Yonsei medical journal
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Yonsei medical journal · Apr 2020
ReviewNusinersen as a Therapeutic Agent for Spinal Muscular Atrophy.
The reduction of survival motor neuron (SMN) protein causes spinal muscular atrophy (SMA), an autosomal recessive neuromuscular disease. Nusinersen is an antisense oligonucleotide, approved by the FDA, which specifically binds to the repressor within SMN2 exon 7 to enhance exon 7 inclusion and augment production of functional SMN protein. ⋯ This review of nusinersen will discuss its action mechanism, cellular uptake, trafficking mechanisms, and administration approaches to cross the blood-brain barrier. Furthermore, nusinersen clinical trials will be assessed in terms of pharmacokinetics, tolerability and safety, the clinical outcomes of multiple intrathecal doses, and a discussion on the primary and secondary endpoints.
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Yonsei medical journal · Mar 2020
ReviewKinematically Aligned Total Knee Arthroplasty with Patient-Specific Instrument.
Kinematically aligned total knee arthroplasty (TKA) is a new alignment technique. Kinematic alignment corrects arthritic deformity to the patient's constitutional alignment in order to position the femoral and tibial components, as well as to restore the knee's natural tibial-femoral articular surface, alignment, and natural laxity. Kinematic knee motion moves around a single flexion-extension axis of the distal femur, passing through the center of cylindrically shaped posterior femoral condyles. ⋯ PSI was recently introduced as a new technology with the goal of improving the accuracy of operative technique, avoiding practical issues related to the complexity of navigation and robotic system, such as the costs and higher number of personnel required. There are several limitations to implement the kinematically aligned TKA with the implant for mechanical alignment. Therefore, it is important to design an implant with the optimal shape for restoring natural knee kinematics that might improve patient-reported satisfaction and function.
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Yonsei medical journal · Feb 2020
ReviewGamma Knife Radiosurgery on the Trigeminal Root Entry Zone for Idiopathic Trigeminal Neuralgia: Results and a Review of the Literature.
Trigeminal neuralgia (TN) is a chronic disorder of the trigeminal nerve characterized by repeated electrical shock-like sensations on one side of the face. It can cause severe pain in the face and disrupt or impair quality of life in patients. ⋯ Moreover, GKRS can be of use for drug-resistant TN patients who are poor surgical candidates due to medical comorbidities, patients of older age, or patients who refuse invasive therapy. We reviewed the rationale, effects, safety, and current treatment policies of GKRS for TN in view of our institution's results and a review of the literature to date.
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Yonsei medical journal · Oct 2019
Review"Hairiness" is a Facsimile of Reorganized Cytoskeletons: A Cytopathic Effect of Coxiella burnetii.
In 1993, I reported that Coxiella burnetii transforms human B cells into hairy cells (cbHCs), the first hairy cell reported outside of hairy cell leukemia (HCL). Over last few decades, advances in molecular biology have provided evidence supporting that C. burnetii induces hairiness and inhibits the apoptosis of host cells. The present review summarizes new information in support of cbHC. ⋯ Accordingly, I propose that C. burnetii induces reorganization of the cytoskeleton and inhibits apoptosis as cytopathic effects that are not target cell specific. The observed hairiness in cbHC appears to be a fixed image of dynamic nature, and hairy cells in HCL are distinct among lymphoid cells in circulation. As the cytoskeleton plays key roles in maintaining cell structural integrity in health and disease, the pathophysiology of similar cytopathic effects should be addressed in other diseases, such as myopathies, B-cell dyscrasias, and autoimmune syndromes.
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Yonsei medical journal · Aug 2019
ReviewEosinophilic Granulomatosis with Polyangiitis: Experiences in Korean Patients.
Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. ⋯ The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.