Pycnodysostosis: Clinicoradiographic Report of a Rare Case
Identifieur interne : 000098 ( Main/Exploration ); précédent : 000097; suivant : 000099Pycnodysostosis: Clinicoradiographic Report of a Rare Case
Auteurs : Mayur D. Dhameliya ; Ajit D. Dinkar ; Manisha Khorate ; Sapna S. Raut DessaiSource :
- Contemporary Clinical Dentistry [ 0976-237X ] ; 2017.
Abstract
Pycnodysostosis is an uncommon autosomal recessive sclerosing bone disorder which is characterized by short stature and generalized diffuse osteosclerosis. Patients usually have a large head with separated sutures, open fontanels, aplasia of frontal sinuses, obtuse mandibular gonial angle, and acroosteolysis of the distal phalanges. This case report showed a 25-year-old female with features pathognomonic of pycnodysostosis. The emphasis is mainly on the early diagnosis as it has an important role in the general health of such patients and prevention of complications.
Url:
DOI: 10.4103/ccd.ccd_1105_16
PubMed: 28566865
PubMed Central: 5426146
Affiliations:
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Raut Dessai</name><affiliation><nlm:aff id="aff1"></nlm:aff></affiliation></author></analytic><series><title level="j">Contemporary Clinical Dentistry</title><idno type="ISSN">0976-237X</idno><idno type="eISSN">0976-2361</idno><imprint><date when="2017">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Pycnodysostosis is an uncommon autosomal recessive sclerosing bone disorder which is characterized by short stature and generalized diffuse osteosclerosis. Patients usually have a large head with separated sutures, open fontanels, aplasia of frontal sinuses, obtuse mandibular gonial angle, and acroosteolysis of the distal phalanges. This case report showed a 25-year-old female with features pathognomonic of pycnodysostosis. 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