Examples of using Kuvan in English and their translations into German
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Medicine
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Colloquial
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Official
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Ecclesiastic
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Financial
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Ecclesiastic
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Political
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Computer
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Programming
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Official/political
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Political
What is Kuvan?
Ml+ 10 ml Kuvan solution and transfer it to a glass or cup for administration.
There are limited data regarding the long-term use of Kuvan.
If your child that was prescribed Kuvan is under 4 years of age.
Kuvan is intended for use in children with HPA due to PKU who are at least four years old.
The recommended starting dose of Kuvan in adults and children with PKU is 10 mg for each kg of body weight.
Kuvan treatment should only be continued in patients who respond adequately to the medicine.
BH4 deficiency The usual starting dose of Kuvan in adult and paediatric patients with BH4 deficiency is 2 to 5 mg for each kg of body weight.
Kuvan has not been specifically studied in paediatric patients under 4 years of age see section 5.1.
The results of these studies demonstrate the efficacy of Kuvan to reduce blood phenylalanine levels and to increase dietary phenylalanine tolerance.
Kuvan is used to treat hyperphenylalaninaemia(HPA) or phenylketonuria(PKU) in adults and children of all ages.
In PKU treatment, a main study compared the reductions in blood phenylalanine in 88 patients treated with either Kuvan or placebo a dummy treatment.
PKU The usual starting dose of Kuvan in adult and paediatric patients with PKU is 10 mg for each kg of body weight.
Kuvan should be used with caution in patients over 65 years of age, and in patients with liver or kidney problems.
By replacing the BH4 that the body cannot produce, Kuvan reduces the harmful excess of phenylalanine in the blood and increases the dietary tolerance to phenylalanine.
Kuvan should not be used in patients who may be hypersensitive(allergic) to sapropterin hydrochloride or any of the other ingredients.
If inadequate control of blood phenylalanine levels is observed during treatment with Kuvan, the patient' s adherence to the prescribed treatment, and diet, should be reviewed before considering an adjustment of the dose of Kuvan.
Kuvan is used to treat high blood levels of phenylalanine in adults and children of all ages with the genetic disorders phenylketonuria(PKU) or tetrahydrobiopterin(BH4) deficiency.
If an unsatisfactory reduction in blood phenylalanine levels is observed,then the dose of Kuvan can be increased weekly to a maximum of 20 mg/ kg/ day, with continued weekly monitoring of blood phenylalanine levels over a one month period.
Kuvan is used to treat hyperphenylalaninaemia(HPA, high levels of phenylalanine in the blood) in patients with the genetic disorders phenylketonuria(PKU) or tetrahydrobiopterin(BH4) deficiency.
The recommended starting dose of Kuvan in adults and children with BH4 deficiency is 2 to 5 mg for each kg of body weight.
In addition Kuvan allowed patients with PKU who were not on a diet restriction to increase their daily phenylalanine intake by 17.5 mg per kg body weight after 10 weeks compared with 3.3 mg/kg with placebo.
The rationale for administration of Kuvan in patients with BH4 Deficiency is to replace the deficient levels of BH4, thereby restoring the activity of phenylalanine hydroxylase.
Kuvan is also indicated for the treatment of hyperphenylalaninaemia(HPA) in adult and paediatric patients with tetrahydrobiopterin(BH4) deficiency who have been shown to be responsive to such treatment see section 4.2.
Treatment with Kuvan must be started and supervised by a doctor who has experience in the treatment of PKU and BH4 deficiency.
Kuvan has not been specifically studied in children under 4 years of age, although the published literature indicates that more than 600 children of 0 to 4 years old with PKU, have been exposed to treatment with an un-registered preparation of BH4, including at least 35 who received therapy≥ 2 months.
It is of primary importance to initiate Kuvan treatment as early as possible to avoid the appearance of non-reversible clinical manifestations of neurological disorders in paediatric patients and cognitive deficits and psychiatric disorders in adults due to sustained elevations of blood phenylalanine.
When Kuvan plus diet restriction was compared with diet restriction alone, the average daily phenylalanine intake that was tolerated after 26 weeks was 81 mg/kg in the Kuvan group and 50 mg/kg in the group on diet restriction alone.
The rationale for administration of Kuvan in patients with BH4-responsive PKU is to enhance the activity of the defective phenylalanine hydroxylase and thereby increase or restore the oxidative metabolism of phenylalanine sufficient to reduce or maintain blood phenylalanine levels, prevent or decrease further phenylalanine accumulation, and increase tolerance to phenylalanine intake in the diet.