RESUMEN
OBJECTIVE: This article aims to demonstrate the clinical application of a comprehensive workflow that integrates digital tools for accurate color matching, and its immediate implementation in the restoration of anterior teeth. CLINICAL CONSIDERATIONS: Two patients demonstrating dissatisfaction regarding a maxillary central incisor had an old restoration replaced resorting to a digital workflow to enhance the predictability of the new direct restoration. OptiShade allowed the precise assessment of tooth color and the CompoShade application provided precise color and material selection, as well as the determination of a layering strategy. Precision and accuracy of the colorimeter and the composite layering application were demonstrated clinically in vivo. CONCLUSIONS: Implementing a digital workflow with the integration of OptiShade measurements and the CompoShade layering recipe calculation, as well as the respective realistic try-in, enables the achievement of precise color matching for anterior composite restorations. CLINICAL SIGNIFICANCE: The employment of a digital colorimeter and layering recipes simplify shade matching, and optimizes composite resin clinical use and success. The implementation of a realistic try-in, previous to the definitive restoration, allows the clinician to perform any adjustments if needed.
RESUMEN
BACKGROUND: Schwartz-Jampel syndrome (SJS) is a very rare inherited disorder characterized by multiple skeletal deformities, limited joint mobility, micrognathia, blepharophimosis, myotonia, and growth retardation. SJS is caused by mutations in the gene encoding perlecan (heparan sulfate proteoglycan). Anesthetic management of these patients is challenging. The use of spinal anesthesia in these patients has not been reported. CASE PRESENTATION: A 14-year-old boy was scheduled for inguinal hernia and hydrocele repair. The diagnosis of SJS was based on his dysmorphic features, electromyographic (EMG) pattern and genetic testing. General anesthesia may encounter difficult airway management, resistance to muscle relaxants, or possibility of malignant hyperthermia. Regional anesthesia may be difficult or even harmful due to skeletal deformities. We report successful management of spinal anesthesia and surgery was done. The patient had an uneventful recovery and was discharged home. We describe the special precautions against pitfalls for using this technique in patients with SJS. CONCLUSION: Spinal anesthesia may be an effective and safe technique for patients with SJS and it may.
RESUMEN
Aggresomes are inclusion bodies for misfolded/aggregated proteins. Despite the role of misfolded/aggregated proteins in neurological disorders, their role in cancer pathogenesis is poorly defined. In the current study we aimed to investigate whether aggresomes-positivity could be used to improve the disease subclassification and prognosis prediction of pediatric medulloblastoma. Ninety three pediatric medulloblastoma tumor samples were retrospectively stratified into three molecular subgroups; WNT, SHH and non-WNT/non-SHH, using immunohistochemistry and Multiplex Ligation Probe Amplification. Formation of aggresomes were detected using immunohistochemistry. Overall survival (OS) and event-free survival (EFS) were determined according to risk stratification criteria. Multivariate Cox regression analyses were carried out to exclude confounders. Aggresomes formation was detected in 63.4% (n = 59/93) of samples. Aggresomes were non-randomly distributed among different molecular subgroups (P = 0.00002). Multivariate Cox model identified aggresomes' percentage at ≥20% to be significantly correlated with patient outcome in both OS (HR = 3.419; 95% CI, 1.30-8.93; P = 0.01) and EFS (HR = 3; 95% CI, 1.19-7.53; P = 0.02). The presence of aggresomes in ≥20% of the tumor identified poor responders in standard risk patients; OS (P = 0.02) and EFS (P = 0.06), and significantly correlated with poor outcome in non-WNT/non-SHH molecular subgroup; OS (P = 0.0002) and EFS (P = 0.0004).