Medicine
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Peripheral nerve injuries (PNIs) in the upper extremities is an important medical problem, causing significant morbidity at a relatively young age. The epidemiology of PNI in South Korea has not been comprehensively evaluated. The purpose of our study was to examine the incidence of upper extremity PNI in South Korea based on an analysis of nationwide data and to investigate the association between PNI and patients' demographic characteristics. ⋯ We investigated the incidence trend and epidemiologic characteristics of upper extremity peripheral nerve injury in South Korea from 2008 to 2018. A decreasing tendency of annual incidence was observed from 2013 onwards. Finger and digital nerve were most commonly injured, and the incidence of PNI was higher in the summer and in men.
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Gastrointestinal bleeding caused by a ruptured pancreatic pseudoaneurysm is rare, and a pseudoaneurysm of the posterior inferior pancreaticoduodenal artery is especially rare. A 68-year-old man was hospitalized after presenting intermittent black stools and dyspnea accompanied by severe anemia. Angiographic examination revealed that Pseudoaneurysm of the posterior inferior pancreaticoduodenal artery. ⋯ posterior inferior pancreaticoduodenal artery pseudoaneurysmIt is rare and difficult to diagnose, gastrointestinal bleeding is a serious complication, vascular interventional embolization is effective.
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Niemann-Pick disease Type C (NPC) is a genetic, incurable, neurodegenerative disorder. This orphan disease is most frequently caused by mutations in the NPC1 protein, resulting in intralysossomal cholesterol accumulation. NPC1 is found in neuronal cell bodies, axon terminals and synaptosomes, suggesting it plays a role in lysosomal degradation pathway and in synaptic transmission. Neuronal function is especially vulnerable to NPC1 deficiency and synaptic changes seem a key element in disease development. Currently, Miglustat (Zavesca®) is the only approved treatment for NPC. However, preclinical evidence showed that low-dose Efavirenz reverted synaptic defects through pharmacological activation of the enzyme CYP46. ⋯ NPC is still an unmet medical need. Although different therapeutic approaches are under study, this is the first clinical trial (to the best of our knowledge) studying the effects of Efavirenz in adult- and late-juvenile-onset NPC. Despite the small sample size and the single-arm design, we expect the results to show Efavirenz's capacity of activating the CYP46 enzyme to compensate for NPC1 deficiency and correct synaptic changes, therefore compensating cognitive and psychiatric changes in these patients. This study may provide direct benefit to enrolled patients in terms of slowing down the disease progression.
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Case Reports
Case report: Moyamoya disease, the culprit in an adult female presenting with left sided numbness.
Moyamoya disease is a rare progressive cerebrovascular disease that is characterized by stenosis of the vascular branches of the internal carotid artery and the development of abnormal, fragile collateral vessels. Patients with Moyamoya disease mainly present with signs and symptoms of cerebral ischemia or cerebral hemorrhage with a typically progressive course. ⋯ This case highlights the importance of considering Moyamoya disease in the differential diagnosis of patients presenting with sudden neurological symptoms. High-resolution vessel wall MRI is a useful tool to diagnose this disease.
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Treatment methods for proximal femoral fractures, when the fractures run from the femoral basal neck to the subtrochanteric area, have not yet been fully reported. Thus, we aimed to clarify osteosynthesis methods based on the fracture frequency and clinical results. We classified the proximal femoral fractures using the Area classification method based on the location (area) of the fracture line. ⋯ Poor results such as cutout or excessive telescoping of lag screw occurred in 11.7% of cases and 17.2% of lateral trochanteric wall fractures. Even in cases where long nails and multiple lag screws were used for femoral trochanteric fractures whose fracture line ran from the femoral basal neck to subtrochanteric area were used, the failure rate was high in the presence of a lateral wall fracture. Therefore, it is necessary to consider careful post-operative treatment for proximal femoral fractures with lateral wall fracture, whose fracture line runs from femoral basal neck to subtrochanteric area.