Kubalayonghaejeong 100 mg (Sapropterin Dihydrochloride)

Adult dose

1) Phenylketonuria The starting dose of Kuvan in adult and pediatric patients with phenylketonuria is 10 mg/kg (body weight) once daily. This dose is usually adjusted within the range of 5 - 20 mg/kg/day to achieve and maintain an appropriate blood phenylalanine level, as determined by the physician. 2) Tetrahydrobiopterin (BH4) deficiency The starting dose of Kuvan in adult and pediatric patients with BH4 deficiency is a total daily dose of 2 – 5 mg/kg (body weight). The dose may be adjusted up to 20 mg/kg/day. Blood phenylalanine levels should be measured before starting this medication and 1 week after starting this medication at the recommended starting dose. If the reduction in blood phenylalanine levels is unsatisfactory, the dose of this medication may be increased weekly up to a maximum of 20 mg/kg per day, while continuing to monitor blood phenylalanine levels weekly. Patients whose blood phenylalanine levels have not decreased by 30% or more after 1 month of treatment at a dose of 20 mg/kg per day, or who have not achieved the therapeutic blood phenylalanine target determined by the treating physician, are considered nonresponders to this medication. Treatment should be discontinued, and this medication should not be continued. Once a response to this medication has been confirmed, the dose may be adjusted within the range of 5-20 mg/kg/day according to the response. The daily dose should be calculated based on body weight and rounded to the nearest 100 mg. In patients with phenylketonuria, Kuvan should be administered as a single daily dose, preferably at the same time each morning. In patients with BH4 deficiency, the total daily dose should be divided into 2-3 doses administered throughout the day.

Child dose

The pediatric dose is the same as the adult dose. Children weighing 20 kg or less, 2 mg/kg/day: 2 kg — 4 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 3 ml Children weighing 20 kg or less, 2 mg/kg/day: 3 kg — 6 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 5 ml Children weighing 20 kg or less, 2 mg/kg/day: 4 kg — 8 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 6 ml Children weighing 20 kg or less, 2 mg/kg/day: 5 kg — 10 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 8 ml Children weighing 20 kg or less, 2 mg/kg/day: 6 kg — 12 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 10 ml Children weighing 20 kg or less, 2 mg/kg/day: 7 kg — 14 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 11 ml Children weighing 20 kg or less, 2 mg/kg/day: 8 kg — 16 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 13 ml Children weighing 20 kg or less, 2 mg/kg/day: 9 kg — 18 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 14 ml Children weighing 20 kg or less, 2 mg/kg/day: 10 kg — 20 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 16 ml Children weighing 20 kg or less, 2 mg/kg/day: 11 kg — 22 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 18 ml Children weighing 20 kg or less, 2 mg/kg/day: 12 kg — 24 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 19 ml Children weighing 20 kg or less, 2 mg/kg/day: 13 kg — 26 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 21 ml Children weighing 20 kg or less, 2 mg/kg/day: 14 kg — 28 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 22 ml Children weighing 20 kg or less, 2 mg/kg/day: 15 kg — 30 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 24 ml Children weighing 20 kg or less, 2 mg/kg/day: 16 kg — 32 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 26 ml Children weighing 20 kg or less, 2 mg/kg/day: 17 kg — 34 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 27 ml Children weighing 20 kg or less, 2 mg/kg/day: 18 kg — 36 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 29 ml Children weighing 20 kg or less, 2 mg/kg/day: 19 kg — 38 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 30 ml Children weighing 20 kg or less, 2 mg/kg/day: 20 kg — 40 mg/day, 1 dissolving tablet, dissolution volume 80 ml, administered solution volume 32 ml Children weighing 20 kg or less, 5 mg/kg/day: 2 kg — 10 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 4 ml Children weighing 20 kg or less, 5 mg/kg/day: 3 kg — 15 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 6 ml Children weighing 20 kg or less, 5 mg/kg/day: 4 kg — 20 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 8 ml Children weighing 20 kg or less, 5 mg/kg/day: 5 kg — 25 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 10 ml Children weighing 20 kg or less, 5 mg/kg/day: 6 kg — 30 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 12 ml Children weighing 20 kg or less, 5 mg/kg/day: 7 kg — 35 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 14 ml Children weighing 20 kg or less, 5 mg/kg/day: 8 kg — 40 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 16 ml Children weighing 20 kg or less, 5 mg/kg/day: 9 kg — 45 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 18 ml Children weighing 20 kg or less, 5 mg/kg/day: 10 kg — 50 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 20 ml Children weighing 20 kg or less, 5 mg/kg/day: 11 kg — 55 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 22 ml Children weighing 20 kg or less, 5 mg/kg/day: 12 kg — 60 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 24 ml Children weighing 20 kg or less, 5 mg/kg/day: 13 kg — 65 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 26 ml Children weighing 20 kg or less, 5 mg/kg/day: 14 kg — 70 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 28 ml Children weighing 20 kg or less, 5 mg/kg/day: 15 kg — 75 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 30 ml Children weighing 20 kg or less, 5 mg/kg/day: 16 kg — 80 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 32 ml Children weighing 20 kg or less, 5 mg/kg/day: 17 kg — 85 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 34 ml Children weighing 20 kg or less, 5 mg/kg/day: 18 kg — 90 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 36 ml Children weighing 20 kg or less, 5 mg/kg/day: 19 kg — 95 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 38 ml Children weighing 20 kg or less, 5 mg/kg/day: 20 kg — 100 mg/day, 1 dissolving tablet, dissolution volume 40 ml, administered solution volume 40 ml Children weighing 20 kg or less, 10 mg/kg/day: 2 kg — 20 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 4 ml Children weighing 20 kg or less, 10 mg/kg/day: 3 kg — 30 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 6 ml Children weighing 20 kg or less, 10 mg/kg/day: 4 kg — 40 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 8 ml Children weighing 20 kg or less, 10 mg/kg/day: 5 kg — 50 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 10 ml Children weighing 20 kg or less, 10 mg/kg/day: 6 kg — 60 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 12 ml Children weighing 20 kg or less, 10 mg/kg/day: 7 kg — 70 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 14 ml Children weighing 20 kg or less, 10 mg/kg/day: 8 kg — 80 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 16 ml Children weighing 20 kg or less, 10 mg/kg/day: 9 kg — 90 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 18 ml Children weighing 20 kg or less, 10 mg/kg/day: 10 kg — 100 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 20 ml Children weighing 20 kg or less, 10 mg/kg/day: 11 kg — 110 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 22 ml Children weighing 20 kg or less, 10 mg/kg/day: 12 kg — 120 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 24 ml Children weighing 20 kg or less, 10 mg/kg/day: 13 kg — 130 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 26 ml Children weighing 20 kg or less, 10 mg/kg/day: 14 kg — 140 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 28 ml Children weighing 20 kg or less, 10 mg/kg/day: 15 kg — 150 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 30 ml Children weighing 20 kg or less, 10 mg/kg/day: 16 kg — 160 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 32 ml Children weighing 20 kg or less, 10 mg/kg/day: 17 kg — 170 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 34 ml Children weighing 20 kg or less, 10 mg/kg/day: 18 kg — 180 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 36 ml Children weighing 20 kg or less, 10 mg/kg/day: 19 kg — 190 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 38 ml Children weighing 20 kg or less, 10 mg/kg/day: 20 kg — 200 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 40 ml Children weighing 20 kg or less, 20 mg/kg/day: 2 kg — 40 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 8 ml Children weighing 20 kg or less, 20 mg/kg/day: 3 kg — 60 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 12 ml Children weighing 20 kg or less, 20 mg/kg/day: 4 kg — 80 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 16 ml Children weighing 20 kg or less, 20 mg/kg/day: 5 kg — 100 mg/day, 1 dissolving tablet, dissolution volume 20 ml, administered solution volume 20 ml Children weighing 20 kg or less, 20 mg/kg/day: 6 kg — 120 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 24 ml Children weighing 20 kg or less, 20 mg/kg/day: 7 kg — 140 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 28 ml Children weighing 20 kg or less, 20 mg/kg/day: 8 kg — 160 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 32 ml Children weighing 20 kg or less, 20 mg/kg/day: 9 kg — 180 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 36 ml Children weighing 20 kg or less, 20 mg/kg/day: 10 kg — 200 mg/day, 2 dissolving tablets, dissolution volume 40 ml, administered solution volume 40 ml Children weighing 20 kg or less, 20 mg/kg/day: 11 kg — 220 mg/day, 3 dissolving tablets, dissolution volume 60 ml, administered solution volume 44 ml Children weighing 20 kg or less, 20 mg/kg/day: 12 kg — 240 mg/day, 3 dissolving tablets, dissolution volume 60 ml, administered solution volume 48 ml Children weighing 20 kg or less, 20 mg/kg/day: 13 kg — 260 mg/day, 3 dissolving tablets, dissolution volume 60 ml, administered solution volume 52 ml Children weighing 20 kg or less, 20 mg/kg/day: 14 kg — 280 mg/day, 3 dissolving tablets, dissolution volume 60 ml, administered solution volume 56 ml Children weighing 20 kg or less, 20 mg/kg/day: 15 kg — 300 mg/day, 3 dissolving tablets, dissolution volume 60 ml, administered solution volume 60 ml Children weighing 20 kg or less, 20 mg/kg/day: 16 kg — 320 mg/day, 4 dissolving tablets, dissolution volume 80 ml, administered solution volume 64 ml Children weighing 20 kg or less, 20 mg/kg/day: 17 kg — 340 mg/day, 4 dissolving tablets, dissolution volume 80 ml, administered solution volume 68 ml Children weighing 20 kg or less, 20 mg/kg/day: 18 kg — 360 mg/day, 4 dissolving tablets, dissolution volume 80 ml, administered solution volume 72 ml Children weighing 20 kg or less, 20 mg/kg/day: 19 kg — 380 mg/day, 4 dissolving tablets, dissolution volume 80 ml, administered solution volume 76 ml Children weighing 20 kg or less, 20 mg/kg/day: 20 kg — 400 mg/day, 4 dissolving tablets, dissolution volume 80 ml, administered solution volume 80 ml

This drug should not be used by patients who are allergic to this medication’s active ingredient or any other components. Caution is advised when using this medication in elderly patients aged 65 years or older, as the safety and efficacy have not been adequately confirmed. Because data on the use of this medication in pregnant women is limited, it should only be used if necessary while strictly managing blood phenylalanine levels before and during pregnancy. Patients taking levodopa should be cautious when using this medication together, as it may increase the risk of seizures, agitation, and hypersensitivity reactions. Caution is necessary for patients with kidney or liver dysfunction, as the safety and efficacy of this medication have not been established. During therapy, patients should continue to maintain a phenylalanine-restricted diet, regularly monitor blood phenylalanine and tyrosine levels, and monitor nutritional status and psychomotor development. If blood phenylalanine levels drop too low, developmental disorders may occur, so blood levels need to be managed appropriately, and food intake should also be regulated. Patients with a tendency for seizures should be particularly careful, as this medication may cause or worsen seizures. Gastrointestinal inflammatory symptoms such as gastritis or esophagitis may occur after taking this medication, and if symptoms are severe, serious complications such as esophageal strictures, ulcers, or bleeding may develop, so patient status should be observed carefully. This medication may interact with certain anticancer drugs or infection treatments, requiring caution when used together, and it should also be used carefully with drugs having vasodilatory effects. Pregnant or nursing mothers should consult a physician about the use of this medication, and if nursing, it should be used cautiously due to uncertainty regarding whether the drug is excreted in breast milk. Overdose may result in symptoms such as dizziness; therefore, caution should be taken not to exceed the recommended dosage, and patients with heart issues related to QT interval shortening should be particularly careful. This medication should be kept out of reach of children, and transferring it to another container may pose risks to its quality and safety. The desiccant capsule inside the bottle should not be swallowed, and any unused medications should be disposed of safely in accordance with local regulations.

Headache, runny nose, decreased amino acid levels (phenylketonuria), vomiting, rhinitis, sore throat, congestion, cough, diarrhea, abdominal pain, hypersensitivity reactions (serious allergic reactions and rash, etc.), indigestion, gastritis, nausea, esophageal pain, oropharyngeal pain, pharyngitis, esophagitis, dizziness

This medication must be stored in a tightly closed container to prevent exposure to air or moisture. The suitable storage temperature is in a cool place between 2 degrees and 25 degrees. Avoid extremely hot or cold places.

Appearance

White to light yellow round soluble tablet

Package unit

30 tablets/bottle, 120 tablets/bottle