Internal medicine journal
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Internal medicine journal · Mar 2024
Cigarette smoking and risk of adult-onset Still disease: a propensity score matching analysis.
Environmental factors play an important role in the pathogenesis of rheumatic diseases. Smoking is thought to be a risk factor for autoimmune rheumatic diseases. ⋯ Smoking probably is not a risk factor for AOSD and did not affect the response to treatment.
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Internal medicine journal · Mar 2024
Case ReportsDRESS syndrome with multiorgan involvement and HHV-6 reactivation in the absence of a drug trigger.
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction where patients present with fever, morbilliform rash and multiorgan manifestations, which may include acute renal failure, acute respiratory distress syndrome and eosinophilic myocarditis. We present a case of a 60-year-old woman with acute heart failure, DRESS syndrome features and human herpesvirus 6 reactivation in the absence of a drug trigger. She was diagnosed with eosinophilic myocarditis and successfully treated with corticosteroid therapy.
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Internal medicine journal · Mar 2024
Clinical spectrum, biochemical profile and disease progression of Kennedy disease in an Indian cohort.
Kennedy disease (KD) is a slowly progressive lower motor neuron degenerative disease. The prevalence of KD is unknown in India. ⋯ This study showed phenotypic heterogeneity in the Indian cohort. The age of onset was earlier with a slowly progressive indolent course as compared with other ethnic cohorts. This highlights the importance of considering the KD diagnosis in patients with the indolent course and suspected ALS diagnosis even with ptosis and fatigability in an appropriate clinical context.
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Internal medicine journal · Mar 2024
Left ventricular assist devices for treatment of refractory advanced heart failure: the Western Australian experience.
Left ventricular (LV) assist devices (LVADs) can prolong survival and improve quality of life in end-stage heart failure. ⋯ Excellent outcomes can be achieved with LVADs in appropriately selected patients.
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Internal medicine journal · Mar 2024
Rapid home therapy infusion of velaglucerase alfa in naïve patients with Gaucher disease.
Enzyme replacement therapy (ERT) has revolutionised the management of patients with Gaucher disease (GD). In 2018, we published the safety and efficacy of rapid 10-min infusion of velaglucerase alfa in previously treated patients, mostly on low-dose therapy. ⋯ Home therapy with rapid infusion of high-dose velaglucerase alfa was a safe, effective and preferable alternative for patients with GD naïve to treatment. We believe that shortening the infusion time improves the QoL of patients with GD who have a lifelong commitment to intravenous therapy.