RESUMEN
BACKGROUND: We report a case and its 4-year follow-up of Osteoglophonic dysplasia (OD), a rare disease that disturbs both skeletal and dental development, which is usually caused by heterozygous FGFR1 mutations. CASE PRESENTATION: This article presents a case where a 6-year-old male patient suffered dysregulation of tooth eruption and was diagnosed with osteogenic dysplasia from a fibroblast growth factor receptor 1 (FGFR1) heterozygote mutation. However, the number of teeth is within the normal range, and their roots are well developed. Several interventions were implemented with varying degrees of results. The details of the 4-year follow-up showed that the signs of OD were more pronounced, including dwarfism, frontal bossing, delayed skeletal maturation, anteverted nares, micrognathia, and prominent ears, but the patient's impacted teeth and edentulous jaws remained unchanged. CONCLUSIONS: FGFR1 heterozygote mutation and OD present significant difficulty for teeth eruption and subsequent intervention. Further measures ought to be taken in recognizing various symptoms presented by the patient. This case supports the significance of careful inquiry, comprehensive physical examination and correct diagnosis as indispensable steps for clinical practice in patients with unerupted teeth. Additionally, the detailed case and its 4-year follow-up length may provide new insights into osteogenic dysplasia and patients with impacted teeth while encouraging further exploration in treatment methods.
Asunto(s)
Receptor Tipo 1 de Factor de Crecimiento de Fibroblastos , Erupción Dental , Niño , Estudios de Seguimiento , Heterocigoto , Humanos , Masculino , Mutación/genética , Osteocondrodisplasias , Receptor Tipo 1 de Factor de Crecimiento de Fibroblastos/genética , Erupción Dental/genéticaRESUMEN
BACKGROUND: The mechanics of double key loop (DKL) are not well defined, and this finite element study was designed to explore its force system. METHODS: A simplified 3-dimensional finite element model of single and double key loops with an archwire between the lateral incisor and second premolar was established in Ansys Workbench 17.0. Activation in Type-1 (retraction at the distal end), Type-2 (retraction at the distal key) and Type-3 (Type-2 plus ligation between keys) was simulated. The vertical force, load/deflection ratio and moment/force ratio of stainless-steel and titanium-molybdenum alloy (TMA) loops were calculated and compared. RESULTS: The double key loop generated approximately 40% of the force of a single key loop. Type-2 loading of DKL showed a higher L/D ratio than Type-1 loading with a similar M/F ratio. Type-3 loading of DKL showed the highest M/F ratio with a similar L/D ratio as single key loop. The M/F ratio in Type-3 loading increased with the decreasing of retraction force. The DKL of TMA produced approximately 40% of the force and moment compared with those of SS in all loading types. When activated at equal distances below 1 mm, the M/F ratios of SS and TMA DKL with equal preactivation angles were almost the same. CONCLUSION: The M/F ratio on anterior teeth increases with the preactivation angle and deactivation of DKL. The M/F ratio at a certain distance of activation mainly depends on the preactivation angle instead of the wire material. TMA is recommended as a substitute for SS in DKL for a lower magnitude of force.
Asunto(s)
Diseño de Aparato Ortodóncico , Alambres para Ortodoncia , Aleaciones Dentales , Análisis del Estrés Dental , Análisis de Elementos Finitos , Humanos , Estrés Mecánico , Técnicas de Movimiento DentalRESUMEN
BACKGROUND: Temporomandibular joint osteoarthritis (TMJ-OA) presents significant challenges due to its complex etiology, often insidious onset, high incidence, and progressive structural deterioration. While research has explored genetic and molecular factors, treatment outcomes remain suboptimal, emphasizing the need for a deeper understanding of disease progression. OBJECTIVE: This study employs a specific mandibular shift rat model to explore the dynamic progression of TMJ-OA-like lesions and evaluate the potential for self-repair at different stages, aiming to inform early diagnosis and preventative strategies. METHODS: Seventy-two female Sprague-Dawley rats were randomized into three groups: a control group (n=24; average weight: 157.23±1.63â¯g) receiving sham surgery. an experimental group (n=24; average weight: 157.78±1.88â¯g) subjected to mandibular shift induction, and a removal group (n=24; average weight: 158.11±2.20â¯g) experiencing mandibular shift for one, two, or four weeks followed by a one-month recovery period (designated as 1w Removal, 2w Removal and 4w Removal, respectively). Histomorphological and molecular analyses were conducted at designated time points. RESULTS: Rats in the 1-week removal group exhibited substantial recovery in condylar morphology, cartilage thickness, extracellular matrix composition, and expression of OA-related genes. Conversely, the 4-week removal group mirrored the experimental group, indicating limited self-repair capacity at later stages. The 2-week removal group presented with variable outcomes, with some animals showing signs of recovery and others resembling the experimental group, indicating a potential transitional phase in the disease process. CONCLUSION: Recovery from early-stage TMJ-OA involves eliminating provoking factors such as occlusal interference or reducing joint loading. However, advanced stages exhibit diminished self-repair capabilities, necessitating additional therapeutic interventions. These findings emphasize the importance of early diagnosis and intervention in TMJ-OA management.
Asunto(s)
Modelos Animales de Enfermedad , Progresión de la Enfermedad , Osteoartritis , Ratas Sprague-Dawley , Animales , Femenino , Osteoartritis/patología , Ratas , Trastornos de la Articulación Temporomandibular/patología , Articulación Temporomandibular/patología , Mandíbula/patologíaRESUMEN
AIMS: Mechanical stress overload in the temporomandibular joint (TMJ) is an important cause of TMJ osteoarthritis (TMJOA). Whether secreted frizzled-related proteins (SFRPs) play important roles in the development of mechanical stress-induced TMJOA remains controversial. In this study, we investigated the roles of the Wnt/ß-catenin signaling and SFRPs in the progression of mechanical stress-induced TMJOA. METHODS: We investigated the progression of mechanical stress-induced TMJOA using an in vivo model via modified increased occlusal vertical dimension (iOVD) malocclusion and an in vitro model in which isolated chondrocytes were subjected to mechanical stress. The effects of inhibition of Wnt/ß-catenin signal on TMJOA induced by mechanical stress were studied by in vitro drug added and in vivo intra-articular injection of XAV-939. TMJOA progression, Wnt/ß-catenin signaling and SFRPs was assessed by Cone beam computed tomography (CBCT) analysis, histochemical and immunohistochemical (IHC) staining, quantitative real-time PCR (qRT-PCR), Western blotting (WB), and immunofluorescence (IF) staining. RESULTS: Our in vivo results showed that iOVD-induced mechanical stress in the TMJ disrupted mandible growth, induced OA-like changes in TMJ cartilage, and increased OA-related cytokine expression. In addition, iOVD activated Wnt/ß-catenin signaling and suppressed Sfrp1, Sfrp3, and Sfrp4 expression in condylar cartilage. Moreover, our in vitro study showed that stress disrupted homeostasis, activated Wnt/ß-catenin signaling and inhibited SFRP3 and SFRP4 expression in chondrocytes. Suppression of Wnt/ß-catenin signaling with XAV-939 promoted SFRP3 and SFRP4 expression and rescued mechanical stress-induced cartilage degeneration in vivo and in vitro. CONCLUSIONS: Our work suggests that mechanical stress reduces SFRPs expression both in vivo and in vitro and promotes TMJOA via Wnt/ß-catenin signaling. Suppression of Wnt/ß-catenin signaling promotes SFRPs expression, especially SFRP3 and SFRP4 expression, and rescues mechanical stress-induced cartilage degeneration. Wnt/ß-catenin signaling and SFRPs may represent potential therapeutic targets for TMJOA.
Asunto(s)
Osteoartritis , beta Catenina , Condrocitos/metabolismo , Humanos , Péptidos y Proteínas de Señalización Intracelular , Osteoartritis/metabolismo , Estrés Mecánico , Articulación Temporomandibular/diagnóstico por imagen , beta Catenina/metabolismoRESUMEN
Mandibular deviation affects the biomechanical environment of the temporomandibular joint (TMJ) and causes thinning of cartilage on the deviated side. We aimed to evaluate, using a rat model, the effect of mandibular functional deviation on the TMJ in relation to the functional roles of integrin ß family members. The effects of experimental functional deviation on the TMJ of 6-week-old Sprague-Dawley female rats, randomly assigned to control (n = 42) and experimental groups (n = 42), were evaluated at 3 days and 1, 2, 4, and 8 weeks by histological staining, immunofluorescence, real-time quantitative polymerase chain reaction, and micro-computed tomography. The results showed that the experimental functional shift changed the shape of condyles, thinned the cartilage, and increased the proportion of the hypertrophic layer on the deviated sides of condyles. In addition, the extracellular matrix of the condyle cartilage exhibited degradation at 1 week and subchondral trabecular bone was lost at 4 and 8 weeks. Osteoarthritis (OA)-like changes occurred in the left and right condyles of rats in the experimental group and were aggravated over time. Integrin ß family expression, especially integrin ß2 , was altered from week 1, possibly related to the OA-like changes. These data may provide insight into the onset of TMJ OA.