Inborn errors of metabolism:
Indications for NULIBRY:
To reduce the risk of mortality in patients with molybdenum cofactor deficiency (MoCD) Type A.
Adults and Children:
Initiate treatment if patient has a diagnosis or presumptive diagnosis; discontinue if presumptive diagnosis is not confirmed by genetic testing. Administer by IV infusion pump once daily at a rate of 1.5mL/min. <37 weeks (preterm neonates): initially 0.4mg/kg, then 0.7mg/kg at Month 1, and 0.9mg/kg at Month 3. ≥37 weeks (term neonates): initially 0.55mg/kg, then 0.75mg/kg at Month 1, and 0.9mg/kg at Month 3. ≥1yr: 0.9mg/kg.
Avoid or minimize exposure to direct sun or UV light (eg, UVA or UVB phototherapy). Advise use of precautionary measures for photosensitivity (eg, protective clothing, hats, sunscreen). Pregnancy. Nursing mothers.
Cyclic pyranopterin monophosphate (cPMP).
Do not mix or administer with other drugs.
Catheter-related complications, pyrexia, viral infection, pneumonia, otitis media, vomiting, cough/sneezing, viral upper respiratory infection, gastroenteritis, bacteremia, diarrhea; photosensitivity.
Generic Drug Availability:
Single-dose vial (5mL)—1