Fast Five Quiz: Juvenile Hypophosphatasia Management

Eric T. Rush, MD, FAAP, FACMG


July 22, 2022

Hypocalcemia may occur in patients with HPP being treated with asfotase alfa. Monitoring calcium levels is important to identify those who require supplementation, per Kishnani and colleagues.

Other laboratory assessments in patients with HPP treated with asfotase alfa include:

  • ALP activity

  • Plasma PLP

  • Plasma inorganic pyrophosphate

  • Urine phosphoethanolamine

  • Vitamin D

  • PO4

  • Routine blood tests: complete blood cell count, liver function, and electrolytes

  • Renal panel

PTH can detect alterations in bone metabolism. PTH is dependent on long-term calcium levels and should be performed when calcium levels are persistently abnormal.

Anti-asfotase alfa IgG (antidrug antibody) tests may be considered in patients with poor response following successful treatment with enzyme replacement therapy.

Learn more about HPP.

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