Severe pegaspargase hypersensitivity reaction rates (grade ≥3) with intravenous infusion vs. intramuscular injection: analysis of 54,280 doses administered to 16,534 patients on children's oncology group (COG) clinical trials.

PEGylated asparaginase (pegaspargase) can be administered via intramuscular (IM) injection or intravenous (IV) infusion with a hypersensitivity reaction (HSR) incidence ranging 3-41%. We evaluated grade ≥3 HSRs when given IM vs. IV on six Children's Oncology Group (COG) leukemia trials (2003-2015) to determine differences in HSR rates.

Allogeneic Hematopoietic Cell Transplantation Using Treosulfan-Based Conditioning for Treatment of Marrow Failure Disorders.

Hematopoietic cell transplantation (HCT) is effective in the treatment of inherited marrow failure disorders and other nonmalignant diseases. Conventional myeloablative conditioning regimens have been associated with high transplant-related mortality, particularly in patients with comorbid conditions. Here we report on 14 patients with marrow failure disorders (Shwachman-Diamond syndrome, n = 3; Diamond Blackfan anemia, n = 4; GATA2 deficiency, n = 2; paroxysmal nocturnal hemoglobinuria, n = 4; and an undefined marrow failure disorder, n = 1) who underwent HCT on a prospective, phase II, multicenter clinical trial.

Hematopoietic Stem Cell Transplantation Activity in Pediatric Cancer between 2008 and 2014 in the United States: A Center for International Blood and Marrow Transplant Research Report.

This Center for International Blood and Marrow Transplant Research report describes the use of hematopoietic stem cell transplantation (HSCT) in pediatric patients with cancer, 4408 undergoing allogeneic (allo) and3076 undergoing autologous (auto) HSCT in the United States between 2008 and 2014. In both settings, there was a greater proportion of boys (n = 4327; 57%), children < 10 years of age (n = 4412; 59%), whites (n = 5787; 77%), and children with a performance score ≥ 90% at HSCT (n = 6187; 83%). Leukemia was the most common indication for an allo-transplant (n = 4170; 94%), and among these, acute lymphoblastic leukemia in second complete remission (n = 829; 20%) and acute myeloid leukemia in first complete remission (n = 800; 19%) werethe most common.

Society of Pediatric Psychology Workforce Survey: Factors Related to Compensation of Pediatric Psychologists.

Objective: To summarize compensation results from the 2015 Society of Pediatric Psychology (SPP) Workforce Survey and identify factors related to compensation of pediatric psychologists.

Methods: All full members of SPP ( n  = 1,314) received the online Workforce Survey; 404 (32%) were returned with usable data. The survey assessed salary, benefits, and other income sources.

Differing perspectives on parent access to their child's electronic medical record during neonatal intensive care hospitalization: a pilot study.

Objective: To improve informed medical decision-making, principles for family-centered neonatal care recommend that parents have access to their child's medical record on an ongoing basis during neonatal intensive unit care (NICU) hospitalization. Currently, many NICUs do not allow independent parent access to their child's electronic medical record (EMR) during hospitalization. We undertook a cross-sectional survey pilot study of medical professionals and parents to explore opinions regarding this practice.

Modified Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy for Hepatoblastoma in a Small Infant: How Far Can We Push the Envelope?

: For patients with hepatoblastoma, a timely and complete resection of the tumor is critical to the patient's tumor recurrence-free survival. Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS), a 2-stage hepatectomy procedure, has revolutionized the surgical management of large hepatic tumors with insufficient future liver remnant (FLR) at presentation. Although existing data support the utility of ALPPS in adults with primary and metastatic hepatobiliary malignancy, the literature in children is scarce.

Scimitar Syndrome-Complex Surgical Revision 3 Decades After Repair.

We present a case of a 39-year-old woman with scimitar syndrome who had a 2-patch repair 3 decades previously and presented with a right-to-left shunt of the inferior vena cava (IVC) to the left atrium resulting from baffle dehiscence. We discuss details of our reoperative repair.

Dose-adapted post-transplant cyclophosphamide for HLA-haploidentical transplantation in Fanconi anemia.

We developed a haploidentical transplantation protocol with post-transplant cyclophosphamide (CY) for in vivo T-cell depletion (TCD) using a novel adapted-dosing schedule (25 mg/kg on days +3 and +4) for Fanconi anemia (FA). With median follow-up of 3 years (range, 37 days to 6.2 years), all six patients engrafted.

Society of Pediatric Psychology Workforce Survey: Development of Survey Methods, Sample Characteristics, and Lessons Learned.

There are few detailed workforce studies of specialty fields within professional psychology, and none have been reported for pediatric psychology since 2006. Availability of such data could facilitate more-informed decision making by students and trainees, psychologists pursuing employment opportunities, and psychologists involved in employment or compensation negotiations. This article describes the work of a task force of the American Psychological Association (APA) Division 54 (Society of Pediatric Psychology) in the design, construction, pretesting, distribution, and data management for the Society of Pediatric Psychology (SPP) Workforce Survey.

Nutritional Practices and Growth in Premature Infants After Surgical Necrotizing Enterocolitis.

Objective: The aim of the study was to describe the nutritional provisions received by infants with surgical necrotizing enterocolitis (NEC) and the associated effects on short-term growth.

Methods: Through the Children's Hospitals Neonatal Database, we identified infants born ≤32 weeks' gestation with surgical NEC from 5 regional neonatal intensive care units for 4 years. Excluded infants had isolated intestinal perforation and died <14 days postoperatively.

Clinicopathologic analysis of malignant effusions in pediatric patients.

Introduction: The utility of cytologic evaluation of effusion fluids in adults is well established but has been less well documented in the pediatric population. We examined the clinicopathologic characteristics of children with malignant effusions to establish the value of cytologic examination of these specimens.

Materials And Methods: Pleural, pericardial, peritoneal, and intraoperative washing specimens obtained between January 2000 and October 2015 were identified via surgical pathology database search.

Dried Blood Spot Reference Intervals for Steroids and Amino Acids in a Neonatal Cohort of the National Children's Study.

Background: Reference intervals from children are limited by access to healthy children and their limited blood volumes. In this study we set out to fill gaps in pediatric reference intervals for amino acids and steroid hormones using dried blood spots (DBS) from a cohort of the National Children's Study.

Methods: Deidentified DBS annotated with age, birthweight, sex, and geographic location were obtained from 310 newborns aged 0-4 days and analyzed for 25 amino acids and 4 steroid hormones using LC-MS/MS.

Analysis of in pediatric and adult glaucoma and other ocular phenotypes.

Purpose: The gene encodes an enzyme that is a member of the cytochrome P450 superfamily. Mutations in have been mainly reported in recessive pediatric ocular phenotypes, such as primary congenital glaucoma (PCG) and congenital glaucoma with anterior segment dysgenesis (CG with ASD), with some likely pathogenic variants also identified in families affected with adult-onset primary open angle glaucoma (POAG).

Methods: We examined in 158 pediatric patients affected with PCG (eight), CG with ASD (22), CG with other developmental ocular disorders (11), juvenile glaucoma with or without additional ocular anomalies (26), and ASD or other developmental ocular conditions without glaucoma (91); in addition, a large cohort of adult patients with POAG (193) and POAG-negative controls (288) was examined.

Infant gastrostomy outcomes: The cost of complications.

Purpose: Comparative outcomes of enhanced percutaneous endoscopic gastrostomy (PEG) and laparoscopic gastrostomy (LG) have not been elucidated in infants. We describe the outcomes and procedural episodic expenditures of PEG versus LG in this high-risk population.

Methods: One hundred eighty-three gastrostomies in children under 1year were reviewed from our institution spanning 1/2011-6/2015.

Placental TLR/NLR expression signatures are altered with gestational age and inflammation.

Objective: To quantify changes in placental expression of Toll-like receptors (TLRs) and nuclear oligomerization domain (NOD)-like receptors (NLRs) gene with (1) advancing gestational age (GA) and (2) exposure to chorioamnionitis (CA) and preterm premature rupture of membrane (PPROM).

Methods: Placental tissue was collected at the time of birth from 83 subjects with live birth pregnancies from 24- to 40-week gestation between 2009 and 2013. Real-time RT-PCR analysis of 13 TLR/NLR genes involved in bacterial sensing was performed using specific probes.

Equestrian-Related Traumatic Brain Injury in the Pediatric Population.

Background: Equestrian activities can be undertaken for competition or leisure. Accidents can lead to bodily injuries. In particular, traumatic brain injury (TBI) can be devastating.

Does FLT3 mutation impact survival after hematopoietic stem cell transplantation for acute myeloid leukemia? A Center for International Blood and Marrow Transplant Research (CIBMTR) analysis.

Background: Patients with FMS like tyrosine kinase 3 (FLT3)-mutated acute myeloid leukemia (AML) have a poor prognosis and are referred for early allogeneic hematopoietic stem cell transplantation (HCT).

Methods: Data from the Center for International Blood and Marrow Transplant Research (CIBMTR) were used to evaluate 511 adult patients with de novo AML who underwent HCT during 2008 through 2011 to determine whether FLT3 mutations had an impact on HCT outcomes.

Results: In total, 158 patients (31%) had FLT3 mutations.

Whole exome sequencing identifies multiple diagnoses in congenital glaucoma with systemic anomalies.

The genetic basis of congenital glaucoma with systemic anomalies is largely unknown. Whole exome sequencing (WES) in 10 probands with congenital glaucoma and variable systemic anomalies identified pathogenic or likely pathogenic variants in three probands; in two of these, a combination of two Mendelian disorders was found to completely explain the patients' features whereas in the third case only the ocular findings could be explained by the genetic diagnosis. The molecular diagnosis for glaucoma included two cases with compound heterozygous or homozygous pathogenic alleles in CYP1B1 and one family with a dominant pathogenic variant in FOXC1; the second genetic diagnosis for the additional systemic features included compound heterozygous mutations in NPHS1 in one family and a heterozygous 18q23 deletion in another pedigree.

Adverse events in children implanted with ventricular assist devices in the United States: Data from the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS).

Background: Ventricular assist devices (VADs) have been used in children on an increasing basis in recent years. One-year survival rates are now >80% in multiple reports. In this report we describe adverse events experienced by children with durable ventricular assist devices, using a national-level registry (PediMACS, a component of INTERMACS) METHODS: PediMACS is a national registry that contains clinical data on patients who are <19 years of age at the time of VAD implantation.

Isolated traumatic retroclival hematoma: case report and review of literature.

Background: Retroclival hematomas are a rare entity. The pathology can be categorized into epidural hematoma or subdural hematoma based on the anatomy of the tectorial membrane. Frequently, the etiology is related to accidental trauma, though other mechanisms have been observed, including coagulopathy, non-accidental trauma, and pituitary apoplexy.