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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[]; 11 (ä): ä PMID27663197show 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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Orphanet+J+Rare+Dis 2016 ; 11 (ä): ä
