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KUVAN® Resource Library

 

 

 

 

 

Information For Prescribers

KUVAN® PBS Prescribing Codes

A quick guide to look up the KUVAN® PBS authority prescribing codes – now available for adults with PKU from 1 April 2023

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KUVAN® Dosing Information

A quick guide to the recommended number of KUVAN® 100mg tablets and/or 500mg powder to prescribe, depending on your patient’s body weight

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KUVAN® product information

The Product Information (PI) provides health professionals with a summary of the scientific information relevant to the safe and effective use of this prescription medicine. This information is intended for use in Australia only.

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Publications

The effect of improved dietary control on cognitive and psychiatric functioning in adults with phenylketonuria: The ReDAPT study.

This study demonstrated improvements in cognitive function, anxiety, and depression ratings associated with resumption of dietary control of PKU. These fndings support the importance of lifelong treatment for PKU in improving the cognitive and psychiatric sequelae of the disease. (Burgess NM, et al. Orphanet J Rare Dis. 2021;16(1):35.)

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Long-term safety profile and efficacy of sapropterin: The PKUDOS registry experience.

Data from the PKUDOS registry suggest that sapropterin has a tolerable safety profile and is associated with a significant and persistent decrease in blood Phe levels while improving dietary Phe tolerance. (Longo N, et al. Mol Genet Metab. 2015;114(4):557-563.)

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Suboptimal outcomes in patients with PKU treated early with diet alone: Revisiting the evidence

Despite the remarkable success of public health programs that have instituted newborn screening and early introduction of dietary therapy for PKU, there is a growing body of evidence that suggests that neurocognitive, psychosocial, quality of life, growth, nutrition, bone pathology and maternal PKU outcomes are suboptimal. (Enns GM, et al. Mol Genet Metab. 2010;101(2-3):99-109.)

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Australasian consensus guidelines for the management of phenylketonuria (PKU) throughout the lifespan

Australasian PKU Lifespan Guidelines endorsed by ASIEM (Inwood AC, et al. Australasian consensus guidelines for the management of maternal phenylketonuria (PKU). Available at: https://rb.gy/vg33a.)

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Patient/Caregiver Resources

Quick facts about PKU and KUVAN® for patients

Designed for patients who have been prescribed KUVAN®, this booklet contains information about PKU, how KUVAN® works, and how to administer it effectively.

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Related Resources

KUVAN® Video Library

Phenylketonuria (PKU) Resource Library

Contact Us

Please contact us for more information.

Products available in Australia

BRINEURA® (cerliponase alfa)

BRINEURA® is indicated for the treatment of neuronal ceroid lipofuscinosis type 2 (CLN2) disease, also known as tripeptidyl peptidase 1 (TPP1) deficiency.

KUVAN® (sapropterin dihydrochloride)

KUVAN® is indicated for the treatment of hyperphenylalaninemia (HPA) in sapropterin-responsive adult and paediatric patients with phenylketonuria (PKU) or tetrahydrobiopterin (BH4) deficiency.

NAGLAZYME® (galsulfase (rch))

NAGLAZYME® is indicated as long term enzyme replacement therapy in patients with Mucopolysaccharidosis VI (MPS VI, Nacetylgalactosamine 4- sulfatase deficiency, Maroteaux-Lamy syndrome).

VIMIZIM® (elosulfase alfa (rch))

VIMIZIM® is indicated for the treatment of MPS IVA (Mucopolysaccharidosis type IVA, morquio A syndrome).

VOXZOGO® (vosoritide)

VOXZOGO® is indicated for the treatment of achondroplasia in patients 2 years of age and older whose epiphyses are not closed. The diagnosis of achondroplasia should be confirmed by appropriate genetic testing.