Identification of genetic risk factors for cerebellar mutism in pediatric brain tumor patients

Following posterior fossa surgery for resection of childhood medulloblastoma and primitive neuroectodermal tumor (M/PNET), cerebellar mutism (CM) may develop. This is a condition of absent or diminished speech in a conscious patient with no evidence of oral apraxia, which can be accompanied by other symptoms of the posterior fossa syndrome complex, which includes ataxia and hypotonia. Little is known about the etiology. Therefore, we conducted a SNP, gene, and pathway-level analysis to assess the role of host genetic variation on the risk of CM in M/PNET subjects following treatment. Cases (n= 20) and controls (n= 53) were recruited from the Childhood Cancer Epidemiology and Prevention Center, in Houston, TX. DNA samples were genotyped using the Illumina Human 1M Quad SNP chip. Ten pathways were identified from logistic regression used to identify the marginal effect of each SNP on CM risk. The minP test was conducted to identify associations between SNPs categorized to genes and CM risk. Pathways were assessed to determine if there was a significant enrichment of genes in the pathway compared to all other pathways. There were 78 genes that reached the threshold of min P ≤0.05 in 948 genes. The Neurotoxicity pathway was the most significant pathway after adjusting for multiple comparisons (q=0.040 and q=0.005, using Fisher's exact test and a test of proportions, respectively). Most genes within the Neurotoxicity pathway that reached a threshold of minP ≤0.05 were known to have an apoptosis function, possibly inducing neuronal apoptosis in the dentatothalamocortical pathway, and may be important in CM etiology in this population. This is the first study to assess the potential role of genetic risk factors on CM. As an exploratory study, these results should be replicated in a larger sample. ^

Debriefing after resuscitations in a pediatric emergency department

Multiple guidelines recommend debriefing of actual resuscitations to improve clinical performance. We implemented a novel standardized debriefing program using a Debriefing In Situ Conversation after Emergent Resuscitations Now (DISCERN) tool. Following the development of the evidence-based DISCERN tool, we conducted an observational study of all resuscitations (intubation, CPR, and/or defibrillation) at a pediatric emergency department (ED) over one year. Resuscitation interventions, patient survival, and physician team leader characteristics were analyzed as predictors for debriefing. Each debriefing's participants, time duration, and content were recorded. Thematic content of debriefings was categorized by framework approach into Team Emergency Assessment Measure (TEAM) elements. There were 241 resuscitations and 63 (26%) debriefings. A higher proportion of debriefings occurred after CPR (p<0.001) or ED death (p<0.001). Debriefing participants always included an attending and nurse; the median number of staff roles present was six. Median interval (from resuscitation end to start of debriefing) & debriefing durations were 33 (IQR 15,67) and 10 minutes (IQR 5,12), respectively. Common TEAM themes included co-operation/coordination (30%), communication (22%), and situational awareness (15%). Stated reasons for not debriefing included: unnecessary (78%), time constraints (19%), or other reasons (3%). Debriefings with the DISCERN tool usually involved higher acuity resuscitations, involved most of the indicated personnel, and lasted less than 10 minutes. This qualitative tool could be adapted to other settings. Future studies are needed to evaluate for potential impacts on education, quality improvement programming, and staff emotional well-being.^

Weight-for-age percentile and its association to community acquired methicillin resistant Staphylococcus aureus among pediatric patients in Houston, TX

BACKGROUND: Weight has been implicated as a risk factor for symptomatic community-acquired methicillin resistant Staphylococcus Aureus (CA-MRSA). Information from Texas Children's Hospital (TCH) in Houston, TX was used to implement a case-control study to assess weight-for-age percentile (WFA), race and seasonal exposure as risk factors. ^ METHODS: A retrospective chart review to collect data from TCH was conducted covering the time period January 1st, 2008 to May 31st, 2011. Cases were confirmed and identified by the infectious disease department and were matched on a 1:1 ratio to controls that were seen by the emergency department for non-infected fractures from June 1st, 2008 to May 31st, 2011. Data abstraction was performed using TCH's electronic medical records (EMR) system (EPIC ®). ^ RESULTS: Of 702 CA-MRSA identified cases, ages 9 to 16.99, 564 (80.3%) had the variable `weight' present in their EMR, were not duplicates and not determined to be outliers. Cases were randomly matched to a pool of available controls (n=1864) according to age and gender, yielding 539 1:1 matched pairs (95.5% case matching success) with a total study sample size, N=1078. Case median age was 13.38 years with the majority being White (66.05%) and male (59.4%). Adjusted conditional logistic regression analysis of the matched pairs identified the following risk factors to presenting with CA-MRSA infection among pediatric patients, ages 9 to 16.99 years: a) Individual weight in the highest (75th-99.9th) WFA quartile (OR=1.36; 95% confidence interval [CI]=1.06-1.74; P= 0.016), b) Infection during summer months (OR: 1.69; 95% CI=1.2-2.38; P= 0.003), c) patients of African American race/ethnicity (OR= 1.48; 95% CI=1.13-1.95; P= 0.004). ^ CONCLUSIONS: Pediatric patients, 9 to 16.99 years of age, in the highest WFA quartile (75th-99.9th), or of African-American race had an associated increased risk of presenting with CA-MRSA infection. Furthermore, children in this population were at a higher risk of contracting CA-MRSA infection during the summer season.^

Surgery, its principles and practice,

"The first six volumes of this work recorded the progress of Surgery down to 1913. Then came the Great War ... Hence, immediately after the Armistice ... I began to assemble a full staff of experts, whose work appears in Volumes VII and VIII."--Preface, v.7, 1921.

Mitral Valve Surgery for Rheumatic Lesions in Young Patients

BACKGROUND: The appropriateness of rheumatic mitral valve repair remains controversial due to the risks of recurrent mitral dysfunction and need for reoperation. The aims of this study were to determine the overall short- and long-term outcomes of pediatric rheumatic mitral valve surgery in our center. METHODS: Single-center, observational, retrospective study that analyzed the results of rheumatic mitral valve surgery in young patients, consecutively operated by the same team, between 1999 and 2014. RESULTS: We included 116 patients (mean age = 12.6 ± 3.5 years), of which 66 (57%) were females. A total of 116 primary surgical interventions and 22 reoperations were performed. Primary valve repair was possible in 86 (74%) patients and valve replacement occurred in 30 (26%). Sixty percent of the patients were followed up beyond three months after surgery (median follow-up time = 9.2 months [minimum = 10 days; maximum = 15 years]). Long-term clinical outcomes were favorable, with most patients in New York Heart Association functional class I (89.6%) and in sinus rhythm (85%). Freedom from reoperation for primary valve repair at six months, five years, and ten years was 96.4% ± 0.25%, 72% ± 0.72%, and 44.7% ± 1.34%, respectively. Freedom from reoperation for primary valve replacement at six months, five years, and ten years was 100%, 91.7% ± 0.86%, and 91.7% ± 0.86%, respectively. Mitral stenosis as the primary lesion dictated early reintervention. CONCLUSIONS: Despite the greater rate of reoperation, especially when the primary lesion was mitral stenosis, rheumatic mitral valve repair provides similar clinical outcomes as compared with replacement, with the advantage of avoiding anticoagulation.

Analysis of Outcome of Intraplueral Streptokinase in Pediatric Empyema Thoracis even in Advanced Stages: A Prospective Study

Background: Empyema thoracis in children causes significant morbidity. Standard treatment of Empyema thoracis includes tube drainage and antibiotics. But the tube drainage often fails. Intrapleural Streptokinase has been used in empyema thoracis with good success rate. Objectives: We evaluated the efficacy of intra-pleural Streptokinase in management of empyema thoracis even in advanced stages. Patients and Methods: A total of 28 patients with empyema thoracis requiring intercostal tube drainage aged zero to twelve years were included in the study who were admitted in Pediatric intensive care unit. 15,000 units/kg of Streptokinase was instilled into the pleural cavity. Response was assessed by clinical outcome, after unclamping and subsequent chest radiography and serial chest ultrasounds. Results: Streptokinase enhanced drainage in all patients with complete resolution of empyema thoracis in 26 patients. Two patients were referred for surgery. Only 7.2% required surgery. Streptokinase was equally effective if started before or after seven days. Conclusions: Intrapleural Streptokinase is the preferred treatment for treating pediatric empyema thoracis even in advanced stages and can avoid surgery.

Pediatric Intussusception in Northern Iran: Comparison of Recurrent With Non-Recurrent Cases

Background: Intussusception represents as the invagination of a part of the intestine into itself and is the most common cause of intestinal obstruction in infants and children between 6 months to 3-years-old. Objectives: The objective of this study was to determine the recurrence rate and predisposing factors of recurrent intussusception. Patients and Methods: The medical records of children aged less than 13-years-old with confirmed intussusception who underwent reduction at a tertiary academic care in northern Iran (Mazandran), from 2001 to 2013 were reviewed. Data were extracted and recurrence rate was determined. The two groups were compared by chi square, Fisher, Mann-Whitney and t-test. Diagnosed cases of intussusception consisted of 237 children. Results: Average age of the patients was 19.57 ± 19.43 months with a peak of 3 to 30 months. Male to female ratio was 1.65 and this increased by aging. Recurrence rate was 16% (38 cases). 87 (36.7%) underwent surgery. These were mainly children under one year old. In 71% (40) of episodes recurrence occurred 1 to 7 times within 6 months. The recurrence occurred in 29 (23.5%) children in whom a first reduction was achieved with barium enema (BE) and 5 (5.7%) children who had an operative reduction (P < 0.001) in the first episode. Pathological leading points (PLPs) were observed in 5 cases; 2.6% in recurrence group versus 2% in non-recurrence group (P = 0.91). Three patients had intestinal polyp, 2 patient’s lymphoma and Mackle’s diverticulum. Age (P = 0.77) and sex (P = 0.38) showed no difference between the two groups. PLPs were observed in 1.4% of children aged 3 months to 5 years. This was 13.3%, in older children (P = 0.02). Conclusions: The recurrence of intussusception was related to the method of treatment in the first episode and it was 5-fold higher in children with BE than in operative reduction. Recurrent intussusceptions were not associated with PLPs, they were more idiopathic.

Pediatric deceased donation — a report of the Transplantation Society meeting in Geneva

The Ethics Committee of The Transplantation Society convened a meeting on pediatric deceased donation of organs in Geneva, Switzerland, on March 21 to 22, 2014. Thirty-four participants from Africa, Asia, the Middle East, Oceania, Europe, and North and South America explored the practical and ethical issues pertaining to pediatric deceased donation and developed recommendations for policy and practice. Their expertise was inclusive of pediatric intensive care, internal medicine, and surgery, nursing, ethics, organ donation and procurement, psychology, law, and sociology. The report of the meeting advocates the routine provision of opportunities for deceased donation by pediatric patients and conveys an international call for the development of evidence-based resources needed to inform provision of best practice care in deceased donation for neonates and children.