Nitisiinone capsules must strictly follow specific storage conditions to ensure drug stability, and metabolic monitoring and dietary management should be coordinated during medication.

1. Storage conditions

(1) Temperature control: It should be stored in a room temperature environment of 20 ° C to 25 ° C, allowing short-term exposure to a temperature range of 15 ° C to 30 ° C.

(2) Moisture prevention measures: Keep the original packaging sealed and do not remove the built-in desiccant to prevent the capsule from getting damp and deteriorating.

(3) Light avoidance requirement: Store in a non transparent container to avoid direct sunlight that may cause the drug to decompose.

2. Basic medication requirements

(1) Dose adjustment: The initial dose of 1mg/kg/d is taken in two doses, adjusted according to the plasma tyrosine level, and maintained at the target value of 200-500 μ mol/L.

(2) Medication method: Swallow the whole capsule without chewing, and take it with a small amount of water to avoid capsule adhesion.

3. Dietary management

(1) Absolute taboo: Do not consume high tyrosine foods (cheese/fermented soy products) and artificial sweeteners containing phenylalanine.

(2) Protein control: Daily animal protein intake ≤ 0.5g/kg, priority should be given to low-fat meats such as chicken breast.

4. Monitoring requirements

(1) Biochemical monitoring: Monitor plasma tyrosine and phenylalanine levels weekly, and check liver function indicators monthly.

(2) Ophthalmic examination: Perform slit lamp examination every quarter to detect early corneal crystal deposition.

5. Special populations

(1) Pregnant women are prohibited from using it: it has clear embryonic toxicity, and women of childbearing age should take dual contraceptive measures during medication.

(2) Breastfeeding period: Medications can be secreted into breast milk, and breastfeeding should be suspended during the treatment period.

6. Exception handling

(1) Omission treatment: Take the medication immediately upon discovery, skip if it is close to the next medication time, and prohibit double dosage.

(2) Adverse reactions: If corneal opacity or abnormal liver function occurs, the medication should be stopped immediately and medical attention should be sought.

7. Drug interactions

(1) Prohibition of co use: Avoid co use with liver enzyme inducers (such as rifampicin), as it may reduce the efficacy.

(2) Caution in combination: When used in combination with warfarin, INR monitoring frequency should be increased.