en POLSKI
ISSN: 1734-1558
Forum Ortodontyczne / Orthodontic Forum
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SCImago Journal & Country Rank
1/2021
vol. 17
 
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abstract:
Case report

Coexistence of the 22q11.2 deletion syndrome and SMMCI A case study of monozygotic twins – a preliminary report

Konrad Małkiewicz
1
,
Natalia Mąkal
1
,
Jakub Bartczak
2
,
Krzysztof Zeman
3
,
Agnieszka Moll-Maryńczak
3
,
Sylwia Majewska-Beśka
4

1.
Department of Orthodontics, the Medical University of Łódź
2.
Outpatient Clinic of Orthodontics, Central Teaching Hospital, the Medical University of Łódź
3.
Department of Paediatrics, Immunology and Nephrology, Polish Mother's Memorial Hospital Research Institute in Łódź
4.
Department of the Developmental Age Dentistry, Chair of Developmental Age Dentistry, the Medical University of Łódź
Forum Ortod 2021; 17 (1): 41-9
Online publish date: 2021/05/04
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Di George syndrome is a chromosomal deletion syndrome that occurs relatively frequently. It was described in the 1960s as a clinical triad involving thymic hypoplasia, immune dysfunction, and cardiac defects. The 22q11.2 deletion syndrome is now considered as a broader concept and encompasses a set of multiorgan anomalies of varying severity.

Aim
The aim of this study is to present cases of six-year-old monozygotic female twins with confirmed 22q11.2 deletion syndrome, including the presence of a solitary median maxillary central incisor (SMMCI) in one of the sisters.

Material and methods
Cases of six-year-old female twins treated at the Department and Outpatient Clinic of Orthodontics and Department and Outpatient Clinic of Developmental Age Dentistry, Central Teaching Hospital of the Medical University of Łódź were evaluated. The case study was based on the analysis of collected medical records, general medical and dental history, clinical examination and additional investigations. The literature search was mainly based on the PubMed database.

Case report
6-year-old female twins diagnosed with the 22q11.2 deletion syndrome presented at outpatient clinics of the Central Teaching Hospital, the Medical University of Łódź. Features of a solitary median maxillary central incisor were observed in one sister.

Summary
Although the genetic basis of facial skeleton and stomatognathic developmental disorders remains unclear in many cases and needs to be clarified by further studies, there is no doubt that affected patients should receive the broadest possible complex dental care to the greatest extent possible within the framework of guaranteed benefits.

keywords:

22q112 deletion syndrome, SMMCI, twins, orthodontic treatment