Internal medicine
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Patients with acute myeloid leukemia (AML) harboring FMS-like tyrosine kinase 3 (FLT3)-internal tandem duplication mutation are associated with a poor survival outcome, even those receiving allogeneic stem cell transplantation (Allo-SCT). An additional treatment strategy with allo-SCT is therefore required to reduce relapse in these patients. ⋯ We herein report a 49-year-old woman with R/R AML who was successfully treated with pre- and post-transplant gilteritinib. Post-transplant gilteritnib yielded a durable response with possible exacerbation of graft-versus-host disease.
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A 55-year-old woman was admitted to our hospital because of gait disturbance and urinary retention that acutely emerged 1 week after severe acute respiratory syndrome coronavirus 2 infection. Acute inflammatory myelopathy was clinically suspected, based on bilateral lower-limb weakness with an extensor plantar response and an elevated immunoglobulin G level in the cerebrospinal fluid. ⋯ Her lower-limb weakness was partially ameliorated with immunosuppressive therapy. Postinfectious myelopathy is a rare neurological complication of coronavirus disease 2019 and can develop with normal radiological findings.
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We herein report a 68-year-old Japanese man with sporadic variant transthyretin (ATTRv) amyloidosis harboring the novel variant A97D (p. A117D) in TTR. He had slow development of asymmetric neuropathy, unintentional weight loss, mild autonomic failure and mild cardiomyopathy. ⋯ In silico analyses predicted that TTR A97D (p. A117D) altered the structure and function of the TTR protein. ATTRv amyloidosis is often difficult to diagnose in non-endemic regions due to its diverse phenotypes, such as atypical peripheral nerve involvement and a rare family history.
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Pulmonary hypertension (PH) often complicates chronic lung disease. However, there are few reports of PH associated with diffuse panbronchiolitis, and there is no effective treatment. ⋯ She received erythromycin, carbocysteine, and home oxygen therapy (1 L O2/min). After 4 months of therapy, the respiratory function (diffusing capacity of the lungs for carbon monoxide: 23.3% to 76.1%) and PH (mean pulmonary arterial pressure: 50 to 28 mmHg; pulmonary vascular resistance: 680 to 518 dynes・sec・cm-5; pre- vs post-therapy, respectively) had improved markedly.
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Observational Study
Elevated Crude Mortality in Obese Chronic Kidney Disease Patients with Loss of Exercise Habit: A Cohort Study of the Japanese General Population.
Objective The relationship between obesity and risk of death in chronic kidney disease (CKD) patients remains controversial. In addition, no clear evidence has been accumulated regarding whether or not exercise improves mortality in CKD patients. Methods The original cohort was based on a Japanese general population of 685,889 people from 40 to 74 years old who had undergone annual specific health checkups. ⋯ In the obese CKD population, mortality was higher with loss of exercise habits (0.96%) than in those continuously maintaining exercise habits (0.52%). The age- and sex-adjusted hazard ratio for all-cause death was 2.23 in the group with weight gain compared to the group with stable weight (p<0.01) and 2.08 in the group with loss of exercise habits compared to those who maintained exercise habits (p<0.01). Conclusion This observational cohort study suggested that loss of exercise habits as well as weight gain of more than 1 kg/m2/year might worsen all-cause mortality in the obese CKD population.