Long-term Follow-up Assessing Renal Angiomyolipoma Treatment Patterns, Morbidity, and Mortality: An Observational Study in Tuberous Sclerosis Complex Patients in the Netherlands

Eijkemans, Marinus J.C.; Wal, Willem van der; Reijnders, Leida J.; Roes, Kit C. B.; Doorn-Khosrovani, Sahar van Waalwijk van Barjesteh; Pelletier, Corey; Magestro, Matthew; Zonnenberg, Bernard A.

doi:10.1053/j.ajkd.2015.05.016

Cited by 111 publications

(121 citation statements)

References 20 publications

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“…Therefore, beginning to monitor the patients when h/she is approaching the age of 10 years would result in the early detection of AMLs; in other words, active surveillance of patients, which initiates no later than the age of 10 years and maintains during their life while increasing the number of monitoring with advancing age, is highly suggested for AMLs patients associated with TSC (36). All studies put emphasis on early detection and treatment to prevent progressive damage to renal tissue and enhance patient outcomes (38). A tumor size of no more than 4 cm was suggested for the observation of asymptomatic AMLs (9).…”

Section: Treatmentmentioning

confidence: 99%

“…When tumor is large with high vascularity, embolization showed less efficacious outcomes than partial nephrectomy (8). Irrespective of the embolization type (elective or nonelective), patients who receive this treatment experience significant anemia, hypertension, and reduced kidney function (38). The most serious complications, which are often reported in patients undergoing embolization, include pain and increased temperature, known as postembolization syndrome.…”

Section: Treatmentmentioning

confidence: 99%

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Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

et al. 2017

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Recent prophylactic treatment options and diagnostic tools show a suboptimal improvement in patients with renal angiomyolipoma (AML). This study was an attempt to review management strategies applied before invasive options. An extensive research on medical databases such as PubMed and Scopus was performed from 1999 to 2016 using the following keywords: 'kidney', 'renal', and 'angiomyolipoma'. All related studies on patients treating with conservative or minimally-invasive procedures were included. However, reports on surgical treatments were excluded. Treatment strategies have been selected based on outcomes resulting from computed tomography (CT) and magnetic resonance (MR) imaging. Fat content and tumor size are the most common used indications for AMLs. Unenhanced CT and chemical shift imaging provide good evidence in case of fat-poor AMLs. Chemical analysis and percutaneous biopsy are recommended in case of a diagnostic challenge. A tumor size of 6 cm or larger in diameter might necessitate invasive treatment, while AMLs patients with tumors around 4 cm may require prophylactic treatment. Of all treatment options, embolization indicated a high risk of reintervention. Moreover, everolimus and sirolimus as 2 inhibitors of mammalian target of rapamycin (mTOR) have been evaluated in clinical trials and demonstrated promising outcomes. In conclusion, advances in imaging techniques along with mTOR inhibitors and embolic materials potentiate functional outcomes in AMLs.

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Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

et al. 2017

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“…A lack of appropriate transition in care may lead to higher costs, as patients may present to emergency services with complications that could have been prevented by pro-active monitoring 22 and management by TSC clinics which are under-utilized 16 . Given the complex and variable nature of TSC as a life-long disease 16 , it is likely to pose a disproportionate economic burden on healthcare services 10,[23][24][25][26] . Therefore, greater understanding of the healthcare resources utilized to manage TSC and potential points of sub-optimal care will enable the delivery of more efficient and effective care to patients.…”

Section: Introductionmentioning

confidence: 99%

The economic burden of tuberous sclerosis complex in the UK: A retrospective cohort study in the Clinical Practice Research Datalink

Kingswood

Crawford

Johnson

et al. 2016

Journal of Medical Economics

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Background: Tuberous sclerosis complex (TSC) is a multi-system genetic disorder characterized by the development of diverse clinical manifestations. The complexity of this disease is likely to result in substantial challenges and costs in disease management throughout the patient's lifetime. This retrospective database study aims to quantify healthcare resources utilized by TSC patients. Methods: TSC patients in the Clinical Practice Research Datalink linked to the Hospital Episodes Statistics database were identified between January 1987 and June 2013. Analyses were conducted over the most recent 3-year period of data and stratified by pediatric (< 18) and adult patients. Prescriptions, procedures, diagnostic tests, and healthcare encounters were reported in comparison with a matched comparator cohort. Costs and key economic drivers by primary organ system manifestations were also examined. Results: A total of 286 patients with TSC were identified and consistently reported 2-fold greater resource use than the matched presumably healthy controls. Despite this comparatively greater resource use, half of TSC patients did not record any procedures, and 20% of patients did not record any diagnostic tests; however, inpatient hospitalizations were greater for the TSC cohort (3.1 vs 1.3), but length of stay was comparable. TSC patients had costs totaling £12,681 per patient over the 3-year period, a figure 2.7-fold greater than the total costs in the comparator cohort (£4,777). Costs for patients with specific primary manifestations were even greater, with brain manifestations incurring £22,139 per affected patient. Kidney and nervous system manifestations were the main cost drivers. Conclusions: The economic burden of TSC and its impact on NHS healthcare resources is mostly attributable to the broad spectrum of manifestations that develop within multiple organ systems. TSC patients may benefit from co-ordinated care based on their requirement for high numbers of healthcare visits across specialties.

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“…Classical findings include the triad of adenoma sebaceum, seizures, and mental retardation. The inheritance is autosomal dominant; however, spontaneous mutation is described up to 66 % [1][2][3][4][5][6][7][8][9].…”

Section: Case Reportmentioning

confidence: 99%

Renal angiomyolipomas in tuberous sclerosis

Röper-Kelmayr¹,

Sokol²,

Fellner³

et al. 2017

Glob Imaging Insights

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Long-term Follow-up Assessing Renal Angiomyolipoma Treatment Patterns, Morbidity, and Mortality: An Observational Study in Tuberous Sclerosis Complex Patients in the Netherlands

Abstract: Despite the use of preventive selective arterial embolization, patients with TSC exhibit clinically significant kidney disease and excess mortality, largely because of kidney-related complications.

Cited by 111 publications

References 20 publications

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

The economic burden of tuberous sclerosis complex in the UK: A retrospective cohort study in the Clinical Practice Research Datalink

Renal angiomyolipomas in tuberous sclerosis

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