World Kidney Day 2016: Kidney Disease & Children World Kidney Day 2016: Kidney Disease & Children. Act Early to Prevent It!

World Kidney Day 2016: Kidney Disease & Children World Kidney Day 2016: Kidney Disease & Children. Act Early to Prevent It! Kidney disease affects millions of people worldwide, including many children who may be at risk at an early age. It is therefore crucial that we encourage and facilitate education, early detection and a healthy life style in children, to fight the increase of preventable kidney diseases and to treat children with inborn and acquired disorders of the kidneys worldwide.

Pediatric Kidney Disease – An Update Edited by the ASPN Clinical Affairs Committee

Glomerulonephritis (GN) Juan Kupferman, MD Albert Einstein School of Medicine

GN: Background GN is an important cause of morbidity and mortality in patients of all ages GN is characterized by glomerular injury and inflammation GN is defined by the presence of some or all of these clinico-biochemical characteristics Hematuria (always present) Proteinuria Edema Hypertension Most cases are due to an immunologic response to a variety of etiologic agents Normal glomerulus Acute glomerulonephritis

GN: Background GN has multiple etiologies and could be primary (affecting only the kidneys) or secondary to a systemic disease Most common: Post-Streptococcal GN (PSGN) IgA nephropathy (IgAN) Henoch-Schonlein purpura (HSP) Systemic lupus erythematosis (SLE)

GN: Background Acute post-Streptococcal GN is the most common glomerulonephritis in childhood Typically with low complement C3 and evidence of recent Streptococcal infection Other microorganisms can cause similar findings IgA nephropathy typically presents with recurrent episodes of gross hematuria Complement is normal Normal capillary loop (EM) Post-Streptococcal GN (EM)

GN: Background Henoch-Schonlein purpura nephritis occurs during or after resolution of the acute disease Similar renal histologic findings to IgA nephropathy Systemic lupus erythematosus can cause focal to diffuse proliferative kidney disease ANA is positive and complements C3 and C4 may be decreased

GN: Treatment Specific therapy depends on etiology Treatment goals Reduce glomerular inflammation Induce and maintain remission of proteinuria Improve kidney function, if decreased Control complications due to fluid retention (i.e. hypertension, edema) Therapeutic agents include Antiproteinuric agents (ACE inhibitors or ARBs) Fish oil (in IgA nephropathy) Steroids Immunosuppresive agents Cyclophosphamide Mycophenolate mofetil Rituximab Plasmapheresis

GN: New developments Hematuria could be a risk factor for chronic kidney disease progression in glomerular diseases -Moreno et al. Hematuria. Pediatr Nephrol 2015 Urine biomarkers, such as monocyte chemoattractant protein-1/CCL2 and neutrophil gelatinase-associated lipocalin, may be useful tools in the assessment of disease activity in lupus nephritis -Abulaban et al. Biomarkers. Curr Rheumatol Rep 2015

GN: Summary Characterized by glomerular injury and inflammation, often due to an immunologic response to a variety of etiologic agents There are a variety of immunomodulator therapies that must be tailored to the specific etiology Novel biomarkers and medications are being developed to enhance our ability to diagnose, predict prognosis, and treat. References: KDIGO guidelines at http://www.kdigo.orgclinical_practice_guidelines/pdf/KDIGO-GN-Guideline.pdf Henderson et al. Lupus nephritis 2012 Vecchio et al. IgA nephropathy. Cochrane Database Syst Rev 2015 Moreno et al. Hematuria. Pediatr Nephrol 2015 Abulaban et al. Biomarkers. Curr Rheumatol Rep 2015 Niaudet. Glomerulonephritis. Uptodate 2016 Walters. ANCA vasculitis. Pediatr Nephrol 2016

Happy World Kidney Day 2016! Your pediatric nephrology community continues to work hard to improve clinical care, foster education, and advance the science regarding kidney disease in children! We appreciate your support and all you do for children’s health care!