Internal medicine
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We herein report the case of a 78-year-old woman who was diagnosed as having disseminated herpes zoster (DHZ) complicated with probable varicella-zoster pneumonia during maintenance therapy for microscopic polyangiitis. Because the patient had severe renal dysfunction, amenamevir administration was started to avoid any neurotoxicity of acyclovir, which is suggested to be optimal for treatment. ⋯ This is the first report suggesting the efficacy of amenamevir in the treatment of severe herpes zoster infection with coexisting DHZ and probable varicella-zoster pneumonia. Amenamevir could thus be a treatment option for severe varicella zoster virus infections.
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The PRPS1 gene encodes phosphoribosyl pyrophosphate synthetase 1 (PRS-1). The phenotypes associated with PRPS1 mutations include DFN2 (mild PRS-1 deficiency), X-linked Charcot-Marie-Tooth disease type 5 (CMTX5) (moderate PRS-1 deficiency), Arts syndrome (severe PRS-1 deficiency), and PRS-1 superactivity1. ⋯ We herein report a Japanese patient with CMTX5 who had a novel hemizygous mutation c.82 G>C in PRPS1. Despite showing a typical clinical picture, the decrease in enzyme activity measured in the patient's erythrocytes was milder than in previously reported cases.
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Objective The Lewis lead configuration is an alternative bipolar chest lead and it can help detect atrial activity. The utility of the Lewis lead to distinguish orthodromic atrioventricular reentrant tachycardia (AVRT) from typical atrioventricular nodal reentrant tachycardia (AVNRT) by visualizing the apparent retrogradely conducted P waves was investigated. Methods Forty-four patients with paroxysmal supraventricular tachycardia (PSVT) were included in this study. ⋯ P waves were more often visible in the Lewis lead configuration than in the standard leads (66% vs. 45%). The RP interval was significantly longer for AVRTs than for AVNRTs (88±17 vs. 154±34 ms, p<0.001), which yields 89% sensitivity and 71% specificity for distinguishing these 2 tachyarrhythmias with a cut-off point of 100 ms. Conclusion A Lewis lead configuration may help to make an accurate diagnosis among the reentrant supraventricular tachycardias prior to procedures, owing to its ability to locate P waves.
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The efficacy of early immunosuppressive therapy without invasive therapy, such as endovascular or surgical revascularization, for pulmonary hypertension due to Takayasu arteritis (TAK-PH) remains to be elucidated. We herein report two cases of TAK-PH due to pulmonary arteritis successfully treated with early immunosuppressive therapy. A literature review of 42 cases of TAK-PH with pulmonary artery involvement showed that the cases treated with immunosuppressive therapy early after the onset (within 12 months) had a higher erythrocyte sedimentation rate and better outcome without invasive therapy than those treated later. TAK-PH may be successfully treated with immunosuppressive therapy without invasive therapy when diagnosed early with high disease activity.
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Guillain-Barré syndrome (GBS) has occasionally occurred in people who have received coronavirus disease 2019 (COVID-19) vaccines. Dysgeusia is rare symptom of GBS. ⋯ Although autoantibodies against glycolipids were not detected, immunotherapy with intravenous immunoglobulin and methylprednisolone pulse therapy effectively ameliorated the symptoms. Our report suggests that the COVID-19 vaccine may induce various clinical subtypes of GBS, including a rare variant with sensory ataxia and dysgeusia.