title: Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism.
abstract: OBJECTIVE
  Analysis of mortality and risk factors for mortality in the use of renal replacement therapy to correct metabolic disturbances associated with confirmed or suspected inborn errors of metabolism.
  
  
  METHODS
  A retrospective review of an institutional review board-approved pediatric acute renal failure data base at the University of Michigan. Eighteen patients underwent 21 renal replacement therapy treatments for metabolic disturbances caused by urea cycle defects (n = 14), organic acidemias (n = 5), idiopathic hyperammonemia (n = 1), and Reye syndrome (n = 1).
  
  
  RESULTS
  There were 14 boys (74%) and 4 girls (26%), with a mean age and weight of 56.2 +/- 71.0 months and 18.5 +/- 19.2 kg, respectively, at the initiation of renal replacement therapy. Overall treatment mortality rate was 57.2% (12 of 21 treatments), with 11 of the 18 patients (61.1%) dying before hospital discharge. Two-year follow-up on those patients demonstrated that 5 patients (71.4%) remained alive. Initial therapy with hemodialysis was associated with improved survival. Ten treatments (47.6%) required transition to another form of renal replacement therapy to maintain ongoing metabolic control, with a mean duration of 6.1 +/- 9.8 days. Time to renal replacement therapy >24 hours was associated with an increased risk of mortality, whereas a blood pressure >5th percentile for age at the initiation of therapy and the use of anticoagulation were associated with a decreased risk of mortality.
  
  
  CONCLUSIONS
  Renal replacement therapy can correct the metabolic disturbances that accompany suspected or confirmed inborn errors of metabolism. Our experience demonstrates an approximately 60% mortality rate associated with renal replacement treatment, with more than 70% of survivors living longer than 2 years.
authors.affiliation: Department of Nephrology, Children's National Medical Center, Washington, DC 20010, USA. KMCBRYDE@cnmc.org
authors.forename: Kevin D
authors.initials: KD
authors.lastname: McBryde
authors.forename: David B
authors.initials: DB
authors.lastname: Kershaw
authors.forename: Timothy E
authors.initials: TE
authors.lastname: Bunchman
authors.forename: Norma J
authors.initials: NJ
authors.lastname: Maxvold
authors.forename: Theresa A
authors.initials: TA
authors.lastname: Mottes
authors.forename: Timothy L
authors.initials: TL
authors.lastname: Kudelka
authors.forename: Patrick D
authors.initials: PD
authors.lastname: Brophy
mesh_terms: D000138:Acidosis / Q000209:etiology / Q000628:therapy; D058186:Acute Kidney Injury / Q000209:etiology / Q000628:therapy*; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology / Q000628:therapy; D033461:Hyperuricemia / Q000209:etiology / Q000628:therapy; D007022:Hypotension / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications / Q000401:mortality / Q000628:therapy*; D017582:Renal Replacement Therapy; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis