xlh information for dentistsxlhnetwork.org/.../6125/xlh-information-for-dentists.pdfmicrosoft word -...
TRANSCRIPT
XLH is the most common form of inherited rickets.
Genetic Defect: loss-‐of-‐function mutations in PHEX, leading to elevated circulating levels of Fgf23, resulting in impaired renal conservation of phosphate
Systemic Effects: rickets, osteomalacia, growth retardation, and dental effects
Oral Manifestations: “Spontaneous” abscesses, possible delay of eruption, delay/lack of apical closure, increased frequency of malocclusion, periodontal disease
Requests for additional information or specific questions may be directed to*: Leslie Blackburn, DDS, MA [email protected] [email protected] *Please include XLH in the email subject line.
1 Long Wharf Drive, Suite 403 New Haven, CT, 06510
http://www.ynhh.org/medical-‐services/dental_pediatricaspx
Yale-‐New Haven Pediatric Dental Center
XLH: A Review
X-‐Linked Hypophosphatemia
Information for
Dental Practitioners Questions?
1
*Note: Most of the XLH literature is in the form of case studies and also the findings are extremely varied; thus the abnormalities noted below may be present in any combination (or not at all) in any one individual.
Structural Defects: Enamel: Possible hypoplasia or hypocalcification; some suggestion that patients may have thin or more easily abraded enamel and microcracks.
Dentin: 1-‐Mantle dentin and most circumpulpal dentin is normal, dentin intermediate to those areas displays large interglobular areas with unmerged calcospherites. 2-‐Dentinal (tubular) clefts in the area of the pulp horns. 3-‐Decreased ability to form tertiary dentin.
Pulp: Large pulp chambers with high pulp horns (sometimes extending to the DEJ).
Cementum: May be thin.
Alveolar bone: Possible absence of lamina dura.
2
Oral Sequelae Observed in XLH:
“Spontaneous” Abscesses periapical abscesses seen without evidence of caries or trauma; reported in both primary and permanent dentitions *This is the most prevalent oral manifestation of XLH. Increased Risk of Iatrogenic Damage because the pulp horns are so high, it is easy to have an iatrogenic pulp exposure during routine restorative care Periodontal Disease possible increase in risk of periodontal disease, and also increase in severity of periodontal disease Increased Sensitivity During Periodontal Treatment if present, may be due to increased severity of disease and possible thinning of cementum
Structural Defects in XLH Teeth and their sequelae
Treatment in XLH Patients
Preventive Routine exams to monitor for spontaneous abscesses are critical.
Dietary guidance and oral hygiene instruction should be emphasized.
Sealants may be efficacious to prevent access through cracked, abraded, or compromised enamel and dentin.
Nightguards may be considered to protect easily abraded enamel.
Interceptive Restorative treatment should be undertaken with care to avoid iatrogenic pulp exposure due to large pulp chambers and high pulp horns.
As XLH patients who experience spontaneous abscesses often have many such abscesses over the course of a lifetime, root canal therapy and other such measures to save affected teeth are preferable to extraction.