popularity norms are non-present. In prosocial classrooms (low aggressive and high prosocial popularity norms), friendship maintenance based on prosocial behavior is enhanced, whereas aggressive friendship processes are largely mitigated. Instead, when aggressive popularity norms are equally strong as prosocial norms (mixed classrooms) or even stronger than prosocial norms (aggressive classrooms), aggression is more important for friendship processes than prosocial behavior. These findings show that the prosocial behavior of popular peers may only buffer against aggressive friendship processes and stimulate prosocial friendship processes if these popular peers (or other popular peers in the classroom) abstain from aggression.</p

Optimizing working-space in laparoscopy: Measuring the effect of mechanical bowel preparation in a porcine model

Background: Adequate working space is a prerequisite for safe and efficient minimal access surgery. No objective data exist in literature about the effect of mechanical bowel preparation (MBP) on working space in laparoscopic surgery. We objectively measured this effect with computed tomography in a porcine laparoscopy model. Methods: Using standardized anesthesia, twelve 20-kg pigs without MBP and eight 20-kg pigs with MBP were studied with computed tomography at intra-abdominal pressure (IAP) levels of 0, 5, 10, and 15 mmHg. Volumes and dimensions of the pneumoperitoneum were measured on reconstructed CT images and compared between the pigs with and those without MBP. Results: A reproducible and statistically significant increase of approximately 500 ml in pneumoperitoneum volume was found in the MBP group at all levels of IAP. This represents a 43 % relative increase at a pneumoperitoneum pressure of 5 mmHg, 21 % at IAP 10 mmHg, and 18 % at IAP 15 mmHg. Peak inspiratory pressure was lower at IAP 0 and 5 mmHg in the MBP group. Anteroposterior diameter in the group with MBP was lower at 0 mmHg, but abdominal dimensions were similar in both groups at all other IAPs. This shows that the gain in working space is due to a diminished volume of the intra-abdominal content and not to compression or displacement of the bowel. Conclusions: MBP increases working space by reducing bowel content. Especially at low intra-abdominal working pressures, the increase in working space associated with MBP could represent an important benefit in challenging laparoscopic surgery

Bullying and Victimization Trajectories in the First Years of Secondary Education:Implications for Status and Affection

Bullying is known to be associated with social status, but it remains unclear how bullying involvement over time relates to social position (status and affection), especially in the first years at a new school. The aim of this study was to investigate whether (the development of) bullying and victimization was related to the attainment of status (perceived popularity) and affection (friendships, acceptance, rejection) in the first years of secondary education (six waves). Using longitudinal data spanning the first- and second year of secondary education of 824 adolescents (51.5% girls; M(age) T1 = 12.54, SD = 0.45) in the SNARE-study, joint bullying and victimization trajectories were estimated using parallel Latent Class Growth Analysis (LCGA). The four trajectories (decreasing bully, stable high bully, decreasing victim, uninvolved) were related to adolescents’ social position using multigroup analysis that examined differences in slope and intercepts (T1 and T6) of social positions, and indicated that the relative social position of the different joint trajectories was determined at the start of secondary education and did not change over time, with one exception: adolescents continuing bullying were besides being popular also increasingly rejected over time. Although bullying is functional behavior that serves to optimize adolescents’ social position, anti-bullying interventions may account for the increasing lack of affection that may hinder bullies’ long-term social development

Pulmonary vascular development in congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primarily from the associated PH, for which the therapeutic modalities are limited and frequently not evidence based. The vascular defects associated with PH, which is characterised by increased muscularisation of arterioles and capillaries, start to develop early in gestation. Pulmonary vascular development is integrated with the development of the airway epithelium. Although our knowledge is still incomplete, the processes involved in the growth and expansion of the vasculature are beginning to be unravelled. It is clear that early disturbances of this process lead to major pulmonary growth abnormalities, resulting in serious clinical challenges and in many cases death in the newborn. Here we provide an overview of the current molecular pathways involved in pulmonary vascular development. Moreover, we describe the abnormalities associated with CDH and the potential therapeutic approaches for this severe abnormality

The gut microbiome in patients with intestinal failure: Current evidence and implications for clinical practice

Intestinal failure (IF) is the reduction of gut function or mass below a minimum needed to absorb nutrients and fluids, such that patients are dependent on parenteral nutrition (PN). Patients with IF have an altered gut microbiome. Our aim was to review and evaluate the current evidence on gut microbiome and its metabolic activity as well as its association with disease characteristics in adults and children with IF. We performed a PubMed literature search for articles published after 2000 using the following terms: intestinal failure, microbiome, microbiota, short-chain fatty acids, short bowel syndrome and parenteral nutrition. Literature search was restricted to human studies only. The gut microbiome diversity is remarkably reduced and community structure is altered with a noticeable over-abundance of Proteobacteria, especially the Enterobacteriaceae family. A substantial increase in Lactobacillus level is often reported in patients with IF. Gut microbiome characteristics have been associated with poor growth, liver disease, D-lactic acidosis and duration of intestinal adaptation. Differences in microbiome characteristics have been found between patients on PN and those whose guts have adapted and have been weaned off PN. Future research with prospective sample collection should explore the value of the gut microbiome as a biomarker to guide clinical practice and as a modifiable therapeutic target to optimize outcomes of patients with IF

Changes in vasoactive pathways in congenital diaphragmatic hernia associated pulmonary hypertension explain unresponsiveness to pharmacotherapy

Background: Patients with congenital diaphragmatic hernia (CDH) have structural and functional different pulmonary vessels, leading to pulmonary hypertension. They often fail to respond to standard vasodilator therapy targeting the major vasoactive pathways, causing a high morbidity and mortality. We analyzed whether the expression of crucial members of these vasoactive pathways could explain the lack of responsiveness to therapy in CDH patients. Methods: The expression of direct targets of current vasodilator therapy in the endothelin and prostacyclin pathway was analyzed in human lung specimens of control and CDH patients. Results: CDH lungs showed increased expression of both ETA and ETB endothelin receptors and the rate-limiting Endothelin Converting Enzyme (ECE-1), and a decreased expression of the prostaglandin-I2 receptor (PTGIR). These data were supported by increased expression of both endothelin receptors and ECE-1, endothelial nitric oxide synthase and PTGIR in the well-established nitrofen-CDH rodent model. Conclusions: Together, these data demonstrate aberrant expression of targeted receptors in the endothelin and prostacyclin pathway in CDH already early during development. The analysis of this unique patient material may explain why a significant number of patients do not respond to vasodilator therapy. This knowledge could have important implications for the choice of drugs and the design of future clinical trials internationally

Implementation of a children's hospital-wide central venous catheter insertion and maintenance bundle

Background: Central venous catheter-associated bloodstream infections in children are an increasingly recognized serious safety problem worldwide, but are often preventable. Central venous catheter bundles have proved effective to prevent such infections. Successful implementation requires changes in the hospital system as well as in healthc