ArticlePediatric nephrology (Berlin, Germany)2018
Renal progression factors in young patients with tuberous sclerosis complex: a retrospective cohort study.
Article in Pediatric nephrology (Berlin, Germany), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed.
- Effectiveness and safety of everolimus treatment in patients with tuberous sclerosis complex in real-world clinical practice.Orphanet journal of rare diseases · 2023Trial
- Deciding "what" to screen for and "when": The importance of natural history information.American journal of human genetics · 2026Review
- Tuberous sclerosis complex.Nature reviews. Disease primers · 2026Review
- Multicystic Kidney Disease in a Family With Tuberous Sclerosis Complex.Nephrology (Carlton, Vic.) · 2026Article
- Case Report: Rapid progression to end-stage renal disease within 3 years in tuberous sclerosis complex challenging conventional risk stratification.Frontiers in medicine · 2026Article
- Kidneys in Children with Tuberous Sclerosis Complex-An Up-to-Date Review.Journal of clinical medicine · 2025Review
- The role and mechanism of TSC in kidney diseases: a literature review.BMC nephrology · 2025Review
- Tuberous sclerosis complex-associated kidney disease in children.Pediatric nephrology (Berlin, Germany) · 2025Review
- The extent of kidney involvement in paediatric tuberous sclerosis complex.Pediatric nephrology (Berlin, Germany) · 2024Article
- Nutritional status as a predictive factor for paediatric tuberous sclerosis complex-associated kidney angiomyolipomas: a retrospective analysis.European journal of pediatrics · 2024Article
- 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 · 2024Review
- Comprehensive Insights Into Renal Perivascular Epithelioid Cell Neoplasms: From Molecular Mechanisms to Clinical Practice.World journal of oncology · 2024Review
- Cystic kidney disease in tuberous sclerosis complex: current knowledge and unresolved questions.Pediatric nephrology (Berlin, Germany) · 2023Article
- Review
- Blood pressure and glomerular filtration rate in youth with tuberous sclerosis complex.European journal of pediatrics · 2022Article
- A rare case of tuberous sclerosis complex-associated renal cell carcinoma.SA journal of radiology · 2022Article
- Review
- Early Vascular Aging in Children With Tuberous Sclerosis Complex.Frontiers in pediatrics · 2021Article
- Renal Manifestations of Tuberous Sclerosis Complex: Key Findings From the Final Analysis of the TOSCA Study Focussing Mainly on Renal Angiomyolipomas.Frontiers in neurology · 2020Article
Corrections and comments
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Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundRenal pathology in tuberous sclerosis complex (TSC) is characterized by the growth of angiomyolipoma and renal cysts, and in rare cases renal cell carcinoma. Other consequences of renal involvement in TSC, including hypertension, proteinuria, and hyperfiltration, are not well studied. We aimed to analyze the early manifestations of the renal TSC phenotype in a young TSC cohort and to explore common, modifiable risk factors.
methodsIn this retrospective cohort study, TSC patients attending the TSC clinics of two tertiary hospitals were included. Data on demographics, history, genotype, kidney function, hematuria, proteinuria, blood pressure, and renal imaging were collected.
resultsEighty patients were included, with a median age of 0.8 years (0.0-63.0) at first presentation, and a median follow-up time of 10.2 (0.4-41.0) years. Mutation analysis was available in 64 patients (80%). Renal lesions (cysts or angiomyolipoma) were observed in 55/73 (75%). Thirty-two percent (19/60) were hypertensive, 8/51 (16%) had proteinuria, and 18/71 (25%) had hyperfiltration (median eGFR 154 ml/min/m
conclusionsBesides renal cysts and angiomyolipoma, the modifiable progression factors hypertension, proteinuria, and hyperfiltration occur frequently and early in TSC patients. This represents a preventive treatment target.
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