Premium
Medical and dental characteristics of children with chromosome 22q11.2 deletion syndrome at the Royal Children's Hospital, Melbourne
Author(s) -
Wong Deborah H.,
Rajan Sadna,
Hallett Kerrod B.,
Manton David J.
Publication year - 2021
Publication title -
international journal of paediatric dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.183
H-Index - 62
eISSN - 1365-263X
pISSN - 0960-7439
DOI - 10.1111/ipd.12755
Subject(s) - medicine , hypoparathyroidism , pediatrics , heart disease , retrospective cohort study , dentition , digeorge syndrome , population , dentistry , psychiatry , environmental health
Background Chromosome 22q11.2 deletion syndrome (22q11.2DS) is a multifaceted syndrome with a variable phenotype. Few studies have described the associated dental characteristics and their relationship with medical co‐morbidities; and no Australian data exist. Aim To determine the clinical manifestations and correlations between oral and medical conditions in children with 22q11.2DS. Design A retrospective observational study. Children genetically diagnosed with 22q11.2DS at the Royal Children's Hospital Melbourne were selected; their medical and dental characteristics were collated and analysed. Results The study population (n = 57; mean age 11.5 years, range 2‐27 years) experienced a range of medical conditions involving multiple medical systems; of whom 44 (77.2%) had caries experience, 7 (12.3%) developmentally missing teeth, and 31 (54.4%) developmental defects of enamel (DDE). Smaller proportions of primary teeth were affected by DDE in children with congenital heart disease (2.2% vs 9.7%; P = .02), and cardiac surgery (0.2% vs 9%; P = .001). Conversely, children with hypoparathyroidism (n = 2) had significantly higher proportions of primary teeth affected by DDE (27.5% vs 4%; P = .02). Conclusions Significant associations existed between medical conditions (congenital heart disease, history of cardiac surgery, and hypoparathyroidism) and primary dentition DDE in children with 22q11.2 DS.