Identifying Predictors of Longitudinal Decline in the Level of Medical Care Received by Adult Survivors of Childhood Cancer: A Report from the Childhood Cancer Survivor Study.

Objectives: Characterize longitudinal changes in the use of medical care in adult survivors of childhood cancer.

Data Sources: The Childhood Cancer Survivor Study, a retrospective cohort study of 5+ year survivors of childhood cancer.

Study Design: Medical care was assessed at entry into the cohort (baseline) and at most recent questionnaire completion.

Capturing the clinical utility of genomic testing: medical recommendations following pediatric microarray.

Interpretation of pediatric chromosome microarray (CMA) results presents diagnostic and medical management challenges. Understanding management practices triggered by CMA will inform clinical utility and resource planning. Using a retrospective cohort design, we extracted clinical and management-related data from the records of 752 children with congenital anomalies and/or developmental delay who underwent CMA in an academic pediatric genetics clinic (2009-2011).

Intestinal obstruction in pregnancy by ondansetron.

Background: Ondansetron use for nausea and vomiting during pregnancy has increased in the last years, although its maternal and fetal safety is not conclusive.

Case: We describe a case of intestinal obstruction in a pregnant woman with severe nausea and vomiting of pregnancy treated with ondansetron, which is known to slow gut motility.

Conclusion: The spontaneous reporting system of WHO confirms that this potentially life threatening complication is more common than what the peer review literature may suggest and needs to be looked into carefully, especially in view of the wide spread off-label use for NVP.

Meta-analysis of SHANK Mutations in Autism Spectrum Disorders: a gradient of severity in cognitive impairments.

SHANK genes code for scaffold proteins located at the post-synaptic density of glutamatergic synapses. In neurons, SHANK2 and SHANK3 have a positive effect on the induction and maturation of dendritic spines, whereas SHANK1 induces the enlargement of spine heads. Mutations in SHANK genes have been associated with autism spectrum disorders (ASD), but their prevalence and clinical relevance remain to be determined.

Structural network analysis of brain development in young preterm neonates.

Preterm infants develop differently than those born at term and are at higher risk of brain pathology. Thus, an understanding of their development is of particular importance. Diffusion tensor imaging (DTI) of preterm infants offers a window into brain development at a very early age, an age at which that development is not yet fully understood.

The development of scales to measure childhood cancer survivors' readiness for transition to long-term follow-up care as adults.

Purpose: To develop and validate scales to measure constructs that survivors of childhood cancer report as barriers and/or facilitators to the process of transitioning from paediatric to adult-oriented long-term follow-up (LTFU) care.

Methods: Qualitative interviews provided a dataset that were used to develop items for three new scales that measure cancer worry, self-management skills and expectations about adult care. These scales were field-tested in a sample of 250 survivors aged 15-26 years recruited from three Canadian hospitals between July 2011 and January 2012.

Development and validation of the pediatric liver transplantation quality of life: a disease-specific quality of life measure for pediatric liver transplant recipients.

Objectives: To develop and validate a Pediatric Liver Transplantation Quality of Life (PeLTQL) questionnaire via an international multicenter collaboration.

Study Design: Item generation with 146 child and/or parent interviews (92 pediatric liver transplantation [LT] recipients) and 3 focus groups generated over 300 items. An item reduction questionnaire with 76 questions was completed by 320 participants (212 pediatric LT recipients).

Time course of cochlear injury discharge (excitotoxicity) determined by ABR monitoring of contralateral cochlear events.

The dynamics of cochlear excitotoxicity can be monitored from effects on the contralateral ear. After unilateral mechanical ablation of the cochlea (in a mouse model) we observed immediate elevations in auditory brainstem evoked response (ABR) thresholds in the contralateral ear. Threshold elevations peaked at 2-3 h post ablation, and returned to baseline levels after 5-6 h.

The protective effects of nausea and vomiting of pregnancy against adverse fetal outcome--a systematic review.

Unlabelled: Studies have suggested that nausea and vomiting of pregnancy (NVP) may confer favorable pregnancy outcome, when compared to women not experiencing NVP. However, this was never examined systematically.

Methods: We systematically reviewed all human studies examining potential effects of NVP on rates of miscarriage, intrauterine growth restriction, congenital malformations, prematurity and developmental achievements.

Advantageous information provided by magnetoencephalography for patients with neocortical epilepsy.

Purpose: We evaluated whether magnetoencephalography (MEG), in addition to surgery, was valuable for the diagnosis and management of epileptic syndromes in patients with neocortical epilepsy (NE).

Methods: We studied MEG in 73 patients (29 females; aged 1-26years; mean 10.3years) for the clinical diagnosis of epilepsy and for preoperative evaluation.

Preferential cephalic redistribution of left ventricular cardiac output during therapeutic hypothermia for perinatal hypoxic-ischemic encephalopathy.

Objective: To determine the relationship between left ventricular cardiac output (LVCO), superior vena cava (SVC) flow, and brain injury during whole-body therapeutic hypothermia.

Study Design: Sixteen newborns with moderate or severe hypoxic-ischemic encephalopathy were studied using echocardiography during and immediately after therapeutic hypothermia. Measures were also compared with 12 healthy newborns of similar postnatal age.

Defining consensus: a systematic review recommends methodologic criteria for reporting of Delphi studies.

Objective: To investigate how consensus is operationalized in Delphi studies and to explore the role of consensus in determining the results of these studies.

Study Design And Settings: Systematic review of a random sample of 100 English language Delphi studies, from two large multidisciplinary databases [ISI Web of Science (Thompson Reuters, New York, NY) and Scopus (Elsevier, Amsterdam, NL)], published between 2000 and 2009.

Results: About 98 of the Delphi studies purported to assess consensus, although a definition for consensus was only provided in 72 of the studies (64 a priori).

Fatal combined immunodeficiency associated with heterozygous mutation in STAT1.

Background: Mutations in the gene for the signal transducer and activator of transcription 1, STAT1, have been shown to be associated with death at an early age due to overwhelming viral infection (complete STAT1 deficiency) or, more commonly, selective deficiencies to mycobacterial or fungal infection (typically heterozygous STAT1 mutations).

Objectives: To define the molecular basis of progressive combined immunodeficiency in a group of patients with fatal infections.

Methods: We studied a group of unrelated patients who displayed an unusual progressive form of combined immunodeficiency.

A comparative analysis of early child health and development services and outcomes in countries with different redistributive policies.

Background: The social environment is a fundamental determinant of early child development and, in turn, early child development is a determinant of health, well-being, and learning skills across the life course. Redistributive policies aimed at reducing social inequalities, such as a welfare state and labour market policies, have shown a positive association with selected health indicators. In this study, we investigated the influence of redistributive policies specifically on the social environment of early child development in five countries with different political traditions.

Hemophagocytic lymphohistiocytosis associated with parechovirus 3 infection.

Hemophagocytic lymphohistiocytosis (HLH) denotes the common final pathway of a potentially fatal hyperinflammatory condition of diverse etiologies. We describe the first case of documented HLH associated with human parechovirus 3. A monoallelic Ala91Val mutation was found in the PRF1 gene, but the contribution of this mutation to HLH remains controversial.

Resuscitation intensity at birth is associated with changes in brain metabolic development in preterm neonates.

Introduction: Intensive resuscitation at birth has been linked to intraventricular haemorrhage (IVH) in the preterm neonate. However, the impact of less intensive resuscitation on more subtle alterations in brain metabolic development is largely unknown. Our objective was to determine the relationship between the intensity of neonatal resuscitation following preterm birth on brain metabolic development.

Total intravenous anesthesia affecting spike sources of magnetoencephalography in pediatric epilepsy patients: focal seizures vs. non-focal seizures.

Purpose: Magnetoencephalography (MEG) provides source localization of interictal spikes. This study evaluated the inhibitory effects of propofol on MEG spike sources (MEGSSs) among different types of seizures in patients who underwent two separate MEG studies with and without total intravenous anesthesia (TIVA) using propofol.

Methods: We studied 19 children (1-14 years; mean, 6.

Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study.

Background: A bias favoring biventricular (BV) repair exists regarding choice of repair pathway for patients with pulmonary atresia with intact ventricular septum (PAIVS). We sought to determine the implications of moving borderline candidates down a BV route in terms of late functional health status (FHS) and exercise capacity (EC).

Methods: Between 1987 and 1997, 448 neonates with PAIVS were enrolled in a multi-institutional study.

Pain assessment and intensity in hospitalized children in Canada.

Unlabelled: Numerous acute pediatric pain assessment measures exist; however, pain assessment is not consistently performed in hospitalized children. The objective of this study was to determine the nature and frequency of acute pain assessment in Canadian pediatric hospitals and factors influencing it. Pain assessment practices and pain intensity scores documented during a 24-hour period were collected from 3,822 children aged 0 to 18 years hospitalized on 32 inpatient units in 8 Canadian pediatric hospitals.

Pulmonary alveolar proteinosis in patients with adenosine deaminase deficiency.

Background: Inherited defects in the function of adenosine deaminase (ADA) cause severe combined immunodeficiency (SCID) and affect many other cells and tissues.

Objectives: We sought to characterize the frequency and features of pulmonary alveolar proteinosis (PAP) in patients with ADA deficiency.

Methods: Clinical and laboratory features of all patients with SCID caused by ADA deficiency in a single center were analyzed.

Airway pressure release ventilation improves pulmonary blood flow in infants after cardiac surgery.

Objective: We hypothesized that spontaneous inspiratory effort transmitted to the pleural space during airway pressure release ventilation would result in increased lung perfusion after surgery for tetralogy of Fallot or following a cavopulmonary shunt as a consequence of transient decreases in intrapleural pressure.

Design: Prospective crossover cohort study.

Setting: A tertiary care cardiac pediatric intensive care unit.

Hematopoietic stem cell transplantation for CD3δ deficiency.

Background: CD3δ deficiency is a fatal form of severe combined immunodeficiency that can be cured by hematopoietic stem cell transplantation (HSCT). The presence of a thymus loaded with T-cell progenitors in patients with CD3δ deficiency may require special considerations in choosing the regimen of conditioning and the type of HSCT.

Objectives: To study the outcome of CD3δ deficiency by using various modalities of stem cell transplantation.