Objective To explore the genetic mutation characteristics, clinical manifestations, and treatment outcomes of catecholaminergic polymorphic ventricular tachycardia (CPVT), and to construct a quantitative scoring system for the severity of CPVT. The correlation between the mutations in different structural domains of the RyR2 gene and clinical manifestations and prognosis was analyzed. Methods By searching the PubMed and Web of Science databases for CPVT-related case reports published up to December 2024, data such as patient age, clinical manifestations, gene mutation sites, and treatment responses were collected. The quality of the literature was assessed using the CARE guidelines. The χ2 test was used to compare the severity and treatment response differences among different RyR2 structural domain mutation groups, and an innovative quantitative scoring system based on symptoms and efficacy was established. Results A total of 80 articles were included, with 102 patients in total. The quality of the literature was reliable. The age of the patients ranged from 1 to 84 years, with a higher proportion of children under 10 years old (25.5%). Female patients (54.9%) outnumbered males (45.1%). For CPVT patients, a quantitative scoring system was developed, with a total score of 2 to 10 points. Among them, 2 to 4 points were classified as mild, 5 to 7 points as moderate, and 8 to 10 points as severe. The results showed that severe patients often had a history of cardiac arrest and were resistant to treatment. Out of the 102 CPVT patients, RyR2 gene mutations accounted for 53.9% (55/102) of patients. Among them, the proportion of severe patients with N-terminal structural domain mutations was significantly higher than other regions, indicating that the RyR2 gene mutation structural domain has a significant impact on the severity of CPVT (χ2=17.530, P=0.008). The proportion of patients with mutations in the central hinge region who were ineffective with β-blockers reached 42.9% (3/7), which was significantly higher than other regions. Left cardiac sympathectomy was performed in 24 cases, and postoperative symptoms were almost completely controlled, significantly better than the drug treatment group.Conclusion Mutations in the N-terminal structural domain of the RyR2 gene are significantly correlated with the severity of CPVT. Left cardiac sympathectomy has gradually become an effective intervention for refractory cases. The scoring system proposed in this study can provide a basis for clinical stratified treatment. In the future, there is a need to expand the sample size to verify mutation-specific treatment strategies.

BACKGROUND: Teeth can be used as a raw material for preparing bone substitutes due to their similar chemical composition to bone. The objective of our study was to evaluate the effect of odontogenic biphasic calcium phosphate (BCP) incorporating dentin noncollagenous proteins (DNCPs) on osteogenesis and stability in maxillary sinus augmentation. METHODS: The composition, structure and morphology of the odontogenic BCP were tested by X-ray powder diffraction (XRD), Brunauer–Emmett–Teller, and scanning electron microscopy methods. The biocompatibility and osteoinduction of DNCPs and materials were examined in vitro and their bone regeneration capacity was verified in vivo. RESULTS: The results showed that the cells adhered and proliferated well on the DNCP-loaded BCP scaffold. The odontogenic BCP and DNCPs promoted osteogenic differentiation of cells, The new bone formation in the BCP groups and DNCP subgroups was significantly higher than the new bone formation in the control, and the new bone quality was better.The bone regeneration effect of odontogenic BCP was similar to the effect of deproteinized bovine bone mineral, but b-TCP did not maintain the height and volume of bone reconstruction. CONCLUSION In conclusion, the combined application of DNCPs and odontogenic BCP is an effective strategy for tissue engineering osteogenesis in the maxillary sinus region. The biomimetic strategy could provide a new approach for patients requiring maxillary sinus lifting.

Objective: To investigate the mechanical property,biocompatibility,and osteogenic differentiation ability of chitosan-β-TCP( CS-β-TCP) scaffold,meanwhile,to study the possibility of the composite as a scaffold to repair bone defect. Methods: Briefly,the CS-β-TCP composite scaffold was fabricated utilizing bidirectional lyophilization technique. Then,the scaffold micro-structure was observed by scanning electron microscopy( SEM),X-ray diffraction( XRD) and energy disperse spectroscopy( EDS) were employed to analyze the ingredients and elements distribution of scaffold,respectively. Additionally,the compression strength of the scaffold was tested by mechanical universal testing machine. The biocompatibility of the scaffold and the cell viability research were characterized via CCK-8 assay and Live/Dead staining,respectively,and the cell adhesion was studied by DPAI/Phalloidine fluorescence staining. qRT-PCR was employed to investigate the expression level of osteogenic-related gene such as BMP2,RUNX2 and COL1. ALP staining was carried out to measure the osteogenic differentiation effect of BMSCs. Results: The CS-β-TCP scaffold was comprised of bulk parallel,aligned and thin lamellas with many porous structures. β-TCP particles were evenly distributed over CS framework layers and the CS-β-TCP scaffold possess excellent elastic property and biocompatibility,moreover,the cell seeded on scaffold revealed high cell viability and continuous proliferation. qRT-PCR and ALP staining results demonstrated that the CS-β-TCP scaffold could induce osteogenic differentiation of BMSC. Conclusion To sum up,the CS-β-TCP scaffold expressed desired mechanical and biological properties,and could induce BMSC differentiate into osteoblast,the composite scaffold provided a promising strategy for bone defect regeneration.