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Osteogenesis imperfecta lethal in infancy: case report and scanning electron microscopic studies of the deciduous teeth.
Am J Med Genet. 1982 Dec; 13(4):359-68.AJ

Abstract

Radiologic evaluation of the skeleton and scanning electron microscopic studies of the teeth were performed on an infant boy with a lethal osteogenesis imperfecta (OI) syndrome who died at 10 mo of pneumonia. The skeletal findings included ribs that were focally expanded by fracture calluses, flat vertebral bodies, and wide limb bones. On fractured tooth surfaces, the enamel and dentin were normal as was the dentin calcification front. Although microscopic abnormalities have been noted in teeth from previously reported infants with lethal OI, a few studies also report infants with normal teeth. These differences in dental findings may indicate heterogeneity in OI lethal in infancy. Results of our study indicate that, until the primary biochemical defects in the OI syndromes are elucidated, examination of teeth from other infants with lethal OI and detailed evaluation of other clinical and skeletal features will aid in delineating heterogeneity and variation in expression in lethal OI.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

7158636

Citation

Levin, L S., et al. "Osteogenesis Imperfecta Lethal in Infancy: Case Report and Scanning Electron Microscopic Studies of the Deciduous Teeth." American Journal of Medical Genetics, vol. 13, no. 4, 1982, pp. 359-68.
Levin LS, Rosenbaum KN, Brady JM, et al. Osteogenesis imperfecta lethal in infancy: case report and scanning electron microscopic studies of the deciduous teeth. Am J Med Genet. 1982;13(4):359-68.
Levin, L. S., Rosenbaum, K. N., Brady, J. M., & Dorst, J. P. (1982). Osteogenesis imperfecta lethal in infancy: case report and scanning electron microscopic studies of the deciduous teeth. American Journal of Medical Genetics, 13(4), 359-68.
Levin LS, et al. Osteogenesis Imperfecta Lethal in Infancy: Case Report and Scanning Electron Microscopic Studies of the Deciduous Teeth. Am J Med Genet. 1982;13(4):359-68. PubMed PMID: 7158636.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Osteogenesis imperfecta lethal in infancy: case report and scanning electron microscopic studies of the deciduous teeth. AU - Levin,L S, AU - Rosenbaum,K N, AU - Brady,J M, AU - Dorst,J P, PY - 1982/12/1/pubmed PY - 1982/12/1/medline PY - 1982/12/1/entrez SP - 359 EP - 68 JF - American journal of medical genetics JO - Am J Med Genet VL - 13 IS - 4 N2 - Radiologic evaluation of the skeleton and scanning electron microscopic studies of the teeth were performed on an infant boy with a lethal osteogenesis imperfecta (OI) syndrome who died at 10 mo of pneumonia. The skeletal findings included ribs that were focally expanded by fracture calluses, flat vertebral bodies, and wide limb bones. On fractured tooth surfaces, the enamel and dentin were normal as was the dentin calcification front. Although microscopic abnormalities have been noted in teeth from previously reported infants with lethal OI, a few studies also report infants with normal teeth. These differences in dental findings may indicate heterogeneity in OI lethal in infancy. Results of our study indicate that, until the primary biochemical defects in the OI syndromes are elucidated, examination of teeth from other infants with lethal OI and detailed evaluation of other clinical and skeletal features will aid in delineating heterogeneity and variation in expression in lethal OI. SN - 0148-7299 UR - https://www.unboundmedicine.com/medline/citation/7158636/Osteogenesis_imperfecta_lethal_in_infancy:_case_report_and_scanning_electron_microscopic_studies_of_the_deciduous_teeth_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0148-7299&date=1982&volume=13&issue=4&spage=359 DB - PRIME DP - Unbound Medicine ER -