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Clinical manifestations of terminal osseous dysplasia and pigmentary defects in a young girl.

Abstract

Terminal osseous dysplasia and pigmentary defects is a rare X-linked dominant disorder with prenatal male lethality. Affected females display multiple systemic abnormalities such as limb deformities and pigmented lesions of the face and scalp. Phenotypic expression of the syndrome varies among the affected individuals. In this case report, we describe the syndromic dental and oral abnormalities in a female child aged 3 and 1/2 years. A widened bigonial width of the mandible and a brachyfacial pattern are observed. Intraoral findings include multiple frenulae, shallow mucobuccal fold, hypodontia, conical incisors, and other developmental structural defects.

Authors+Show Affiliations

Department of Pediatric Dentistry, The University of Texas Health Science Center at San Antonio, Dental School - MSC 7888, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900, USA. linb1@uthscsa.eduNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

12738953

Citation

Lin, Brent P J., et al. "Clinical Manifestations of Terminal Osseous Dysplasia and Pigmentary Defects in a Young Girl." Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, vol. 95, no. 5, 2003, pp. 607-13.
Lin BP, Lin MI, Berlocher WC. Clinical manifestations of terminal osseous dysplasia and pigmentary defects in a young girl. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95(5):607-13.
Lin, B. P., Lin, M. I., & Berlocher, W. C. (2003). Clinical manifestations of terminal osseous dysplasia and pigmentary defects in a young girl. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, 95(5), 607-13.
Lin BP, Lin MI, Berlocher WC. Clinical Manifestations of Terminal Osseous Dysplasia and Pigmentary Defects in a Young Girl. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95(5):607-13. PubMed PMID: 12738953.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical manifestations of terminal osseous dysplasia and pigmentary defects in a young girl. AU - Lin,Brent P J, AU - Lin,Michael I J, AU - Berlocher,William C, PY - 2003/5/10/pubmed PY - 2003/7/25/medline PY - 2003/5/10/entrez SP - 607 EP - 13 JF - Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics JO - Oral Surg Oral Med Oral Pathol Oral Radiol Endod VL - 95 IS - 5 N2 - Terminal osseous dysplasia and pigmentary defects is a rare X-linked dominant disorder with prenatal male lethality. Affected females display multiple systemic abnormalities such as limb deformities and pigmented lesions of the face and scalp. Phenotypic expression of the syndrome varies among the affected individuals. In this case report, we describe the syndromic dental and oral abnormalities in a female child aged 3 and 1/2 years. A widened bigonial width of the mandible and a brachyfacial pattern are observed. Intraoral findings include multiple frenulae, shallow mucobuccal fold, hypodontia, conical incisors, and other developmental structural defects. SN - 1079-2104 UR - https://www.unboundmedicine.com/medline/citation/12738953/Clinical_manifestations_of_terminal_osseous_dysplasia_and_pigmentary_defects_in_a_young_girl_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079210402917099 DB - PRIME DP - Unbound Medicine ER -