Yonsei medical journal
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Yonsei medical journal · Dec 2023
Nusinersen for Spinal Muscular Atrophy Type I with Chronic Respiratory Failure: A Retrospective Study in South Korea.
To analyze the efficacy and safety of nusinersen in patients with spinal muscular atrophy (SMA) type I with chronic respiratory failure. ⋯ Nusinersen is safe and effective in patients with SMA type I, even those with chronic respiratory failure and those on permanent ventilation. No significant adverse effects of nusinersen were observed.
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Yonsei medical journal · Dec 2023
Case ReportsSARS-CoV-2-Infected Pregnant Woman Requiring 38 Days of Extracorporeal Membrane Oxygenation Experiences Rectal Ulcer Bleeding: A Case Report.
Pregnancy has been shown to be associated with an adverse clinical course and symptomatic patients with severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection. Extracorporeal membrane oxygenation (ECMO) is rarely used in pregnant or postpartum women with severe coronavirus disease 2019 (COVID-19). ⋯ Despite being placed on ECMO for 38 days and receiving a massive transfusion of 95 packs of red blood cells, she recovered and was discharged on hospital day 112. ECMO can be used in most patients with severe COVID-19, including pregnant patients, although potential coagulopathy complications must be considered.
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Yonsei medical journal · Dec 2023
Case ReportsThe First Korean Adult Case of Progressive Familial Intrahepatic Cholestasis Type 7 with Novel USP53 Splicing Variants by Next Generation Sequencing.
Progressive familial intrahepatic cholestasis (PFIC) is a group of rare genetic disorders caused by defects in biliary epithelial transporters. It mostly presents as low γ-glutamyltransferase cholestasis. Recently, USP53 has been identified as one of the novel genes associated with PFIC. ⋯ At 4.5 years after the initial presentation, the patient's symptom and high bilirubin level were normalized after administration of high-dose ursodeoxycholic acid. Recognition of this disease entity is important for prompt diagnosis and management. USP53 is recommended for the work-up of low γ-glutamyltransferase cholestasis.
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Yonsei medical journal · Dec 2023
Drug Retention Rate and Factors Associated with Discontinuation of Interleukin-17 Inhibitors in Patients with Axial Spondyloarthritis.
To assess the drug retention rate of interleukin-17 inhibitors (IL-17is) over long-term observation in patients with axial spondyloarthritis (axSpA) in whom treatment with tumor necrosis factor inhibitors (TNFis) failed and to determine baseline factors associated with discontinuation of IL-17is. ⋯ In patients with axSpA in whom TNFis failed, the long-term drug retention rate of IL-17is appears to be acceptable, with a 3-year drug retention rate of approximately 60%. Current smoking was associated with a higher risk of discontinuing IL-17is, whereas previous use of ≥3 TNFis was associated with a lower risk of discontinuing IL-17is.
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Yonsei medical journal · Dec 2023
Erratum to "National Trends in Hospitalization for Ambulatory Care Sensitive Conditions among Korean Adults between 2008 and 2019" by Park H, et al. (Yonsei Med J 2022 Oct;63(10):948-955).
This corrects the article on p. 948 in vol. 63, PMID: 36168248.