Int J Pediatr Otorhinolaryngol
August 2023
Objectives: Pediatric cranial base pathology is anatomically complex and surgical treatment is oftentimes difficult to conceptualize for patients and their families. Three-dimensional (3D) models of the sinuses and cranial base have the potential to enhance patient understanding in numerous domains. Our objective is to assess the use of 3D models in pre-operative parental and patient counseling prior to endoscopic endonasal skull base surgery in the pediatric population.
View Article and Find Full Text PDFBackground: The rapid expansion and anticipated U.S Food and Drug Administration regulation of 3D printing at the point-of-care necessitates the creation of robust quality management systems. A critical component of any quality management system is a document control system for the organization, tracking, signature collection, and distribution of manufacturing documentation.
View Article and Find Full Text PDFStudies of human movement usually collect data from multiple repetitions of a task and use the average of all movement trials to approximate the typical kinematics or kinetics pattern for each individual. Few studies report the expected accuracy of these estimated mean kinematics or kinetics waveforms for each individual. The purpose of this study is to demonstrate how simultaneous confidence bands, which is an approach to quantify uncertainty across an entire waveform based on limited data, can be used to calculate margin of error (MOE) for waveforms.
View Article and Find Full Text PDFThis study explores the relationships between hemispheric and cerebellar white matter lesions and motor and cognitive impairments in male carriers of Fragile-X Mental Retardation 1 () premutation alleles, and in a subgroup of these carriers affected with Fragile X-Associated Tremor/Ataxia syndrome (FXTAS). Regional and total white matter hyperintensities () on MRI, assessed using semiquantitative scores, were correlated with three motor rating scales (ICARS, UPDRS, Tremor), and neuropsychological measures of non-verbal reasoning, working memory and processing speed, in a sample of 30 male premutation carriers aged 39-81 years, and separately in a subsample of 17 of these carriers affected with FXTAS. There were significant relationships between in the infratentorial region and all three motor scales, as well as several cognitive measures-Prorated IQ, Matrix Reasoning, Similarities, and the Symbol Digit Modalities Test (SDMT), in the total sample of carriers, as well as in the FXTAS group separately.
View Article and Find Full Text PDFThe fragile X premutation (PM) allele contains a CGG expansion of 55-200 repeats in the gene's promoter. Male PM carriers have an elevated risk of developing neurological and psychiatric changes, including an approximately 50% risk of the fragile X-associated tremor/ataxia syndrome (FXTAS). The aim of this study was to assess the relationships of regional white matter hyperintensities () semi-quantitative scores, clinical status, motor (UPDRS, ICARS, Tremor) scales, and cognitive impairments, with -specific genetic changes, in a sample of 32 unselected male PM carriers aged 39-81 years.
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