Genetic syndromes with premature loss of teeth: A retrospective study and a suggested classification

Mehrez, Mennat I.; Hassib, Nehal; Sayed, Inas; Aboul-Ezz, Eman; Ramzy, Magda I.; El-Hadidi, Saher M.; Abdel-Kader, Mohmed; Bellah, Nermeen El Moataz; Aglan, Mona; Ismail, Samira; Hosny, Laila; Otaify, Ghada; El-Husseini, Rasha; Esmail, Asmaa M.; El-Desouky, Dina; Temtamy, Samia A.

doi:10.4103/MXE.MXE_23_19

Genetic syndromes with premature loss of teeth: A retrospective study and a suggested classification

Authors

Abstract

Aim
The aim was to identify some of the disorders that feature premature loss of teeth (PLOT) and categorize them in a classification that might help in the diagnosis of these syndromes.
Patients and methods
In this study, the authors investigated the presence of PLOT in a cohort of patients over a 6-year period. The authors searched the files for PLOT as a chief complaint and then as a feature. The authors used the London Dysmorphology Database and Online Mendelian Inheritance in Man to find disorders that feature PLOT to review their files if present. Children who had no teeth at the primary evaluation were recalled as well as children on replacement therapies. Criteria for PLOT involved looseness, early loss, and remaining roots. In primary dentition, PLOT was from 2 to 5 years, whereas in permanents, it was at 6 years.
Results
Of 2044 patients, 57 had PLOT. They constituted 19 disorders, which are cherubism, Coffin-Lowry syndrome, congenital insensitivity to pain with anhidrosis, Fanconi–Bickel syndrome, Hajdu-Cheney syndrome, hypophosphatasia, hypophosphatemic rickets, kyphomelic dysplasia, lacrimoauriculodentodigital, Langerhans cell histiocytosis, mandibuloacral dysplasia, microcephalic osteodysplastic primordial dwarfism, Oculodentodigital dysplasia, osteogenesis imperfecta, osteopetrosis, juvenile Paget, polyostotic fibrous dysplasia, vitamin D-dependent rickets 1A, and vitamin D-dependent rickets 2A. Only three cases remain under investigation. The classification was constructed according to the dental findings.
Conclusion
PLOT due to genetic disorders could occur owing to caries, trauma, jaw lesions, and short roots and not only aggressive periodontitis. Both mandibuloacral dysplasia and nail–patella syndrome are probably not PLOT syndromes.

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