Sapropterin

What is Sapropterin

Sapropterin is indicated in conjunction with a phenylalanine-restricted diet to reduce blood phenylalanine concentrations in patients with hyperphenylalaninemia due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).

Sapropterin is a synthetic form, 6R-BH4, of tetrahydrobiopterin and was the first FDA-approved drug to treat PKU. 

Brand Name

KUVAN

Indications

  • phenylketonuria (PKU)

Side Effects

  1. abdominal pain
  2. agitation
  3. anaphylactoid reactions
  4. arthralgia
  5. bleeding
  6. cough
  7. diarrhea
  8. dizziness
  9. dyspepsia
  10. esophagitis
  11. gastritis
  12. GI bleeding
  13. headache
  14. hyperactivity
  15. irritability
  16. myocardial infarction
  17. nasal congestion
  18. nausea
  19. peripheral edema
  20. pharyngitis
  21. polyuria
  22. rash
  23. rhinitis
  24. rhinorrhea
  25. seizures
  26. vomiting

Monitoring Parameters

  • serum phenylalanine (Phe) concentrations

Contraindications

  • anorexia nervosa
  • breast-feeding
  • bulimia nervosa
  • children
  • fever
  • GI disease
  • hepatic disease
  • infants
  • infection
  • neonates
  • pregnancy
  • renal impairment

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