Chronic renal failure and its treatment in tuberous sclerosis

Schillinger, F; Montagnac, R

doi:10.1093/ndt/11.3.481

Cited by 37 publications

(17 citation statements)

References 18 publications

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“…Twenty-nine deaths were recorded during the observation period, and the mortality rate was nearly 5-times higher than that in the ageand gender-matched Dutch general population during the same time period (for further details on mortality among patients in our sample, see Eijkemans et al 25 ). The finding that female patients were more likely to have kidney impairment is consistent with the Schillinger et al 26 study based on a survey of 260 French dialysis centers. The association between advanced CKD and the TSC2 gene mutation is consistent with established knowledge that this mutation is associated with a more severe phenotype 9,11,24 .…”

Section: Discussionsupporting

confidence: 88%

Kidney involvement in tuberous sclerosis complex: the impact on healthcare resource use and costs

Vekeman

Magestro

Karner

et al. 2015

Journal of Medical Economics

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Objective: Tuberous sclerosis complex (TSC) is associated with non-malignant kidney lesions-angiomyolipomatathat may be associated with chronic kidney disease (CKD). This study investigated the relationship between renal angiomyolipomata and CKD in TSC, including the impact on healthcare resource utilization (HCRU) and costs. Methods:This was a retrospective, longitudinal cohort study based on medical record data spanning January 1990-April 2012 for 369 TSC patients treated at a specialty center in the Netherlands. Cohorts were established based on CKD stage and angiomyolipoma size. Rates of HCRU (physician visits, monitoring, and interventions) were compared across cohorts using rate ratios. Healthcare costs were compared across cohorts using cost differences. Regression models were used to identify predictive factors for HCRU and healthcare costs. Results:Sixteen per cent of patients reached CKD stage 3 or higher during follow-up. Patients at more advanced stages of CKD more frequently had either large or multiple small angiomyolipomata and higher HCRU rates and healthcare costs. In the multivariate analyses, male gender, CKD stage 41, angiomyolipoma size !3.5 cm, embolization, and the presence of moderate or severe lymphangioleiomyomatosis (LAM) were associated with greater HCRU (p 0.002 for all comparisons). Definite (vs suspected) TSC diagnosis, CKD stage 5 (vs CKD stage 1), angiomyolipoma size !3.5 cm, and moderate or severe LAM were associated with higher costs (p ¼ 0.050 for TSC diagnosis, p 0.002 for other comparisons). Costs in CKD stage 5 were driven primarily by dialysis. Conclusions:A substantial proportion of patients with TSC developed moderate-to-severe CKD, which was associated with renal angiomyolipomata and increased HCRU and costs.

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Section: Discussionsupporting

confidence: 88%

Kidney involvement in tuberous sclerosis complex: the impact on healthcare resource use and costs

Vekeman

Magestro

Karner

et al. 2015

Journal of Medical Economics

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“…9 The presence of numerous renal cysts can cause renal failure; however, its prevalence was reported to be 1-3%. 10,11 AMLs occupy an important place in management of adult TSC patients, since 6…”

Section: Resultsmentioning

confidence: 99%

Management of Renal Angiomyolipomas Associated With Tuberous Sclerosis Complex

et al. 2004

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“…Renal disease poses a significant burden on patients with TSC and renal failure was identified as a leading cause of death in TSC in one study [ 23 ]. In France, the prevalence of TSC with end-stage renal failure was reported to be 0.7 cases per million and that of end-stage renal failure in TSC to be 1 per 100 [ 24 ]. It is more frequent in females (63.1%) and was diagnosed at a mean age of 29 years [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

Renal manifestations of tuberous sclerosis among children: an Indian experience and review of the literature

Korula

Ekbote

Kumar

et al. 2014

Clinical Kidney Journal

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ObjectiveThe objective of this study was to describe the renal manifestations in children 0–18 years of age diagnosed with tuberous sclerosis complex (TSC) at a tertiary hospital in South India.MethodsData of children with TSC, who presented to Christian Medical College Vellore Hospital from January 2008 to January 2013, were analysed by a retrospective chart review. The cases were identified from outpatient records and underwent ultrasonography, urine analysis and examination of serum creatinine to recognize renal involvement.ResultsTwenty-five children with TSC were identified. Two children did not have imaging studies available and were excluded from the analysis. The age of the included children ranged from 5 days to 15 years with a median age of 8 years. Seventy-four per cent (17/23) were males. Ten of the 23 children had evidence of renal involvement (43.5%). Of the 10 children with renal involvement, 6 had angiomyolipoma (60%), 5 had renal cysts (50%) and 1 had suspected renal cell carcinoma. In two children both angiomyolipoma (AML) and cysts were noted. One child was found to have proteinuria. The rest of the children had no evidence of proteinuria and had normal creatinine clearance.ConclusionWe conclude that all children with TSC should be screened for renal involvement and regular follow-up should be arranged.

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Chronic renal failure and its treatment in tuberous sclerosis

Abstract: Tuberous sclerosis with end-stage renal failure is rare. These patients require dialysis and renal transplantation, but we recommend binephrectomy after starting dialysis and before transplantation, given the risk of cancer and bleeding related to angiomyolipomas.

Cited by 37 publications

References 18 publications

Kidney involvement in tuberous sclerosis complex: the impact on healthcare resource use and costs

Kidney involvement in tuberous sclerosis complex: the impact on healthcare resource use and costs

Management of Renal Angiomyolipomas Associated With Tuberous Sclerosis Complex

Renal manifestations of tuberous sclerosis among children: an Indian experience and review of the literature

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