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.
Nat Rev Nephrol
; 20(6): 402-420, 2024 06.
Article
in En
| MEDLINE
| ID: mdl-38443710
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Tuberous Sclerosis
Limits:
Humans
Language:
En
Journal:
Nat Rev Nephrol
/
Nature reviews. Nephrology (Online)
Journal subject:
NEFROLOGIA
Year:
2024
Document type:
Article
Affiliation country:
Country of publication: