Nusinersen Initiation After Onset of Weakness Does Not Prevent Progression of Hip Instability.

Background: We report changes in the natural history of hip instability with nusinersen treatment among patients with spinal muscular atrophy (SMA) type II after onset of weakness, historically wheelchair-bound but now potentially ambulatory in the era of disease-modifying therapy.

Methods: Patients with genetically confirmed diagnoses of SMA type II who received intrathecal nusinersen from January 1, 2018, to June 30, 2022, were screened for inclusion. Patients with less than 6 months of follow-up, or prior hip surgeries were excluded.

Suppression of apoptosis impairs phalangeal joint formation in the pathogenesis of brachydactyly type A1.

Apoptosis occurs during development when a separation of tissues is needed. Synovial joint formation is initiated at the presumptive site (interzone) within a cartilage anlagen, with changes in cellular differentiation leading to cavitation and tissue separation. Apoptosis has been detected in phalangeal joints during development, but its role and regulation have not been defined.

Virtual reality-based surgical planning simulator for tumorous resection in FreeForm Modeling: an illustrative case of clinical teaching.

The importance of virtual reality (VR) has been emphasized by many medical studies, yet it has been relatively under-applied to surgical operation. This study characterized how VR has been applied in clinical education and evaluated its tutorial utility by designing a surgical model of tumorous resection as a simulator for preoperative planning and medical tutorial. A 36-year-old male patient with a femoral tumor who was admitted to the Affiliated Jiangmen Traditional Chinese Medicine Hospital was randomly selected and scanned by computed tomography (CT).

Percutaneous endoscopic lumbar discectomy in lumbar disc herniation with posterior ring apophysis fracture: A case report in a 15-year-old child.

Rationale: Lumbar disc herniation (LDH) with posterior ring apophysis fracture (PRAF) is rather rare in children, and in all age-stratified LDH patients, the incidence of RAF was 5.3% to 7.5%.

Scoliosis in osteogenesis imperfecta: identifying the genetic and non-genetic factors affecting severity and progression from longitudinal data of 290 patients.

Background: Scoliosis is widely prevalent among osteogenesis imperfecta (OI) patients, and is progressive with age. However, factors affecting scoliosis in OI are not well known.

Methods: We retrospectively retrieved longitudinal radiographic and clinical records of consecutive OI patients seeking treatments at our hospital from 2014 to 2022, graded their pre-operative spinal conditions into four outcome groups, estimated their progression rates, and descriptively and inferentially analyzed the genetic and non-genetic factors that may affect the outcomes and progression rates.

Electromyographic Discrepancy in Paravertebral Muscle Activity Predicts Early Curve Progression of Untreated Adolescent Idiopathic Scoliosis.

Study Design: This study adopted a prospective cohort study design.

Purpose: This study aimed to examine electromyogram (EMG) discrepancy in paravertebral muscle activity and scoliosis progression, determine how vertebral morphology and EMG discrepancy evolve during scoliosis progression, and identify differences in EMG activity between individuals with and without adolescent idiopathic scoliosis (AIS).

Overview Of Literature: Higher EMG activity is observed in the convex side of scoliotic curves, but not in populations without scoliosis, suggesting that higher EMG activity is a causative factor for curve progression.

Virtual operation for hip joint replacement implemented by Sensable_FreeForm_Modelling: A surgical drill.

Objective: To design a virtual operation of joint replacement for surgical drills using a haptic device, SenSable_FreeForm_Modelling (SFM), to enhance surgeons' efficiency and enable "Virtual tutorial without reality" for interns.

Method: A patient with hip joint osteoarthritis is randomly selected to perform Total Hip Replacement (THR). The hip images were input into Mimics in the format of *.

Application of silver nanoparticles for improving motor recovery after spinal cord injury via reduction of pro-inflammatory M1 macrophages.

Silver nanoparticles (AgNPs) possess anti-inflammatory activities and have been widely deployed for promoting tissue repair. Here we explored the efficacy of AgNPs on functional recovery after spinal cord injury (SCI). Our data indicated that, in a SCI rat model, local AgNPs delivery could significantly recover locomotor function and exert neuroprotection through reducing of pro-inflammatory M1 survival.

Clinical features and molecular characterization of Chinese patients with FKBP10 variants.

Background: Osteogenesis imperfecta (OI) is a group of rare skeletal dysplasia. Long bone deformity and scoliosis are often associated with progressively deforming types of OI. FKBP65 (encoded by FKBP10, OMIM *607063) plays a crucial role in the processing of type I procollagen.

Retrospective analyses of clinical features in 28 Chinese patients with type V osteogenesis imperfecta: new perspectives in an old issue.

Unlabelled: Type V osteogenesis imperfecta (OI) is a form of OI characterized by radial head dislocation (RHD), calcification of interosseous membrane (CIM), and hyperplastic callus (HPC). In this study, we characterized the clinical features of 28 type V OI patients. We presented that dysfunctions of elbow, hip joint, and abnormal epiphyseal growth plate were associated with ectopic calcification and summarized the history of HPC progression and treatment.

Patient-reported outcomes in a Chinese cohort of osteogenesis imperfecta unveil psycho-physical stratifications associated with clinical manifestations.

Background: Osteogenesis imperfecta (OI) is a rare congenital disorder of the skeletal system, inflicting debilitating physical and psychological distress on patients and caregivers. Over the decades, much effort has been channeled towards understanding molecular mechanisms and developing new treatments. It has recently become more apparent that patient-reported outcome measurements (PROM) during treatment, healing and rehabilitation are helpful in facilitating smoother communication, refining intervention strategies and achieving higher quality of life.

SSVEP-Based Brain Computer Interface Controlled Soft Robotic Glove for Post-Stroke Hand Function Rehabilitation.

Soft robotic glove with brain computer interfaces (BCI) control has been used for post-stroke hand function rehabilitation. Motor imagery (MI) based BCI with robotic aided devices has been demonstrated as an effective neural rehabilitation tool to improve post-stroke hand function. It is necessary for a user of MI-BCI to receive a long time training, while the user usually suffers unsuccessful and unsatisfying results in the beginning.

Phenotypic Spectrum and Molecular Basis in a Chinese Cohort of Osteogenesis Imperfecta With Mutations in Type I Collagen.

Osteogenesis imperfecta (OI) is a rare inherited connective tissue dysplasia characterized with skeletal fragility, recurrent fractures and bone deformity, predominantly caused by mutations in the genes or that encode the chains of type I collagen. In the present study, clinical manifestations and genetic variants were analysed from 187 Chinese OI patients, majority of whom are of southern Chinese origin. By targeted sequencing, 63 and 58 OI patients were found carrying mutations in and respectively, including 8 novel and 7 novel variants.

PRIMUS: Comprehensive proteomics of mouse intervertebral discs that inform novel biology and relevance to human disease modelling.

Mice are commonly used to study intervertebral disc (IVD) biology and related diseases such as IVD degeneration. Discs from both the lumbar and tail regions are used. However, little is known about compartmental characteristics in the different regions, nor their relevance to the human setting, where a functional IVD unit depends on a homeostatic proteome.

Regulation and Role of Transcription Factors in Osteogenesis.

Bone is a dynamic tissue constantly responding to environmental changes such as nutritional and mechanical stress. Bone homeostasis in adult life is maintained through bone remodeling, a controlled and balanced process between bone-resorbing osteoclasts and bone-forming osteoblasts. Osteoblasts secrete matrix, with some being buried within the newly formed bone, and differentiate to osteocytes.

Effectiveness of scoliosis-specific exercises for alleviating adolescent idiopathic scoliosis: a systematic review.

Background: Adolescent idiopathic scoliosis (AIS) is the most common pediatric spinal deformity with reported complications including pain, mental health concern and respiratory dysfunction. The scoliosis-specific exercise (SSE) is prescribed throughout pubertal growth to slow progression although effects are unclear. This review aims to establish the effectiveness of SSE for alleviating AIS in terms of reducing Cobb angle, improving trunk asymmetry and quality of life (QoL).

A Novel Method to Measure the Sagittal Curvature in Spinal Deformities: The Reliability and Feasibility of 3-D Ultrasound Imaging.

The objective of this study was to test the reliability of sagittal spinal curvature measurements using 3-D ultrasound in patients with adolescent idiopathic scoliosis (AIS). Ultrasound spinous process angle (USSPA) and ultrasound laminae angle (USLA) were measured on sagittal ultrasound images, while the Cobb angle (XCA) was measured on sagittal X-ray images. Intra-class correlation coefficients (ICC) for the intra- and inter-observer variability, linear regression analysis and Bland-Altman method, including mean absolute difference (MAD), were investigated to evaluate the reliability and validity of the two ultrasound angles compared with XCA.

Significance of recumbent curvature in prediction of in-orthosis correction for adolescent idiopathic scoliosis.

Background:: Prediction of in-orthosis curvature at pre-orthosis stage is valuable for the treatment planning for adolescent idiopathic scoliosis, while the position of spinal curvature assessment that is effective for this prediction is still unknown.

Objectives:: To compare the spinal curvatures in different body positions for predicting the spinal curvature rendered by orthosis.

Study Design:: A prospective cohort study.

Patterns of coronal curve changes in forward bending posture: a 3D ultrasound study of adolescent idiopathic scoliosis patients.

Purpose: The Adam's forward bending test is the most commonly used approach to assess the spine deformity in adolescent idiopathic scoliosis (AIS) patients. However, there are noticeable differences in the hump appearance from standing to forward bending. This phenomenon has yet to be understood due to limitations of conventional radiographs.

An effective assessment method of spinal flexibility to predict the initial in-orthosis correction on the patients with adolescent idiopathic scoliosis (AIS).

Background: Spinal flexibility is an essential parameter for clinical decision making on the patients with adolescent idiopathic scoliosis (AIS). Various methods are proposed to assess spinal flexibility, but which assessment method is more effective to predict the effect of orthotic treatment is unclear.

Objective: To investigate an effective assessment method of spinal flexibility to predict the initial in-orthosis correction, among the supine, prone, sitting with lateral bending and prone with lateral bending positions.

Microfluidic fabrication of polymeric core-shell microspheres for controlled release applications.

We report a facile and robust microfluidic method to fabricate polymeric core-shell microspheres as delivery vehicles for biomedical applications. The characteristics of core-shell microspheres can be precisely and easily tuned by manipulating the microfluidic double emulsion templates. The addition of a shell can significantly improve the versatility as well as functionality of these microspheres as delivery vehicles.

In vivo stimulation of bone formation by aluminum and oxygen plasma surface-modified magnesium implants.

A newly developed magnesium implant is used to stimulate bone formation in vivo. The magnesium implant after undergoing dual aluminum and oxygen plasma implantation is able to suppress rapid corrosion, leaching of magnesium ions, as well as hydrogen gas release from the biodegradable alloy in simulated body fluid (SBF). No released aluminum is detected from the SBF extract and enhanced corrosion resistance properties are confirmed by electrochemical tests.