Piet L. J. M. Leroy

Safe and effective procedural sedation for gastrointestinal endoscopy in children.

Objective: The aim of the study was to assess, by a review of published evidence, the safest and most effective way to provide procedural sedation (PS) in children undergoing gastrointestinal endoscopy (GIE). Methods: The databases MEDLINE, Cochrane Library, and Embase were used. Search terms “endoscopy, gastrointestinal” or “endoscopy, digestive system” were combined with “sedation,” “conscious sedation,” “moderate sedation,” “deep sedation,” and “hypnotics and sedatives.” The final review was restricted to studies reporting specifically on safety (incidences of adverse events) and/or effectiveness (time characteristics, need for supplemental sedation, need for restraint, procedural success, provider satisfaction, and patient comfort) of PS for GIE in children younger than 18 years. Results: The search yielded 182 references and the final selection included 11 randomized controlled trials (RCTs) and 15 non-RCTs. Six sedation categories were identified: propofol, opioid/benzodiazepine, premedication, ketamine-, sevoflurane-, and midazolam-based. Only a few RCTs have compared different categories. Opioid/benzodiazepine- and propofol-based PS have a similar safety profile and a low incidence of major adverse events. Propofol-based sedation turned out to be the most effective regimen, with effectiveness comparable to general anesthesia. The addition of midazolam, fentanyl, remifentanil, and/or ketamine to propofol may increase the effectiveness without creating more adverse events. Data on midazolam-, ketamine- and sevoflurane-based sedation were generally too limited to draw conclusions. Conclusions: Despite a lack of RCTs containing all aspects of effectiveness and safety, the present evidence indicates propofol-based PS to be the best practice for PS in children undergoing GIE. Propofol can be safely administered by specifically trained nonanesthesiologists.

Severe hemolysis and methemoglobinemia following fava beans ingestion in glucose-6-phosphatase dehydrogenase deficiency—case report and literature review

IntroductionReduced concentrations of glucose-6-phospate dehydrogenase (G6PD) render erythrocytes susceptible to hemolysis under conditions of oxidative stress. In favism, the ingestion of fava beans induces an oxidative stress to erythrocytes, leading to acute hemolysis.DiscussionThe simultaneous occurrence of methemoglobinemia has been reported only scarcely, despite the fact that both phenomena are the consequence of a common pathophysiologic mechanism. The presence of methemoglobinemia has important diagnostic and therapeutic consequences. We report a previously healthy boy who presented with combined severe hemolytic anemia and cyanosis due to methemoglobinemia, following the ingestion of fava beans. His condition was complicated by the development of transient acute renal failure. A G6PD-deficiency was diagnosed. We review the literature on the combination of acute hemolysis and methemoglobinemia in favism. Pathophysiologic, diagnostic, and therapeutic aspects of this disorder are discussed.

Invasive pulmonary and central nervous system aspergillosis after near-drowning of a child: case report and review of the literature.

Invasive aspergillosis is extremely rare in immunocompetent children. Here we describe the clinical, radiologic, and laboratory course of fatal invasive pulmonary and central nervous system aspergillosis in a previously healthy child after a near-drowning incident with submersion in a pond. Findings were compared with data from the literature, which is reviewed. Serum Aspergillus galactomannan levels were determined retrospectively and were compared with the results of routine microbiological and radiologic examinations, showing a significant diagnostic and therapeutic delay of the routine diagnostic approach in comparison with the use of the Aspergillus galactomannan assay. This delay may have contributed to the fatal course. Serial determination of serum Aspergillus galactomannan may be helpful in diagnosing invasive aspergillosis early in case of pulmonary disease after near-drowning and may contribute to an early appropriate treatment. Currently voriconazole, eventually in combination with caspofungin, should be considered as the drug of choice in the management of invasive aspergillosis after near-drowning.

Pediatric illness severity measures predict delirium in a pediatric intensive care unit.

Context:Delirium in children is a serious but understudied neuropsychiatric disorder. So there is little to guide the clinician in terms of identifying those at risk. Objective:To study, in a pediatric intensive care unit (PICU), the predictive power of widely used generic pediatric mortality scoring systems in relation to the occurrence of pediatric delirium (PD). Design and Methods:Four-year prospective observational study, 2002–2005. Predictors used were the Pediatric Index of Mortality (PIM) and Pediatric Risk of Mortality (PRISM II). Setting:A tertiary 8-bed PICU in the Netherlands. Patients:877 critically ill children who were acutely, nonelectively, and consecutively admitted. Main Outcome Measure:Pediatric delirium. Main Results:Out of 877 children with mean age 4.4 yrs, 40 were diagnosed with PD (Cumulative incidence: 4.5%), 85% of whom (versus 40% with nondelirium) were mechanically ventilated. The area under the curve was 0.74 for PRISM II and 0.71 for the PIM, with optimal cut-off points at the 60th centile (PRISM: sensitivity: 76%; specificity: 62%; PIM: sensitivity: 82%; specificity: 62%). A PRISM II or PIM score above the 60th centile was strongly associated with later PD in terms of relative risk (PRISM II: risk ratio = 4.9; 95% confidence interval: 2.3–10.1; PIM: RR = 6.7; 95% confidence interval: 3.0–15.0). Given the low incidence of PD, values for positive predictive value were lower (PRISM II: 8.3%; PIM: 8.9%, rising to, respectively, 10.1% and 10.6% in mechanically ventilated patients) and values for negative predictive value were higher (PRISM II: 98.3%; PIM: 98.7%). Limitations:Given the relatively low incidence of delirium, a low detection rate biased toward the most severe cases cannot be excluded. Conclusions:Given the fact that PIM and PRISM II are widely used mortality scoring instruments, prospective associations with PD suggest additional value for ruling in, or out, patients at risk of PD.

Adherence to safety guidelines on paediatric procedural sedation: the results of a nationwide survey under general paediatricians in The Netherlands

Objectives Following two fatal accidents during paediatric procedural sedation (PS), the authors investigated the level of adherence to established safety standards on PS in a nationwide cohort of fully trained general paediatricians, entrusted with PS. Study design and methods: Sample survey Safety guidelines on PS were split into four domains (“Presedation Assessment”, “Monitoring during PS”, “Recovery after PS” and “Facilities and Competences for Emergencies and Rescue”). Each domain was operationalised into sub-domains and items. Items were presented within a questionnaire list as procedural points of attention on which respondents could give their personal adherence score. Percentages of full adherence were calculated. Non-adherence was defined as gradual deviation from full adherence. After factor and reliability analysis, observed scores were summed up to scales, and results were transformed to a 0–10 report mark (RM). An RM of ≥9 is considered as a satisfactory level of adherence while an RM <6 is considered as unacceptably low. Results Full adherence was rare. For most (sub) domains, only a minority of respondents achieved a satisfactory level of adherence. Large numbers of respondents had scores below 6. Conclusions Potentially unsafe PS practices are common under Dutch general paediatricians, despite the availability of guidelines. The design of guidelines should include a goal-directed plan for implementation including training, initiatives for continuous quality assurance and improvement and repeated measurements of adherence to guidelines.

Minimizing limb amputations in meningococcal sepsis by early microsurgical arteriolysis.

PURPOSE Surgical treatment of children with meningococcal sepsis has mainly involved debridement of necrotic skin and amputation of limbs. This resulted in major functional impairment. On the contrary, when early microsurgical arteriolysis was performed, freeing up the blood vessels, the impaired blood flow could be restored, thereby significantly reducing the amputation levels. METHODS We prospectively evaluated 14 patients affected by meningococcal sepsis. In 7 patients, microsurgical arteriolysis was performed; standard sepsis treatment was performed on the remaining 7. Ischemia levels on admission were compared with permanent amputation levels after 1 year. RESULTS Statistically significant decreases (P = .005) in ischemia values were achieved by the arteriolysis, in comparison with final amputation percentages. The functional impairment of the affected limbs was highly reduced compared with the probable loss of function observed on admission. CONCLUSIONS Our findings show that early microsurgical arteriolysis is a reliable method to reduce the devastating amputations normally found in patients with meningococcal sepsis. This significantly improves the functional outcome in severely ischemic limbs in meningococcal induced septic children.

Acute hepatic injury in four children with Dravet syndrome : Valproic acid, topiramate or acetaminophen?

We describe four children with Dravet syndrome treated with the combination of valproic acid (VPA) and topiramate (TPM) who developed transient liver toxicity. The time-interval between fever, administration of acetaminophen, epileptic status and liver enzyme disturbances in our four cases suggests that accumulation of toxic acetaminophen-metabolites is possibly responsible for liver toxicity. If acetaminophen and its metabolites cause those liver problems in children treated with the combination of VPA and TPM, the advice to use acetaminophen for treating fever in children using this combination, should be changed. Only future clinical observations and research can solve this clinical dilemma.

Pets or meds: how to tackle misery in a paediatric intensive care unit

Sir: Rates of mood disorder and delirium in patients admitted to an intensive care unit are high and associated with a poor prognosis, as evidenced by longer hospital stay, poorer functional and cognitive outcomes and higher mortality rates after discharge from hospital. Among the adult population, the incidence is particularly high in geriatric patients [1]. In paediatric patients, similarly high rates of psychiatric disorder, mainly mood disorder and delirium, are observed, with reported rates of 5–40% [2, 3]. Targeted pharmacological interventions may be used for primary and secondary prevention, but a common-sense population approach towards prevention in the form of extensive psychosocial interventions may be more productive, particularly in high-risk paediatric intensive care unit (PICU) settings [3–5]. Thus, parents’ presence and comfort throughout the day (and night), familiar music, favourite toys, pictures, lighting schedules and sometimes even fragrances may be used productively in all admitted patients. One of our patients, a 12-year-old girl, became less talkative, displayed a sad facial expression, and lost interest in the surrounding environment. She was known with spina bifida, Arnold Chiari type II malformation, and central hypoventilation during sleep. She was hospitalized because she required non-invasive ventilation during the night, and for a plastic surgery procedure. As she clearly developed an adjustment disorder with depressed mood, an intensification of the routine psychosocial protocol was attempted – guided by the words of colleague David Mrazek: “During the prolonged hospitalization of young children, the single most important aspect of treatment is to ensure that the primary attachment figures are

Beyond the drugs: nonpharmacologic strategies to optimize procedural care in children.

Purpose of review Painful and/or stressful medical procedures mean a substantial burden for sick children. There is good evidence that procedural comfort can be optimized by a comprehensive comfort-directed policy containing the triad of nonpharmacological strategies (NPS) in all cases, timely or preventive procedural analgesia if pain is an issue, and procedural sedation. Recent findings Based both on well-established theoretical frameworks as well as an increasing body of scientific evidence NPS need to be regarded an inextricable part of procedural comfort care. Summary Procedural comfort care must always start with a child-friendly, nonthreatening environment in which well-being, confidence, and self-efficacy are optimized and maintained. This requires a reconsideration of the medical spaces where we provide care, reduction of sensory stimulation, normalized professional behavior, optimal logistics, and coordination and comfort-directed and age-appropriate verbal and nonverbal expression by professionals. Next, age-appropriate distraction techniques and/or hypnosis should be readily available. NPS are useful for all types of medical and dental procedures and should always precede and accompany procedural sedation. NPS should be embedded into a family-centered, care-directed policy as it has been shown that family-centered care can lead to safer, more personalized, and effective care, improved healthcare experiences and patient outcomes, and more responsive organizations.

Vanishing spleen after Nissen fundoplication: a case report

Nissen fundoplication is a generally accepted treatment for severe gastro-oesophageal reflux after conservative management has failed. The surgical techniques and the complications that may develop following the operation have been well described. However, necrosis of the spleen is a rare complication. We report here a patient with Down syndrome with a vanishing spleen after a Nissen fundoplication, who died of overwhelming pneumococcal septic shock 7 months after the operation. Vascular anomaly in Down syndrome, inadvertent ligation of the splenic artery or volvulus of the spleen may have caused a compromised splenic arterial circulation.Conclusion Nissen fundoplication may be associated with vanishing spleen and, consequently, with devastating consequences.