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ISSN 2174-0798 (Print)
Organo oficial de la Asociación Latinoamericana de Odontopediatría (ALOP)

2022, Number 1

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Rev Odotopediatr Latinoam 2022; 12 (1)

Dental eruption aspects of Cornelia de Lange syndrome. Case Report

Palencia TLR, Santaella PJB, Zamudio AYC, Rondón JND

Language: Spanish
References: 32
Page:
PDF size: 317.58 Kb.


ABSTRACT

Cornelia de Lange Syndrome (SCDL) is a genetic abnormality with a prevalence of 1: 62,000-1: 45,000 of births. It is mainly attributed to mutations in the NIPBL, SMC3 and SMC1A genes. It is characterized by presenting general physical alterations, cognitive and language alteration; and orofacial features such as sinofridia, hirsutism, there is also malocclusion, delayed eruption, crowding, anodontia, limb malformation, prenatal and postnatal developmental delay, and other congenital malformations. Objective: To analyze the case of a patient with Cornelia de Lange syndrome and its relationship with some findings reported in the literature, especially dental eruption. We present a 2-year-old and 5-month-old female nursing patient from Valencia with a genetic diagnosis of Cornelia de Lange Syndrome, plumbemia, and biliary lithiasis, who attended the Pediatric Dentistry Postgraduate consultation at the University of Carabobo for presenting delay in tooth eruption. Clinical history, general clinical examination where psychomotor retardation, language and phenotypic characteristics of the syndrome are observed. A clinical intraoral evaluation shows gingival ridges with normal insertion of braces and absence of dental units (delayed eruption). The dental eruption can be affected in patients diagnosed with Cornelia de Lange Syndrome, both in its chronology and in the eruption sequence.


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