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Acta Dermato-venereologica Mar 2020Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue...
Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue fragility. However, there are limited published data on the dental manifestations of EDS. This review systematically assessed the spectrum of published dental anomalies in various types of EDS. Twenty-four individual case reports/series and 3 longer case-control studies, reporting on a total of 84 individuals with a clinical diagnosis of EDS, were included in the data analysis. The main dental features listed in classical EDS were pulp calcification and localized root hypoplasia. Common dental abnormalities observed in vascular EDS were pulp shape modifications (52.2%), exceeding root length (34.8%), and molar root fusion (47.8%). Dentinogenesis imperfecta is a consistent finding in osteogenesis imperfecta/EDS overlap syndrome. Data on dental manifestations in other types of EDS are both rare and generally inconclusive.
Topics: Dental Pulp Calcification; Ehlers-Danlos Syndrome; Humans; Tooth Abnormalities; Tooth Diseases; Tooth Root
PubMed: 32147746
DOI: 10.2340/00015555-3428 -
Journal of Endodontics Mar 2019The primary aim was to explore the criteria used in characterization of reparative cells and mineralized matrices formed after treatment of pulp exposures, and the...
INTRODUCTION
The primary aim was to explore the criteria used in characterization of reparative cells and mineralized matrices formed after treatment of pulp exposures, and the sequence of relative events. The secondary aim was to evaluate whether the reparative events depend on the experimental model species, age, and therapeutic intervention.
METHODS
A literature search of databases using different combinations of the key words was undertaken. Data analysis was based only on studies having histological or histochemical assessment of the pulp tissue responses. The search yielded 86 studies, 47 capping material-based and 39 bioactive application-based experiments, which provided data on morphological or functional characterization of the mineralized matrices and the associated cells.
RESULTS
In 64% of capping material-based and 72% of bioactive application-based experiments, a 2-zone mineralized matrix formation (atubular followed by tubular) was detected, whereas characterization of odontoblastic differentiation is provided in only 25.5% and 46.1% of the studies, respectively. In 93.3% of the studies showing odontoblast-like cells, differentiated cells were in association with tubular mineralized matrix formation. Analyses further showed that cell- and matrix-related outcomes do not depend on experimental model species, age, and therapeutic intervention.
CONCLUSIONS
The evidence of the reviewed scientific literature is that dental pulp cells secrete a dentin-like matrix of tubular morphology in relation to primitive forms of atubular or osteotypic mineralized matrix. Furthermore, data analysis showed that dental pulp cells express in vivo the odontoblastic phenotype, and secrete matrix in a predentin-like pattern, regardless of the model species, age, and therapeutic intervention used.
Topics: Aging; Animals; Calcification, Physiologic; Cell Differentiation; Databases, Bibliographic; Dental Pulp; Dentin, Secondary; Dentinogenesis; Extracellular Matrix Proteins; Humans; Odontoblasts; Species Specificity
PubMed: 30803530
DOI: 10.1016/j.joen.2018.12.002 -
BMC Oral Health Apr 2024Decellularized extracellular matrix (dECM) from several tissue sources has been proposed as a promising alternative to conventional scaffolds used in regenerative...
BACKGROUND
Decellularized extracellular matrix (dECM) from several tissue sources has been proposed as a promising alternative to conventional scaffolds used in regenerative endodontic procedures (REPs). This systematic review aimed to evaluate the histological outcomes of studies utilizing dECM-derived scaffolds for REPs and to analyse the contributing factors that might influence the nature of regenerated tissues.
METHODS
The PRISMA 2020 guidelines were used. A search of articles published until April 2024 was conducted in Google Scholar, Scopus, PubMed and Web of Science databases. Additional records were manually searched in major endodontic journals. Original articles including histological results of dECM in REPs and in-vivo studies were included while reviews, in-vitro studies and clinical trials were excluded. The quality assessment of the included studies was analysed using the ARRIVE guidelines. Risk of Bias assessment was done using the (SYRCLE) risk of bias tool.
RESULTS
Out of the 387 studies obtained, 17 studies were included for analysis. In most studies, when used as scaffolds with or without exogenous cells, dECM showed the potential to enhance angiogenesis, dentinogenesis and to regenerate pulp-like and dentin-like tissues. However, the included studies showed heterogeneity of decellularization methods, animal models, scaffold source, form and delivery, as well as high risk of bias and average quality of evidence.
DISCUSSION
Decellularized ECM-derived scaffolds could offer a potential off-the-shelf scaffold for dentin-pulp regeneration in REPs. However, due to the methodological heterogeneity and the average quality of the studies included in this review, the overall effectiveness of decellularized ECM-derived scaffolds is still unclear. More standardized preclinical research is needed as well as well-constructed clinical trials to prove the efficacy of these scaffolds for clinical translation.
OTHER
The protocol was registered in PROSPERO database #CRD42023433026. This review was funded by the Science, Technology and Innovation Funding Authority (STDF) under grant number (44426).
Topics: Tissue Scaffolds; Regenerative Endodontics; Animals; Extracellular Matrix; Decellularized Extracellular Matrix; Dental Pulp; Models, Animal; Tissue Engineering; Regeneration
PubMed: 38689279
DOI: 10.1186/s12903-024-04266-x -
Gene May 2020Osteogenesis imperfecta (OI) is a rare disease characterized by increased bone fragility and predisposition to fractures, bone deformities and other major signs such as...
Rare splicing mutation in COL1A1 gene identified by whole exomes sequencing in a patient with osteogenesis imperfecta type I followed by prenatal diagnosis: A case report and review of the literature.
BACKGROUND
Osteogenesis imperfecta (OI) is a rare disease characterized by increased bone fragility and predisposition to fractures, bone deformities and other major signs such as dentinogenesis imperfecta, blue sclera and deafness. Over 90% of OI cases are caused by mutations in the COL1A1 and COL1A2 genes and the inheritance is autosomal dominant.
METHODS
We present a case of a couple requesting genetic counseling, because the man was diagnosed with OI on a clinical and radiological basis and the woman was pregnant. Whole exomes sequencing (WES) was performed in order to identify the mutation (s), followed by prenatal diagnosis.
RESULTS
WES identified a rare splicing mutation c.1155 + 1G > C in the COL1A1 gene recognized to be pathogenic and subsequently confirmed by next generation sequencing. The carrier state of the mutation was excluded for the fetus, so the pregnancy was further pursued and a healthy baby was born at term.
CONCLUSIONS
WES is a new and effective technique for detecting pathogenic variants in monogenic diseases and it is preferable to use such a technique in diseases with genetic heterogeneity especially when time does not allow another time-consuming diagnostic technique such classical Sanger sequencing. WES offers possibility to expand the global spectrum of OI pathogenic variants enabling the diagnosis of the disease.
Topics: Adult; Alternative Splicing; Collagen Type I; Collagen Type I, alpha 1 Chain; Exome; Female; Genotype; High-Throughput Nucleotide Sequencing; Humans; Male; Mutation; Osteogenesis Imperfecta; Pedigree; Pregnancy; Prenatal Diagnosis; Protein Isoforms; Exome Sequencing
PubMed: 32165296
DOI: 10.1016/j.gene.2020.144565