Mandible customized distraction osteogenesis in Treacher Collins patient: Literature review, report of a case and post-distraction analysis

Introduction: Treacher-Collins syndrome is a rare genetic disorder with variable phenotypic expression. Related micrognathia can cause upper airway obstruction in neonatal patients because of posterior tongue displacement. Many approaches have been described to treat upper airway obstruction in such patients; the use of mandibular distraction to this purpose was first proposed in 1994. To our knowledge, only a few authors have employed customized devices to perform mandibular distraction in these syndromic patients.This paper presents a systematic review of the use of customized devices to perform mandibular distraction osteogenesis in patients affected by Treacher-Collins syndrome and upper airway obstruction. A case report of a Treacher-Collins syndrome patient treated in our institution is also presented. Results: Eight articles fulfilled the inclusion criteria and were included in this systematic review; demographic, clinical and surgical features of 13 cases were evaluated.Preoperative assessment, virtual surgical planning and a surgical protocol for a Treacher-Collins patient were performed and set-out in detail; a post-distraction 3D analysis workflow was developed and proposed.The patient had an airway obstruction resolution after the distractors removal and was progressively decannulated. The post-distraction 3D analysis revealed that the oropharynx and hypopharynx airway space acquired greater volumetric dimension in a more antero-superior position; this advancement was comparable to the antero-superior advancement of the distracted mandible. Conclusion: In the author's opinion, customized devices for mandibular distraction osteogenesis and post-distraction tridimensional analysis should be objected of further investigation in the light of their potential advantage in diagnosis and treatment of syndromic malformation

Human neural progenitor cell models to study the antiviral effects and neuroprotective potential of approved and investigational human cytomegalovirus inhibitors

: Human cytomegalovirus (HCMV) is the viral leading cause of congenital defects in newborns worldwide. Many aspects of congenital CMV (cCMV) infection, which currently lacks a specific treatment, as well as the main determinants of neuropathogenesis in the developing brain during HCMV infection are unclear. In this study, we modeled HCMV infection at different stages of neural development. Moreover, we evaluated the effects of both approved and investigational anti-HCMV drugs on viral replication and gene expression in two different neural progenitor cell lines, i.e., human embryonic stem cells-derived neural stem cells (NSCs) and fetus-derived neuroepithelial stem (NES) cells. Ganciclovir, letermovir, nitazoxanide, and the ozonide OZ418 reduced viral DNA synthesis and the production of infectious virus in both lines of neural progenitors. HCMV infection dysregulated the expression of genes that either are markers of neural progenitors, such as SOX2, NESTIN, PAX-6, or play a role in neurogenesis, such as Doublecortin. Treatment with antiviral drugs had different effects on HCMV-induced dysregulation of the genes under investigation. This study contributes to the understanding of the molecular mechanisms of cCMV neuropathogenesis and paves the way for further consideration of anti-HCMV drugs as candidate therapeutic agents for the amelioration of cCMV-associated neurological manifestations

Spectral Mismatch Effect to the Open-circuit Voltage in the Indoor Characterization of Multi-junction Thin-film Photovoltaic Modules

The spectral mismatch between the standard AM1.5g spectral irradiance and the spectrum of a solar simulator is well-known to affect the measurement of the shortcircuit current in the current-voltage (IV) characterization of both single and multi-junction photovoltaic (PV) devices, where it may result in deviations larger than 10% even on Class A simulators. Multi-junction devices may also give spectral mismatch effects on the open-circuit voltage, as discussed in this work. The effect is larger when the measurement is performed using isotype reference cells, that are conventionally preferred to broadband ones, giving a better estimation of the short-circuit current. Experimental evidence of systematic differences in the open-circuit voltage of 2-junction PV module measurement campaign are given, that may arise from such effect. The results stress the importance of using both isotype and broadband cells as reference in IV measurements of multi-junction PV modules, as well as a spectral mismatch correction procedure for the open-circuit voltage in a future standard procedure.JRC.F.7-Renewable Energ