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2016 ; 11
(1
): 127
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Efficacy and safety of i v sodium benzoate in urea cycle disorders: a
multicentre retrospective study
#MMPMID27663197
Husson MC
; Schiff M
; Fouilhoux A
; Cano A
; Dobbelaere D
; Brassier A
; Mention K
; Arnoux JB
; Feillet F
; Chabrol B
; Guffon N
; Elie C
; de Lonlay P
Orphanet J Rare Dis
2016[Sep]; 11
(1
): 127
PMID27663197
show ga
BACKGROUND: The efficacy and safety of intra-venous (i.v.) sodium benzoate for
treating acute episodes of hyperammonemia in urea cycle enzyme disorders (UCD) is
well known. However, published data do not provide a clear picture of the
benefits and risks of this drug. We report a retrospective multicentre study on
the use of i.v. sodium benzoate in patients treated for UCD between 2000 and 2010
in the 6 French reference centres for metabolic diseases. RESULTS: Sixty-one
patients with UCDs - 22 ornithine transcarbamylase (20 confirmed, 2 suspected),
18 arginino-succinate synthetase, 15 carbamoyl phosphate synthetase, 3
arginosuccinate lyase, 1 arginase deficiency, 1 N-acetylglutamate synthetase, 1
HHH syndrome - required i.v. sodium benzoate over the course of 95 acute episodes
(NH3?>?100 ?mol/L or high-risk situations, i.e., gastroenteritis, surgery). Forty
out of 61 patients experienced only one episode of decompensation (neonatal coma,
68.6 %). The most frequent cause of late decompensation was infection (55.5 %). A
loading dose of i.v. sodium benzoate (median 250 mg/kg over 2 h) was administered
for 41/95 acute episodes. The median maintenance dose was 246.1 mg/kg/day,
administered via peripheral venous infusion in all cases except one via a central
line. The total median duration of i.v. sodium benzoate treatment per episode was
2 days (0-13 days). The median durations of hospitalization in intensive care and
metabolic units were 4 days (0-17 days) and 10 days (0-70 days), respectively.
Eight patients died during the neonatal coma (n?=?6) or surgery (n?=?2). The
median plasma ammonium level before treatment was 245.5 ?mol/L
(20.0-2274.0 ?mol/L); it decreased to 40.0 ?mol/L in patients who were alive
(13.0-181.0 ?mol/L) at the end of treatment with i.v. sodium benzoate. A decrease
in ammonium level to???100 ?mol/L was obtained in 92.8 % of episodes (64/69 of
the episodes recorded for the 53 surviving patients). Five patients required
another treatment for hyperammonemia (sodium phenylacetate?+?sodium benzoate,
haemofiltration). Eighteen side effects were reported related to the i.v.
infusion (local diffusion, oedema). CONCLUSION: This 10-year retrospective study
shows that i.v. sodium benzoate associated with an emergency regimen is an
effective and safe treatment for acute episodes of UCD.
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