Central project CP3 is intended to create a registry for pediatric and adult patients which allows histopathological parameters to be linked with clinical information and biomaterials - the so-called FOrMe Registry (The German Focal Segmental Glomerulosclerosis and Minimal Change Disease Registry).
The primary goals of the FOrMe Registry are
- Gaining insight into intracellular signaling pathways
- Finding novel diagnostic markers for, eventually, targeted treatment
- Generating new study ideas
Initially, this registry will be limited to the Cologne area (about 5 million inhabitants), but will go multi-centric after local initiation involving additional centers throughout Germany. This registry will be compatible with the established NEPTUNE (Nephrotic Syndrome Study Network) registry with respect to their operating procedures, structure, and data fields.
Virtual slides will be precisely annotated according to the NEPTUNE standard by an experienced nephropathologist after scanning and storing. Blood, tissue and urine samples will be stored in the Biobank of the Institute of Pathology (BioMaSOTA) of the University Hospital of Cologne. Genetic data and clinical data will be stored and made available through the tranSMART web-based user interface.
It is of major importance for the success of FOrMe that it will be a living registry with continuous support by the input of investigators, enabling scientific analyses in return. Any co-worker may request for scientific evaluations that are approved by a steering committee before data exchange. This open exchange with all co-workers within this resource to deliver data will be a real added value to the scientific community.
Tight collaboration of FOrMe with existing registries all over the world such as Hamburger Glomerulonephritis-Register, EURenOmics, PodoNet, Neptune, MWPNC, ChinaDiKip and others will boost podocyte research in Germany and internationally.
Inclusion Criteria (Pediatric)
- Idiopathic nephrotic syndrome
- Written informed consent of persons having care and custody of the child
Inclusion criteria (Adult)
- All patients (age ≥ 18) with renal-biopsy proven FSGS or MCD
Project related publications
Bockmeyer CL, Säuberlich K, Wittig J, Eßer M, Roeder SS, Vester U, Hoyer PF, Agustian PA, Zeuschner P, Amann K, Daniel C, Becker JU. Sci Rep. 2016 Aug 24;(6):31992.
Becker JU, Hoerning A, Schmid KW, Hoyer PF. Immigrating progenitor cells contribute to human podocyte turnover. Kidney Int. 2007 Dec;72(12):1468-73.
Gellermann J, Weber L, Pape L, Tönshoff B, Hoyer P, Querfeld U, for the Gesellschaft für Pädiatrische Nephrologie (GPN), Nephrotic Syndrome Study Group. Mycophenolate Mofetil vs. Cyclosporin A in Children with Frequently Relapsing Nephrotic Syndrome. J Am Soc Nephrol 2011, 24:1-9
Hackl Á, Cseprekál O, Geßner M, Liebau MC, Habbig S, Ehren R, Müller C, Taylan C, Dötsch J, Weber LT. Mycophenolate-mofetil therapy in children with idiopathic nephrotic syndrome. Does therapeutic drug monitoring make a difference? Ther Drug Monit 2016 38(2):274-279
Bartram MP, Habbig S, Pahmeyer C, Höhne M, Weber LT, Thiele H, Altmüller J, Kottoor N, Wenzel A, Krueger M, Schermer B, Benzing T, Rinschen MR, Beck BB. Three-layered proteomic characterization of a novel ACTN4 mutation unravels its pathogenic potential in FSGS. Human Molecular Genetics 2016 25(6):1152-1164
Büscher AK, Beck BB, Melk A, Hoefele J, Kranz B, Bamborschke D, Baig S, Lange-Sperandio B, Jungraithmayr T, Weber LT, Kemper MJ, Tönshoff B, Hoyer PF, Konrad M, Weber S; German Pediatric Nephrology Associatio. Rapid Response to Cyclosporin A and Favorable Renal Outcome in Nongenetic Versus Genetic Steroid-Resistant Nephrotic Syndrome. Clin J Am Soc Nephrol. 2016 11(2):245-53
Burst V, Grundmann F, Kubacki T, Greenberg A, Becker I, Rudolf D, Verbalis J. Thiazide-Associated Hyponatremia, Report of the Hyponatremia Registry: An Observational Multicenter International Study.
Am J Nephrol. 2017 Apr 19;45(5):420-430.
Jayne DR, Bruchfeld AN, Harper L, Schaier M, Venning MC, Hamilton P, Burst V, Grundmann F, Jadoul M, Szombati I, Tesař V, Segelmark M, Potarca A, Schall TJ, Bekker P; CLEAR Study Group.
Randomized Trial of C5a Receptor Inhibitor Avacopan in ANCA-Associated Vasculitis. J Am Soc Nephrol. 2017 Apr 11.