Patient triage to specialist outpatient clinics-time to standardize terminology.

Objectives: The objectives of this study were to evaluate existing nomenclature used for outpatient referrals triage and propose a system, which meets the needs of McMaster Children's Hospital and can be modified for use in other hospitals.

Methods: We reviewed triage practices in the Department of Pediatrics, McMaster Children Hospital, Hamilton, Ontario and conducted a literature search to assess terminology used for outpatient referrals triage at other institutions.

Results: There is lack of unified terminology for triaging outpatient referrals.

Epidermal growth factor receptor deficiency: Expanding the phenotype beyond infancy.

Transmembrane tyrosine kinase receptors represent a fundamental mechanism for transducing extracellular signals into the activation of signaling cascades responsible for intercellular communication, embryogenesis and tissue integrity. The epidermal growth factor receptor (EGFR) is a canonical member of this family, regarded for its dysregulated function in various malignancies. Here, we describe a young female born prematurely with friable and immature skin who developed chronic diarrhea, recurrent gastrointestinal and respiratory infections, as well as an ichthyotic and inflammatory papulopustular rash accompanied by alopecia.

Anterior Ischemic Optic Neuropathy in a Child Receiving Chronic Hemodialysis.

Anterior ischemic optic neuropathy (AION) occurs due to hypoperfusion of the optic nerve and is a rare complication in patients receiving maintenance dialysis. To date, AION has only been reported in 22 children, all of whom were receiving peritoneal dialysis. We report the first case of AION in a 2-year 11-month-old child receiving chronic hemodialysis secondary to polycystic kidney disease from a phosphomannomutase 2 gene mutation.

Deviations from the expected relationship between serum FGF23 and other markers in children with CKD: a cross-sectional study.

Background: High levels of fibroblast growth factor-23 (FGF23) are associated with mortality. In chronic kidney disease (CKD), FGF23 levels rise as renal function declines. We analyzed the contribution of laboratory values to the variance of FGF23 levels in relationship to a curve of expected FGF23 levels for a given GFR.

A cross-sectional study measuring vanadium and chromium levels in paediatric patients with CKD.

Objectives: Although many secondary effects of high levels of vanadium (V) and chromium (Cr) overlap with symptoms seen in paediatric patients with chronic kidney disease (CKD), their plasma V and Cr levels are understudied.

Design: Ancillary cross-sectional study to a prospective, longitudinal, randomised controlled trial.

Setting: Children's Hospital of Western Ontario, London Health Sciences Centre, London, Ontario, Canada.

High prevalence of elevated molybdenum levels in pediatric CKD patients. A cross-sectional and longitudinal study .

Aims: Many of the secondary effects of high levels of molybdenum (Mo) overlap with symptoms commonly seen in pediatric patients with chronic kidney disease (CKD). We measured plasma Mo levels and examined the relationship between Mo levels and kidney function.

Materials And Methods: The study was carried out at the London Health Sciences Centre in London, Ontario, Canada with 36 children and adolescents 4 - 18 years of age with CKD.

Process Mapping in a Pediatric Emergency Department to Minimize Missed Urinary Tract Infections.

Urinary tract infections (UTIs) are common in young children and are seen in emergency departments (EDs) frequently. Left untreated, UTIs can lead to more severe conditions. Our goal was to undertake a quality improvement (QI) initiative to help minimize the number of children with missed UTIs in a newly established tertiary care pediatric emergency department (PED).

HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome.

Steroid-sensitive nephrotic syndrome (SSNS) accounts for >80% of cases of nephrotic syndrome in childhood. However, the etiology and pathogenesis of SSNS remain obscure. Hypothesizing that coding variation may underlie SSNS risk, we conducted an exome array association study of SSNS.

Effects of acute exercise on markers of inflammation in pediatric chronic kidney disease: a pilot study.

Background: Children and adolescents with chronic kidney disease (CKD) are chronically exposed to high levels of inflammation, placing them at an increased risk of secondary health complications. Regular exercise may represent an effective therapy to reduce inflammation. The aims of this pilot study were to determine the effects of acute exercise on inflammation and immune cell counts in CKD.

Strong anion determination in biological fluids by capillary electrophoresis for clinical diagnostics.

New methods for quantitative analysis of strong anions are required for diagnostic testing of human diseases. Current techniques suffer from poor selectivity and/or long analysis times that are not amenable for labile anions in high-saline or volume-restricted samples. We introduce a rapid assay (<5 min) based on capillary electrophoresis (CE) with indirect UV detection for simultaneous analysis of sulfate, sulfite, and chloride in human urine, plasma, and sweat specimens.

Cracking haematuria in adolescent with negative work up.

A 16-year-old girl was referred to our nephrology clinic with persistent visible haematuria (2 months), no urinary tract infection, proteinuria ranging from negligible to 1.5 g/l, normal kidney function and otherwise negative work up including immunology screen. After negative ultrasound scans of the kidneys and bladder, normal renal biopsy and normal cystoscopy, a CT angiogram was obtained.

The dangers of substance abuse in adolescents with chronic kidney disease: a review of the literature.

Although there exist no specific data on the prevalence of substance abuse among children and adolescents with chronic kidney diseases (CKD), the magnitude of this problem should not be underestimated, as almost half of twelfth-graders in the U.S. admit to a history of using illegal drugs at least once when asked (National Institute on Drug Abuse, 2011).

Measurement of saliva tacrolimus levels in pediatric renal transplant recipients.

The aim of this study was to investigate whether a strong and clinically applicable correlation exists between saliva and whole-blood tacrolimus levels measured by high-performance liquid chromatography-tandem mass spectrometry. A high degree of correlation would potentially allow pain-free saliva sample collection to replace blood sampling for the measurement of tacrolimus levels. Enrolled in the study were 37 children (24 boys) aged 8-18 years [median (IQR) 16.

Dual left anterior descending artery distribution.

Dual left anterior descending (LAD) coronary artery with distribution of the vessels from the left main coronary artery and the right aortic sinus of Valsalva is a rare coronary anomaly. Here, we report such a rare anomaly in a young female with anterior wall myocardial infarction and stenting of the 'short' LAD coronary artery, which was subsequently confirmed in the operating room and by multi-slice cardiac computerized tomography after surgery.

Clinical features in 17 paediatric patients with Wegener granulomatosis.

The aim of this report was to describe childhood patients with Wegener granulomatosis (WG) from one centre, to analyse the variety of clinical manifestations seen and compare the data with other published paediatric and adult series. The records of 17 patients with WG who were under the care of Great Ormond Street Hospital for Children (GOSH) from 1981 to 1998 were reviewed. We analysed presenting features before admittance to GOSH and the clinical signs observed whilst the children were under the care of the hospital.