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Familial pseudohyperkalemia (FP) is a dominantly inherited condition caused by variants in the gene ABCB6 resulting in red blood cell (RBC) membrane protein defects. FP is generally asymptomatic. However, FP RBCs have an increased permeability to monovalent cations when stored below 37°C. Transfusion of RBC components donated by FP individuals can induce hyperkalemia and may be causally related to transfusion-associated hyperkalemic cardiac arrest, particularly in neonates and infants. Therefore it is necessary to accurately evaluate the frequency of FP occurrence in the Korean population and assess whether FP RBCs have significantly higher supernatant potassium levels. Efforts should be made to recognize the effects of blood products collected from FP donors on blood transfusion recipients to reduce the risk of hyperkalemia, especially in fetuses, infants, and patients at risk of this condition.
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Background@#The importance of establishing a regional stroke safety net for the treatment of acute stroke is increasingly emphasized. In this study, we analyzed effectiveness of transferring the suspected acute ischemic stroke (AIS) patients through the 1899-0215 hotline system from 119 ambulances and nearby hospitals. @*Methods@#From July 2015 to July 2022, we analyzed data from 632 suspected acute stroke patients through the hotline. Furthermore, we investigated the proportion of patients in the target population who were eligible for reperfusion therapy and compared cases transferred by 119 emergency services (EMS) with those transferred from nearby hospitals. @*Results@#Six hundred thirty-two suspected acute stroke patients were transferred to our stroke center through the 1899-0215 hotline system. The accuracy of diagnosing acute stroke among transferred patients is 81.0% for 119 EMS and 80.5% for cases transferred from nearby hospitals. Of the 200 patients transferred from hospitals, they were transferred from 47 nearby hospitals with an average transfer distance of 38.2 km and an average transfer time of 65 minutes. Among 291 patients diagnosed with AIS, The onset-to-door time was significantly shorter (p<0.01) for patients transferred by 119 EMS. The proportion of patients who received reperfusion therapy was significantly higher (p<0.01) for those transferred by 119 EMS. @*Conclusions@#The hotline service will become a useful tool for establishing a regional stroke network in the Busan area, and its effectiveness will be particularly useful for 119 EMS. Measures are needed in the Busan metropolitan region to ensure the speediness of transferring acute stroke patients between hospitals.
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Intravascular lymphoma is characterized by an exclusively intravascular distribution of tumor cells. Intravascular natural killer/T-cell lymphoma (IVNKTL) is extremely rare, highly aggressive, commonly Epstein-Barr virus (EBV)–positive, and predominantly affects the skin and central nervous system. Here we report a case of IVNKTL diagnosed in a 67-year-old female, presenting with persistent intermittent fever and skin rashes throughout the body. Incisional biopsy of an erythematous lesion on the chest exhibited aggregation of medium to large-sized atypical lymphoid cells confined to the lumen of small vessels that were positive for CD3, granzyme B, and CD56 on immunohistochemistry and EBV-encoded RNA in situ hybridization. EBV DNA was also detected in serum after diagnosis. With a review of 26 cases of IVNKTL to date, we suggest that active biopsy based on EBV DNA detection may facilitate early diagnosis of IVNKTL.
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Background@#This study investigated the associations between transfusion of different types of red blood cell (RBC) preparations and kidney allograft outcomes after kidney transplantation (KT) over a 16-year period in Korea using a nationwide population-based cohort. @*Methods@#We investigated the reported use of RBCs during hospitalization for KT surgery, rejection, and graft failure status using nationwide data from the National Health Information Database (2002–2017). The associations between the type of perioperative RBC product and transplant outcomes were evaluated among four predefined groups: no RBC transfusion, filtered RBCs, washed RBCs, and packed RBCs (pRBCs). @*Results@#A total of 17,754 KT patients was included, among which 8,530 (48.0%) received some type of RBC transfusion. Of the patients who received RBC transfusion, 74.9%, 19.7%, and 5.4% received filtered RBCs, pRBCs, or washed RBCs, respectively. Regardless of the type of RBC products, the proportions of acute rejection and graft failure was significantly greater in patients receiving transfusion (P < 0.001). Cox proportional hazards regression analyses showed that the filtered RBC and pRBC groups were significantly associated with both rejection and graft failure. The washed RBC group also had hazard ratios greater than 1.0 for rejection and graft failure, but the association was not significant. Rejection-free survival of the pRBC group was significantly lower than that of the other groups (P < 0.001, log-rank test), and graft survival for the no RBC transfusion group was significantly greater than in the other groups (P < 0.001, log-rank test). @*Conclusion@#Perioperative RBC transfusion was associated with poor graft outcomes.Notably, transfusion of pRBCs significantly increased transplant rejection. Therefore, careful consideration of indications for RBC transfusion and selection of the appropriate type of RBCs is necessary, especially for patients at high risk of rejection or graft failure.
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Transfusion is an essential life-sustaining treatment for many patients. However, unnecessary transfusion has been reported to be related to worse patient outcomes. Further, owing to the recent pandemic, blood supply has been more challenging to maintain. Many studies have been conducted to elucidate the optimal transfusion threshold for many clinical conditions, and most suggested that a restrictive transfusion strategy has advantages over a liberal transfusion strategy. Hematologic disorders, which require chronic transfusion in many cases, have not been the main subjects of such studies, and only little evidence is available regarding the optimal transfusion threshold in these patients.According to several recent studies, a liberal transfusion strategy is preferable for patients with hematologic disorders due to their quality of life. A patient-centered approach is needed for proper management of hematologic disorders.
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Objective@#The aims of our study were to identify distinct trajectories of cognitive function using the group-based trajectory model. We also investigate which demographic factors act as risk factors for cognitive decline in each group. @*Methods@#The data from the Seoul National University Hospital Healthcare System Gangnam Center, from 2005 to 2019. The number of study subjects was 637. We used a group-based model to identify cognitive function trajectories. Multinomial logistic regression was employed to define risk factors for cognitive function decline. @*Results@#The cognitive function trajectories among adults over 40 years of age were heterogeneous. We identified four trajectories: high (27.3%), medium (41.0%), low (22.7%), and rapid decline (9.1%). Older age, male, low educational level, bad dietary habits, diabetes mellitus, technical worker, and lower income increased the likelihood of a cognitive function decline. @*Conclusion@#A younger age, a higher educational level, professional worker, good dietary habits, no diabetes mellitus, and no obesity improved cognitive function. A combination of these factors can improve “cognitive reserve” and delay cognitive decline. Interventions to prevent cognitive decline are needed after identification of high-risk groups for cognitive decline.
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The unexpected antibody screening test is one of the most important pretransfusion tests. The tests are usually performed using commercial cells, which in Asia may not include clinically important antigens. To overcome this limitation, some laboratories use additional screening cells, including Di a or Mi a . However, these efforts are not supported by the Korean National Health Insurance system. The estimated total medical costs for using additional screening cells are less than the estimated total medical costs for the management of potential hemolytic transfusion reactions caused by missing clinically important antibodies. Hence, it is recommended that the Health Insurance system should be modified to take this into account and address the issue.
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Del-Nido cardioplegia (DNc) is a single-dose cardioplegia that is widely used in human medicine because of its long duration. In this report, we describe two cases of open-heart surgery with cardiopulmonary bypass (CPB) using DNc. One dog was diagnosed with partial atrioventricular septal defect, and the other dog was diagnosed with myxomatous mitral valve disease stage D. Both dogs were treated with open-heart surgery with DNc to induce temporary cardiac arrest. No complications from DNc were observed, and the patients were discharged. Veterinary heart surgeons should consider DNc as an option for temporary cardiac arrest during open-heart surgery with CPB.
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Purpose@#To investigate the outcomes of patients who underwent surgical correction of congenital superior oblique muscle paralysis in adulthood. @*Methods@#We retrospectively analyzed the medical records of 35 patients who underwent surgical correction of congenital superior oblique muscle paralysis after the age of 18 years. At the final follow-up, success was defined as residual hypertropia < 4 prism diopters (PD), whereas failure was defined as residual hypertropia ≥ 5 PD or overcorrection. Clinical features were compared between the success and failure groups to identify factors affecting the surgical outcome. At the final follow-up, the resolution of symptoms, such as head tilt and diplopia, was also recorded. @*Results@#Of the 35 patients, 24 (68.6%) and 11 (31.4%) were classified into the success and failure groups, respectively. Preoperative vertical deviation was larger in the failure group compared to the success group (23.1 vs. 15.2 PD, respectively; p < 0.05). Of the 31 patients with preoperative head tilt, head tilt resolved in 29 (93.5%). Of the 19 patients with diplopia, it resolved in all except 1 who was overcorrected. @*Conclusions@#The preoperative vertical angle of the superior oblique muscle may affect the outcome of surgery performed in adulthood for correcting congenital superior oblique paralysis. Head tilt, diplopia, and subjective symptoms are typically resolved postoperatively.
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Background@#For acute ischemic stroke (AIS) patients with history of prior stroke (PS) and diabetes mellitus (DM), intravenous recombinant tissue plasminogen activator (IV-tPA) therapy in the 3- to 4.5-hour window is off-label in Korea. This study aimed to assess the safety and efficacy of IV-tPA in these patients. @*Methods@#Using data from a prospective multicenter stroke registry between January 2009 and March 2021, we identified AIS patients who received IV-tPA in the 3- to 4.5-hour window, and compared the outcomes of symptomatic intracranial hemorrhage (SICH), 3-month mortality, 3-month modified Rankin Scale (mRS) score 0-1 and 3-month mRS distribution between patients with both PS and DM (PS/DM, n=56) versus those with neither PS nor DM, or with only one (non-PS/DM, n=927). @*Results@#The PS/DM group versus the non-PS/DM group was more likely to have a prior disability, hypertension, hyperlipidemia, coronary heart disease and less likely to have atrial fibrillation. The PS/DM and the non-PS/DM groups had comparable rates of SICH (0% vs. 1.7%; p>0.999) and 3-month mortality (10.7% vs. 10.2%; p=0.9112). The rate of 3-month mRS 0-1 was non-significantly lower in the PS/DM group than in the non-PS/DM group (30.4% vs. 40.7%; adjusted odds ratio [95% confidence interval], 0.81 [0.41-1.59]). @*Conclusions@#In the 3- to 4.5-hour window, AIS patients with PS/DM, as compared to those with non-PS/DM, might benefit less from IV-tPA. However, given the similar risks of SICH and mortality, IV-tPA in the late time window could be considered in patients with both PS and DM.
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Background@#There is growing evidence that abnormal liver function tests (LFTs) are common in patients with coronavirus disease 2019 (COVID-19). However, it is not known whether viral involvement in the liver differs according to the strain. We investigated the impact on liver injury in patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Delta (B.1.617.2) variants. @*Materials and Methods@#We conducted a single-center, retrospective cohort study, including 372 patients admitted during the pre-Delta period (PDP: between February 1 and November 30, 2020) and 137 patients admitted during the Delta period (DP: between August 1 and August 31, 2021). Initial liver injury was defined as alanine aminotransferase (ALT) or aspartate aminotransferase (AST) levels ≥3 × the upper limit of normal (ULN) or alkaline phosphatase (ALP) or total bilirubin ≥2 × the ULN within 3 days from admission. @*Results@#Of 509 patients with COVID-19 included in our study, 38 (7.5%) patients had initial liver injury. The DP group had a significantly higher rate of initial liver injury than the PDP group (PDP: 5.9% vs. DP: 11.7%, P = 0.028). The DP group (adjusted odds ratio [aOR]: 2.737, 95% confidence interval [CI]: 1.322 – 5.666) was independently associated with initial liver injury. During hospitalization, 160 (31.4%) patients had severe COVID-19. The DP group and initial liver injury had higher odds of progressing to severe COVID-19 (aOR: 2.664, 95% CI: 1.526 - 4.648, and aOR: 4.409, 95% CI: 1.816 - 10.707, respectively). The mediation analysis suggested that initial liver injury mediates the relationship between SARS-CoV-2 Delta variant infection and severe COVID-19 (unstandardized beta coefficient = 0.980, Standard error = 0.284, P = 0.001). @*Conclusion@#Initial liver injury is more common in COVID-19 patients with Delta variants. Also, Delta variants and initial liver injury are associated with poor clinical outcomes.
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Leukoreduction is a process in which the white blood cells (WBCs) in cellular products are intentionally reduced to bring down the risk of adverse transfusion reactions, such as febrile nonhemolytic transfusion reactions or human leukocyte antigen alloimmunization. So far, Korea has not considered leukoreduction of plasma products. However there have been recommendations for leukoreduction to improve patient outcomes. The authors have experience in measuring WBCs and WBC fragment counts in plasma products and have shown that the WBC and their fragments could be efficiently removed using leukoreduction filters. Hence, it may be beneficial to begin discussions on the necessity of using leukoreduction of plasma products.
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Mitral regurgitation (MR) is the most common heart disease in small-breed dogs. Mitral repair, which comprise artificial chorda tendineae implantation and mitral annuloplasty, has become the mainstay of treatment in the veterinary field. We report on two small dogs who underwent mitral repair surgery for MR. A Hegar dilator was used during mitral annuloplasty for accurate and reproducible surgery. In both cases, mitral regurgitant flow almost disappeared after surgery, and clinical signs improved. The treatment regimen was terminated 3 months after the surgery. We concluded that using a Hegar dilator may facilitate mitral valve repair surgery.
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Antithrombotic therapy is a cornerstone of acute ischemic stroke (AIS) management and secondary stroke prevention. Since the first version of the Korean Clinical Practice Guideline (CPG) for stroke was issued in 2009, significant progress has been made in antithrombotic therapy for patients with AIS, including dual antiplatelet therapy in acute minor ischemic stroke or high-risk transient ischemic stroke and early oral anticoagulation in AIS with atrial fibrillation. The evidence is widely accepted by stroke experts and has changed clinical practice. Accordingly, the CPG Committee of the Korean Stroke Society (KSS) decided to update the Korean Stroke CPG for antithrombotic therapy for AIS. The writing members of the CPG committee of the KSS reviewed recent evidence, including clinical trials and relevant literature, and revised recommendations. A total of 35 experts were invited from the KSS to reach a consensus on the revised recommendations. The current guideline update aims to assist healthcare providers in making well-informed decisions and improving the quality of acute stroke care. However, the ultimate treatment decision should be made using a holistic approach, considering the specific medical conditions of individual patients.
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The deep inferior epigastric perforator (DIEP) flap has been widely used for autologous breast reconstruction after mastectomy. In the conventional surgical method, a long incision is needed at the anterior fascia of the rectus abdominis muscle to obtain sufficient pedicle length; this may increase the risk of incisional hernia. To shorten the incision, several trials have investigated the use of endoscopic/robotic devices for pedicle harvest; however, making multiple additional incisions for port insertion and operating in the intraperitoneal field were inevitable. Here, we describe the first case, in which a DIEP free flap was successfully made using the da Vinci SP model. Our findings can help surgeons perform operations in smaller fields with a single port in the extraperitoneal space. Moreover, this method is expected to lead to fewer donor-related complications and faster healing.
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Kimura disease is characterized by painless subcutaneous masses, usually in the head and neck area as well as serum/tissue eosinophilia and elevated serum IgE levels. Various treatment methods, such as surgical excision, corticosteroids, and radiotherapy, have been suggested, but none of these are considered standard therapy due to high recurrence rates. Dupilumab is a human monoclonal antibody that binds to the alpha subunit of IL-4 receptor for IL-4 and IL-13 and inhibits T helper 2 (Th2) response, which plays an important role in the pathogenesis of allergic diseases, such as atopic dermatitis, asthma, and nasal polyps. Kimura disease has also been shown to increase CD4+Th2 cells and Th2 cytokines. We report a case of a 12-year-old boy who had subcutaneous neck and orbital masses of Kimura disease concurrent with allergic rhinitis, decreased lung function, and IgG subclass deficiency. The masses did not improve with omalizumab or immunomodulatory agents, but resolved with dupilumab. Considering potential side effects of steroids or immunosuppressant agents, dupilumab may be an option for initial treatment with Kimura diseases in children.
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Purpose@#: People who recovered from the COVID-19 complain of psychological symptoms such as stress, anxiety, and depression by psycho-social stress due to social labelling in the early stage of the epidemic. Stress, depression, anxiety, and degree of stress were compared between those recovered from early infection and those recovered from late infection. @*Methods@#: The ‘Perceived Stress Scale’ and ‘Hospital Anxiety-Depression Scale’ was compared for patients recovering from early (February-May 2020) infection (n=20) and patients recovering from two years later (March-April 2022) infection (n=21). @*Results@#: The median age of patients recovering from early/late illness was 51.3/46.1 years. The average score of the perceived stress scale was higher in the early period(19.6±6.52), compare to late period(15.3±6.49), and severe stress was more common in early period(40%) compare to late period(14%). The average score of hospital anxiety and depression were significantly higher in early period(15.4±5.45), compare to late period(9.6±5.44). The average score of the anxiety domain was significantly higher in the early period(6.6±3.25) than late period(4.7±2.96), and severe anxiety(8point~) was more common in early period(45%) compare to late period(9.5%). The average score in depression domain was higher in the early period(8.4±3.86) than late period(6.1±3.21), and the severe depression(8point~) was more common in early period(70%) compare to late period(19%). @*Conclusion@#: Those who recovered after early stages of the COVID-19 epidemic showed significantly higher levels of stress perception, anxiety, and depression than patients in the late stages of the epidemic two years later.
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Purpose@#To retrospectively analyze the relative frequency and describe the clinical features of sagging eye syndrome in Korean patients from a single center. @*Methods@#We retrospectively analyzed the medical records of patients with diplopia, aged over 40 years, who visited Chosun University Hospital from January 2018 to December 2020. The relative frequency of sagging eye syndrome was examined by classifying the cause of diplopia. Clinical features, such as age, sex, strabismus type, angle of deviation, treatment method, and prognosis were evaluated. @*Results@#A total of 128 patients were identified, of which 23 (18%) were diagnosed with sagging eye syndrome, including 12 male patients (52.2%) and 11 female patients (47.9%). Their mean age was 74.6 ± 7.6 years (range, 61–89 years), and all patients were over 60 years. Among the 62 patients with diplopia and age over 60 years, the relative frequency of sagging eye syndrome was 23 (37.1%), the highest among all age groups. Among the 23 patients with sagging eye syndrome, nine patients (39.1%) had only distance esotropia, with a mean distance esotropia value of 10.1 ± 8.6 prism diopters (PD) (range, 4–25 PD) at the first visit; nine patients (39.1%) had a combination of esotropia and vertical strabismus, with a mean esotropia value of 6.2 ± 4.8 PD (range, 2–12 PD) and a vertical angle of 4.7± 3.2 PD (range, 2–10 PD); and five patients (21.7%) had only vertical strabismus, with an average vertical strabismus angle of 3.3 ± 1.6 PD (range, 4–8 PD). Furthermore, 17 out of 23 patients (73.9%) used prism glasses, in whom the symptoms of diplopia disappeared. Finally, only three patients (13.0%) underwent surgery. @*Conclusions@#Sagging eye syndrome in Korean patients was identified in those over 60 years with a similar male-to-female ratio. Moreover, nonsurgical treatments, such as prism glasses, largely helped relieve the symptoms of sagging eye syndrome.
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The Rh blood group system has C, D, E, c, and e as the main antigens, but ce(f) has been reported as a compound antigen. Anti-f(ce) is an unexpected antibody (Ab) against the ce(f) compound antigen. This paper reports a case with anti-f(ce) and anti-M Abs in a patient with liver cirrhosis. A 47-year-old male patient was repeatedly admitted to hospital due to recurrent hepatic encephalopathy. He showed disorientation and was admitted. A packed red blood cells (pRBCs) transfusion was required, and Ab identification test identified anti-f(ce) and anti-M Abs. Anti-f(ce) Ab can cause fetal neonatal hemolytic disease and a clinically serious hemolytic transfusion reaction (HTR), and anti-M Ab can cause a HTR when it reacts at 37℃. RBCs with Rh haplotype of CDe and negative for M antigen were transfused to the patient. There was no HTR. The possibility of an anti-f(ce) Ab was not considered when an unexpected Ab screening/identification test was performed. It was simply reported as an ‘unknown alloantibody’. Therefore, laboratory physicians should consider Abs to the Rh compound antigen when Abs to Rh antigens are identified, and efforts should be made to identify them to gain basic knowledge about Abs against Rh compound antigens.