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- Title
Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature.
- Authors
Yazdizadeh, Mina; Sharifi, Maryam; Torabi Parizi, Arefeh; Alipour, Firoozeh; Ghasempuor, Maryam; Zanguei, Elham; Yazdizadeh, Maryam
- Abstract
Background: Progressive familial intrahepatic cholestasis is a heterogeneous group of disorders, leading to intrahepatic cholestasis, with the possibility of chronic liver failure and biliary cirrhosis. Oligodontia is either the manifestation of a specific syndrome or is non-syndromic. To the best of our knowledge, this is the first case report of type 3 progressive familial intrahepatic cholestasis and concurrent oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition in the medical and dental literature. Case presentation: We present the dental and medical histories and comprehensive dental management of a girl with type 3 progressive familial intrahepatic cholestasis and several dental anomalies, who was referred to a dental clinic due to severe dental caries and pain. Conclusion: Our findings suggest that PFIC with manifestations as oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition, might indicate an unknown syndrome; otherwise, the craniofacial anomalies are the manifestations of an independent disease coinciding with PFIC. Moreover, our case is a good example of the importance of timely medical and dental care in confining further health-related complications. The patient was able to ingest without any pain or discomfort after receiving proper dental management.
- Subjects
CHOLESTASIS; PAIN; MIXED dentition; ORAL hygiene; CIRRHOSIS of the liver; HYPODONTIA; TEETH abnormalities; CRANIOSYNOSTOSES; DENTISTRY; DENTAL caries; DENTITION; PEDIATRIC dentistry; LIVER failure
- Publication
BMC Oral Health, 2023, Vol 23, Issue 1, p1
- ISSN
1472-6831
- Publication type
Article
- DOI
10.1186/s12903-022-02593-5