PAEDIATRIC DENTISTRY - 3rd Ed. (2005)

Prepubertal periodontitis/systemic diseases:

• very rare;


• autosomal mode of inheritance;


• aggressive periodontal destruction.

The lesions of juvenile or childhood hypophosphatasia become apparent before 2
years of age. Bone defects are usually quite mild with bowing of the legs, proptosis,
and wide-open fontanelles being prominent signs. Dental features are resorption of
alveolar bone (in the absence of marked gingivitis), premature exfoliation of anterior
deciduous teeth, hypoplasia or complete absence of cementum, and the presence of
'small teeth' that have enlarged pulp chambers as a consequence of defective
mineralization (644HFig. 11.18 (a) and (b)). The aplastic or hypoplastic cementum and a
weakened periodontal attachment is thought to render the patients susceptible to
infection with periodontopathogens.

The diagnosis of hypophosphatasia is confirmed biochemically by low activity of
serum ALP and a raised level of phosphoethanolamine in a 24-h urine sample.

645H

Fig. 11.18 (a) Premature loss of BA|AB

in a 3 1/2-year-old child with

hypophosphatasia. The have already

erupted. (b) Extensive root resorption led

to exfoliation of at 4 years of age.

(Reproduced by kind permission of Dr I.

L. Chapple, Professor of Periodontology,

Birmingham, UK.)

11.12.8 Down syndrome

Children with Down syndrome (trisomy 21) do not suffer aggressive periodontal
disease, However, there are a significant number of local and general risk factors that
may exist as a result of the syndrome. Local factors that may serve to increase dental
plaque retention are: