Internal medicine
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We herein report a 78-year-old woman with Gaucher disease (GD) who was initially diagnosed with GD type 1, had been receiving long-term enzyme replacement therapy since 58 years old, and developed neurological manifestations in her 70s. The neurological manifestations included myoclonic seizures and progressive cognitive decline. Although it is rare for GD patients to first develop neurologic manifestations at such an advanced age, physicians engaged in long-term care for GD patients should be alert for this possibility.
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Objective Azacitidine (AZA) has been the standard of care for elderly patients with high-risk myelodysplastic syndromes (MDS). However, reliable clinical predictors of outcome have yet to be identified. The prognostic value of fetal hemoglobin (HbF) levels has been reported for decitabine therapy. ⋯ Results The median follow-up duration was 13.0 (range 1.5-93.5) months. The HbF-elevated group was associated with a significantly higher hematologic improvement rate (76.9% vs. 25%, p=0.009) and better overall survival (median, 21.0 vs. 13.0 months, p=0.048) than the HbF-normal group. Conclusion These results suggest that elevated pretreatment HbF levels can predict better outcomes in patients with MDS/AML treated with AZA.
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Objective To examine the trends and relationships between nonalcoholic fatty liver disease (NAFLD) and hypertension, type 2 diabetes mellitus (T2DM), and dyslipidemia from fiscal year (FY) 2008, when specific health checkups (SHCs) were initiated in Japan, to FY 2019 and the relationship between NAFLD trends and dietary nutrition. Methods A total of 48,332 participants (25,121 men and 23,211 women) diagnosed with NAFLD who underwent health checkups, including ultrasonography, from FY 2008 to FY 2019 were included. A fatty liver was diagnosed using ultrasonography. ⋯ The dietary nutrient intake increased with regard to the total fat, fat/energy ratio, saturated fatty acids, monounsaturated fatty acids, and n6/n3 fatty acid ratio in both men and women, and these nutrient trends were correlated with NAFLD prevalence (all p≤0.0005). Conclusion In Japan, NAFLD increased in both men and women regardless of the BMI and age, even after starting SHCs. An unbalanced fat intake may be one of the major reasons for this increase.