Cargando…

Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature

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 o...

Descripción completa

Detalles Bibliográficos
Autores principales: Yazdizadeh, Mina, Sharifi, Maryam, Torabi Parizi, Arefeh, Alipour, Firoozeh, Ghasempuor, Maryam, Zanguei, Elham, Yazdizadeh, Maryam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9827620/
https://www.ncbi.nlm.nih.gov/pubmed/36624442
http://dx.doi.org/10.1186/s12903-022-02593-5
_version_ 1784867087048507392
author Yazdizadeh, Mina
Sharifi, Maryam
Torabi Parizi, Arefeh
Alipour, Firoozeh
Ghasempuor, Maryam
Zanguei, Elham
Yazdizadeh, Maryam
author_facet Yazdizadeh, Mina
Sharifi, Maryam
Torabi Parizi, Arefeh
Alipour, Firoozeh
Ghasempuor, Maryam
Zanguei, Elham
Yazdizadeh, Maryam
author_sort Yazdizadeh, Mina
collection PubMed
description 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.
format Online
Article
Text
id pubmed-9827620
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-98276202023-01-10 Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature Yazdizadeh, Mina Sharifi, Maryam Torabi Parizi, Arefeh Alipour, Firoozeh Ghasempuor, Maryam Zanguei, Elham Yazdizadeh, Maryam BMC Oral Health Case Report 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. BioMed Central 2023-01-09 /pmc/articles/PMC9827620/ /pubmed/36624442 http://dx.doi.org/10.1186/s12903-022-02593-5 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Yazdizadeh, Mina
Sharifi, Maryam
Torabi Parizi, Arefeh
Alipour, Firoozeh
Ghasempuor, Maryam
Zanguei, Elham
Yazdizadeh, Maryam
Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
title Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
title_full Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
title_fullStr Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
title_full_unstemmed Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
title_short Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
title_sort dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9827620/
https://www.ncbi.nlm.nih.gov/pubmed/36624442
http://dx.doi.org/10.1186/s12903-022-02593-5
work_keys_str_mv AT yazdizadehmina dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature
AT sharifimaryam dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature
AT torabipariziarefeh dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature
AT alipourfiroozeh dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature
AT ghasempuormaryam dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature
AT zangueielham dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature
AT yazdizadehmaryam dentalmanagementofapediatricpatientwithprogressivefamilialintrahepaticcholestasishavingdentalanomaliesacasereportandbriefreviewoftheliterature