Cementum and Dentin in Hypophosphatasia

Bos, T. van den; Handoko, G.; Niehof, A.; Ryan, Lawrence M.; Coburn, Stephen P.; Whyte, Michael P.; Beertsen, W.

doi:10.1177/154405910508401110

Cited by 127 publications

(98 citation statements)

References 17 publications

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“…The enamel seems not to be directly affected in HPP patients, although enamel hypoplasia, predisposing to dental caries, has been reported. Even if the primary teeth are most characteristically affected, HPP may also cause alterations in the permanent teeth, requiring counseling and early interventions [49][50][51][52][53].…”

Section: Dental Problems In Hppmentioning

confidence: 99%

Hypophosphatasia: an overview of the disease and its treatment

Bianchi

2015

Osteoporos Int

108

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This review presents the current knowledge on hypophosphatasia, a rare genetic disease of very variable severity (from lethal to mild) and clinical presentation, caused by defective production of tissue-non-specific alkaline phosphatase (TNSALP). Hypophosphatasia can affect babies in utero as well as infants, children, and adults. The article first presents the genetics of TNSALP and its many known mutations underlying the disease. Then, it presents the epidemiology, classification, and clinical presentation of the six different forms of the disease (perinatal lethal, prenatal benign, infantile, childhood, adult, and odontohypophosphatasia) as well as the essential diagnostic clues. The last section on treatment presents a survey of the therapeutic approaches, up to the ongoing phase 2 studies of enzyme replacement therapy.

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Section: Dental Problems In Hppmentioning

confidence: 99%

Hypophosphatasia: an overview of the disease and its treatment

Bianchi

2015

Osteoporos Int

108

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“…There is no increased gingival inflammation or periodontitis and the immune response to infections is not defective [88].…”

Section: Resultsmentioning

confidence: 99%

Untitled

2015

Int J Oral Dent Health

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“…In normal individuals, the tooth root surface is covered with cementum tissue, which attaches to alveolar bone via the periodontal ligament. However, in those with hypophosphatasia, the root is not able to adequately attach to absorbed alveolar bone due to disturbed cementum, leading to spontaneous early exfoliation of the affected tooth [5][6][7][8]. Dentists can screen for possible cases of hypophosphatasia if they have such knowledge and then refer the suspected patient to a pediatrician for more detailed examinations.…”

Section: Discussionmentioning

confidence: 99%

Report of Two Dental Patients Diagnosed with Hypophosphatasia

Okawa

Kitaoka²,

Saga³

et al. 2016

J Clin Case Rep

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Hypophosphatasia is a rare inherited skeletal disorder characterized by defective bone mineralization and tissue non-specific alkaline phosphatase (TNSALP) deficiency, with mutations in the gene encoding the TNSALP isozyme the cause. As for dental manifestations, premature loss of primary teeth due to disturbed cementum formation is well known and tooth roots in affected patients are not able to adequately attach to absorbed alveolar bone due to malformed cementum. We report spontaneous early exfoliation along with mild to severe mobility of primary anterior teeth in 2 child patients referred to pediatric dentists from a general dental practitioner. Case 1 was a 1-year-7-month-old boy with 3 mandibular incisors exfoliated, while Case 2 was a 3-year-3-month-old girl with 1 mandibular central incisor exfoliated. The possibility of hypophosphatasia was considered and the patients were referred to the Pediatric Clinic of Osaka University Medical Hospital, where each was diagnosed with hypophosphatasia (odonto type). We performed repeated periodontal treatments and applied removable partial dentures in both cases, which solved esthetic and functional problems. Although the incidence is quite low, it is important to diagnose hypophosphatasia as early as possible for planning general and dental preventive approaches. In patients with mild forms, such as odonto and childhood types, early exfoliation of primary teeth identified in a dental examination can occasionally lead to early diagnosis of hypophosphatasia. In addition, dental findings obtained by a dentist are useful to identify patients with a risk of hypophosphatasia, who can then be immediately referred to a pediatrician for more detailed systemic examinations. Thus, a cooperative relationship between pediatricians and pediatric dentists should be regarded as necessary. This is the first known report to describe cases of hypophosphatasia diagnosed after referral from a pediatric dentist because of typical dental findings for the disorder.

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Cementum and Dentin in Hypophosphatasia

Cited by 127 publications

References 17 publications

Hypophosphatasia: an overview of the disease and its treatment

Hypophosphatasia: an overview of the disease and its treatment

Untitled

Report of Two Dental Patients Diagnosed with Hypophosphatasia

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