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 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.

Etiological Factors Involved in Molar-Incisor Hypomineralization in 7 to 12-Year-Old Children in Tehran.

Objectives: Molar-incisor hypomineralization (MIH) lesions are common in children. The prevalence of MIH is variable in different communities. However, information regarding the prevalence of MIH in the Iranian population is limited. This study sought to assess the prevalence and etiological factors of MIH in 7-12-year-old children in Tehran. Materials and Methods: This descriptive, cross-sectional study evaluated 1028 elementary students between 7-12 years, selected from different districts of Tehran in 2017. The frequency of MIH in the maxillary and mandibular molars and incisors was determined by clinical examination. In order to assess the role of different factors in the development of MIH, a questionnaire was filled out by the mothers regarding problems during their pregnancy, medical history of children, and age of occurrence of systemic conditions (if any). The effect of different factors on the development of MIH was analyzed by the logistic regression test. Results: The prevalence of MIH was found to be 25.6%. The delivery condition of the mother (P<0.001), history of urinary tract infection (P<0.011), history of chickenpox (P<0.018), and frequent use of amoxicillin during childhood (P<0.041) significantly affected the occurrence of MIH. The most commonly involved teeth were the mandibular left first molars. Conclusion: The prevalence of MIH in our study population was within the range reported in the literature. Considering the relatively high prevalence of MIH in 7-12-year-old children, pediatric dentists should pay special attention to treatment of MIH.