We provide you with the best medicine purchasing service
Quickly morph client-centric results through performance based applications. Proactively facilitate professional human capital for cutting-edge.
Download Now
Sodium benzoate
Hyperammonaemia in urea cycle disorders and in undiagnosed patients with hyperammonaemia, no evidence to support use of sodium benzoate in acute crises of propionic or methylmalonic academia (101).
Acute crises: 0.25 g/kg (<20 kg) or 5.5 g/m2 (>20kg) as a loading dose followed by same dose as maintenance dose over 24 hours (22, 104).
In difficult to control chronic hyperammonemia, a dose of 250 mg/kg PO divided in 3 doses may be considered (101).
In NKH up to 750 mg/kg/day have been used (57).
Powder
Endocrine metabolic: Hyperammonemia (5%), Hypokalemia (7%), Metabolic acidosis (4%)
Hematologic: Anemia (4%), Disseminated intravascular coagulation (3%)
Neurologic: Cerebral edema (5%), Coma (3%), Neurotoxicity, Seizure (6%)
4
22. Kranen S, Keough D, Gordon RB, Emmerson BT. Xanthine-containing calculi during allopurinol therapy. J Urol 1985;133(4):658-9
57. Gahl WA, Thoene JG, Schneider JA. Cystinosis. N Engl J Med 2002;347(2):111-21 doi: 10.1056/NEJMra020552[published Online First: Epub Date]|.
101. Lu FL, Wang PJ, Hwu WL, Tsou Yau KI, Wang TR. Neonatal type of nonketotic hyperglycinemia. Pediatric neurology 1999;20(4):295-300
104. Wraith JE, Clarke LA, Beck M, et al. Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human alpha-L-iduronidase (laronidase). J Pediatr 2004;144(5):581-8 doi: 10.1016/j.jpeds.2004.01.046[published Online First: Epub Date]|.
