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Early tooth loss in children: a warning sign of childhood hypophosphatasia

From Volume 44, Issue 4, April 2017 | Pages 317-321

Authors

Sara L Hughes

BDS, MFDS RCS(Glasg)

Dental Foundation Trainee Year 2, Unit of Paediatric Dentistry, University Dental Hospital, Cardiff, UK

Articles by Sara L Hughes

Rachel C Parkes

BDS, MFDS RCS(Glasg)

Specialist Trainee in Paediatric Dentistry, Unit of Paediatric Dentistry, University Dental Hospital, Cardiff, UK

Articles by Rachel C Parkes

Nicholas Drage

BDS, FDS RCS(Eng), FDS RCPS(Glas), DDR RCR

Consultant Maxillofacial Radiologist, Cardiff University, School of Dentistry, Heath Park, Cardiff CF14 4XY

Articles by Nicholas Drage

Mechelle Collard

Consultant in Paediatric Dentistry, University Dental Hospital, Cardiff, UK

Articles by Mechelle Collard

Abstract

Premature exfoliation of primary teeth may be the first manifestation of this serious condition and the general dental practitioner plays an important role in recognizing dental anomalies and referring patients at an appropriate time. This is imperative to ensuring early diagnosis and good quality patient care. This article describes the case of a 4-year-old boy affected by childhood hypophosphatasia, who presented with premature exfoliation of his primary teeth as the first manifestation of this condition. An overview of the condition is outlined including a discussion of the likelihood of permanent dentition involvement.

CPD/Clinical Relevance: Oral manifestations of hypophosphatasia may be the first and the only signs of this condition and may be the basis upon which a diagnosis is made.

Article

A 4-year-old boy (JJ) was referred to the Unit of Paediatric Dentistry at the University Dental Hospital, Cardiff by a consultant paediatrician regarding premature exfoliation of his primary teeth. JJ's parents were concerned about the rapid loss of his teeth along with delayed growth, for which he was under the care of both a geneticist and a paediatrician. Despite numerous medical investigations, a definitive diagnosis had not been confirmed.

JJ's first tooth erupted at the age of 15 months, became progressively mobile and exfoliated shortly afterwards. A total of 11 primary anterior teeth excluding the URC exfoliated promptly after eruption and JJ's mother reported difficulties with speech and mastication. JJ has a fit and healthy 6-year-old brother and no known family history of dental abnormalities.

Extra-orally, JJ was of small stature with a dolichocephalic head shape. Intra-orally, the soft tissues were healthy, the dentition was caries free and oral hygiene was good. Teeth present included all primary molars and the URC, all other primary teeth having exfoliated (Figure 1). The URC was grade 2 mobile.

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