@article{WeiderSchlagenhaufSeefried2022,
  author    = {Weider, Margareta and Schlagenhauf, Ulrich and Seefried, Lothar},
  title     = {Oral health status of adult hypophosphatasia patients: A cross-sectional study},
  series = {Journal of Clinical Periodontology},
  volume    = {49},
  journal   = {Journal of Clinical Periodontology},
  number    = {12},
  doi       = {10.1111/jcpe.13718},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-293777},
  pages     = {1253 -- 1261},
  year      = {2022},
  abstract  = {Aim This study evaluated the oral health status of adult patients with hypophosphatasia (HPP). Materials and Methods Parameters of oral health assessment comprised decayed/missing/filled teeth (DMFT) index, probing pocket depth and clinical attachment level (CAL) as well as documentation of tooth loss and periodontal health status according to CCD/AAP criteria. Findings were compared with national reference data (DMS V survey) reporting oral health status in age-related controls. Within-group comparisons were made between the HPP patients harbouring one versus two alkaline phosphatase liver/bone/kidney type (ALPL) gene variants. Results Of 80 HPP patients (64 female) with a mean age of 46.4 years (range 24-78) and one (n = 55) or two (n = 18) variants (n = 7 lacking testing) within the ALPL gene, those with two variants displayed substantially higher tooth loss rate (14.0 ± 9.3) than those affected by only one ALPL variant (4.1 ± 5.4), who did not differ substantially from healthy DMS V controls. While DMFT score and severe periodontal diseases (PDs) of HPP patients with one variant only increased with progressing age, the two-variant sub-cohort age independently exhibited increased DMFT scores and a higher rate of severe PDs. Conclusions HPP patients affected by two variants of the ALPL gene exhibited a higher risk of periodontitis and tooth loss than the general population, while patients with one variant developed clinically relevant oral disease symptoms with progressing ageing.},
  language  = {en}
}