Perioperative Management and In-Hospital Outcomes After Minimally Invasive Repair of Pectus Excavatum: A Multicenter Registry Report From the Society for Pediatric Anesthesia Improvement Network.

Background: There are few comparative data on the analgesic options used to manage patients undergoing minimally invasive repair of pectus excavatum (MIRPE). The Society for Pediatric Anesthesia Improvement Network was established to investigate outcomes for procedures where there is significant management variability. For our first study, we established a multicenter observational database to characterize the analgesic strategies used to manage pediatric patients undergoing MIRPE.

Modern Management of the Failed Bladder Exstrophy Closure: A 50-yr Experience.

Background: A failed closure of classic bladder exstrophy (CBE) has a negative long-term impact on the patient and the health care system.

Objective: To investigate the outcomes of CBE patients with failed primary bladder closure.

Design, Setting, And Participants: A database of 1317 exstrophy-epispadias complex patients was retrospectively reviewed for CBE patients with failed primary bladder closure from 1965 to 2017 with subsequent repeat closure.

Postchemotherapy Immunization Practices for Non-HSCT Pediatric Oncology Patients.

Pediatric oncology patients treated with antineoplastic therapy have impaired immune systems that lead to loss of protective antibodies. They require reimmunization to protect against vaccine-preventable diseases. There are a paucity of studies on the clinical practice of pediatric oncologists and the available recommendations are heterogenous.

Predictors of a successful primary bladder closure in cloacal exstrophy: A multivariable analysis.

Purpose: To investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors' hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure.

Predictors and outcomes of perioperative blood transfusions in classic bladder exstrophy repair: A single institution study.

Background: Primary bladder closure of classic bladder exstrophy (CBE) is a major operation that occasionally requires intraoperative or postoperative (within 72 h) blood transfusions.

Objective: This study reported perioperative transfusion rates, risk factors for transfusion, and outcomes from a high-volume exstrophy center in primary bladder closure of CBE patients.

Study Design: A prospectively maintained, institutional exstrophy-epispadias complex database of 1305 patients was reviewed for primary CBE closures performed at the authors' institution (Johns Hopkins Hospital) between 1993 and 2017.

The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy.

Introduction: Newborns with classic bladder exstrophy (CBE) may present with a bladder template that is inadequate for closure in the neonatal period (figure). In these cases, a delayed primary closure (DPC) is conducted to permit growth of the bladder template. This study reports the surgical and long-term urinary continence outcomes of poor template CBE patients undergoing DPC and compares them to patients who underwent DPC for reasons unrelated to bladder quality (i.

Combined Bladder Neck Reconstruction and Continent Stoma Creation as a Suitable Alternative for Continence in Bladder Exstrophy: A Preliminary Report.

Objective: To explore the use of concomitant bladder neck reconstruction (BNR) and creation of a continent stoma (CS) in patients who are not quite eligible for BNR but still strongly desire volitional voiding.

Methods: The authors retrospectively reviewed an institutional database of patients with exstrophy-epispadias complex who underwent BNR-CS between 2000 and 2015. Indications for a BNR-CS, perioperative outcomes, and continence status were evaluated.

Neuroimaging Findings of Organic Acidemias and Aminoacidopathies.

Although individual cases of inherited metabolic disorders are rare, overall they account for a substantial number of disorders affecting the central nervous system. Organic acidemias and aminoacidopathies include a variety of inborn errors of metabolism that are caused by defects in the intermediary metabolic pathways of carbohydrates, amino acids, and fatty acid oxidation. These defects can lead to the abnormal accumulation of organic acids and amino acids in multiple organs, including the brain.

Achieving urinary continence in cloacal exstrophy: The surgical cost.

Introduction: Cloacal exstrophy (CE) is a severe midline congenital abnormality that requires numerous surgical corrections to achieve an acceptable quality of life. Candidates for urinary continence undergo multiple procedures, most often continent bladder diversions, to become socially dry. Here, the authors investigate the number of genitourinary interventions that patients with CE undergo to attain urinary continence.

Provider Communication, Prompts, and Feedback to Improve HPV Vaccination Rates in Resident Clinics.

Background And Objectives: Human papillomavirus (HPV) vaccination rates lag behind vaccination rates for other adolescent vaccines; a bundled intervention may improve HPV vaccination rates. Our objective is to evaluate the impact of quality improvement (QI) training plus a bundled practice-based intervention (provider prompts plus communication skills training plus performance feedback) on improving HPV vaccinations in pediatric resident continuity clinics.

Methods: Staff and providers in 8 resident clinics participated in a 12-month QI study.

Bladder Re-augmentation in Classic Bladder Exstrophy: Risk Factors and Prevention.

Objective: To characterize the causes of re-augmentation in patients with classic bladder exstrophy (CBE).

Methods: A prospectively maintained institutional database of 1327 exstrophy-epispadias complex patients was reviewed for patients with CBE who underwent more than 1 augmentation cystoplasty (AC) procedure. Data regarding bladder capacities, complications following AC, and reasons for re-augmentation were evaluated.

Pelvic and lower extremity immobilization for cloacal exstrophy bladder and abdominal closure in neonates and older children.

Introduction: Successful bladder closure in cloacal exstrophy (CE) is best accomplished through a multidisciplinary team and attention to pre- and postoperative technique. This study from a high volume exstrophy center investigates outcomes and complications of primary and reoperative bladder closures in patients immobilized with spica cast or patients with external fixation (EF) and skin traction.

Methods: The authors reviewed an institutionally approved and daily updated database of 1311 patients with exstrophy-epispadias complex and identified patients with cloacal exstrophy born between 1975 and 2015 who had undergone primary or reoperative bladder closures.

Congenital Neuronal Ceroid Lipofuscinosis with a Novel CTSD Gene Mutation: A Rare Cause of Neonatal-Onset Neurodegenerative Disorder.

Neuronal ceroid lipofuscinoses represent a heterogeneous group of early onset neurodegenerative disorders that are characterized by progressive cognitive and motor function decline, visual loss, and epilepsy. The age of onset has been historically used for the phenotypic classification of this group of disorders, but their molecular genetic delineation has now enabled a better characterization, demonstrating significant genetic heterogeneity even among individuals with a similar phenotype. The rare Congenital Neuronal Ceroid Lipofuscinosis (CLN10) caused by mutations in the gene encoding for cathepsin D is associated with a dramatic presentation with onset before or around birth.

Advanced Pediatric Neurosonography Techniques: Contrast-Enhanced Ultrasonography, Elastography, and Beyond.

Recent technical advances in neurosonography continue broadening the diagnostic utility, sensitivity, and specificity of ultrasound for detecting intracranial abnormalities bed side. The clinical and functional applications of neurosonography have significantly expanded since the 1980s when transcranial Doppler sonography first allowed anatomic and hemodynamic delineation of the intracranial vessels through the thin temporal skull. In the past few years, contrast-enhanced ultrasonography, elastography, 3D/4D reconstruction tools, and high-resolution microvessel imaging techniques have further enhanced the diagnostic significance of neurosonography.

Building a Community of Practice for Researchers: The International Network for Simulation-Based Pediatric Innovation, Research and Education.

The scope and breadth of simulation-based research is growing rapidly; however, few mechanisms exist for conducting multicenter, collaborative research. Failure to foster collaborative research efforts is a critical gap that lies in the path of advancing healthcare simulation. The 2017 Research Summit hosted by the Society for Simulation in Healthcare highlighted how simulation-based research networks can produce studies that positively impact the delivery of healthcare.

Cystectomy in the Pediatric Exstrophy Population: Indications and Outcomes.

Objective: To explore the long-term outcomes and indications for cystectomy in patients with bladder exstrophy. Although rare, cystectomy is the final surgical alternative to bladder repair among these patients with a poor quality bladder template.

Methods: A prospectively maintained database of 1298 patients with exstrophy-epispadias complex was reviewed for patients who underwent cystectomy between 1970 and 2015 at the authors' institution.

Climate Change-Related Water Disasters' Impact on Population Health.

Purpose: Rising global temperatures have resulted in an increased frequency and severity of cyclones, hurricanes, and flooding in many parts of the world. These climate change-related water disasters (CCRWDs) have a devastating impact on communities and the health of residents. Clinicians and policymakers require a substantive body of evidence on which to base planning, prevention, and disaster response to these events.

Early Preterm Birth Across Generations Among Whites and African-Americans: A Population-Based Study.

Objectives To determine the extent to which non-Latina White and African-American mother's gestational age is associated with extremely early (<30 weeks), modestly early (30-33 weeks), and late (34-36 weeks) infant preterm birth (PTB) rates. Methods Race-specific stratified and multivariable logistic regression analyses were performed on the Illinois Transgenerational Birth File of non-Latino White and African-American infants (born 1989-1991) and their mothers (born 1956-1976). Results White mothers (n = 184) born at <30 weeks had a greater extremely early infant PTB rate than White mothers (n = 131,980) born at term: 1.

The Surgical Management of Bladder Polyps in the Setting of Exstrophy Epispadias Complex.

Objective: To investigate the surgical course for treating bladder polyps in patients with exstrophy epispadias complex (EEC). EEC bladder polyps are unique pathologic entities, with a distinct difference between polyps discovered at birth and polyps developed after failed exstrophy closure.

Methods: A prospectively maintained database of 1300 patients with EEC was reviewed for bladder exstrophy patients with bladder polyps.

The effect of step stool use and provider height on CPR quality during pediatric cardiac arrest: A simulation-based multicentre study.

Objectives: We aimed to explore whether a) step stool use is associated with improved cardiopulmonary resuscitation (CPR) quality; b) provider adjusted height is associated with improved CPR quality; and if associations exist, c) determine whether just-in-time (JIT) CPR training and/or CPR visual feedback attenuates the effect of height and/or step stool use on CPR quality.

Methods: We analysed data from a trial of simulated cardiac arrests with three study arms: No intervention; CPR visual feedback; and JIT CPR training. Step stool use was voluntary.

Patient-Reported Impact of Pelvic Organ Prolapse on Continence and Sexual Function in Women With Exstrophy-Epispadias Complex.

Objective: This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function.

Design: Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire.

Effectiveness of the 13-valent pneumococcal conjugate vaccine against invasive pneumococcal disease in South African children: a case-control study.

Background: The 13-valent pneumococcal conjugate vaccine (PCV13) was designed to include disease-causing serotypes that are important in low-income and middle-income countries. Vaccine effectiveness estimates are scarce in these settings. South Africa replaced PCV7 with PCV13 in 2011 using a 2 + 1 schedule.