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Etiopathogenic analysis of the caries on three patients with Noonan Syndrome
Elena Barberia Leache1, Dolores Saavedra Ontiveros, Miriam Maroto Edo
1Catedrático de la U.C.M. Facultad de Odontología, Madrid. barberia @ odon.ucm.es
Introduction:
Noonan syndrome is a genetic disease frequently characterized by short height, winged neck and a thoracic defect, among other alterations. However, there are no reports that mention oral health complications. In this article three children are presented with this syndrome in which multiple caries are observed, determining in each case which factors increase their susceptibility to caries.
Clinical Cases:
1. A 4 year old girl who suffered neonatal sepsis, pyelocalyceal ecstasia, congenital hypertrophic pyloric stenosis, cardiopathy, hypoacusia and psychomotor delay. Presented multiple cavitated caries, requiring the extraction of the 4 incisors. 2. A 4-year-old boy afflicted with myocardiopathy pyelocalyceal ecstasia and psychomotor delay. Exhibited multiple cavitated caries of atypical locations. 4 incisors were extracted and replaced with preformed crowns. 3. A 9-year-old boy affected with plagiocephaly, laryngomalasia, hiatal hernia, urinary infections and psycho-motor delay. Presented multiple profound and extensive caries, which needed pulp treatment and preformed crown placement.
Discussion:
The caries in all three patients were similar and of rapid progression. The most relevant factors were: reflux and vomiting of 2 of the patients and continuous and prolonged ingestion of fruit juices of the other. In all three of them there was abnormal exposure to both intrinsic and extrinsic acids in the oral cavity, so as muscular hypotony and psychomotor deficiency that hindered the autoclisis and oral hygiene. It is inferred that in all 3 cases the initial pathology was a description of demineralization of chemical origin concordant to dental erosion, upon which an immediate colonization of microorganisms arose, accelerating tissue destruction. It is concluded that the systemic and familial conditions of children with Noonan Syndrome favor erosion and the establishment of caries that are potentially harmful foci, of which adequate and preventive measures should be implemented.