Clinical practice recommendations for kidney involvement in tuberous sclerosis complex: a consensus statement by the ERKNet Working Group for Autosomal Dominant Structural Kidney Disorders and the ERA Genes &amp; Kidney Working Group.

Mekahli, Djalila; Müller, Roman-Ulrich; Marlais, Matko; Wlodkowski, Tanja; Haeberle, Stefanie; de Argumedo, Marta López; Bergmann, Carsten; Breysem, Luc; Fladrowski, Carla; Henske, Elizabeth P; Janssens, Peter; Jouret, François; Kingswood, John Christopher; Lattouf, Jean-Baptiste; Lilien, Marc; Maleux, Geert; Rozenberg, Micaela; Siemer, Stefan; Devuyst, Olivier; Schaefer, Franz

doi:10.1038/s41581-024-00818-0

Clinical practice recommendations for kidney involvement in tuberous sclerosis complex: a consensus statement by the ERKNet Working Group for Autosomal Dominant Structural Kidney Disorders and the ERA Genes & Kidney Working Group.

Mekahli, Djalila

;

Müller, Roman-Ulrich

;

Marlais, Matko

;

Wlodkowski, Tanja

;

Bissler, John

;et.al.

(2024) Nature Reviews Nephrology — Vol. 20, n° 6, p. 402-420 (2024)

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Authors

Mekahli, Djalila
Author
Müller, Roman-Ulrich
Author
Marlais, Matko
Author
Wlodkowski, Tanja
Author
Devuyst, OlivierUCLouvain
Author
Bissler, John
Author

Abstract

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the presence of proliferative lesions throughout the body. Management of TSC is challenging because patients have a multifaceted systemic illness with prominent neurological and developmental impact as well as potentially severe kidney, heart and lung phenotypes; however, every organ system can be involved. Adequate care for patients with TSC requires a coordinated effort involving a multidisciplinary team of clinicians and support staff. This clinical practice recommendation was developed by nephrologists, urologists, paediatric radiologists, interventional radiologists, geneticists, pathologists, and patient and family group representatives, with a focus on TSC-associated kidney manifestations. Careful monitoring of kidney function and assessment of kidney structural lesions by imaging enable early interventions that can preserve kidney function through targeted approaches. Here, we summarize the current evidence and present recommendations for the multidisciplinary management of kidney involvement in TSC.

Affiliations

UCLouvainSSS/IREC/NEFR - Pôle de Néphrologie

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Mekahli, D., Müller, R.-U., Marlais, M., Wlodkowski, T., Haeberle, S., de Argumedo, M. L., Bergmann, C., Breysem, L., Fladrowski, C., Henske, E. P., Janssens, P., Jouret, F., Kingswood, J. C., Lattouf, J.-B., Lilien, M., Maleux, G., Rozenberg, M., Siemer, S., Devuyst, O., et al. (2024). Clinical practice recommendations for kidney involvement in tuberous sclerosis complex: a consensus statement by the ERKNet Working Group for Autosomal Dominant Structural Kidney Disorders and the ERA Genes & Kidney Working Group. Nature Reviews Nephrology, 20(6), 402-420. https://doi.org/10.1038/s41581-024-00818-0 (Original work published 2024)