Nephrology Dialysis Transplantation, Vol 14, Issue 4 910-918, Copyright © 1999 by Oxford University Press
F Schaefer, E Straube, J Oh, O Mehls and E Mayatepek
Background. Certain inborn errors of metabolism become
manifest during the neonatal period by acute accumulation of neurotoxic
metabolites leading to coma and death or irreversible neurological damage.
Outcome critically depends on the immediate elimination of the accumulated
neurotoxins. Recent technological progress provides improved tools to
optimize the efficacy of neonatal dialysis. Methods.
We report our experience with continuous venovenous
haemodialysis (CVVHD) in six neonates with hyperammonaemic coma due to
urea-cycle disorders or propionic acidaemia and in one child with leucine
accumulation due to maple-syrup urine disease (MSUD), in comparison with
five patients managed by peritoneal dialysis (PD) (2 hyperammonaemia, 3
MSUD). Application of a new extracorporeal device specifically designed for
use in small children permitted the establishment of stable blood circuits
utilizing small-sized catheters, and the tight control of balanced
dialysate flows over wide flow ranges. Results. Plasma
ammonia or leucine levels were reduced by 50% within 7.1%±4.1 h
by CVVHD and within 17.9±12.4 h by PD
(P<0.05). Also, total dialysis time was shorter
with CVVHD (25±21 h) than with PD (73±35 h,
P<0.02). A comparison of the CVVHD results with
published literature confirmed superior metabolite removal compared to PD,
and suggested comparable efficacy as achieved with continuous
haemofiltration techniques. Apart from accidental pericardial tamponade
during catheter insertion in one case, no major complications were noted
with CVVHD. In three of the five PD patients, dialysis was comprised by
mechanical complications. None of the MSUD patients but four children with
urea-cycle disorders died, two during the acute period and two late during
the first year of life, with signs of severe mental delay. Of the eight
children presenting with hyperammonaemic coma, the four with the most rapid
dialytic ammonia removal rate (50% reduction in >7 h) survived with
no or moderate mental retardation, whereas slower toxin removal was always
associated with a lethal outcome. Simulation studies showed that the
efficacy of neonatal CVVHD is limited mainly by blood-flow restrictions.
Conclusion. While CVVHD is the potentially most
efficacious dialytic technique for treating acute metabolic crises in
neonates, utmost care must be taken to provide an adequately sized vascular
access. Keywords: ammonia; CVVHD; dialysis; leucine;
metabolism; neonates
ORIGINAL ARTICLES
Dialysis in neonates with inborn errors of metabolism
Divisions of Pediatric Nephrology and Metabolic Diseases, University Children's Hospital, Im Neuenheimer Feld 150, D-69120 Heidelberg, Germany; Corresponding author; E-mail: franz_schaefer@med.uni-heidelberg.de
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