Peri-Operative Patient Safety - An Interactive Workshop for Section 3 CPD Credits Developed in Collaboration with the CMPA.

Background: The Royal College of Physicians and Surgeons of Canada requires physicians to collect credit in continuing professional development courses including Section 3 credits which require feedback and self-assessment. This study aims to examine the effectiveness of offering Section 3 credits in a conference setting using an interactive workshop on peri-operative patient safety developed in collaboration with the Canadian Medical Protective Association (CMPA). Both the knowledge gained and the attitudes towards the conference were analysed.

Implementing Obstetrics Quality Improvement, Driven by Medico-legal Risk, is Associated With Improved Workplace Culture.

Objective: This study implemented a quality improvement program based on knowledge of medico-legal risk in obstetrics and sought to evaluate the impact of this program on workplace culture.

Methods: The study conducted needs assessments with front-line providers working in the obstetrical unit of the Queensway Carleton Hospital, an urban community hospital in Ottawa, Ontario, and included the safety, communication, operational reliability, and engagement (SCORE) survey. The study investigators delivered training in quality improvement science and co-developed three projects that were based on their alignment with local needs and aggregate medico-legal risk data: an organized team response to the need for an immediate cesarean section, a protocol for managing patients who present at term with pre-labour rupture of membranes, and regular morning team briefings.

Characterization of a novel disease-associated mutation within NPHS1 and its effects on nephrin phosphorylation and signaling.

Mutations in the transmembrane protein nephrin (encoded by NPHS1) underlie nearly half of all cases of congenital nephrotic syndrome (CNS), which is caused by aberrations in the blood filtering function of glomerular podocytes. Nephrin directly contributes to the structure of the filtration barrier, and it also serves as a signaling scaffold in podocytes, undergoing tyrosine phosphorylation on its cytoplasmic tail to recruit intracellular effector proteins. Nephrin phosphorylation is lost in several human and experimental models of glomerular disease, and genetic studies have confirmed its importance in maintenance of the filtration barrier.

Parental Health Literacy and Outcomes of Childhood Nephrotic Syndrome.

Objective: Determine the association of parental health literacy with treatment response among children with nephrotic syndrome.

Methods: This was a cohort study of children aged 1-18 with nephrotic syndrome and their parent. Health literacy was measured using the validated Short Test of Functional Health Literacy in Adults assessing reading comprehension and numeracy.

Ethnic Differences in Incidence and Outcomes of Childhood Nephrotic Syndrome.

Background And Objectives: Ethnic differences in outcomes among children with nephrotic syndrome are unknown.

Design, Setting, Participants, & Measurements: We conducted a longitudinal study at a single regional pediatric center comparing ethnic differences in incidence from 2001 to 2011 census data and longitudinal outcomes, including relapse rates, time to first relapse, frequently relapsing disease, and use of cyclophosphamide. Among 711 children, 24% were European, 33% were South Asian, 10% were East/Southeast Asian, and 33% were of other origins.

Parental attitudes to genetic testing differ by ethnicity and immigration in childhood nephrotic syndrome: a cross-sectional study.

Background: Studies in the USA report differences in opinion among parents of different ethnic groups toward genetic testing for their child; however, there are no studies that address this issue in the diverse ethnic and immigrant population in Canada.

Objective: This study aims to determine whether ethnicity and immigration status influences parental interest in clinical genetic testing for a potentially progressive kidney disease.

Design: This is a cross-sectional study.

A New Grading System for the Management of Antenatal Hydronephrosis.

Background And Objectives: Standard clinical assessments do not predict surgical intervention in patients with a moderate degree of upper tract hydronephrosis. This study investigated whether combined measures of renal calyceal dilation and anteroposterior diameter (APD) of the renal pelvis at the first postnatal ultrasound better predict surgical intervention beyond standard assessments of the APD or Society of Fetal Urology (SFU) grading system.

Design, Setting, Participants, & Measurements: A retrospective cohort of 348 children with antenatal hydronephrosis followed from 2003 to 2013 were studied.

The rationale and design of Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT): a prospective cohort study of childhood nephrotic syndrome.

Background: Nephrotic syndrome is one of the most commonly diagnosed kidney diseases in childhood and its progressive forms can lead to chronic kidney disease (CKD) and/or end-stage renal disease (ESRD). There have been few longitudinal studies among a multi-ethnic cohort to determine potential risk factors influencing disease susceptibility, treatment response, and progression of nephrotic syndrome. Temporal relationships cannot be studied through cross-sectional study design.

Notch promotes dynamin-dependent endocytosis of nephrin.

Notch signaling in podocytes causes proteinuria and glomerulosclerosis in humans and rodents, but the underlying mechanism remains unknown. Here, we analyzed morphologic, molecular, and cellular events before the onset of proteinuria in newborn transgenic mice that express activated Notch in podocytes. Immunohistochemistry revealed a loss of the slit diaphragm protein nephrin exclusively in podocytes expressing activated Notch.

Genetics of proteinuria: an overview of gene mutations associated with nonsyndromic proteinuric glomerulopathies.

Heritable causes of proteinuria are rare and account for a relatively small proportion of all cases of proteinuria affecting children and adults. Yet, significant contributions to understanding the mechanistic basis for proteinuria have been made through genetic and molecular analyses of a small group of syndromic and nonsyndromic proteinuric disorders which are caused by mutations encoding structural components of the glomerular filtration barrier. Technological advances in genomic analyses and improved accessibility to mutational screening at clinically approved laboratories have facilitated diagnosis of proteinuria in the clinical setting.

Lunatic Fringe-mediated Notch signaling is required for lung alveogenesis.

Distal lung development occurs through coordinated induction of myofibroblasts, epithelial cells, and capillaries. Lunatic Fringe (Lfng) is a beta(1-3) N-acetylglucosamine transferase that modifies Notch receptors to facilitate their activation by Delta-like (Dll1/4) ligands. Lfng is expressed in the distal lung during saccular development, and deletion of this gene impairs myofibroblast differentiation and alveogenesis in this context.

Remission of resistant MPGN type I with mycophenolate mofetil and steroids.

Very few studies have been published on how to treat children with membranoproliferative glomerulonephritis type I (MPGN I), and as yet there is only one report on the use of mycophenolate mofetil (MMF) in children with MPGN I. We report a 12-year-old boy who presented with edema, hypertension, nephrotic range proteinuria, and microscopic hematuria following an upper respiratory tract infection. Laboratory tests revealed a serum creatinine of 90 micromol/l, albumin of 20 g/l, and a C3 of 0.

Ectopic notch activation in developing podocytes causes glomerulosclerosis.

Genetic evidence supports an early role for Notch signaling in the fate of podocytes during glomerular development. Decreased expression of Notch transcriptional targets in developing podocytes after the determination of cell fate suggests that constitutive Notch signaling may oppose podocyte differentiation. This study determined the effects of constitutive Notch signaling on podocyte differentiation by ectopically expressing Notch's intracellular domain (NOTCH-IC), the biologically active, intracellular product of proteolytic cleavage of the Notch receptor, in developing podocytes of transgenic mice.

Expression of Hairy/Enhancer of Split genes, Hes1 and Hes5, during murine nephron morphogenesis.

Hairy/Enhancer of Split (Hes) genes encode transcriptional repressors that function as downstream targets of activated Notch receptors in cell fate decisions during tissue development. During nephrogenesis, multiple Notch pathway genes are co-expressed in multi-potent epithelial progenitors (i.e.

Elevated SMAD1/beta-catenin molecular complexes and renal medullary cystic dysplasia in ALK3 transgenic mice.

Renal dysplasia, the most frequent cause of childhood renal failure in humans, arises from perturbations in a complex series of morphogenetic events during embryonic renal development. The molecular pathogenesis of renal dysplasia is largely undefined. While investigating the role of a BMP-dependent pathway that inhibits branching morphogenesis in vitro, we generated a novel model of renal dysplasia in a transgenic (Tg) model of ALK3 receptor signaling.

The molecular control of renal branching morphogenesis: current knowledge and emerging insights.

Mammalian kidney development requires the formation of a patterned, branched network of collecting ducts, a process termed renal branching morphogenesis. Disruption of renal branching morphogenesis during human kidney development results in renal dysplasia, the major cause of renal failure in young children. Genetic evidence, combined with in vitro data, have implicated transcription factors, secreted growth factors, and cell surface signaling peptides as critical regulators of renal branching morphogenesis.