Peritoneal dialysis in the treatment of metabolic crises caused by inherited disorders of organic and amino acid metabolism

Acta Paediatr Scand. 1989 Sep;78(5):706-11. doi: 10.1111/j.1651-2227.1989.tb11130.x.

Abstract

Four neonates who presented with coma secondary to hyperammonaemia resulting in central respiratory failure were treated with peritoneal dialysis for between 16 and 120 hours. Underlying diseases were maple-syrup-urine disease, propionic acidaemia and citrullinaemia in two patients. Clinical improvement was observed in three patients within 16 to 72 hours after institution of peritoneal dialysis. Biochemical analysis revealed a rapid reduction in plasma concentration of leucine, isoleucine and valine as well as their alpha-keto-analogues in the infant suffering from maple-syrup-urine disease. Correction of ammonia, glycine, alanine and propionic acid concentrations was observed in the infant with propionic acidaemia 24-72 hours after institution of peritoneal dialysis. Severe hyperammonaemia (1,000-2,500mumol/l) in two infants with citrullinaemia before peritoneal dialysis was treated successfully in one infant; whereas the second infant showed no clinical improvement despite amelioration of biochemical parameters. Glucose-absorption from peritoneal dialysis solution was in the range of 216-441 mg/kg/h.

Publication types

  • Case Reports

MeSH terms

  • Amino Acid Metabolism, Inborn Errors / complications
  • Amino Acid Metabolism, Inborn Errors / therapy*
  • Ammonia / blood*
  • Citrulline / blood*
  • Creatinine / blood
  • Female
  • Humans
  • Infant, Newborn
  • Male
  • Maple Syrup Urine Disease / complications
  • Maple Syrup Urine Disease / therapy*
  • Peritoneal Dialysis*
  • Propionates / blood*
  • Tyrosine / blood

Substances

  • Propionates
  • Citrulline
  • Tyrosine
  • Ammonia
  • Creatinine