Literature DB >> 27132503

Long-term Clinical Morbidity in Patients With Renal Angiomyolipoma Associated With Tuberous Sclerosis Complex.

John Bissler1, Katherine Cappell2, Hearns Charles3, Xue Song4, Zhimei Liu5, Judith Prestifilippo5, Christopher Gregory2, John Hulbert6.   

Abstract

OBJECTIVE: To estimate the incidence rates of kidney-related clinical outcomes among patients with tuberous sclerosis complex (TSC)-related angiomyolipoma (AML) compared to an age-matched control cohort in the United States.
MATERIALS AND METHODS: This was a retrospective, observational study. Administrative data from the MarketScan Research Databases were used to select patients with TSC and renal AML. An age-matched group with no TSC or renal AML was identified for comparison. Outcomes were incidence rates per 100 patient-years and number of months to development of hematuria, chronic kidney disease, renal hemorrhage, kidney failure, and inpatient death.
RESULTS: Among the commercially insured TSC-renal AML patients (N = 605) and matched controls (N = 1815), 37.2% were <18 years old. Among Medicaid TSC-renal AML patients (N = 246) and matched controls (N = 738), 38.6% were aged <18. In the commercial sample, in both age groups (<18 and ≥18), the incidence rate of each clinical outcome measured was higher in the TSC-renal AML cohort than in the control cohort, with several differences reaching statistical significance. Compared with younger patients, older TSC-renal AML patients had higher incidence rates of clinical outcomes (hematuria: 20.4 vs 8.7; chronic kidney disease: 9.6 vs 3.5; renal hemorrhage 2.7 vs 0.7; kidney failure: 1.9 vs 0.4) and took less time on average to develop each clinical outcome. A similar pattern of results was observed among patients with Medicaid insurance.
CONCLUSION: TSC-renal AML patients are at significantly higher risk for renal morbidity relative to the general population.
Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

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Year:  2016        PMID: 27132503     DOI: 10.1016/j.urology.2016.04.027

Source DB:  PubMed          Journal:  Urology        ISSN: 0090-4295            Impact factor:   2.649


  11 in total

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Journal:  Int Urol Nephrol       Date:  2021-04-25       Impact factor: 2.370

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10.  Renal angiomyolipoma in patients with tuberous sclerosis complex: findings from the TuberOus SClerosis registry to increase disease Awareness.

Authors:  J Chris Kingswood; Elena Belousova; Mirjana P Benedik; Tom Carter; Vincent Cottin; Paolo Curatolo; Maria Dahlin; Lisa D' Amato; Guillaume Beaure d'Augères; Petrus J de Vries; José C Ferreira; Martha Feucht; Carla Fladrowski; Christoph Hertzberg; Sergiusz Jozwiak; John A Lawson; Alfons Macaya; Ruben Marques; Rima Nabbout; Finbar O'Callaghan; Jiong Qin; Valentin Sander; Matthias Sauter; Seema Shah; Yukitoshi Takahashi; Renaud Touraine; Sotiris Youroukos; Bernard Zonnenberg; Anna C Jansen
Journal:  Nephrol Dial Transplant       Date:  2019-03-01       Impact factor: 5.992

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