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Abstract: INFO26

FOrMe, the German Focal Segmental Glomerulosclerosis and Minimal Change Disease Registry: An Update

Session Information

  • Informational Posters
    November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

  • No subcategory defined

Authors

  • Osterholt, Thomas, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Todorova, Polina, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Ehren, Rasmus JC, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Grundmann, Franziska, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Benzing, Thomas, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Weber, Lutz Thorsten, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Volker, Linus A., Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Brinkkoetter, Paul T., Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
Description

Background:
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are rare diseases but common causes of nephrotic syndrome and associated with development of chronic kidney failure.
Methods:
In 2019 we established a registry for MCD and FSGS in Germany that is funded by the German Research Foundation (DFG) and is part of Clinical Research Unit (CRU) 329 "Molecular Mechanisms of Podocytic Diseases - Nephrology on the Way to Precision Medicine" and the STOP-FSGS-Network. In 2022, five additional medical centers joint the registry. The registry collects clinical data throughout the disease course at regular intervals, complemented by biosampling and a genetic work-up. We additionally established a digital histopathology work-flow that enables the multicentric evaluation of renal biopsies by an expert panel.
Results:
Ninety-eight patients have enrolled in the FOrMe registry, 73 adults and 25 pediatric patients accumulating 625 patient-years.
The mean age at diagnosis was 38 years (IQR 19-52 years) for adult and 4 years (IQR 2-7 years) for children. Thirty-five (47.9%) patients were classified as adult FSGS and 35 (47.9%) as MCD. The number of different immunosuppressant medications ranged from 1 to up to 4 medications to achieve or maintain remission. In genetic analyses of 41 patients, we found potential causative mutations in 7/41 (17%) analyzed adult patients and 7/11 (64%) of pediatric patients.
Conclusion
The German FOrMe registry is recruiting since April 2019 and a significant number of patients could be included. The crosslinking of highly granular and carefully reviewed clinical data, laboratory values, genetics and pathology enables new insights in the disease course of MCD and FSGS in central Europe. In combination with biosampling, the FOrMe registry enables a translational research and may spark new diagnostic and therapeutic approaches. The high yield of genetic mutations in this population argues for a low threshold for genetic analysis in MCD- and FSGS-patients. With the inclusion of 5 new participating centers and 5 awaiting participating centers, we hope for a frutile development and exchange with other international registries in the upcoming years.

Funding

  • German Research Foundation (DFG)
Abstract: INFO26

FOrMe, the German Focal Segmental Glomerulosclerosis and Minimal Change Disease Registry: An Update

Session Information

  • Informational Posters
    November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category:

  • No subcategory defined

Authors

  • Osterholt, Thomas, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Todorova, Polina, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Ehren, Rasmus JC, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Grundmann, Franziska, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Benzing, Thomas, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Weber, Lutz Thorsten, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Volker, Linus A., Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Brinkkoetter, Paul T., Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
Description

Background:
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are rare diseases but common causes of nephrotic syndrome and associated with development of chronic kidney failure.
Methods:
In 2019 we established a registry for MCD and FSGS in Germany that is funded by the German Research Foundation (DFG) and is part of Clinical Research Unit (CRU) 329 "Molecular Mechanisms of Podocytic Diseases - Nephrology on the Way to Precision Medicine" and the STOP-FSGS-Network. In 2022, five additional medical centers joint the registry. The registry collects clinical data throughout the disease course at regular intervals, complemented by biosampling and a genetic work-up. We additionally established a digital histopathology work-flow that enables the multicentric evaluation of renal biopsies by an expert panel.
Results:
Ninety-eight patients have enrolled in the FOrMe registry, 73 adults and 25 pediatric patients accumulating 625 patient-years.
The mean age at diagnosis was 38 years (IQR 19-52 years) for adult and 4 years (IQR 2-7 years) for children. Thirty-five (47.9%) patients were classified as adult FSGS and 35 (47.9%) as MCD. The number of different immunosuppressant medications ranged from 1 to up to 4 medications to achieve or maintain remission. In genetic analyses of 41 patients, we found potential causative mutations in 7/41 (17%) analyzed adult patients and 7/11 (64%) of pediatric patients.
Conclusion
The German FOrMe registry is recruiting since April 2019 and a significant number of patients could be included. The crosslinking of highly granular and carefully reviewed clinical data, laboratory values, genetics and pathology enables new insights in the disease course of MCD and FSGS in central Europe. In combination with biosampling, the FOrMe registry enables a translational research and may spark new diagnostic and therapeutic approaches. The high yield of genetic mutations in this population argues for a low threshold for genetic analysis in MCD- and FSGS-patients. With the inclusion of 5 new participating centers and 5 awaiting participating centers, we hope for a frutile development and exchange with other international registries in the upcoming years.

Abstract: INFO26

FOrMe, the German Focal Segmental Glomerulosclerosis and Minimal Change Disease Registry: An Update

Session Information

  • Informational Posters
    November 05, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category:

  • No subcategory defined

Authors

  • Osterholt, Thomas, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Todorova, Polina, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Ehren, Rasmus JC, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Grundmann, Franziska, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Benzing, Thomas, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Weber, Lutz Thorsten, Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Volker, Linus A., Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
  • Brinkkoetter, Paul T., Uniklinik Koln, Koln, Nordrhein-Westfalen, Germany
Description

Background:
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are rare diseases but common causes of nephrotic syndrome and associated with development of chronic kidney failure.
Methods:
In 2019 we established a registry for MCD and FSGS in Germany that is funded by the German Research Foundation (DFG) and is part of Clinical Research Unit (CRU) 329 "Molecular Mechanisms of Podocytic Diseases - Nephrology on the Way to Precision Medicine" and the STOP-FSGS-Network. In 2022, five additional medical centers joint the registry. The registry collects clinical data throughout the disease course at regular intervals, complemented by biosampling and a genetic work-up. We additionally established a digital histopathology work-flow that enables the multicentric evaluation of renal biopsies by an expert panel.
Results:
Ninety-eight patients have enrolled in the FOrMe registry, 73 adults and 25 pediatric patients accumulating 625 patient-years.
The mean age at diagnosis was 38 years (IQR 19-52 years) for adult and 4 years (IQR 2-7 years) for children. Thirty-five (47.9%) patients were classified as adult FSGS and 35 (47.9%) as MCD. The number of different immunosuppressant medications ranged from 1 to up to 4 medications to achieve or maintain remission. In genetic analyses of 41 patients, we found potential causative mutations in 7/41 (17%) analyzed adult patients and 7/11 (64%) of pediatric patients.
Conclusion
The German FOrMe registry is recruiting since April 2019 and a significant number of patients could be included. The crosslinking of highly granular and carefully reviewed clinical data, laboratory values, genetics and pathology enables new insights in the disease course of MCD and FSGS in central Europe. In combination with biosampling, the FOrMe registry enables a translational research and may spark new diagnostic and therapeutic approaches. The high yield of genetic mutations in this population argues for a low threshold for genetic analysis in MCD- and FSGS-patients. With the inclusion of 5 new participating centers and 5 awaiting participating centers, we hope for a frutile development and exchange with other international registries in the upcoming years.