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20mg/kg every 2 weeks IV infusion over 4 hours.(5-7)

50 mg single-use vials for reconstitution to yield (5mg/mL)

IV

Pneumonia, respiratory failure, respiratory distress,catheter-related infection, respiratory syncytial virus infection, gastroenteritis, fever and infusion reactions.

1b

5. Fernandes J, Saudubray JM, Van den Berghe G. Inborn metabolic diseases : diagnosis and treatment. 3rd rev. ed. Berlin ; New York ; London: Springer, 2000.

6. Blau N. Physician's guide to the treatment and follow-up of metabolic diseases. Berlin: Springer, 2006.

7. Saudubray JM, Van den Berghe G, Walter J. Inborn metabolic diseases : diagnosis and treatment. 5th ed. Berlin: Springer, 2012.