The impact of enzyme replacement therapy on the oral health manifestations of hypophosphatasia among children: a scoping review

• Published in: European archives of paediatric dentistry Vol. 24; no. 4; pp. 429 - 440
• Main Authors: Smart, G., Jensen, E. D., Poirier, B. F., Sethi, S.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-08-2023; Springer Nature B.V
• Subjects: Bones; Child; Child, Preschool; Children; Dental enamel; Dentistry; Endodontics; Enzyme Replacement Therapy - adverse effects; Enzyme Replacement Therapy - methods; Enzymes; Humans; Hypercalcemia; Hypophosphatasia - drug therapy; Infant; Medicine; Mutation; Oral Health; Oral hygiene; Phosphatase; Systematic Review; Teeth; Therapy; Canada; United States > US; Japan; Odontohypophosphatasia; Children; Enzyme replacement therapy; Hypophosphatasia
• ISSN: 1818-6300; 1996-9805
• DOI: 10.1007/s40368-023-00796-0

Purpose
A scoping review to describe the use of enzyme replacement therapy (ERT) in the form of asfotase alfa to decrease the severity of oral manifestations in children with hypophosphatasia (HPP).
Methods
Six databases were searched using keywords and index terms related to “hypophosphatasia,” “children,” and “enzyme replacement therapy.” Duplicates were removed and two independent reviewers screened the titles and abstracts to identify articles for full-text review. Extracted data was summarised narratively.
Results
The systematic search identified 3548 articles, with 171 suitable for full-text review and a final 22 that met inclusion criteria. Enzyme replacement therapy generally resulted in a reduction in the presence and severity of oral manifestations of HPP. However, numerous studies failed to report specific details regarding the nature of oral health outcomes and there were reported cases of further loss of primary teeth.
Conclusions
The available evidence suggests that that ERT in the form of asfotase alfa for HPP in infants and young children leads to improved oral health outcomes. It is recommended that the outcomes are improved with earlier initiation of ERT. Further, well-designed clinical research is required to assess oral health improvements and decreased morbidity associated with the early loss of teeth.