Current medical research and opinion
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To systematically review the literature and summarize the health-related quality-of-life (HRQoL) of patients undergoing treatment for mucopolysaccharidoses (MPS), a rare, hereditary lysosomal storage disorder. ⋯ This systematic literature review revealed the substantial humanistic burden of individuals affected by MPS as well as caregivers. Significant variation in HRQoL scores was observed, however studies indicate that the quality-of-life of MPS patients is influenced primarily by severity of disease (MPS type and phenotype), and then by time of diagnosis, pain, cognitive involvement, mobility, dependence, and time of treatment initiation. Further studies are needed to assess the global humanistic burden of MPS, particularly in MPS III, VI, VII, and IX subtypes, in adults, and for a longer follow-up period. Considering the vast array of HRQoL assessment tools available and used in this study, researchers should also consider using scales with condition-specific measures to ensure appropriate estimates of effectiveness.
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Observational Study
Combining biomarkers of BNIP3 L, S100B, NSE and accessible measures to prediction sepsis-associated encephalopathy: a prospective observational study.
Accurate identification of delirium in sepsis patients is crucial for guiding clinical diagnosis and treatment. However, there are no accurate biomarkers and indicators at present. We aimed to identify which combinations of cognitive impairment-related biomarkers and other easily accessible assessments best predict delirium in sepsis patients. ⋯ The logistic regression showed that the combination model was strongly correlated with cognitive dysfunction in sepsis patients.
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Although pulmonary hypertension (PH) and Eisenmenger's syndrome (ES) are common complications in adult congenital heart disease (ACHD), the frequency of diagnostic tests and the incidence of PH/ES in patients with ACHD in Japanese clinical practice are unclear. Therefore, we sought to clarify the frequency of diagnostic tests and incidence of PH/ES in patients with ACHD using the Medical Data Vision (MDV) database, the largest anonymized database of diagnosis procedure combination hospitals in Japan. ⋯ We have clarified the frequency of diagnostic tests related to PH/ES and the incidence of PH/ES in patients with ACHD in clinical practice in Japan, including non-specialist institutions for PH.
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Pressure-Enabled Drug Delivery (PEDD), a method using pressure to advance catheter-delivered drug distribution, can improve treatment for hepatocellular carcinoma (HCC) and liver metastases, but real-world evidence is limited. We compared baseline patient characteristics, clinical complexity, and post-procedure healthcare resource utilization (HRUs) and clinical complications for PEDD and non-PEDD procedures. ⋯ Despite higher baseline disease burden and complexity, post-procedure HRU and clinical complications for PEDD patients were similar to non-PEDD patients. The complex baseline clinical profile may reflect selection of challenging cases for PEDD use. Future studies should validate the benefits observed with PEDD embolization in larger samples with greater statistical power.
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Cloninger's psychobiosocial model of personality proposes that consistent patterns of health behavior are determined by the complex interaction of different neurobiological processes of the patient's temperament and character dimensions. Poor medication adherence is a pervasive problem among glaucoma patients and can lead to increased morbidity and disability. The objective of the present study was to investigate the association between Cloninger's personality dimensions and medication adherence among glaucoma patients. ⋯ The results suggest that POAG patients with higher scores on the Self-Transcendent personality dimension are more likely to experience difficulties adhering to medication regimen. The study highlights the importance of a holistic approach to glaucoma treatment, which takes into account not only the biological aspects of disease but also the psychosocial factors that influence patient behavior. Healthcare providers may need to consider glaucoma patients' personality dimensions, beliefs and values when developing treatment plans and strategies to improve medication adherence.