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362094

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

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Last updated: 29 Dec 2024

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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.

DOI

10.4103/MXE.MXE_23_19

Keywords

Aggressive Periodontitis, classification, premature teeth loss, syndromes

Authors

First Name

Mennat I.

Last Name

Mehrez

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First Name

Nehal

Last Name

Hassib

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nounih@hotmail.com

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0000-0001-8902-9018

First Name

Inas

Last Name

Sayed

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First Name

Eman

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Aboul-Ezz

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First Name

Magda I.

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Ramzy

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First Name

Saher M.

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El-Hadidi

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First Name

Mohmed

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Abdel-Kader

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First Name

Nermeen El Moataz

Last Name

Bellah

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First Name

Mona

Last Name

Aglan

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drmona_aglan@yahoo.com

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First Name

Samira

Last Name

Ismail

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First Name

Laila

Last Name

Hosny

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Orcid

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First Name

Ghada

Last Name

Otaify

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First Name

Rasha

Last Name

El-Husseini

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First Name

Asmaa M.

Last Name

Esmail

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First Name

Dina

Last Name

El-Desouky

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First Name

Samia A.

Last Name

Temtamy

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Volume

8

Article Issue

2

Related Issue

48654

Issue Date

2020-07-01

Receive Date

2019-12-15

Publish Date

2020-07-11

Print ISSN

2090-8571

Online ISSN

2090-763X

Link

https://mxe.journals.ekb.eg/article_362094.html

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https://mxe.journals.ekb.eg/service?article_code=362094

Order

362,094

Publication Type

Journal

Publication Title

Middle East Journal of Medical Genetics

Publication Link

https://mxe.journals.ekb.eg/

MainTitle

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

Details

Type

Article

Created At

29 Dec 2024