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25-year follow-up. Vasculitis-induced chronic damage was observed in all patients. Disease activity decreased and vascular damage remained stable throughout the follow-up period. CONCLUSIONS The clinical features and angiographic type of TA in Iran are different from most Asian countries. Differences in angiographic and clinical features may lead to delayed diagnosis. The issue of delay in diagnosis should create awareness among health care providers that TA is not a very rare disease in Iranians and failure to pay attention to warning symptoms may delay the diagnosis.Background The need for client feedback in assessing healthcare services is widely recognized. However, little is known about the satisfaction of adolescent clients utilizing healthcare services in Malaysia. To the best of our knowledge, there is no validated instrument to measure the satisfaction of adolescent clients attending health clinics in Malaysia. This cross-sectional study aimed to determine the reliability of the Malay version of the Malaysian Ministry of Health's Adolescent Client Satisfaction Questionnaire among adolescents attending health clinics in northeastern Malaysia. Methods A cross-sectional study was conducted in January 2019 among adolescents aged 10-19 years attending four designated health clinics in the northeastern state of Peninsular Malaysia. The test for Cronbach's α was performed to determine the internal consistency reliability. Results There were a total of 85 adolescent clients involved in this study. The mean age of respondents was 15.6 years. The majority of respondents were female, Malay, students, and had attained a secondary level of education. The mean total satisfaction score was 78.35. The internal consistency reliability according to the Cronbach's α of the domain was 0.854, which is considered highly reliable. The corrected item-total correlation for the domain was acceptable as it was ≥0.4. Conclusion The Malay version of the Malaysian Ministry of Health's Adolescent Client Satisfaction Questionnaire has excellent internal consistency reliability. Therefore, it may be recommended as a tool to measure the satisfaction level among adolescents attending health clinics in Malaysia.Background/Aims The inflammatory bowel diseases (IBD), ulcerative colitis (UC), and Crohn's disease (CD) are chronic diseases mostly affecting young patients. this website As they are diseases accompanying patients for their entire life, and the quality of life (QUOL) interacts with disease activity, improving QUOL should be one of the main goals of therapy. This study aims to identify factors contributing to good or impaired QUOL. Methods Questionnaires addressing health-related QUOL and other psychological and social features were positioned on our institutions' webpage and on the webpage of the largest self-help group for IBD in Germany. Patients were subdivided according to their QUOL score with a cutoff of less then 60. We used the Short Inflammatory Bowel Disease Questionnaire, the Assessment of the Demand for Additional Psychological Treatment, and the Fear of Progression Questionnaire Short Form. Results High numbers of patients in both subgroups showed an impaired QUOL (87.34% in UC, 91.08% in CD). Active extraintestinal manifestations, smoking, high fear of progression and high demand for psychotherapy were associated with reduced QUOL. In addition, polypharmacological interventions did not result in a good QUOL, but ostomies are linked to improved QUOL especially in CD patients. Conclusions Scores used in clinical day-to-day-practice mainly focusing on somatic factors do not sufficiently address important aspects concerning QUOL. Most importantly, extraintestinal manifestations show a hitherto underestimated impact on QUOL.Mucopolysaccharidosis type I (MPS I) is caused by genetic deficiency of α-l-iduronidase and impairment of lysosomal catabolism of heparan sulfate and dermatan sulfate. In the brain, these substrates accumulate in the lysosomes of neurons and glial cells, leading to neuroinflammation and neurodegeneration. Their storage also affects lysosomal homeostasis-inducing activity of several lysosomal proteases including cathepsin B (CATB). In the central nervous system, increased CATB activity has been associated with the deposition of amyloid plaques due to an alternative pro-amyloidogenic processing of the amyloid precursor protein (APP), suggesting a potential role of this enzyme in the neuropathology of MPS I. In this study, we report elevated levels of protein expression and activity of CATB in cortex tissues of 6-month-old MPS I (Idua -/- mice. Besides, increased CATB leakage from lysosomes to the cytoplasm of Idua -/- cortical pyramidal neurons was indicative of damaged lysosomal membranes. The increased CATB activity coincided with an elevated level of the 16-kDa C-terminal APP fragment, which together with unchanged levels of β-secretase 1 was suggestive for the role of this enzyme in the amyloidogenic APP processing. Neuronal accumulation of Thioflavin-S-positive misfolded protein aggregates and drastically increased levels of neuroinflammatory glial fibrillary acidic protein (GFAP)-positive astrocytes and CD11b-positive activated microglia were observed in Idua -/- cortex by confocal fluorescent microscopy. Together, our results point to the existence of a novel CATB-associated alternative amyloidogenic pathway in MPS I brain induced by lysosomal storage and potentially leading to neurodegeneration.The alpha-glucosidase- and lipase-inhibitory activities of three phenalenones (1-3) and one phenylpropanoid (4) from the ethyl acetate extracts of a Pseudolophiosptoma sp. are described. They represent the first secondary metabolites reported from the genus Pseudolophiostoma. Scleroderolide (1) and sclerodione (2) exhibited potent α-glucosidase- and porcine-lipase-inhibitory activity during primary screening, with better IC50 values compared to the positive controls, N-deoxynojirimycin and orlistat. In silico techniques were employed to validate the probable biological targets and elucidate the mechanism of actions of phenalenones 1 and 2. Both compounds exhibited strong binding affinities to both alpha-glucosidase and porcine lipase through H-bonding and π-π interactions. Interestingly, favorable in silico ADME (absorption, distribution, metabolism, and excretion) properties such as gastrointestinal absorption were also predicted using software.