title: Characteristics and survival of patients with end stage renal disease and spina bifida in the United States renal data system.
abstract: OBJECTIVE
  We describe the characteristics, treatments and survival of patients with spina bifida in whom end stage renal disease developed from 2004 through 2008 in the United States Renal Data System.
  
  
  METHODS
  We used ICD-9-CM code 741.* to identify individuals with spina bifida using hospital inpatient data from 1977 to 2010, and physician and facility claims from 2004 to 2008. We constructed a 5:1 comparison group of patients with end stage renal disease without spina bifida matched by age at first end stage renal disease service, gender and race/ethnicity. We assessed the risk of mortality and of renal transplantation while on dialysis using multivariate cause specific proportional hazards survival analysis. We also compared survival after the first renal transplant from the first end stage renal disease service to August 2011.
  
  
  RESULTS
  We identified 439 patients with end stage renal disease and spina bifida in whom end stage renal disease developed at an average younger age than in patients without spina bifida (41 vs 62 years, p <0.001) and in whom urological issues were the most common primary cause of end stage renal disease. Compared to patients with end stage renal disease without spina bifida those who had spina bifida showed a similar mortality hazard on dialysis and after transplantation. However, patients with end stage renal disease without spina bifida were more likely to undergo renal transplantation than patients with spina bifida (HR 1.51, 95% CI 1.13-2.03). Hospitalizations related to urinary tract infections were positively associated with the risk of death on dialysis in patients with end stage renal disease and spina bifida (HR 1.42, 95% CI 1.33-1.53).
  
  
  CONCLUSIONS
  Spina bifida was not associated with increased mortality in patients with end stage renal disease on dialysis or after renal transplantation. Proper urological and bladder management is imperative in patients with spina bifida, particularly in adults.
authors.affiliation: Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Bethesda, Maryland. Electronic address: louyang@cdc.gov.
authors.forename: Lijing
authors.initials: L
authors.lastname: Ouyang
authors.affiliation: Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Bethesda, Maryland.
authors.forename: Julie
authors.initials: J
authors.lastname: Bolen
authors.affiliation: Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Bethesda, Maryland.
authors.forename: Rodolfo
authors.initials: R
authors.lastname: Valdez
authors.affiliation: Department of Urology, School of Medicine, University of Alabama at Birmingham, Birmingham.
authors.forename: David
authors.initials: D
authors.lastname: Joseph
authors.affiliation: Division of Nephrology, Harvard Medical School, Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Baum
authors.affiliation: Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Bethesda, Maryland.
authors.forename: Judy
authors.initials: J
authors.lastname: Thibadeau
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006801:Humans; D007223:Infant; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis; D012189:Retrospective Studies; D012307:Risk Factors; D016135:Spinal Dysraphism / Q000150:complications* / Q000401:mortality*; D015996:Survival Rate; D014481:United States; D055815:Young Adult
keywords: chronic; dialysis; kidney failure; spinal dysraphism; transplantation; urinary tract infections