Environmental Factors Associated With Risk of Crohn's Disease Development in the Crohn's and Colitis Canada - Genetic, Environmental, Microbial Project.

Background & Aims: To date, it is unclear how environmental factors influence Crohn's disease (CD) risk and how they interact with biological processes. This study investigates the association between environmental exposures and CD risk and evaluates their association with pre-disease biomarkers.

Methods: We studied 4289 healthy first-degree relatives (FDRs) of patients with CD from the Crohn's and Colitis Canada - Genetic, Environmental, Microbial (CCC-GEM) project.

Feasibility of intranasal human milk as stem cell therapy in preterm infants with intraventricular hemorrhage.

Objective: Intraventricular hemorrhage (IVH) is a common cause of preterm brain injury. Fresh parent's own milk (POM) contains pluripotent stem cells (SCs) that produce neuronal cells in-vitro. The permeable neonatal blood brain barrier potentially allows SC delivery.

Autosomal dominant ApoA4 mutations present as tubulointerstitial kidney disease with medullary amyloidosis.

Sporadic cases of apolipoprotein A-IV medullary amyloidosis have been reported. Here we describe five families found to have autosomal dominant medullary amyloidosis due to two different pathogenic APOA4 variants. A large family with autosomal dominant chronic kidney disease (CKD) and bland urinary sediment underwent whole genome sequencing with identification of a chr11:116692578 G>C (hg19) variant encoding the missense mutation p.

Gut Microbiome Composition Is Associated With Future Onset of Crohn's Disease in Healthy First-Degree Relatives.

Background & Aims: The cause of Crohn's disease (CD) is unknown, but the current hypothesis is that microbial or environmental factors induce gut inflammation in genetically susceptible individuals, leading to chronic intestinal inflammation. Case-control studies of patients with CD have cataloged alterations in the gut microbiome composition; however, these studies fail to distinguish whether the altered gut microbiome composition is associated with initiation of CD or is the result of inflammation or drug treatment.

Methods: In this prospective cohort study, 3483 healthy first-degree relatives (FDRs) of patients with CD were recruited to identify the gut microbiome composition that precedes the onset of CD and to what extent this composition predicts the risk of developing CD.

Utilization of neonatal sedation and anesthesia: an SPR survey.

Background: There is little data regarding the use of sedation and anesthesia for neonatal imaging, with practice patterns varying widely across institutions.

Objective: To understand the current utilization of sedation and anesthesia for neonatal imaging, and review the current literature and recommendations.

Materials And Methods: One thousand, two hundred twenty-six questionnaire invitations were emailed to North American physician members of the Society for Pediatric Radiology using the Survey Monkey platform.

Multi-institutional analysis of treatment modalities in basal ganglia and thalamic germinoma.

Background: Central nervous system (CNS) germinomas are treatment-sensitive tumors with excellent survival outcomes. Current treatment strategies combine chemotherapy with radiotherapy (RT) in order to reduce the field and dose of RT. Germinomas originating in the basal ganglia/thalamus (BGTGs) have proven challenging to treat given their rarity and poorly defined imaging characteristics.

Single lead VDD pacing in children with complete heart block.

Objective: To evaluate clinically transvenous single lead VDD pacing in children with complete heart block.

Design: Case series.

Setting: Tertiary care unit of a pediatric teaching hospital.

Roux-en-Y jejunostomy in the pediatric population.

Surgical access for nutrition is required in a variety of pediatric disorders. In some, the presence of gastroesophageal reflux, poor gastric emptying, and risks for fundoplication favor the use of a jejunostomy. The significant problems associated with the simple loop jejunostomy can be avoided by using the Roux-en-Y configuration.

Helicobacter pylori is not associated with nonspecific abdominal pain in children.

Nonspecific abdominal pain is a significant problem in the pediatric population, and there has been much recent interest in the role that Helicobacter pylori (HP) might play in this disorder. A retrospective review was conducted at our center to determine its prevalence among children with otherwise undiagnosed abdominal pain. The study was conducted over a 45-month period during which 47 patients underwent gastroscopy and antral biopsies in the workup of this problem.

Will a critical level of hyperventilation-induced hypocapnia always induce an absence seizure?

We wished to determine if the degree of hypocapnia correlates with increased frequency of absence seizures and if there is a critical pCO2 at which absence seizures are reliably provoked. Twelve untreated children with newly diagnosed absence epilepsy were continuously monitored by EEG and end-expiratory CO2 recording during quiet respiration and hyperventilation (to absence seizure or exhaustion) while breathing four gas mixtures: (a) room air, (b) 100% O2, (c) 4% CO2 in room air, or (d) 4% CO2 + 96% O2). In quiet respiration, a reduction in number of spike and wave bursts and total seconds of spike and wave was noted in children breathing supplemental CO2 (gases c and d vs.

Transvenous closure of patent ductus arteriosus in a sick 2780g infant.

A three-and-a-half-month-old 2780 g critically ill infant had successful transvenous coil occlusion of a ductus arteriosus. At six-and-a-half months of age the infant died, and autopsy showed coverage of the coil on the aortic aspect and no lumenal narrowing. It was concluded that transvenous coil occlusion of the patent ductus arteriosus in small infants is possible and that th venous route is preferred to be arterial route.

Benign rolandic epilepsy: atypical features are very common.

The objective of this study was to determine the frequency of atypical clinical and electrographic features in children with benign rolandic epilepsy. A retrospective case series design was employed in the setting of a tertiary care pediatric hospital. Forty-two children with benign rolandic epilepsy were seen through our neurology department between January 1, 1991, and December 31, 1993.

Survival in neonatal congenital hernia without extracorporeal membrane oxygenation support.

The experience with high-risk congenital diaphragmatic hernia (CDH) at an institution that does not offer extracorporeal membrane oxygenation (ECMO) was reviewed. Between January 1, 1983 and December 31, 1993, 38 children presented with Bochdalek-type CDH. Excluded were two infants with lethal cardiac anomalies and four presenting after 4 hours of age.

Dexamethasone therapy for bacterial meningitis: Better never than late?

A multicentre randomized controlled trial was conducted in children with bacterial meningitis using dexamethasone or placebo for four days within 24 h of starting antibiotics. Primary outcomes were hearing loss and neurological abnormalities at 12 months after meningitis. The dexamethasone (n=50) and placebo (n=51) groups were similar in age, severity of illness and etiological agent.

Thrombotic complications of saphenous central venous lines.

Authors of recent publications advocate central venous access by saphenous vein cutdown in the thigh. Even relatively inert SILASTIC catheters are recognized to convey a risk of large vein thrombosis when maintained for long periods. Thrombosis of the inferior vena cava (IVC) and tributaries place the patient at risk for a spectrum of problems not associated with superior vena cava (SVC) cannulation.

Acute and chronic toxicity of antiepileptic medications: a selective review.

Acute and chronic toxicity complicates all antiepileptic medications (AED) and is idiosyncratic. Acute toxicity can be categorized into 1) acute brain dysfunction or 2) acute organ dysfunction when AED's are started. Despite promising in vitro lymphocyte testing, anticipation of acute reactions cannot be offered.

Changing pattern of clinical illness in children with group A streptococcal bacteremia.

Objective: To test the hypothesis that bacteremia caused by group A streptococci (gas) has become more common and the presentation of the infection more severe in the Izaak Walton Killam Hospital for Children during the past decade.

Design: Retrospective analysis by laboratory log and chart review.

Setting: A pediatric teaching hospital providing primary and tertiary care.

Follow-up after patch aortoplasty for coarctation of aorta.

Background: Patch aortoplasty (PA) for coarctation of aorta (COA) can lead to aneurysm formation at the repair site. X-ray, echocardiogram and computed tomography are unreliable for diagnosis of this complication.

Objective: To evaluate prospectively patients with PA for COA by magnetic resonance imaging (MRI) to detect presence of aneurysm at the repair site.

Patients' characteristics and consumer satisfaction on an inpatient child psychiatric unit.

This paper reports the results of a chart review and consumer satisfaction evaluation of referrals to a tertiary care child psychiatric inpatient unit. The evaluation involved an examination of the types of child and family difficulties treated during the period of the study; the nature and extent of assessment, treatment and follow-up; treatment outcome and parental perception of factors related to treatment outcome; and the satisfaction of both the parents and referral sources. Results indicated that children admitted for treatment were a heterogeneous group with severe difficulties from families with a number of problems.

Long term therapy of cytomegalovirus retinitis with ganciclovir in a child with acquired immunodeficiency syndrome.

Cytomegalovirus retinitis is the most severe ophthalmological complication of patients with acquired immune deficiency syndrome (aids). Ganciclovir must be given continuously to control progression of the disease or relapse typically occurs. Data in children are limited; this report describes a nine-year-old boy with transfusion-acquired aids who was treated with ganciclovir for 23 months for control of cytomegalovirus retinitis.

Mycoplasma pneumoniae infection associated with central nervous system complications.

We describe two children who had central nervous system complications, encephalitis and meningoencephalitis, temporally associated with Mycoplasma pneumoniae. M pneumoniae was identified as the cause of the illnesses on the basis of at least a fourfold increase in complement fixation antibody titers. Despite extensive viral and bacterial investigation, no evidence of any other pathogen was found.

Inhibition of lymphocyte endothelial adhesion and in vivo lymphocyte migration to cutaneous inflammation by TA-3, a new monoclonal antibody to rat LFA-1.

Lymphocyte function-associated Ag-1 (LFA-1) or CD11a/CD18 mediates lymphocyte adhesion to cultured vascular endothelial cells (EC). Thus, LFA-1 likely plays a major role in lymphocyte migration out of the blood, but there is little information on this in vivo. Small peritoneal exudate lymphocytes (sPEL) and lymph node (LN) lymphoblasts adhere to cytokine-activated EC and preferentially migrate to cutaneous inflammatory sites.