Central project CP3 has created 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 was limited to the Cologne area (about 5 million inhabitants) but is now expanding throughout Germany. This registry is adapted from the established NEPTUNE (Nephrotic Syndrome Study Network) registry with respect to its operating procedures, structure, and data fields.
Virtual slides will be precisely annotated according to a standardized descriptor system by an experienced team of nephropathologists 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 an up-to-date online database (www.forme-register.de). Of note, all registered patients can obtain a genetic workup free of charge. 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 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 benefit to the scientific community.
In addition, we are working on making the database more and more user-friendly. Our goal is to automatically transfer as much clinical data and laboratory parameters as possible into the database and thus preserve the limited resources in the health care system. Another goal is to re-program the database in order to allow further projects to adapt it to their needs at little cost.
PROJECT RELATED PUBLICATIONS
Weber, L.T., Tonshoff, B., Grenda, R., Bouts, A., Topaloglu, R., Gulhan, B., Printza, N., Awan, A., Battelino, N., Ehren, R., Hoyer, P.F., Novljan, G., Marks, S.D., Oh, J., Prytula, A., Seeman, T., Sweeney, C., Dello Strologo, L., and Pape, L. (2021) Clinical practice recommendations for recurrence of focal and segmental glomerulosclerosis/steroid-resistant nephrotic syndrome. Pediatr Transplant, 25(3): e13955.
Schreuder, M.F., Ehren, R., and Weber, L.T. (2021) Inadequate Dosage of Mycophenolate Mofetil Is Likely to Yield Substandard Trial Results and Substandard Patient Care. JAMA Pediatr, 175(8): 869-870.
Ehren, R. and Weber, L.T. (2021) Im Falle des Einsatzes eines Calcineurininhibitorsbei komplizierten Verläufen des nephrotischen Syndroms im Kindesalter. Kinder- und Jugendmedizin, 21(01): 27-35.
Ehren, R., Benz, M.R., Brinkkotter, P.T., Dotsch, J., Eberl, W.R., Gellermann, J., Hoyer, P.F., Jordans, I., Kamrath, C., Kemper, M.J., Latta, K., Muller, D., Oh, J., Tonshoff, B., Weber, S., Weber, L.T., and German Society for Pediatric, N. (2021) Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020. Pediatr Nephrol, 36(10): 2971-2985.
Ehren, R., Benz, M.R., Brinkkotter, P.T., Dotsch, J., Eberl, W.R., Gellermann, J., Hoyer, P.F., Jordans, I., Kamrath, C., Kemper, M.J., Latta, K., Muller, D., Oh, J., Tonshoff, B., Weber, S., Weber, L.T., and German Society for Pediatric, N. (2021) Commentary on "Pediatric Idiopathic Steroid-sensitive Nephrotic Syndrome Diagnosis and Therapy - Short version of the updated German Best Practice Guideline (S2e)". Pediatr Nephrol, 36(10): 2961-2966.
Volker, L.A., Ehren, R., Grundmann, F., Benzing, T., Weber, L.T., and Brinkkotter, P.T. (2019) A newly established clinical registry of minimal change disease and focal and segmental glomerulosclerosis in Germany. Nephrol Dial Transplant, 34(12): 1983-1986.
Vogd, J., Hackl, A., Habbig, S., Lechner, F., Akarkach, A., Weber, L.T., and Ehren, R. (2019) Discontinuation of maintenance therapy in frequently relapsing nephrotic syndrome. Clin Nephrol, 91(1): 25-31.
Schijvens, A.M., Ehren, R., Weber, L.T., and Schreuder, M.F. (2019) The quest for optimal control of relapses in children with nephrotic syndrome. Kidney Int, 95(3): 717.
Ehren, R., Brinkkötter, P., Weber, L.T., and Querfeld, U. (2019) Nephrotisches Syndrom des Kindes- und Jugendalters. Der Nephrologe, 14(3): 184-191.
Benz, M.R., Ehren, R., Kleinert, D., Muller, C., Gellermann, J., Fehrenbach, H., Schmidt, H., and Weber, L.T. (2019) Generation and Validation of a Limited Sampling Strategy to Monitor Mycophenolic Acid Exposure in Children With Nephrotic Syndrome. Ther Drug Monit, 41(6): 696-702.
Querfeld, U. and Weber, L.T. (2018) Treatment strategies for children with steroid-dependent nephrotic syndrome: in need of controlled studies. Pediatr Nephrol, 33(12): 2391.
Querfeld, U. and Weber, L.T. (2018) Mycophenolate mofetil for sustained remission in nephrotic syndrome. Pediatr Nephrol, 33(12): 2253-2265.
Hoefele, J., Beck, B.B., Weber, L.T., and Brinkkötter, P. (2018) Steroid-resistentes nephrotisches Syndrom. medizinische genetik, 30(4): 410-421.
Ehren, R., Benz, M.R., Doetsch, J., Fichtner, A., Gellermann, J., Haffner, D., Hocker, B., Hoyer, P.F., Kastner, B., Kemper, M.J., Konrad, M., Luntz, S., Querfeld, U., Sander, A., Toenshoff, B., Weber, L.T., and Gesellschaft fur Padiatrische, N. (2018) Initial treatment of steroid-sensitive idiopathic nephrotic syndrome in children with mycophenolate mofetil versus prednisone: protocol for a randomised, controlled, multicentre trial (INTENT study). BMJ Open, 8(10): e024882.
Hackl, A., Ehren, R., and Weber, L.T. (2017) Effect of mycophenolic acid in experimental, nontransplant glomerular diseases: new mechanisms beyond immune cells. Pediatr Nephrol, 32(8): 1315-1322.
Ehren, R., Benz, M.R., Hackl, A., and Weber, L.T. (2016) Management des steroidsensiblen nephrotischen Syndroms im Kindes- und Jugendalter. Kinder- und Jugendmedizin, 16(05): 359-365.
