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Cited by 29Alkaline Phosphatase Research Studiesdental development and anomaliesBone and Dental Protein Studies

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Dental outcomes for children receiving asfotase alfa for hypophosphatasia

Schroth, Robert J., Long, Catherine, Lee, Victor H.K., Alai-Towfigh, Hamideh, Rockman-Greenberg, Cheryl·2021·Bone
DOI 10.1016/j.bone.2021.116089
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Abstract

Introduction Hypophosphatasia, a genetic disease impeding development of teeth and bones, is associated with premature exfoliation of primary teeth. Hypophosphatasia is caused by mutations in the ALPL gene, which encodes the tissue non-specific form of alkaline phosphatase. Asfotase alfa (Strensiq®) is a human recombinant bone-targeted alkaline phosphatase. Objectives To review development and exfoliation patterns of primary/permanent teeth in a cohort of patients with hypophosphatasia enrolled in an open-label clinical trial of enzyme replacement therapy (ERT) with asfotase alfa. Methods Data were collected from existing study files of a cohort of patients ≤5 years of age with infantile hypophosphatasia. Children were recruited at the Winnipeg site of a global clinical trial and were treated with ERT. Dental information, including the exfoliation/eruption patterns, were recorded at each visit. Results Eleven children (7 females, 4 males) participated. Participants enrolled as infants (5 infants; mean age 3.0 ± 2.3 months) prematurely lost significantly fewer teeth to hypophosphatasia than patients recruited as preschoolers (6 preschoolers; mean age 52.5 ± 11.3 months), who started on asfotase alfa at a later age. Conclusion The oral health of children with early onset infantile hypophosphatasia may be improved with early and continued administration of ERT, compared to institution of therapy later in childhood.

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  • In HPP, enzyme replacement therapy has shifted care from purely symptomatic management to treatment that significantly benefits teeth still undergoing mineralization, improving their density and structure.

    “For example, in the case of HPP, enzyme replacement therapy has shifted care from purely symptomatic management to treatment that significantly benefits teeth still undergoing mineralization, improving their density and structure (Schroth et al. 2021; Smart et al. 2023).”

    From Dental Management of Genetic Dental Disorders: A Critical Review (2025) · cites this paper as Schroth et al. 2021

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References 25 · Cited by 29

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Metadata

Authors
Schroth, R. J., Long, C., Lee, V. H. K., Alai-Towfigh, H., & Rockman-Greenberg, C.
Affiliations
  • Winnipeg Regional Health Authority (CA)
  • University of Manitoba (CA)
  • Children's Hospital Research Institute of Manitoba (CA)
Corresponding author
Schroth, Robert J.
ORCID
Schroth, Robert J.
Venue
Bone
Publisher
Elsevier BV
Published
2021-11
Volume
152
Pages
116089
ISSN
  • 1873-2763 (ISSN-L)
  • 8756-3282
DOI
10.1016/j.bone.2021.116089
OpenAlex
W3177301449
Cited by
29
References
25
Citations by year
2026: 6 · 2025: 8 · 2024: 6 · 2023: 4 · 2022: 5
FWCI
2.81
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Bone (publishedVersion)
  • PubMed (publishedVersion)
Funders
Alexion Pharmaceuticals
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Alkaline Phosphatase Research Studies
  • Health Sciences › Dentistry › General Dentistry › dental development and anomalies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Bone and Dental Protein Studies
Keywords
hypophosphatasia, asfotase alfa, enzyme replacement therapy, infantile hypophosphatasia, primary teeth, tooth exfoliation, tooth eruption, tooth development, ALPL gene, alkaline phosphatase, oral health, treatment timing
MeSH terms
Alkaline Phosphatase, Child, Child, Preschool, Female, Humans, Hypophosphatasia, Immunoglobulin G, Infant, Infant, Newborn, Male, Recombinant Fusion Proteins, Enzyme Replacement Therapy

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