The FOrMe Registry (The German Focal Segmental Glomerulosclerosis and Minimal Change Disease Registry) (FOrMe)

In a monocentric, later multicentric prospective approach the FOrMe registry (The German Focal Segmental Glomerulosclerosis and Minimal Change Disease Registry) aims to generate a longitudinal cohort of 150 pediatric cases of idiopathic nephrotic syndrome and 350 adult cases of biopsy-proven Minimal Change Disease (MCD) or Focal and Segmental Glomerular Sclerosis (FSGS) over 10 years. The registry will provide a repository for biomaterials such as blood samples, DNA, urine, feces, and tissue biopsies that will be accessible to collaborators to facilitate future research on pathogenesis, diagnostics, and treatment.

Study Overview

• Status: Recruiting
• Conditions: Glomerulosclerosis, Focal Segmental; Minimal Change Disease; Idiopathic Nephrotic Syndrome
• Intervention / Treatment: Other: Biosampling

Detailed Description

Idiopathic Nephrotic Syndrome is characterized by proteinuria, volume retention, hyperlipidemia, hypoalbuminemia. As Minimal Change Disease (MCD) represents by far the most prevalent underlying diagnosis in children older than 1 year, a kidney biopsy is usually deferred in these cases. In adolescence and adults, a kidney biopsy is crucial for the diagnosis because MCD and FSGS account for only 10-15 and 12-35 percent of all cases of nephrotic syndrome respectively. Pathomechanisms as well as optimal treatment remain elusive as systematic trials are scarce and hampered by low incidence and heterogenicity of the clinical presentation. To bridge this informational gap, the investigators identified the need for a German registry of pediatric and adult patients with idiopathic nephrotic syndrome (in children) and biopsy-proven MCD/FSGS (in adults).

The registry will record clinical data of participants regarding basic demographics, initial presentation, hereditary traits, disease course and treatment modalities as well as quality of life, concomitant diseases, and comedication. During the initial visit and to a lesser intent on follow-up visits, biomaterials (blood, urine, DNA, feces, tissue) will be collected and stored in a state-of-the art biobank. This material will be available to collaborators to support research on idiopathic nephrotic syndrome and MCD/FSGS. By the time of completion, the registry will provide data on clinical courses and outcome of approximately 500 patients that can easily be correlated with biomaterials giving insight into risk factors, prognostic parameters, and association with comorbidities.

Tissue sections of all patients that undergo kidney biopsy (all adult and some pediatric patients) will be digitalized, annotated, and analyzed by a panel of nephropathologists. Histopathologic features will be individually assessed and scored according to a set of descriptors that was developed and is used by the American NEPTUNE (Nephrotic Syndrome Study Network).

• Study Type: Observational
• Enrollment (Estimated): 500

Contacts and Locations

• Study Contact: Name: Paul T Brinkkoetter, MD; Phone Number: +49-221-478-4480; Email: paul.brinkkoetter@uk-koeln.de
• Study Contact Backup: Name: Linus A Voelker, MD; Phone Number: +49-221-478-4480; Email: linus.voelker@uk-koeln.de

Study Locations

• Germany
  • Aachen, Germany, 52074
    • Not yet recruiting
    • Uniklinik RWTH Aachen
    • Contact: Claudia Seikrit, MD; Email: cseikrit@ukaachen.de
    • Contact: Kerstin Schröder; Email: keschroeder@ukaachen.de
    • Principal Investigator: Claudia Seikrit, MD
  • Berlin, Germany
    • Recruiting
    • Charite University Hospital
    • Sub-Investigator: Eva-Vanessa Schrezenmeier, MD
    • Contact: Jan P. Halbritter, MD, Prof.; Email: jan.halbritter@charite.de
    • Contact: Silke Kasbohm; Phone Number: + 49 30 450 514183; Email: silke.kasbohm@charite.de
    • Principal Investigator: Jan P. Halbritter, MD, Prof.
    • Sub-Investigator: Natnael Gebremedhin
    • Principal Investigator: Julia Thumfahrt, MD
    • Sub-Investigator: Verena Klämbt, MD
  • Bonn, Germany, 53127
    • Recruiting
    • Kindernierenzentrum Bonn
    • Contact: Gesa Gesa, MD; Phone Number: +49 228 6883860; Email: gesa.schalk@knz-bonn.de
    • Contact: Katja Theissen; Phone Number: +49 228 6883860; Email: Katja.Theisen@knz-bonn.de
    • Principal Investigator: Gesa Schalk, MD
  • Dachau, Germany, 85221
    • Recruiting
    • Kindernephrologie Dachau
    • Contact: Marcus Benz, MD; Phone Number: +49 8131-2995954; Email: marcus.benz@uk-koeln.de
    • Principal Investigator: Marcus Benz, MD
  • Erlangen, Germany
    • Not yet recruiting
    • University Hospital Erlangen
    • Contact: Matthias Galiano, MD; Email: Matthias.Galiano@uk-erlangen.de
    • Contact: Alina Hilger, MD; Email: alina.hilger@uk-erlangen.de
  • Essen, Germany, 45147
    • Recruiting
    • University Hospital Essen
    • Principal Investigator: Andreas Kribben, MD
    • Contact: Lars Pape, MD, Prof.; Phone Number: +49 201 723 2810; Email: lars.pape@uk-essen.de
    • Contact: Andreas Kribben, MD, Prof.; Phone Number: +49 201 723 1868; Email: nephrologie@uk-essen.de
    • Principal Investigator: Lars Pape, MD
    • Sub-Investigator: Christina Okorn, MD
    • Sub-Investigator: Anja Gäckler, MD
  • Heidelberg, Germany, 69120
    • Recruiting
    • University Hospital Heidelberg
    • Contact: Burkhard Tönshoff, MD, Prof; Phone Number: +49 6221 56-4002; Email: Burkhard.Toenshoff@med.uni-heidelberg.de
    • Contact: Maria Luisa Möller-Winheim; Phone Number: +49 6221 56-310376; Email: MariaLuisa.Moeller-Winheim@med.uni-heidelberg.de
    • Principal Investigator: Burkhard Tönshoff, MD, Prof
    • Sub-Investigator: Annika Gold, MD
    • Sub-Investigator: Joanna Sladowska-Kozlowska, MD
  • Leipzig, Germany, 04129
    • Recruiting
    • Klinikum St. Georg
    • Contact: Ralph Wendt, MD; Phone Number: 0341-909-4056; Email: ralph.wendt@sanktgeorg.de
    • Principal Investigator: Ralph Wendt, MD
  • Marburg, Germany
    • Recruiting
    • University Hospital Marburg
    • Principal Investigator: Stefanie Weber, MD
    • Contact: Stefanie Weber, MD; Phone Number: +49 6421-5862659; Email: stefanie.weber@med.uni-marburg.de
    • Contact: Jördis Chrestensen-Fuchs; Phone Number: +49 6421-5864807; Email: Joerdis.Chrestensen-Fuchs@uk-gm.de
  • Münster, Germany
    • Recruiting
    • University Hospital Münster
    • Contact: Jens König, MD; Email: Jens.Koenig@ukmuenster.de
    • Contact: Mee-Ling Maywald, MD; Email: meeling.maywald@ukmuenster.de
    • Principal Investigator: Jens König, MD
    • Principal Investigator: Mee-Ling Maywald, MD
  • Stuttgart, Germany, 70174
    • Recruiting
    • Klinikum Stuttgart
    • Contact: Vedat Schwenger, Prof. MD; Email: v.schwenger@klinikum-stuttgart.de
    • Contact: Castellie Cabata; Email: c.cabata@klinikum-stuttgart.de
    • Principal Investigator: Vedat Scwenger, Prof. MD
  • North Rhine-Westphalia
    • Cologne, North Rhine-Westphalia, Germany, 50937
      • Recruiting
      • University Hospital of Cologne
      • Contact: paul brinkkoetter, MD; Phone Number: +49-221 478-4480; Email: paul.brinkkoetter@uk-koeln.de
      • Contact: Lutz Weber, MD; Phone Number: +49 221 478-4319; Email: lutz.weber@uk-koeln.de
      • Sub-Investigator: Linus Völker, MD
      • Principal Investigator: Paul Brinkkötter, MD, Prof.
      • Principal Investigator: Franziska Grundmann, MD
      • Principal Investigator: Lutz T. Weber, MD, Prof.
      • Sub-Investigator: Stefan Kohl, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Pediatric patients until 18 years of age presenting with or diagnosed with idiopathic nephrotic syndrome or a biopsy-proven diagnosis of MCD or FSGS. Candidate participants need to be willing to provide written informed consent.

Adult patients 18 years and above with a biopsy-proven diagnosis of primary or secondary FSGS or MCD. Candidate participants need to be willing to provide written informed consent.

Description

Inclusion Criteria (cohort A):

• written informed consent
• 17 or less years of age
• idiopathic nephrotic syndrome

Inclusion Criteria (cohort B):

• written informed consent
• older or equal to 18 years of age
• biopsy-proven primary or secondary FSGS or MCD or biopsy-proven recurrence of disease in kidney transplant.

Exclusion Criteria (both cohorts):

• Prior kidney transplant without biopsy-proven recurrence
• A clinical diagnosis of other glomerular disease resulting in secondary MCD or FSGS as judged by the treating physicians.
• Refusal to provide written informed consent
• (Anticipated) incompliance with visit schedule

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
A - Pediatric Cohort; Pediatric patients until 18 years of age presenting with or diagnosed with idiopathic nephrotic syndrome or a biopsy-proven diagnosis of MCD or FSGS.	Other: Biosampling; Biosampling at initial visit and follow-up visits
B -Adult Cohort; Adult patients 18 years and above with a biopsy-proven diagnosis of primary or secondary FSGS or MCD.	Other: Biosampling; Biosampling at initial visit and follow-up visits

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Average Annual Change in estimated glomerular filtration rate (eGFR)	Outcome measure: eGFR loss in ml/min/year. Higher values are considered worse outcome.	5-15 years
Incidence of End-stage Renal Disease (ESRD)	Documented initiation of chronic renal replacement therapy regardless of type.	5-15 years
Incidence of Death	Documented patient death due to any cause	5-15 years
Incidence of Kidney Transplantation	Documented kidney transplantation regardless of type (living donor, cadaveric donor)	5-15 years
Changes in Quality of Life (adults patients)	Patient-reported outcome will be assessed using Quality of Life questionnaires at regular intervals using the SF-36 questionnaire. For reference see https://www.rand.org/health-care/surveys_tools/mos/36-item-short-form/scoring.html The SF-36 questionnaire measures eight health concepts: physical functioning, bodily pain, role limitations due to physical health problems, role limitations due to personal or emotional problems, emotional well-being, social functioning, energy/fatigue, and general health perceptions. All items are scored so that a high score defines a more favorable health state. Lowest and highest possible scores are 0 and 100. Scores represent the percentage of total possible score achieved. Original publication: Ware, J.E., Jr., & Sherbourne, C.D. "The MOS 36-Item Short-Form Health Survey (SF-36): I. Conceptual Framework and Item Selection,". Medical Care, 30:473-483, 1992.	5-15 years
Changes in Quality of Life (pediatric patients)	Patient-reported outcome will be assessed using Quality of Life questionnaires at regular intervals using the PedsQL questionnaire. For reference see https://www.pedsql.org/score.html The PedSQL questionnaire measures four health concepts: Physical Functioning, Emotional Functioning, Social Functioning, and School Functioning. Items are reversed scored and linearly transformed to a 0-100 scale, so that higher scores indicate better HRQOL (Health-Related Quality of Life). To create Scale Scores, the mean is computed as the sum of the items over the number of items answered (this accounts for missing data). To create the Total Scale Score, the mean is computed as the sum of all the items over the number of items answered on all the Scales.	5-15 years

Collaborators and Investigators

• Sponsor: Prof. Dr. Paul Brinkkoetter
• Collaborators: German Research Foundation; Medical Faculty and the Faculty of Natural Sciences of the University of Cologne; Cluster of Excellence on Cellular Stress Responses in Ageing-Associated Diseases; Cologne Center for Genomics
• Investigators: Study Director: Paul T Brinkkoetter, MD, University Hospital of Cologne, Cologne, Germany

Publications and helpful links

General Publications

• Osterholt T, Todorova P, Kuhne L, Ehren R, Weber LT, Grundmann F, Benzing T, Brinkkotter PT, Volker LA. Repetitive administration of rituximab can achieve and maintain clinical remission in patients with MCD or FSGS. Sci Rep. 2023 Apr 28;13(1):6980. doi: 10.1038/s41598-023-32576-7.

Helpful Links

• CRU 329 Homepage

Study record dates

Study Major Dates

• Study Start (Actual): April 1, 2018
• Primary Completion (Estimated): March 31, 2028
• Study Completion (Estimated): March 31, 2033

Study Registration Dates

• First Submitted: December 3, 2018
• First Submitted That Met QC Criteria: May 13, 2019
• First Posted (Actual): May 14, 2019

Study Record Updates

• Last Update Posted (Estimated): September 17, 2025
• Last Update Submitted That Met QC Criteria: September 11, 2025
• Last Verified: September 1, 2025

More Information

Terms related to this study

• Keywords: Nephrotic Syndrome; Minimal change disease; FSGS; MCD; Focal and Segmental Glomerulosclerosis; Focal & Segmental Glomerulosclerosis
• Additional Relevant MeSH Terms: Urogenital Diseases; Male Urogenital Diseases; Kidney Diseases; Urologic Diseases; Female Urogenital Diseases; Female Urogenital Diseases and Pregnancy Complications; Glomerulonephritis; Nephritis; Nephrosis; Nephrotic Syndrome; Glomerulosclerosis, Focal Segmental; Nephrosis, Lipoid; Macular dystrophy, corneal type 1
• Other Study ID Numbers: 005

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: Biosamples as well as patient data will be available to collaborators after revision of research application by a steering committee.
• IPD Sharing Time Frame: 5-15 years
• IPD Sharing Access Criteria: Actively contributing to registry and approval by international steering committee.

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No