Medicine and Health Sciences Commons^™

Rapid Fecal Calprotectin Testing To Assess For Endoscopic Disease Activity In Inflammatory Bowel Disease: A Diagnostic Cohort Study, Lukasz Kwapisz, Mahmoud Mosli, Nilesh Chande, Brian Yan, Melanie Beaton, Jessica Micsko, Pauline W. Mennill, William Barnett, Kevin Bax, Terry Ponich, John Howard, Anthony Tirolese, Robert Lannigan, James Gregor

Paediatrics Publications

Background and Aim: With increasing numbers of patients diagnosed with inflammatory bowel disease (IBD), it is important to identify noninvasive methods of detecting disease activity. The aim of this study is to examine the diagnostic accuracy of fecal rapid calprotectin (FC) testing in the detection of endoscopically active IBD. Patients and Methods: All consecutive patients presenting to outpatient clinics with lower gastrointestinal symptoms were prospectively recruited. Patients provided FC samples. Sensitivity (Sn), specificity (Sp), positive predictive value (PPV), and negative predictive value (NPV) for FC were calculated. Receiver-operator characteristics (ROC) curve was used to identify the ideal FC cutoff that …

Quality Of Life In Children With Adverse Drug Reactions: A Narrative And Systematic Review, Blanca R. Del Pozzo-Magaña, Michael J. Rieder, Alejandro Lazo-Langner

Paediatrics Publications

Aims Adverse drug reactions are a common problem affecting adults and children. The economic impact of the adverse drug reactions has been widely evaluated; however, studies of the impact on the quality of life of children with adverse drug reactions are scarce. The aim was to evaluate studies assessing the health-related quality of life of children with adverse drug reactions. Methods We conducted a systematic review that included the following electronic databases: MEDLINE, EMBASE and the Cochrane Library (including the Cochrane Database of Systematic Reviews, the Database of Abstracts of Reviews of Effects, the Cochrane Controlled Trials Register and the …

Using Simplified Blood Pressure Tables To Avoid Underdiagnosing Childhood Hypertension, Ajay P. Sharma, Javed Mohammed, Benson Thomas, Ram N. Singh, Guido Filler

Paediatrics Publications

Background: Recent studies have revealed that hypertension remains underdiagnosed in a significant number of children despite their recorded office blood pressure (OBP) exceeding the recommended fourth report OBP thresholds. Simplified OBP thresholds have been proposed to reduce this underdiagnosis of hypertension in children. In clinical practice, OBP screened as elevated according to the fourth report OBP thresholds are referred for ambulatory blood pressure (ABP) monitoring to rule out 'white coat' hypertension. Objectives: The present study tested the usefulness of simplified OBP thresholds to screen abnormal OBP for ABP monitoring referral. Methods: A total of 155 subjects were retrospectively analyzed with …

Retinoic Acid And The Development Of The Endoderm, Gregory M. Kelly, Thomas A. Drysdale

Paediatrics Publications

Retinoic acid (RA) is an important signaling molecule in the development of the endoderm and an important molecule in protocols used to generate endodermal cell types from stem cells. In this review, we describe the RA signaling pathway and its role in the patterning and specification of the extra embryonic endoderm and different endodermal organs. The formation of endoderm is an ancient evolutionary feature and RA signaling appears to have coevolved with the vertebrate lineage. Towards that end, we describe how RA participates in many regulatory networks required for the formation of extraembryonic structures as well as the organs of …

Pthrp-Related Hypercalcaemia In Infancy And Congenital Anomalies Of The Kidney And Urinary Tract (Cakut), Nardeen Kodous, Guido Filler, Ajay Parkash Sharma, Tamara Angela Van Hooren

Paediatrics Publications

BACKGROUND: Severe hypercalcaemia is a rare but clinically significant condition in infancy and childhood. Parathyroid hormone-related peptide (PTHrP)-mediated hypercalcaemia resulting from a malignancy is rare and only a handful of case reports have outlined its incidence alongside a benign condition.

OBJECTIVES: To describe the diagnostic workup and management of an infant with hypercalcaemia, renal dysplasia, and elevated PTHrP levels.

DESIGN: Case report.

SETTING: The Victoria Hospital campus of the London Health Sciences Centre in London, Ontario, Canada.

PATIENTS: A child with congenital anomalies of the kidneys and urinary tract (CAKUT), stage 2 chronic kidney disease (CKD), and renal dysplasia who …

Evidence-Based Recommendations For Genetic Diagnosis Of Familial Mediterranean Fever, Gabriella Giancane, Nienke M.Ter Haar, Nico Wulffraat, Sebastiaan J. Vastert, Karyl Barron, Veronique Hentgen, Tilmann Kallinich, Huri Ozdogan, Jordi Anton, Paul Brogan, Luca Cantarini, Joost Frenkel, Caroline Galeotti, Marco Gattorno, Gilles Grateau, Michael Hofer, Isabelle Kone-Paut, Jasmin Kuemmerle-Deschner, Helen J. Lachmann, Anna Simon, Erkan Demirkaya, Brian Feldman, Yosef Uziel, Seza Ozen

Paediatrics Publications

Familial Mediterranean fever (FMF) is a disease of early onset which can lead to significant morbidity. In 2012, Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched with the aim of optimising and disseminating diagnostic and management regimens for children and young adults with rheumatic diseases. The objective was to establish recommendations for FMF focusing on provision of diagnostic tools for inexperienced clinicians particularly regarding interpretation of MEFV mutations. Evidencebased recommendations were developed using the European League against Rheumatism standard operating procedure. An expert committee of paediatric rheumatologists defined search terms for the systematic literature review. …

Modulation Of Type Ii Tgf-Β Receptor Degradation By Integrin-Linked Kinase, Linda Vi, Stellar Boo, Samar Sayedyahossein, Randeep K. Singh, Sarah Mclean, Gianni M. Di Guglielmo, Lina Dagnino

Paediatrics Publications

Cutaneous responses to injury, infection, and tumor formation involve the activation of resident dermal fibroblasts and subsequent transition to myofibroblasts. The key for induction of myofibroblast differentiation is the activation of transforming growth factor-β (TGF-β) receptors and stimulation of integrins and their associated proteins, including integrin-linked kinase (ILK). Cross-talk processes between TGF-β and ILK are crucial for myofibroblast formation, as ILK-deficient dermal fibroblasts exhibit impaired responses to TGF-β receptor stimulation. We now show that ILK associates with type II TGF-β receptors (TβRII) in ligand- and receptor kinase activity-independent manners. In cells with targeted Ilk gene inactivation, cellular levels of TβRII …

Understanding Early Organogenesis Using A Simplified In Situ Hybridization Protocol In Xenopus, Steven J. Deimling, Rami R. Halabi, Stephanie A. Grover, Jean H. Wang, Thomas A. Drysdale

Paediatrics Publications

Organogenesis is the study of how organs are specified and then acquire their specific shape and functions during development. The Xenopuslaevis embryo is very useful for studying organogenesis because their large size makes them very suitable for identifying organs at the earliest steps in organogenesis. At this time, the primary method used for identifying a specific organ or primordium is whole mount in situ hybridization with labeled antisense RNA probes specific to a gene that is expressed in the organ of interest. In addition, it is relatively easy to manipulate genes or signaling pathways in Xenopus and in situ hybridization …

Nfkb1 Activation By The E26 Transformation-Specific Transcription Factors Pu.1 And Spi-B Promotes Toll-Like Receptor-Mediated Splenic B Cell Proliferation, Stephen K.H. Li, Ali K. Abbas, Lauren A. Solomon, Gaëlle M.N. Groux, Rodney P. Dekoter

Paediatrics Publications

Generation of antibodies against T-independent and T-dependent antigens requires Toll-like receptor (TLR) engagement on B cells for efficient responses. However, the regulation of TLR expression and responses in B cells is not well understood. PU.1 and Spi-B (encoded by Sfpi1 and Spib, respectively) are transcription factors of the E26 transformation-specific (ETS) family and are important for B cell development and function. It was found that B cells from mice knocked out for Spi-B and heterozygous for PU.1 (Sfpi1^+/- Spib^-/- [PUB] mice) proliferated poorly in response to TLR ligands compared to wild-type (WT) B cells. The NF-κB family member …

Estimation Of Gfr Using Β-Trace Protein In Children, Samantha H. Witzel, Shih Han S. Huang, Branko Braam, Guido Filler

Paediatrics Publications

Background and objectives Sexmay affect the performance of smallmolecularweight proteins as markers of GFR because of differences in fat mass between the two sexes. The hypothesis was that the diagnostic performance of b-trace protein, a novel marker of GFR, would be significantly better in boys than in girls. Design, setting, participants, & measurements GFR, height, weight, serum creatinine, and β-trace protein were measured in 755 children and adolescents (331 girls) undergoing 99technetium diethylenetriamine penta–acetic acid renal scans from July of 1999 to July of 2006. Boys and girls were separated into formula generation cohorts (284 boys and 220 girls) and …