Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A

Introduction: Dentinogenesis imperfecta (DI) is a genetic disease that affects the dentin of the teeth. The autosomal dominantly inherited disease affects only the tissues of connective tissue (mesodermal) origin of the teeth, so there are no changes in the enamel and the periodontium. Due to poor q...

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Main Authors: Laura Lipták, Klaudia Lipták, Gergely Balaton, Noémi Katinka Rózsa
Format: Article
Language:English
Published: Hungarian Dental Association 2023-12-01
Series:Fogorvosi Szemle
Subjects:
Online Access:https://ojs3.mtak.hu/index.php/fogorv-szemle/article/view/13805
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author Laura Lipták
Klaudia Lipták
Gergely Balaton
Noémi Katinka Rózsa
author_facet Laura Lipták
Klaudia Lipták
Gergely Balaton
Noémi Katinka Rózsa
author_sort Laura Lipták
collection DOAJ
description Introduction: Dentinogenesis imperfecta (DI) is a genetic disease that affects the dentin of the teeth. The autosomal dominantly inherited disease affects only the tissues of connective tissue (mesodermal) origin of the teeth, so there are no changes in the enamel and the periodontium. Due to poor quality dentin, the teeth become discolored and the enamel easily peels off. In the dental treatment of patients with DI, the focus should be on indirect restorations as soon as possible, so the dentist must diagnose the condition as early as possible so that the possibility of early intervention is realized, as well as balancing the decision for early intervention with the long-term survival of the restorations. Case report: A 15-year-old young male patient came to the Department of Paediatric Dentistry and Orthodontics of Semmelweis University, to have the veneers on his lower incisors replaced. He also wanted a comprehensive aesthetic rehabilitation. The anamnesis revealed that the patient’s father also suffers from DI, and the patient lives with hemophilia A. Based and the literature evidence and recommendations, we planned indirect restorations. The decreased occlusal vertical dimension was increased; in the first phase to adapt the neuromuscular system to the increased vertical dimension, polymethyl-methacrylate (PMMA) restorations were prepared. After the patient both aesthetically and functionally having been satisfied with the temporary restorations, the final restoration were planned as a monolithic zirconia crowns and splints for the upper and lower arches. For the cementation resin modified glass ionomer was used to avoid the adhesive technique, which is not recommended in such cases. The prosthodontic phase was preceded by professional oral hygiene treatment, instruction, and motivation. Summary: For patients with DI, the key is the early diagnosis and its treatment is essential. Scientific evidence indicates that indirect restorations should be preferred over direct ones in patients with DI. Close cooperation with the hematologist is necessary during the treatment of the patient with hemophilia A.
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spelling doaj.art-b18d92963a054375ba3d65dafb4fed172023-12-18T21:05:45ZengHungarian Dental AssociationFogorvosi Szemle2498-81702023-12-01116419119710.33891/FSZ.116.4.191-19713805Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia ALaura Lipták0Klaudia Lipták1Gergely Balaton2Noémi Katinka Rózsa3Semmelweis Egyetem, Fogorvostudományi Kar, Gyermekfogászati és Fogszabályozási KlinikaSemmelweis Egyetem, Fogorvostudományi Kar, Fogpótlástani KlinikaSemmelweis Egyetem, Fogorvostudományi Kar, Gyermekfogászati és Fogszabályozási KlinikaSemmelweis Egyetem, Fogorvostudományi Kar, Gyermekfogászati és Fogszabályozási KlinikaIntroduction: Dentinogenesis imperfecta (DI) is a genetic disease that affects the dentin of the teeth. The autosomal dominantly inherited disease affects only the tissues of connective tissue (mesodermal) origin of the teeth, so there are no changes in the enamel and the periodontium. Due to poor quality dentin, the teeth become discolored and the enamel easily peels off. In the dental treatment of patients with DI, the focus should be on indirect restorations as soon as possible, so the dentist must diagnose the condition as early as possible so that the possibility of early intervention is realized, as well as balancing the decision for early intervention with the long-term survival of the restorations. Case report: A 15-year-old young male patient came to the Department of Paediatric Dentistry and Orthodontics of Semmelweis University, to have the veneers on his lower incisors replaced. He also wanted a comprehensive aesthetic rehabilitation. The anamnesis revealed that the patient’s father also suffers from DI, and the patient lives with hemophilia A. Based and the literature evidence and recommendations, we planned indirect restorations. The decreased occlusal vertical dimension was increased; in the first phase to adapt the neuromuscular system to the increased vertical dimension, polymethyl-methacrylate (PMMA) restorations were prepared. After the patient both aesthetically and functionally having been satisfied with the temporary restorations, the final restoration were planned as a monolithic zirconia crowns and splints for the upper and lower arches. For the cementation resin modified glass ionomer was used to avoid the adhesive technique, which is not recommended in such cases. The prosthodontic phase was preceded by professional oral hygiene treatment, instruction, and motivation. Summary: For patients with DI, the key is the early diagnosis and its treatment is essential. Scientific evidence indicates that indirect restorations should be preferred over direct ones in patients with DI. Close cooperation with the hematologist is necessary during the treatment of the patient with hemophilia A.https://ojs3.mtak.hu/index.php/fogorv-szemle/article/view/13805dentinogenesis imperfectahemophilia aindirect restorationmonolithic zirconiafixed denture
spellingShingle Laura Lipták
Klaudia Lipták
Gergely Balaton
Noémi Katinka Rózsa
Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A
Fogorvosi Szemle
dentinogenesis imperfecta
hemophilia a
indirect restoration
monolithic zirconia
fixed denture
title Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A
title_full Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A
title_fullStr Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A
title_full_unstemmed Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A
title_short Prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia A
title_sort prosthetic rehabilitation of a young patient with dentinogenesis imperfecta and hemophilia a
topic dentinogenesis imperfecta
hemophilia a
indirect restoration
monolithic zirconia
fixed denture
url https://ojs3.mtak.hu/index.php/fogorv-szemle/article/view/13805
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AT gergelybalaton prostheticrehabilitationofayoungpatientwithdentinogenesisimperfectaandhemophiliaa
AT noemikatinkarozsa prostheticrehabilitationofayoungpatientwithdentinogenesisimperfectaandhemophiliaa