title: National variation in costs and mortality for leukodystrophy patients in US children's hospitals.
abstract: BACKGROUND
  Inherited leukodystrophies are progressive, debilitating neurological disorders with few treatment options and high mortality rates. Our objective was to determine national variation in the costs for leukodystrophy patients and to evaluate differences in their care.
  
  
  METHODS
  We developed an algorithm to identify inherited leukodystrophy patients in deidentified data sets using a recursive tree model based on International Classification of Disease, 9th Edition, Clinical Modification, diagnosis and procedure charge codes. Validation of the algorithm was performed independently at two institutions, and with data from the Pediatric Health Information System (PHIS) of 43 US children's hospitals, for a 7-year period between 2004 and 2010.
  
  
  RESULTS
  A recursive algorithm was developed and validated, based on six International Classification of Disease, 9th Edition, Clinical Modification, codes and one procedure code that had a sensitivity up to 90% (range 61-90%) and a specificity up to 99% (range 53-99%) for identifying inherited leukodystrophy patients. Inherited leukodystrophy patients comprise 0.4% of admissions to children's hospitals and 0.7% of costs. During 7 years, these patients required $411 million of hospital care, or $131,000/patient. Hospital costs for leukodystrophy patients varied at different institutions, ranging from two to 15 times more than the average pediatric patient. There was a statistically significant correlation between higher volume and increased cost efficiency. Increased mortality rates had an inverse relationship with increased patient volume that was not statistically significant.
  
  
  CONCLUSIONS
  We developed and validated a code-based algorithm for identifying leukodystrophy patients in deidentified national datasets. Leukodystrophy patients account for $59 million of costs yearly at children's hospitals. Our data highlight potential to reduce unwarranted variability and improve patient care.
authors: University of Utah School of Medicine, Salt Lake City, Utah 84108, USA.
authors: Cameron J
authors: CJ
authors: Brimley
authors: Jonathan
authors: J
authors: Lopez
authors: Keith
authors: K
authors: van Haren
authors: Jacob
authors: J
authors: Wilkes
authors: Xiaoming
authors: X
authors: Sheng
authors: Clint
authors: C
authors: Nelson
authors: E Kent
authors: EK
authors: Korgenski
authors: Rajendu
authors: R
authors: Srivastava
authors: Joshua L
authors: JL
authors: Bonkowsky
mesh_terms: D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D017281:Cost of Illness; D005260:Female; D006760:Hospitalization / Q000191:economics*; D006776:Hospitals, Pediatric; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000191:economics / Q000453:epidemiology / Q000401:mortality; D008297:Male; D012189:Retrospective Studies; D014481:United States / Q000453:epidemiology
references: Neurology. 2010 Aug 24;75(8):718-25
references: 20660364
references: AMIA Annu Symp Proc. 2012;2012:281-90
references: 23304298
references: J Pediatr. 2013 Mar;162(3):624-628.e1
references: 23069195
references: PLoS Med. 2012 Jan;9(1):e1001158
references: 22272190
references: JAMA. 2008 May 7;299(17):2048-55
references: 18460665
references: Semin Hematol. 2010 Jan;47(1):70-8
references: 20109614
references: Semin Pediatr Surg. 2002 Feb;11(1):60-3
references: 11815944
references: Neurology. 2009 Feb 24;72(8):750-9
references: 19237705
references: AMIA Annu Symp Proc. 2011;2011:994-1003
references: 22195159
references: J Inherit Metab Dis. 2007 Apr;30(2):175-83
references: 17347913
references: Arch Pediatr Adolesc Med. 2012 Dec;166(12):1155-64
references: 23027409
references: Adv Exp Med Biol. 2012;724:154-71
references: 22411242
references: Expert Rev Neurother. 2011 Oct;11(10):1485-96
references: 21955203