Oral Care for Two Children with Alagille Syndrome from Very Early Childhood

  • YANAGITA Ken-ichi
    Department of Pediatric Dentistry, Kyushu University Hospital
  • YAMAZA Haruyoshi
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Faculty of Dental Science, Kyushu University
  • MASUDA Keiji
    Department of Pediatric Dentistry, Kyushu University Hospital
  • NISHIGAKI Soichiro
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Faculty of Dental Science, Kyushu University
  • OOKUMA Yukiko
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Faculty of Dental Science, Kyushu University
  • OGASAWARA Takako
    Department of Pediatric Dentistry, Kyushu University Hospital
  • NONAKA Kazuaki
    Section of Pediatric Dentistry, Division of Oral Health, Growth and Development, Faculty of Dental Science, Kyushu University

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Other Title
  • 幼少期から口腔管理を行ったAlagille症候群の2例

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Abstract

Alagille syndrome is an extremely rare autosomal dominant hereditary disorder, while hepatic ductular hypoplasia is one of its main characteristics, as well as other four abnormalities appearing in the heart, eyes, vertebrae, and face. Liver transplantation from a living donor is performed for many of these cases. We have supported 2 girls with Alagille syndrome with consistent dental health care from an early age (1 year 7 months, 1 year 11 months old, respectively, at first visit). The notable dental findings in these cases are as follows.1.Dark-green discoloration was observed in all primary teeth.2.Enlarged pulp chambers were found in primary teeth with radiographic examinations.3.No abnormalities were observed in soft tissues.4.Multiple dental caries were detected in 1 of the patients.Based on our experiences with dental health care from a very young age, we concluded that it is very important to support healthy growth and development of children with Alagille syndrome by continuous oral care, starting as soon after birth as possible.

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