This study provides preliminary evidence that exercising on a bike or treadmill may provide short-term improvements in gross motor function for non-ambulant children with cerebral palsy. This needs to be tested in a large-scale randomized trial.
Microvascular disease, or small-vessel disease, is a multisystem disorder with a common pathophysiological basis that differentially affects various organs in some patients. The prevalence of small-vessel disease in the heart has been found to be higher in women compared with men. Additionally, other diseases prominently affecting women, including heart failure with preserved ejection fraction, Takotsubo cardiomyopathy, cerebral small-vessel disease, preeclampsia, pulmonary arterial hypertension (PAH), endothelial dysfunction in diabetes, diabetic cardiomyopathy, rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis, may have a common etiologic linkage related to microvascular disease. To the best of our knowledge this is the first article to investigate this potential linkage. We sought to identify various diseases with a shared pathophysiology involving microvascular/endothelial dysfunction that primarily affect women, and their potential implications for disease management. Advanced imaging technologies, such as magnetic resonance imaging and positron-emission tomography, enable the detection and increased understanding of microvascular dysfunction in various diseases. Therapies that improve endothelial function, such as those used in PAH, may also be associated with benefits across the full spectrum of microvascular dysfunction. A shared pathology across multiple organ systems highlights the need for a collaborative, multidisciplinary approach among medical subspecialty practitioners who care for women with small-vessel disease. Such an approach may lead to accelerated research in diseases that affect women and their quality of life.
HPRT Hypoxanthine-guanine phosphoribosyl transferase LND Lesch-Nyhan disease AIM The aims of this study were to identify all people with Lesch-Nyhan disease (LND) born in the UK between 1988 and 2008, and to obtain a clinical profile including age at diagnosis, genetic background, family history, neurological signs, and medications.METHOD Potential participants were contacted through the British Paediatric Neurology Surveillance Unit. Questionnaires were sent to each child's paediatric neurologist or primary consultant. Two purine laboratories provided metabolic information.RESULTS Twenty-three live males with LND in the 0-to 20-year age band and eight live males over the age of 20 years were identified. Thirty-one live people with LND were identified in the UK in 2008, giving a prevalence of 1 in 2 million people. Over the 20 years of study, there was a mean incidence rate of 0.18 per 100 000 live births, range 0 to 0.5.
This study evaluated the inter-observer reliability and stability over time of the Eating and Drinking Ability Classification System (EDACS) for children and young people with cerebral palsy (CP). Method: Case-records for 97 children with CP were examined to collect retrospective data about eating and drinking abilities, at four time-points, minimum 2 years between each time-point. Sex, Gross Motor Function Classification System (GMFCS) level, presence of feeding tube and orthopaedic issues were recorded from case-records. One speech and language therapist (SaLT1) classified eating and drinking ability using EDACS for all cases at all time-points; SaLT2 assigned EDACS levels for 50 cases at time-point 1; SaLT3 assigned EDACS levels for 24 cases at all time-points. Inter-observer reliability and stability over time were assessed using Intraclass Correlation Coefficient (ICC). Associations between EDACS levels and functioning recorded with other Functional Classification Systems (FCSs) were calculated using Kendall's tau (τ). Results: Out of 97 children, 48 were male, 48 had feeding tubes, and 83 had orthopaedic issues. ICC for EDACS levels recorded by SaLT1 across all time-points was 0.97 (95%CI 0.96-0.98); changes in EDACS levels occurred infrequently and never by more than one level. ICC between SaLT1 and SaLT2 at time-point 1 was 0.8 (95%CI 0.67-0.89); ICC between SaLT1 and SaLT3 across all time-points was 0.95 (95%CI 0.92-0.98). Association between GMFCS and EDACS was moderate (τ = 0.58).
Purpose: Bioprinting is an alternative method for constructing tissues/organs for transplantation. This study investigated the cross-linker influence and post-printing modification using oligochitosan and chitosan for stability improvement. Methods: Oligochitosan was tested as a novel cross-linker to replace Ca 2+ for pectin-based bio-ink. Oligochitosan (2 kD) and different molecular weight of chitosan were used to modify the bioprinted scaffold. Fourier transform infrared (FTIR) spectroscopy and scanning electron microscopy (SEM) were used to characterize the scaffolds. Results: Oligochitosan failed to serve as a viable cross-linker. Successful post-printing modification was confirmed by FTIR and SEM analyses. Conclusion: Regarding post-modification, chitosan-treated scaffolds showed enhanced stability compared to untreated scaffolds. In particular, scaffolds modified with 150 kD chitosan exhibited the highest stability.
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