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Pediatric and Developmental Pathology :... 2023Unique dental conditions in children include odontogenic cysts and tumors, hereditary dental diseases, developmental anomalies, and lesions associated with the eruption... (Review)
Review
Unique dental conditions in children include odontogenic cysts and tumors, hereditary dental diseases, developmental anomalies, and lesions associated with the eruption of the primary or permanent teeth. Many of these conditions have long lasting effects on the adult dentition in terms of affecting esthetics, function, and overall quality of life. Inherited dental syndromes affect the dental hard tissues specifically the enamel, dentin, and/or cementum in a generalized manner, involving both primary and permanent teeth. These conditions manifest in altered quality or quantity of the hard tissues, leading to fragility, tooth loss and dental diseases such as caries, periapical pathology, and periodontal disease. This category includes amelogenesis imperfecta, dentinogenesis imperfecta, dentin dysplasia, hypophosphatasia, and hypophosphatemia. Developmental defects such as regional odontodysplasia are defined by involvement of the primary and permanent dentition in a localized manner, identified in early childhood. This review will elaborate on the histologic findings in these selected dental conditions with a discussion on clinical and radiographic findings, as well as molecular features wherever appropriate.
Topics: Adult; Humans; Child, Preschool; Child; Quality of Life; Tooth; Amelogenesis Imperfecta; Syndrome
PubMed: 37962547
DOI: 10.1177/10935266231207045 -
The Journal of Clinical Pediatric... Sep 2023Regional odontodysplasia (RO) is a rare developmental abnormality of epithelial and mesenchymal dental tissues. Due to its poorly understood etiology, assessing and...
Regional odontodysplasia (RO) is a rare developmental abnormality of epithelial and mesenchymal dental tissues. Due to its poorly understood etiology, assessing and discussing related clinical cases of this dental anomaly is crucial to guide professionals in improving its treatment and outcomes. This article aimed to report the case of a 9-year-old male patient who presented to our department with the main complaint of absent eruption of permanent left mandibular quadrant teeth. This is the first case reported in China from a patient with multiple cutaneous nevi on the face and neck, and based on the retrieved clinical and radiographic features, we described and discussed the treatment and etiology of RO.
Topics: Male; Humans; Child; Odontodysplasia; Neck; Mandible; Tooth Eruption
PubMed: 37732452
DOI: 10.22514/jocpd.2023.068 -
Pediatric and Developmental Pathology :... 2023Evaluation of bone pathology within the head and neck region, particularly the gnathic bonesis is complex, demonstrating unique pathologic processes. In part, this... (Review)
Review
Evaluation of bone pathology within the head and neck region, particularly the gnathic bonesis is complex, demonstrating unique pathologic processes. In part, this variation is due to odontogenesis and the embryological cells that may be involved, which can contribute to disease development and histologic variability. As with any boney pathosis, the key is to have clinical correlation, particularly with radiographic imaging prior to establishing a definitive diagnosis. This review will cover those entities that have a predilection for the pediatric population, and while it is not all inclusive, it should serve as a foundation for the pathologist who is evaluating bony lesions involving the craniofacial skeleton.
Topics: Humans; Child; Bone and Bones; Neck; Neoplasms; Odontogenic Tumors
PubMed: 37232383
DOI: 10.1177/10935266231170744 -
British Dental Journal Apr 2023
Topics: Humans; Odontodysplasia
PubMed: 37117359
DOI: 10.1038/s41415-023-5807-8 -
Journal of Dentistry For Children... Jan 2023Regional odontodysplasia (ROD) is a rare developmental anomaly with distinctive clinical, radiographical and histological findings, affecting both primary and permanent...
Regional odontodysplasia (ROD) is a rare developmental anomaly with distinctive clinical, radiographical and histological findings, affecting both primary and permanent dentitions. The teeth with ROD have an atypical morphology and are usually discolored, with either delayed eruption or complete failure to erupt. Radiographically, the affected teeth have a "ghostly" appearance, with marked radiolucency and decreased radiodensity, showing a thin outline of enamel and dentin, which appear hypomineralized histologically, with poorly structured dentinal tubules and enamel prisms. Calcifications are frequently found in the pulp chambers of the affected teeth. This case report discusses a three-year-old girl who presented with ROD in her mandible as well as the clinical and radiographical features, and treatment of the condition.
Topics: Humans; Female; Child, Preschool; Odontodysplasia; Dentition, Permanent; Tooth, Deciduous; Dental Enamel; Mandible
PubMed: 37106528
DOI: No ID Found -
Oral Diseases Sep 2023This review aimed to summarize recent progress on syndromic dentin defects, promoting a better understanding of systemic diseases with dentin malformations, the... (Review)
Review
OBJECTIVE
This review aimed to summarize recent progress on syndromic dentin defects, promoting a better understanding of systemic diseases with dentin malformations, the molecules involved, and related mechanisms.
SUBJECTS AND METHODS
References on genetic diseases with dentin malformations were obtained from various sources, including PubMed, OMIM, NCBI, and other websites. The clinical phenotypes and genetic backgrounds of these diseases were then summarized, analyzed, and compared.
RESULTS
Over 10 systemic diseases, including osteogenesis imperfecta, hypophosphatemic rickets, vitamin D-dependent rickets, familial tumoral calcinosis, Ehlers-Danlos syndrome, Schimke immuno-osseous dysplasia, hypophosphatasia, Elsahy-Waters syndrome, Singleton-Merten syndrome, odontochondrodysplasia, and microcephalic osteodysplastic primordial dwarfism type II were examined. Most of these are bone disorders, and their pathogenic genes may regulate both dentin and bone development, involving extracellular matrix, cell differentiation, and metabolism of calcium, phosphorus, and vitamin D. The phenotypes of these syndromic dentin defects various with the involved genes, part of them are similar to dentinogenesis imperfecta or dentin dysplasia, while others only present one or two types of dentin abnormalities such as discoloration, irregular enlarged or obliterated pulp and canal, or root malformation.
CONCLUSION
Some specific dentin defects associated with systemic diseases may serve as important phenotypes for dentists to diagnose. Furthermore, mechanistic studies on syndromic dentin defects may provide valuable insights into isolated dentin defects and general dentin development or mineralization.
Topics: Humans; Dentinogenesis Imperfecta; Odontodysplasia; Osteogenesis Imperfecta; Dentin; Vitamin D
PubMed: 37094075
DOI: 10.1111/odi.14589 -
International Journal of Clinical... 2023A rare dental developmental anomaly known as regional odontodysplasia (RO) manifests itself in hypoplastic and hypomineralized teeth with discolored yellow to brown...
UNLABELLED
A rare dental developmental anomaly known as regional odontodysplasia (RO) manifests itself in hypoplastic and hypomineralized teeth with discolored yellow to brown enamel. Radiographically, the affected tooth appears as a shell tooth with a large pulp chamber and a thin layer of teeth structure. The treatment plan is not well-established, and a multidisciplinary approach is often needed. However, a literature review revealed that most of the studies regarding RO are case reports, and more research, including epidemiological, genetic, and experimental studies, is needed to understand this condition better. Additionally, the cause of this anomaly is currently unknown. Removing the affected tooth is a controversial matter, and the treatment options should be chosen on an individual basis, considering the patient's goals for treatment. This report describes an uncommon case of an 8-year-old girl who has RO affecting the front teeth in the upper jaw and a long-standing infection caused by actinomycosis.
HOW TO CITE THIS ARTICLE
Helal N. Regional Odontodysplasia with Actinomycosis Infection: A Rare Case Report. Int J Clin Pediatr Dent 2023;16(1):190-197.
PubMed: 37020769
DOI: 10.5005/jp-journals-10005-2504 -
Contemporary Clinical Dentistry 2022Regional odontodysplasia (RO), also called ghost teeth, is a rare nonhereditary developmental dental anomaly affecting the epidermal and mesenchymal tissues associated...
Regional odontodysplasia (RO), also called ghost teeth, is a rare nonhereditary developmental dental anomaly affecting the epidermal and mesenchymal tissues associated with the development of tooth which can affect both primary and permanent dentition. It can affect the child's overall quality of life and sometimes may lead to skeletal malocclusion. Management of such patients requires a multidisciplinary approach. Essix retainers are being widely used as retention appliances. Various modifications of this appliance are also being attempted. Thus, this article aims to focus on the use of Essix retainer as an interim prosthesis by modifying it with the incorporation of pontics to manage partial edentulousness and mild orthodontic corrections in a 7-year-old child diagnosed with bilateral RO.
PubMed: 36213853
DOI: 10.4103/ccd.ccd_434_21 -
Clinical Case Reports May 2022Regional odontodysplasia (RO) in permanent teeth is a rare developmental anomaly of mineralized tissues. Three-dimensional images and data from CBCT allowed to provide...
Regional odontodysplasia (RO) in permanent teeth is a rare developmental anomaly of mineralized tissues. Three-dimensional images and data from CBCT allowed to provide useful information on the degree of tooth calcification and consequently confirm the diagnosis of RO and establish a treatment strategy to minimize future damages and sequels.
PubMed: 35600036
DOI: 10.1002/ccr3.5890 -
Nucleic Acids Research Jun 2022DDX58 encodes RIG-I, a cytosolic RNA sensor that ensures immune surveillance of nonself RNAs. Individuals with RIG-IE510V and RIG-IQ517H mutations have increased...
DDX58 encodes RIG-I, a cytosolic RNA sensor that ensures immune surveillance of nonself RNAs. Individuals with RIG-IE510V and RIG-IQ517H mutations have increased susceptibility to Singleton-Merten syndrome (SMS) defects, resulting in tissue-specific (mild) and classic (severe) phenotypes. The coupling between RNA recognition and conformational changes is central to RIG-I RNA proofreading, but the molecular determinants leading to dissociated disease phenotypes remain unknown. Herein, we employed hydrogen/deuterium exchange mass spectrometry (HDX-MS) and single molecule magnetic tweezers (MT) to precisely examine how subtle conformational changes in the helicase insertion domain (HEL2i) promote impaired ATPase and erroneous RNA proofreading activities. We showed that the mutations cause a loosened latch-gate engagement in apo RIG-I, which in turn gradually dampens its self RNA (Cap2 moiety:m7G cap and N1-2-2'-O-methylation RNA) proofreading ability, leading to increased immunopathy. These results reveal HEL2i as a unique checkpoint directing two specialized functions, i.e. stabilizing the CARD2-HEL2i interface and gating the helicase from incoming self RNAs; thus, these findings add new insights into the role of HEL2i in the control of antiviral innate immunity and autoimmunity diseases.
Topics: Autoimmune Diseases; DEAD Box Protein 58; DEAD-box RNA Helicases; Humans; Immunity, Innate; Metacarpus; Odontodysplasia; RNA
PubMed: 35580046
DOI: 10.1093/nar/gkac361