Peritoneal dialysis in neonates with inborn errors of metabolism: is it really out of date?
Peritoneal and extracorporeal dialysis are used to treat newborns affected by inborn errors of metabolism to minimize the effects of the acute accumulation of neurotoxic metabolites that can produce irreversible and severe neurological damage and even death. In recent papers, extracorporeal dialysis has been described as more effective than peritoneal dialysis in improving the prognosis in newborns with inborn errors of metabolism and hyperammonemia. However, it appears that the outcome is primarily related to the duration of neonatal hyperammonemic coma. Here we report seven newborns with hyperammonemia caused by inborn errors of metabolism (five with organic acidemias, two with urea-cycle disorders). They received dietetic and pharmacological treatment as well as peritoneal dialysis. Four of the five patients with organic acidemia survived with and without mild neurological impairment (follow-up 3.5-10 years). One died from bacterial sepsis after peritoneal dialysis was discontinued and the peritoneal catheter was removed. One of the two patients affected by urea-cycle disorders, a boy, died during the neonatal period, and the other, a girl, died at the age of 13 months due to severe neurological damage. Our results demonstrate that peritoneal dialysis may still be an effective treatment for neonatal hyperammonemia caused by inborn errors of metabolism. Furthermore, peritoneal dialysis can be administered quickly and easily in all settings, clearly an advantage when fast intervention is so crucial.
Pela
Ivana
I
Pediatrics, Meyer Hospital, Via Luca Giordano 13, Florence 50135, Italy.
Seracini
Daniela
D
Donati
Maria Alice
MA
Lavoratti
Giancarlo
G
Pasquini
Elisabetta
E
Materassi
Marco
M
D000641:Ammonia / Q000097:blood; D005260:Female; D006801:Humans; D022124:Hyperammonemia / Q000628:therapy*; D007231:Infant, Newborn; D008297:Male; D008661:Metabolism, Inborn Errors / Q000628:therapy*; D010530:Peritoneal Dialysis; D014508:Urea / Q000378:metabolism
D000641:Ammonia; D014508:Urea