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At least 1,585 records · Page 88Linked to original sources
PubMed1994

Dental and craniofacial findings in a child affected by glycogen storage disease type III.

This paper reports on previously undescribed dentofacial features a child suffering from Glycogen-Storage Disease type III with hepatomegaly and hypertransaminasemia with muscular involvement. Deficient craniofacial development, reduced width of the upper jaw resulting in posterior cross-bite, and taurodontism of the primary dentition were found. Pathogenetic basis for craniofacial abnormalities is discussed. Cooperation between pediatricians and pediatric dentists is strongly recommended for an early diagnosis and treatment of the dentofacial defects of the syndrome.

Cephalometry↗
PubMed1993

The CHARGE association: report of two cases.

Although many reports of the CHARGE association appear in the literature, the dental findings were never discussed before. In this report of two cases, both patients present with delayed eruption of the permanent teeth and a remarkable similarity of the eruption pattern of the mandibular teeth. One lower permanent central incisor is congenitally missing, while the other is malformed. The lower permanent laterals of JM have erupted lingually and interfere with his speech and function of the tongue. Mandibular retrognathism is present. TM had a V-shaped constricted upper arch, which was expanded in a first phase of orthodontic intervention. Fixed appliances are the future considerations for the correction of the malocclusion.

Abnormalities, Multiple↗
PubMed2006

The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): a case report.

Maroteaux-Lamy syndrome is one of the genetic disorders involving disturbances in mucopolysaccharide metabolism resulting in increased storage of acid mucopolysaccharide in various tissues. The basic defect in Maroteaux-Lamy syndrome is a deficiency of arylsulfatase B, which leads to accumulation of dermatan sulfate in tissues and their urinary excretion. The deposition of mucopolysaccharides leads to a progressive disorder involving multiple organs that often results in death in the second decade of life. This disease, which has several oral and dental manifestations, is first diagnosed on the basis of clinical findings. A large head, short neck, corneal opacity, open mouth associated with an enlarged tongue, enlargement of skull, and a long antero-posterior dimension are the main characteristic features. Dental complications can be severe and include unerupted dentition, dentigerous cystlike follicles, malocclusions, condylar defects, and gingival hyperplasia. An 11-year-old boy with Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) is described in this article, with special emphasis on the oral manifestations.

Alveolar Process↗
PubMed1979

Talon cusps: a dental anomaly in the Rubinstein-Taybi syndrome.

Dental casts of fourteen cases of Rubinstein-Taybi syndrome (RTS) were studied. Two exhibited talon cusps, a dental anomaly which is rare in the normal population. Three others exhibited prominent cingula, which were interpreted as representing a tendency toward talon cusp formation. While additional cases should be examined for this anomaly to determine its frequency in RTS, the presence of talon cusps in a suspected case supports that diagnosis. Teeth exhibiting talon cusps are especially prone to caries and often require early treatment if they are to be retained.

Abnormalities, Multiple↗