Region Jnkpings ln Odontologiska Institutionen
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A case of hypophosphatasia followed from childhood to adulthood over a period of more than thirty years

Slotte C*, Thorstensson B, Bergendal B, Koch G, Lundgren D.
 

Objectives

The aim was to present dental management of a patient with hypophosphatasia, a rare genetic metabolic bone disorder where early loss of permanent teeth is a prevalent symptom.

Methods

In 1974 the patient, at 11 yrs, was referred to the dept. of Paediatric Dentistry with tooth resorptions and marginal bone loss. All first molars were extracted. In 1978, the patient had a trauma when the upper central incisors were exarticulated. The teeth were replanted and endodontically treated.
In 1988 tooth 45 spontaneously exfoliated, and 15 and 47 were extracted due to severe periodontal breakdown. From then on he has had discontinous specialist treatment and failing co-operation.

Results

Thorough periodontal treatment has been given over the years. However, the patient has frequently failed to attend dental appointments. Therefore, together with the patient's smoking habit, periodontal problems have led to further extractions. Most remaining teeth, including the upper central incisors, now exhibit severe progressive attachment loss.
Three titanium implants have been installed unilaterally in the lower jaw, supporting a temporary fixed partial denture(FPD). Breakdown of marginal bone was noted at two of three implants at 2-6 years of follow-up.
The patient is now forty-three years old. Further treatment includes rehabilitation with a removable partial denture in the upper jaw. In the lower jaw, implant placement is considered to support an overdenture or a FPD.

Conclusions

Due to the patient's unwillingness to attend maintenance visits together with his smoking habit, further loss of marginal bone around teeth and implants can be expected.
Therefore, the long-term prognosis of the dentition and the prosthetic restorations in this case is dubious.

Uppdaterad: 2013-04-03
Anna Thofelt, Avdelningen för odontologisk radiologi Jönköping, Folktandvården