Prevalence of Enamel Defects and Caries in Diamond Blackfan Anaemia
Research type
Research Study
Full title
Prevalence of Enamel Defects and Dental Caries in Patients with Diamond Blackfan Anaemia Attending St. Mary's Hospital, London
IRAS ID
158317
Contact name
Marielle Kabban
Contact email
Sponsor organisation
King's College London, Guy's Campus
Research summary
Diamond Blackfan Anaemia (DBA) is a rare genetic disorder. There is lack of information in the literature regarding the dental findings in DBA patients. Willis and Seale (1984) reported findings of a supernumerary (extra) tooth, impacted third molars, and obliterations of nerve canals of erupted teeth in one 15 year old DBA patient. An earlier case reported severe gingival inflammation, multiple tooth decay, and poor healing of recent tooth extraction sites (Sanger and Wilson, 1982). Craniofacial abnormalities described as ear deformities, under-development of the midface and lower jaw and cleft palate have also been reported to be associated with DBA (Gripp et al, 2001). Childhood medical problems especially in the first two years of life have been known as possible factors for defects of tooth enamel (Alaluusua, 2010). DBA patients are high risk for enamel defects. The aim of this study is to report the prevalence of enamel defects and dental decay in patients with DBA. All DBA paediatric patients already have a dental exam as part of their routine appointments at the Paediatric Haematology and Bone Marrow Transplantation Unit at St Mary's Hospital, London. Enamel defects will be scored using validated indices; the modified Dental Developmental Defect (DDE) index (FDI, 1992) and the International Caries Detection and Assessment System (ICDAS) index (Ismail, 2007). The postgraduate student will collate and report these findings.
REC name
South Central - Oxford C Research Ethics Committee
REC reference
14/SC/1264
Date of REC Opinion
23 Sep 2014
REC opinion
Further Information Favourable Opinion