title: Long-term Clinical Morbidity in Patients With Renal Angiomyolipoma Associated With Tuberous Sclerosis Complex.
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.
  
  
  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.
  
  
  CONCLUSIONS
  TSC-renal AML patients are at significantly higher risk for renal morbidity relative to the general population.
authors.affiliation: LeBonheur Children's Hospital and St. Jude's Children's Research Hospital, Memphis, TN.
authors.forename: John
authors.initials: J
authors.lastname: Bissler
authors.affiliation: Truven Health Analytics Inc., Ann Arbor, MI.
authors.forename: Katherine
authors.initials: K
authors.lastname: Cappell
authors.affiliation: NYU Langone Medical Center, New York, NY.
authors.forename: Hearns
authors.initials: H
authors.lastname: Charles
authors.affiliation: Truven Health Analytics Inc., Ann Arbor, MI. Electronic address: xue.song@truvenhealth.com.
authors.forename: Xue
authors.initials: X
authors.lastname: Song
authors.affiliation: Novartis Pharmaceuticals Corporation, East Hanover, NJ.
authors.forename: Zhimei
authors.initials: Z
authors.lastname: Liu
authors.affiliation: Novartis Pharmaceuticals Corporation, East Hanover, NJ.
authors.forename: Judith
authors.initials: J
authors.lastname: Prestifilippo
authors.affiliation: Truven Health Analytics Inc., Ann Arbor, MI.
authors.forename: Christopher
authors.initials: C
authors.lastname: Gregory
authors.affiliation: Urologic Physicians, Edina, MN.
authors.forename: John
authors.initials: J
authors.lastname: Hulbert
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018207:Angiomyolipoma / Q000150:complications*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000453:epidemiology* / Q000209:etiology*; D007680:Kidney Neoplasms / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009017:Morbidity; D012189:Retrospective Studies; D013997:Time Factors; D014402:Tuberous Sclerosis / Q000150:complications*; D055815:Young Adult