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Analysis of a kindred with amelogenesis imperfecta.
J Oral Pathol. 1985 May; 14(5):366-74.JO

Abstract

Amelogenesis imperfecta (AI) is a group of hereditary disorders whose manifestations are generally considered as being confined to the teeth. Pedigree analysis of a kindred revealed 2 siblings affected with AI which was consistent with an autosomal recessive mode of inheritance. Cephalometric evaluation showed both children to have an obtuse gonial angle and steep mandibular plane. These skeletal changes were manifested as a severe anterior open bite. Histological evaluation of the teeth demonstrated numerous enamel changes including altered prism morphology, prism coalescence and disruption, globular inclusions, and irregular crystallite orientation. The clinical and histological data are consistent with autosomal recessive pigmented hypomaturation amelogenesis imperfecta. The enamel defects appear to be caused by the combination of decreased mineral deposition with abnormal crystallite and prism formation. Some areas of enamel hypoplasia seem to exist as well.

Authors

No affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

3925102

Citation

Wright, J T.. "Analysis of a Kindred With Amelogenesis Imperfecta." Journal of Oral Pathology, vol. 14, no. 5, 1985, pp. 366-74.
Wright JT. Analysis of a kindred with amelogenesis imperfecta. J Oral Pathol. 1985;14(5):366-74.
Wright, J. T. (1985). Analysis of a kindred with amelogenesis imperfecta. Journal of Oral Pathology, 14(5), 366-74.
Wright JT. Analysis of a Kindred With Amelogenesis Imperfecta. J Oral Pathol. 1985;14(5):366-74. PubMed PMID: 3925102.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Analysis of a kindred with amelogenesis imperfecta. A1 - Wright,J T, PY - 1985/5/1/pubmed PY - 1985/5/1/medline PY - 1985/5/1/entrez SP - 366 EP - 74 JF - Journal of oral pathology JO - J Oral Pathol VL - 14 IS - 5 N2 - Amelogenesis imperfecta (AI) is a group of hereditary disorders whose manifestations are generally considered as being confined to the teeth. Pedigree analysis of a kindred revealed 2 siblings affected with AI which was consistent with an autosomal recessive mode of inheritance. Cephalometric evaluation showed both children to have an obtuse gonial angle and steep mandibular plane. These skeletal changes were manifested as a severe anterior open bite. Histological evaluation of the teeth demonstrated numerous enamel changes including altered prism morphology, prism coalescence and disruption, globular inclusions, and irregular crystallite orientation. The clinical and histological data are consistent with autosomal recessive pigmented hypomaturation amelogenesis imperfecta. The enamel defects appear to be caused by the combination of decreased mineral deposition with abnormal crystallite and prism formation. Some areas of enamel hypoplasia seem to exist as well. SN - 0300-9777 UR - https://www.unboundmedicine.com/medline/citation/3925102/Analysis_of_a_kindred_with_amelogenesis_imperfecta_ L2 - http://www.diseaseinfosearch.org/result/362 DB - PRIME DP - Unbound Medicine ER -