Postgraduate medical journal
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Fabry disease is a rare inborn error of the enzyme α-galactosidase (α-Gal) and results in lysosomal substrate accumulation in tissues with a wide range of clinical presentations. The disease has attracted a lot of interest over the last years, in particular since enzyme replacement therapy (ERT) has become widely available in 2001. ⋯ Another challenge are patients who develop neutralising antibodies to ERT, which possibly leads to reduced therapy effectiveness. In this article, we summarise the latest developments in the science community regarding diagnostics and management of this rare lysosomal storage disorder and offer an outlook to future treatments.
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VAT had higher correlation coefficient r with ALT and GGT than SAT. VAT, but not SAT, was associated with ALT (males: β=0.15, p=0.01; females: β=0.17, p=0.02) and GGT (males: β=0.39, p<0.0001) in linear regression. VAT remained to be associated with GGT in males (β=0.33, p=0.0001) when was further adjusted. Logistic regression showed that VAT was associated with elevated GGT (OR=2.218, p=0.043) in males but not in females and no such association was observed for SAT. ⋯ Increased VAT, but not SAT, was associated with elevation of hepatic enzymes including ALT and GGT. Moreover, VAT was associated with elevated GGT independent of insulin resistance and subcutaneous fat in males.
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Workforce studies show a declining proportion of UK junior doctors proceeding directly to specialist training, with many taking career breaks. Doctors may be choosing to delay this important career decision. ⋯ Most UK-trained doctors feel rushed into choosing their long-term career specialty. Doctors find this difficult because they lack sufficient medical experience and adequate career advice to make sound choices. Workforce trainers and planners should enable greater flexibility in training pathways and should further improve existing career guidance.
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Various options for the pharmacological treatment of breakthrough cancer pain (BTcP) are available. International guidelines on BTcP treatment are not univocal. A tailored treatment should be based on the assessment of different variables such as BTcP characteristics, oral mucositis, chronic rhinitis and a patient's ability to take medication. ⋯ In clinical practice, the factors that most influenced the pharmacological treatment for BTcP were baseline opioid dosage, setting of assistance and self-ability to take medication. Further research is needed to improve the knowledge on tailored BTcP treatment.