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X-linked hypophosphataemia Clinical criteria: Treatment Phase: Initial treatment - New patient Patient must have a documented confirmation of PHEX pathogenic variant; OR Patient must have a confirmed diagnosis of X-linked hypophosphataemia demonstrated by the presence of all of the following: (i) a serum phosphate concentration below the age adjusted lower limit of normal; (ii) current or historical (for those with growth plate fusion) radiographic X-ray evidence of rickets; (iii) elevated (or inappropriately normal) serum or plasma FGF-23 levels of above the mean of the assay-specific reference range; (iv) renal phosphate wasting demonstrated by a ratio of tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) according to age specific normal ranges using the second morning urine void and paired serum sample measuring phosphate and creatinine. Treatment criteria: Must be treated by a medical practitioner identifying as at least one of the following specialists: (i) paediatric endocrinologist, (ii) paediatric nephrologist, (iii) endocrinologist, (iv) nephrologist. At the time of authority application, medical practitioners must request the appropriate number of vials of appropriate strength(s) to provide sufficient drug, based on the weight of the patient, adequate for 4 weeks, according to the specified dosage in the approved Product Information (PI). A separate authority prescription form must be completed for each strength requested. Up to a maximum of 5 repeats will be authorised. Confirmation of eligibility for treatment with diagnostic reports must be documented in the patient's medical records.
“Burosumab is a recombinant human monoclonal IgG1 antibody that binds to and inhibits the biological activity of fibroblast growth factor 23 (FGF23), present in excess in X-linked hypophosphataemia.”
“The clearance of burosumab is dependent on body weight and estimated to be 0.278 l/day in a typical adult (70kg) XLH patient with corresponding disposition half-life (t 1/2 ) in the serum of approximately 18 days.”
Working under the parallel aged-care framework? Aged-care equivalent →
Curated subset. The full adverse-effect list is in the TGA Product Information; click any citation above to open it.