Oral abnormalities in the Saldino-Noonan syndrome

Virchows Archiv. A, Pathological Anatomy and Histopathology
H Strømme KoppangP S Hoel

Abstract

The oral and dental features of a case of the Saldino-Noonan lethal short rib-polydactyly syndrome (SNS) are described. Natal teeth were noted. The anterior maxillary and mandibular fornices and the central labial frenula were absent. The tongue appeared larger than normal and lacked the sulcus terminalis and the vallate and foliate papillae. The tooth anlagen were abnormal. Microscopic studies revealed small tooth buds, in which hard tissue formation was more advanced than gestational age; abnormalities were noted primarily in the most recently formed dental tissue, indicating that the biochemical defect responsible for this disorder had acted abnormally on dentinogenesis mainly shortly prior to birth. Studies of oral and tooth development should be important to better understanding the abnormal function of the chondrodystrophy genes.

References

Mar 1, 1978·Pediatrics·P Bidot-LópezM J Mahoney
Mar 1, 1979·Oral Surgery, Oral Medicine, and Oral Pathology·D G Gardner, L C Dort
Jan 1, 1978·Scandinavian Journal of Dental Research·G AnnerothG Linge
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Jan 17, 1974·Zeitschrift Für Kinderheilkunde·J SprangerR Krepler
Feb 1, 1972·The American Journal of Roentgenology, Radium Therapy, and Nuclear Medicine·R M Saldino, C D Noonan
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Jan 1, 1980·European Journal of Pediatrics·N KaibaraK Takagishi
May 1, 1957·Oral Surgery, Oral Medicine, and Oral Pathology·E HALS

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Citations

Apr 30, 2016·American Journal of Medical Genetics. Part a·Lucas BoerRoelof-Jan Oostra
Dec 17, 2014·The Journal of Craniofacial Surgery·Fatih CeranSamet Vasfi Kuvat

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