title: Peritoneal dialysis in the treatment of metabolic crises caused by inherited disorders of organic and amino acid metabolism.
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.
authors.affiliation: University Children's Hospital, Ulm/Donau FRG.
authors.forename: L
authors.initials: L
authors.lastname: Gortner
authors.forename: D
authors.initials: D
authors.lastname: Leupold
authors.forename: F
authors.initials: F
authors.lastname: Pohlandt
authors.forename: P
authors.initials: P
authors.lastname: Bartmann
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications / Q000628:therapy*; D000641:Ammonia / Q000097:blood*; D002956:Citrulline / Q000097:blood*; D003404:Creatinine / Q000097:blood; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008375:Maple Syrup Urine Disease / Q000150:complications / Q000628:therapy*; D010530:Peritoneal Dialysis; D011422:Propionates / Q000097:blood*; D014443:Tyrosine / Q000097:blood
chemical_list: D011422:Propionates; D002956:Citrulline; D014443:Tyrosine; D000641:Ammonia; D003404:Creatinine