From the Guidelines
I strongly recommend maintaining the 0.4 mg/kg body weight oral supplementation regimen of burosumab, as this dose has been shown to be effective in raising fasting serum phosphate levels within the lower end of the normal reference range for age, with a maximum dosage of 2.0 mg/kg body weight 1. This dose is typically recommended for patients with X-linked hypophosphataemia, and it is essential to titrate the dose in increments of 0.4 mg/kg body weight to achieve the desired phosphate levels. The pharmacokinetic and pharmacodynamic studies have demonstrated similar results in children and adults, with a drug half-life of ~19 days and peak serum concentrations of burosumab at 7–11 days after injection 1. Some key points to consider when taking burosumab include:
- Monitoring fasting serum phosphate levels every 2 weeks during the first month of treatment, and every 4 weeks for the following 2 months and thereafter as appropriate 1
- Measuring calcium and creatinine levels in urine to calculate the urinary calcium:creatinine ratio 1
- Assessing disease severity through radiographs of the left wrist and/or knees in children who do not respond well to therapy or whose bone deformities worsen despite medical treatment 1 It is crucial to follow the recommended dosage and monitoring schedule to ensure the safe and effective use of burosumab, and to consult with a healthcare provider if any concerns or questions arise.
From the Research
Oral Supplementation Regimen
- The provided studies do not directly address the topic of a 0.4 oral supplementation regimen.
- However, studies 2 and 3 discuss the effects of vitamin D and calcium supplementation on various health outcomes, including bone health and fracture risk.
- Study 2 found that daily supplementation with both vitamin D and calcium was associated with a reduced risk of fracture, while study 3 reported inconsistent findings across studies for bone health outcomes.
- Studies 4, 5, and 6 do not provide relevant information on oral supplementation regimens, as they focus on ethics consultations, guideline recommendations, and external validity in research studies, respectively.