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Objective@#Pesticide poisoning is one of the leading causes of death, and it is important to classify high-risk patients. Lactate measurement could be used to assess the triage level and decide and prioritize treatment even within the same triage group. This study investigates whether point-of-care lactate measurement helps to triage pesticide poisoning patients in the emergency department (ED). @*Methods@#This retrospective study was done for pesticide poisoning patients between January 2018 to December 2021. Demographic data and laboratory results were collected by reviewing medical records. Statistical analysis was performed by dividing patients into death and survival groups. Univariable and multivariable logistic regression analyses were done. Receiver operating characteristic curves were obtained for variables with significant differences, and area under the curves (AUC) were calculated and compared. @*Results@#Among the 288 patients evaluated, there were 24 (8.33%) mortalities and 264 (91.67%) survivals during hospitalization. Age, the Acute Physiology and Chronic Health Evaluation II (APACHE-II) score, pH and base excess, and lactate levels were analyzed via univariable and multivariable logistic regression tests. We found that old age and high lactate were independent factors in predicting mortality for pesticide poisoning patients. AUCs with 95% confidence interval for age and lactate were 0.784 (0.653-0.915) and 0.803 (0.674-0.932), respectively. @*Conclusion@#Lactate measurement may be useful during triage of alert and consciousness patients presenting with pesticide poisoning and having stable vital signs in ED. This study has several limitations, and it is necessary to reconfirm the results through a well-designed prospective study.
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Background/Aims@#Patients with inflammatory bowel disease (IBD) are diagnosed with ankylosing spondylitis (AS) often. However, the disease course of patients with both IBD and AS is not well understood. This study aims to evaluate the effect of concomitant AS on IBD outcomes. @*Methods@#Among the 4,722 patients with IBD who were treated in 3 academic hospitals from 2004 to 2021, 55 were also diagnosed with AS (IBD-AS group). Based on patients’ electronic medical records, the outcomes of IBD in IBD-AS group and IBD group without AS (IBD-only group) were appraised. @*Results@#The proportion of patients treated with biologics or small molecule therapies was significantly higher in IBD-AS group than the proportion in IBD-only group (27.3% vs. 12.7%, P= 0.036). Patients with both ulcerative colitis and AS had a significantly higher risk of biologics or small molecule therapies than patients with only ulcerative colitis (P< 0.001). For univariable logistic regression, biologics or small molecule therapies were associated with concomitant AS (odds ratio, 4.099; 95% confidence interval, 1.863–9.021; P< 0.001) and Crohn’s disease (odds ratio, 3.552; 95% confidence interval, 1.590–7.934; P= 0.002). @*Conclusions@#Concomitant AS is associated with the high possibility of biologics or small molecule therapies for IBD. IBD patients who also had AS may need more careful examination and active treatment to alleviate the severity of IBD.
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Background and Objectives@#The prognostic or safety implication of renin-angiotensinaldosterone system inhibitors (RASi) in hypertrophic cardiomyopathy (HCM) are not well established, mainly due to concerns regarding left ventricular outflow tract (LVOT) obstruction aggravation. We investigated the implications of RASi in a sizable number of HCM patients. @*Methods@#We enrolled 2,104 consecutive patients diagnosed with HCM in 2 tertiary university hospitals and followed up for five years. RASi use was defined as the administration of RASi after diagnostic confirmation of HCM. The primary and secondary outcomes were all-cause mortality and hospitalization for heart failure (HHF). @*Results@#RASi were prescribed to 762 patients (36.2%). During a median follow-up of 48.1months, 112 patients (5.3%) died, and 94 patients (4.5%) experienced HHF. Patients using RASi had less favorable baseline characteristics than those not using RASi, such as older age, more frequent history of comorbidities, and lower ejection fraction. Nonetheless, there was no difference in clinical outcomes between patients with and without RASi use (log-rank p=0.368 for all-cause mortality and log-rank p=0.443 for HHF). In multivariable analysis, patients taking RASi showed a comparable risk of all-cause mortality (hazard ratio [HR], 0.70, 95% confidence interval [CI], 0.43–1.14, p=0.150) and HHF (HR, 1.03, 95% CI, 0.63–1.70, p=0.900). In the subgroup analysis, there was no significant interaction of RASi use between subgroups stratified by LVOT obstruction, left ventricular (LV) ejection fraction, or maximal LV wall thickness. @*Conclusions@#RASi use was not associated with worse clinical outcomes. It might be safely administered in patients with HCM if clinically indicated.
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Objective@#Venom-induced coagulopathy (VIC) is a common snakebite complication that can cause life-threatening hemorrhage. Previous studies have shown that snake venom can cause a decrease in the erythrocyte sedimentation rate (ESR), but this has not been investigated in actual clinical practice. This study evaluated the clinical utility of erythrocyte sedimentation rate as a predictive factor for VIC in patients with a poisonous snakebite. @*Methods@#From January 2012 to December 2021, this study performed a retrospective study of patients with venomous snakebites presenting to a tertiary emergency department. The demographic and laboratory data were collected through a chart review. The patients were divided into two groups, VIC and NoVIC groups. Logistic regression analysis was performed to identify the factors that predicted the presence of VIC, and the receiver operating characteristic (ROC) curve was drawn. @*Results@#One hundred and fifty-three patients were enrolled, and 31 patients (20.3%) developed VIC. The VIC group had significantly lower ESR than the NoVIC group (5.1±5.6 vs. 14.8±13.8; P<0.001). Logistic regression analysis showed that the decreased ESR was associated with the occurrence of coagulopathy (odds ratio, 0.957; 95% confidence interval, 0.917-0.999; P=0.045). The area under the curve was 0.701 in the ROC curve, and the cutoff value was set to 4.5 mm/hr. @*Conclusion@#ESR measured upon arrival at the emergency department was available to predict venom-induced coagulopathy in snakebite patients.
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Background@#Menorrhagia is a common cause of iron deficiency anemia (IDA) in premenopausal women. However, the effects of menorrhagia on IDA in premenopausal women have been underestimated compared to those on other IDA-related disorders (IRDs) such as gastrointestinal malignancies (GIMs). To better understand the relationship between menorrhagia and IDA in premenopausal women, we analyzed the National Health Insurance Service-National Health Information Database (NHIS-NHID). @*Methods@#From 2005 to 2008, data about women between the age of 20 and 59 years were extracted from the NHIS-NHID to create a propensity score-matched case (IDA) and control group. The annual incidence of IDA was calculated per age group. A 10-year follow up of the study population was determined to detect IRDs in case and control groups. We compared the risk of detection (ROD) of IRDs, including GIM and gynecological disorders associated with menorrhagia - leiomyoma of uterus (LM) and adenomyosis (AM), in the case and the control group. @*Results@#From 2005 to 2008, women diagnosed with IDA (n = 535,249) and healthy women as a control group (n = 1,070,498) were identified from the NHIS-NHID. The annual incidence of IDA was 767.4 (2005), 948.7 (2006), 981.6 (2007), and 897.7 (2008) per 100,000 women.The age distribution of IDA was similar each year; IDA was common in women aged 30–39 years (36–37%) and 40–49 years (30–32%), and its incidence was significantly decreased in women aged 50–59 years (< 10%). The ROD of IRDs were significantly higher in the IDA group than in the control group (LM: 20.8% vs. 6.9%, AM: 5.6% vs. 1.6%, and GIM: 2.6% vs.0.7%). The corresponding hazard ratios were 3.89 (95% confidence interval [CI], 3.85–3.93) for LM, 4.99 (95% CI, 4.90–5.09) for AM, and 3.43 (95% CI, 3.32–3.55) for GIM. The ROD of the IRDs varied; the ROD of LM in the IDA group increased with age and decreased in the age group 50–59 years. AM was more frequently detected in women with IDA aged 30–39 years and less in women older than 40 years. The frequency of GIM increased with age. @*Conclusion@#In this study, we found that the gynecologic disease is the main cause of IDA in premenopausal women. Gynecological evaluations should be performed more actively in the clinic to prevent and control IDA and IRDs.
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Background@#Interscalene brachial plexus block (ISB) is a common regional technique to manage acute postoperative pain for arthroscopic rotator cuff tear repair. However, rebound pain may compromise its overall benefit. Our aim was to investigate the primary hypothesis that perineural and intravenous dexamethasone have different effects on rebound pain after resolution of ISB for arthroscopic rotator cuff tear repair. @*Methods@#Patients aged ≥ 20 years scheduled for elective arthroscopic rotator cuff tear repair under general anesthesia with preoperative ISB were included. The participants were randomized to receive dexamethasone either perineurally (perineural group) or intravenously (intravenous group). In the perineural group, patients received ISB with 12 mL of 0.5% ropivacaine containing 5 mg of dexamethasone; simultaneously, 1 mL of 0.9% normal saline was administered intravenously. In the intravenous group, patients received ISB with 12 mL of 0.5% ropivacaine; simultaneously, 1 mL of dexamethasone 5 mg was administered intravenously. The primary outcome was the difference in the pain score (0–10 on numeric rating scale) between before and after ISB resolution. The secondary outcomes were the incidence of rebound pain; onset, duration, and intensity of rebound pain; time to the first analgesic request; and pain-related sleep disturbance. @*Results@#A total of 71 patients were randomized to either perineural group (n = 36) or intravenous group (n = 35). After block resolution, pain scores increased significantly more in the perineural group (mean ± standard deviation, 4.9 ± 2.1) compared to the intravenous group (4.0 ± 1.7, P = 0.043). The duration of ISB was more prolonged in the perineural group (median [interquartile range], 19.9 [17.2–23.1] hours) than the intravenous group (15.1 [13.7–15.9] hours, P < 0.001). The incidence of rebound pain and pain-related sleep disturbance during the first postoperative week was significantly higher in the perineural group than in the intravenous group (rebound pain: 44.4% vs. 20.0%, P = 0.028; sleep disturbance: 55.6% vs. 25.7%, P = 0.011). The duration and intensity of rebound pain were similar between the two groups. @*Conclusion@#Although perineural dexamethasone provided longer postoperative analgesia, intravenous dexamethasone was more beneficial in reducing pain increase after ISB resolution, incidence of rebound pain, and pain-related sleep disturbance.
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Mesenchymal stromal cells (MSCs) have attracted scientific and medical interest due to their self-renewing properties, pluripotency, and paracrine function. However, one of the main limitations to the clinical application of MSCs is their loss of efficacy after transplantation in vivo. Various bioengineering technologies to provide stem cell niche-like conditions have the potential to overcome this limitation. Here, focusing on the stem cell niche microenvironment, studies to maximize the immunomodulatory potential of MSCs by controlling biomechanical stimuli, including shear stress, hydrostatic pressure, stretch, and biophysical cues, such as extracellular matrix mimetic substrates, are discussed. The application of biomechanical forces or biophysical cues to the stem cell microenvironment will be beneficial for enhancing the immunomodulatory function of MSCs during cultivation and overcoming the current limitations of MSC therapy.
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Mastocytosis is a heterogeneous neoplasm characterized by accumulation of neoplastic mast cells in various organs. There are three main types: cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mast cell sarcoma. CM mainly affects children and is confined to the skin, whereas SM affects adults and is characterized by extracutaneous involvement, with or without cutaneous involvement. Most cases of SM have an indolent clinical course; however, some types of SM have aggressive behavior and a poor prognosis. Recent advances in the understanding of the molecular changes in SM have changed the diagnosis and treatment of aggressive and advanced SM subtypes. The International Consensus Classification and World Health Organization refined the diagnostic criteria and classification of SM as a result of accumulation of clinical experience and advances in molecular diagnostics. Somatic mutations in the KIT gene, most frequently KIT D816V, are detected in 90% of patients with SM. Expression of CD30 and any KIT mutation were introduced as minor diagnostic criteria after the introduction of highly sensitive screening methods. SM has a wide spectrum of clinical features, and only a few drugs are effective at treating advanced SM. Currently, the mainstay of SM treatment is limited to the management of chronic symptoms related to release of mast cell mediators. Small-molecule kinase inhibitors targeting the KIT-downstream and KIT-independent pathways were recently approved for treating advanced SM. I describe recent advances in diagnosis of SM, and review the currently available and emerging therapeutic options for SM management.
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Purpose@#We investigated the current status of imaging studies for pediatric blunt cervical spine injury, and applied 3 clinical decision rules to children with blunt trauma of the head or neck in a pediatric emergency center in Korea. The rules included National Emergency X-Radiography Utilization Study (NEXUS) criteria, Canadian Cervical Spine Rule, and Pediatric Emergency Care Applied Research Network risk factors. @*Methods@#This was a retrospective study conducted on 399 children aged 15 years or younger who visited the center after the blunt trauma, and underwent cervical spine radiographs from January 2020 through December 2021. We examined the clinical characteristics per age groups (0-1, 2-5, 6-12, and 13-15 years). Using the 3 rules, we selected children with a potential need for imaging studies (PNI). For this purpose, we analyzed the absence of low-risk variables and the presence of high-risk variables. Predictive performances of the rules were measured for the imaging-confirmed cervical spine injury. @*Results@#The study population (n = 399) had a median age of 5.0 years (interquartile range, 2.0-9.0) and a 64.2% boys’ proportion. Fall (36.6%) was the most common injury mechanism. Two children had the cervical spine injuries. As per NEXUS criteria, Canadian Cervical Spine Rule, and Pediatric Emergency Care Applied Research Network risk factors, 72 (18.0%), 289 (72.4%), and 74 children (18.5%) were classified as those with PNI, respectively. Resultantly, 291 children (72.9%) were classified as having PNI whereas the other 108 (27.1%) were deemed to undergo unnecessary imaging. The 3 rules had nearly 100% sensitivity and negative predictive value, except a 50% sensitivity of NEXUS criteria. @*Conclusion@#Imaging studies can be minimized for children with blunt trauma of the head or neck who are deemed without PNI per the 3 current clinical decision rules. More elaborate criteria are needed to make a timely diagnosis.
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Polycythemia vera (PV) is a rare myeloproliferative disease that causes elevated absolute red blood cell (RBC) mass due to uncontrolled RBC production. Moreover, this condition has been associated with a high risk of ischemic stroke and large vessel stenosis or occlusion, with many studies reporting cerebral infarction in PV patients. Despite these findings, there have been no reports on the vessel wall MRI (VW-MRI) findings of the narrowed vessels in PV-associated ischemic stroke patients. To the best of our knowledge, this is the first report in English regarding the carotid VW-MRI findings of a 30-year-old male diagnosed with PV after being hospitalized due to stroke.
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Purpose@#This study aimed to confirm the decision-making patterns for life-sustaining treatment (LST) and analyze medical service utilization changes after enforcement of the Life-Sustaining Treatment Decision-Making Act. @*Materials and Methods@#Of 1,237 patients who completed legal forms for life-sustaining treatment (hereafter called the LST form) at three academic hospitals and died at the same institutions, 1,018 cancer patients were included. Medical service utilization and costs were analyzed using claims data. @*Results@#The median time to death from completion of the LST form was three days (range, 0 to 248 days). Of these, 517 people died within two days of completing the document, and 36.1% of all patients prepared the LST form themselves. The frequency of use of the intensive care unit, continuous renal replacement therapy, and mechanical ventilation was significantly higher when the families filled out the form without knowing the patient’s intention. In the top 10% of the medical expense groups, the decision-makers for LST were family members rather than patients (28% patients vs. 32% family members who knew and 40% family members who did not know the patient’s intention). @*Conclusion@#The cancer patient’s own decision-making rather than the family’s decision was associated with earlier decision-making, less use of some critical treatments (except chemotherapy) and expensive evaluations, and a trend toward lower medical costs.
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Hypertrophic cardiomyopathy (HCM) is one of the most common inheritable cardiomyopathies. Contemporary management strategies, including the advent of implantable cardioverter-defibrillators and effective anticoagulation, have substantially improved the clinical course of HCM patients; however, the disease burden of HCM is still high in Korea. Sudden cardiac death (SCD), atrial fibrillation and thromboembolic risk, dynamic left ventricular outflow tract (LVOT) obstruction, and heart failure (HF) progression remain important issues in HCM. SCD in HCM can be effectively prevented with implantable cardioverter-defibrillators. However, appropriate patient selection is important for primary prevention, and the 5-year SCD risk score and the presence of major SCD risk factors should be considered. Anticoagulation should be initiated in all HCM patients with atrial fibrillation regardless of the CHA 2 DS 2 -VASc score, and non-vitamin K antagonist oral anticoagulants are the first option. Symptomatic dynamic LVOT obstruction is first treated medically with negative inotropes, and if symptoms persist, septal reduction therapy is considered. The recently approved myosin inhibitor mavacamten is promising. HF in HCM is usually related to diastolic dysfunction, while about 5% of HCM patients show reduced left ventricular ejection fraction <50%, also referred to as “end-stage” HCM. Myocardial fibrosis plays an important role in the progression to advanced HF in patients with HCM. Patients who do not respond to guideline-directed medical therapy can be considered for heart transplantation. The development of imaging techniques, such as myocardial deformation on echocardiography and late gadolinium enhancement on cardiac magnetic resonance, can provide better risk evaluation and decision-making for management strategies in HCM.
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Objective@#After the coronavirus disease 2019 (COVID-19) outbreak, there have been some changes in the way the medical system deals with suspected infectious diseases. These changes may also affect non-COVID-19 patients. Acute appendicitis is a common disease that requires emergent surgical intervention in pediatric patients, and delayed diagnosis and treatment may cause some complications. This study analyzed the impact of the COVID-19 pandemic on the incidence of complicated appendicitis in pediatric patients presenting to emergency departments in South Korea. @*Methods@#The target group (post-COVID group) included patients aged under 15 years and diagnosed with acute appendicitis between February 23 and November 30, 2020. Patients diagnosed during the same period in 2019 were selected as the control group (pre-COVID group). The difference in the incidence of complicated appendicitis before and after the COVID-19 outbreak was investigated, and the association with various variables was analyzed using the odds ratios and 95% confidence intervals. @*Results@#The study enrolled a total of 94 patients: 54 in the pre-COVID group and 40 in the post-COVID group. There was no statistically significant variation in the incidence of complicated appendicitis (31.5% vs 35.0%, P=0.723) between the groups. In-hospital time was longer in the post-COVID group (7 hours vs. 10.5 hours, P=0.014), but pre-hospital time showed no significant difference (16 hours vs. 22 hours, P=0.768). In the multivariable logistic regression analysis, pre-hospital time (odds ratio [OR], 1.08; P=0.004), erythrocyte sedimentation rate (OR, 33.24; P<0.001), and the presence of fever (OR, 21.11, P=0.002) showed a significant correlation. @*Conclusion@#Post the COVID-19 pandemic outbreak, there was no difference in the incidence of pediatric complicated appendicitis in South Korea.
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Objective@#This study aimed to develop texture-modified soups and classify them based on the International Dysphagia Diet Standardization (IDDSI) and National Dysphagia Diet (NDD) criteria. @*Methods@#Ten soups were selected, and a xanthan gum-based thickener was added to adjust their viscosity. Flow tests with a 10 ml syringe and viscosity measurements using a rheometer were carried out according to the IDDSI and NDD criteria. @*Results@#The addition of 1 g of thickener to the selected commercial soups resulted in different viscosity levels depending on the soup type. Under the IDDSI framework, seven soups (R SMG, BMG, KHG, SLT, B SUG, BGG, DGT) were categorized as level 1, two soups (B MYG, ADG) as level 2, and one soup (R DJJ) as level 3. As per NDD guidelines, seven soups (R SMG, BMG, KHG, SLT, B SUG, BGG, DGT) were classified as Nectar-like and three soups (R DJJ, B MYG, ADG) as Honey-like. However, the addition of 2 g of thickener decreased the fluidity of the soups, with two soups (R SMG, SLT) being classified as level 2 and eight soups (R BMG, DJJ, KHG, B SUG, MYG, ADG, BGG, DGT) as level 3 under the IDDSI criteria. Meanwhile, all soups were classified as Honey-like under the NDD criteria. @*Conclusion@#The viscosity was different due to the varying compositions of guk/tang/jjigae, the concentration of the thickener, and time. Swallowing standardsands and other guidelines applicable specifically to Korean-food for dysphagia patients need to be developed in a further study.
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Background@#This study was performed to evaluate etiologies and secular trends in primary amenorrhea in South Korea. @*Methods@#This retrospective multi-center study analyzed 856 women who were diagnosed with primary amenorrhea between 2000 and 2016. Clinical characteristics were compared according to categories of amenorrhea (hypergonadotropic/hypogonadotropic hypogonadism, eugonadism, disorders of sex development) or specific causes of primary amenorrhea. In addition, we assessed secular trends of etiology and developmental status based on the year of diagnosis. @*Results@#The most frequent etiology was eugonadism (39.8%). Among specific causes, Müllerian agenesis was most common (26.2%), followed by gonadal dysgenesis (22.4%). Women with hypergonadotropic hypogonadism were more likely to have lower height and weight, compared to other categories. In addition, the proportion of cases with iatrogenic or unknown causes increased significantly in hypergonadotropic hypogonadism category, but overall, no significant secular trends were detected according to etiology. The proportion of anovulation including polycystic ovarian syndrome increased with time, but the change did not reach statistical significance. @*Conclusion@#The results of this study provide useful clinical insight on the etiology and secular trends of primary amenorrhea. Further large-scale, prospective studies are necessary.
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Postpartum hemorrhage (PPH) is a major cause of maternal mortality and the risk factors for PPH differ among studies. In this large-scale study, we investigated whether the anesthetic method used was associated with PPH after cesarean section. Methods: We extracted data on cesarean sections performed between January 2008 and June 2013 from the National Health Insurance Service database. The anesthetic methods were categorized into general, spinal and epidural anesthesia. To compare the likelihood of PPH among deliveries using different anesthetic methods, crude and adjusted odds ratios (ORs) and 95% confidence intervals were calculated using logistic regression analysis. Results: Data from 330,324 cesarean sections were analyzed, and 21,636 cases of PPH were identified. Univariate analysis showed that general and epidural anesthesia increased the risk of PPH compared to spinal anesthesia. The OR for PPH was highest for morbidly adherent placenta, followed by placenta previa, placental abruption, and hypertension. When other clinical covariates were controlled for, general and epidural anesthesia still remained significant risk factors for PPH compared to spinal anesthesia. Conclusions: This study showed that general and epidural anesthesia elevated the risk of PPH compared to spinal anesthesia during cesarean section. Since we could not consider the potential bias of group differences in indications, more in-depth clinical trials are needed to validate our findings. Obstetric factors such as placental abnormalities had high odds ratios and thus are more important than the choice of anesthetic method, which should be based on the patient’s clinical condition and institutional resources.
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Purpose@#This study aims to investigate the relationship between smoking and alcohol consumption on the risk of suicide among culturally diverse adolescents. @*Methods@#This is a secondary data analysis study using data derived from the 15~17th (2019~2021) Youth Health Behavior Online Survey. Data from 2,922 culturally diverse adolescents were analyzed using descriptive statistics, the Rao-Scott x2 test, and hierarchical logistic regression. @*Results@#After controlling for demographic and individual, family, and social factors as compounding variables, the suicide attempt of culturally diverse adolescents with smoking and alcohol consumption dual use experience was a 1.91 odds ratio (95% CI: 1.02~3.55) compared to culturally diverse adolescents without smoking and alcohol consumption experiences. @*Conclusion@#Our study findings indicate a need to prevent suicide among culturally diverse adolescents with smoking and alcohol consumption experiences. Suicidal prevention programs would greatly benefit from the experiences from culturally diverse adolescents who are smoking and consuming alcohol to better their programs on decreasing suicide attempts.
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Purpose@#This study aims to analyze the mediation role that impulsivity plays between social stigma and SNS cyberbullying when moderated by parental attachment. @*Methods@#This study is a secondary analysis using the raw data from panel survey of adolescents who have dropped out of school provided by the Korea Youth Policy Research Institute, and the total number of samples is 576 cases. SPSS V.25 and PROCESS macro for SPSS V.3.5.2 were used for data analysis. @*Results@#In the relationship between social stigma and SNS cyberbullying behavior, impulsivity showed a statistically significant positive completely mediating effect. Parental attachment was found to control impulsivity as a parameter and SNS cyberbullying as a dependent variable, and thus the mediated moderating effect was verified. @*Conclusion@#Based on the results of this study, it was confirmed that parental attachment plays a role as a regulatory mechanism to lower impulsivity. More specifically, in controlling impulsivity caused by the social stigma of school-dropped adolescents, support based on parental attachment of parents is absolutely necessary.
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Purpose@#At the end of life, communication is a key factor for good care. However, in clinical practice, it is difficult to adequately discuss end-of-life care. In order to understand and analyze how decision-making related to life-sustaining treatment (LST) is performed, the shared decision-making (SDM) behaviors of physicians were investigated. @*Methods@#A questionnaire was designed after reviewing the literature on attitudes toward SDM or decision-making related to LST. A final item was added after consulting experts. The survey was completed by internal medicine residents and hematologists/medical oncologists who treat terminal cancer patients. @*Results@#In total, 202 respondents completed the questionnaire, and 88.6% said that the decision to continue or end LST is usually a result of SDM since they believed that sufficient explanation is provided to patients and caregivers, patients and caregivers make their own decisions according to their values, and there is sufficient time for patients and caregivers to make a decision. Expected satisfaction with the decisionmaking process was the highest for caregivers (57.4%), followed by physicians (49.5%) and patients (41.1%). In total, 38.1% of respondents said that SDM was adequately practiced when making decisions related to LST. The most common reason for inadequate SDM was time pressure (89.6%). @*Conclusion@#Although most physicians answered that they practiced SDM when making decisions regarding LST, satisfactory SDM is rarely practiced in the clinical field. A model for the proper implementation of SDM is needed, and additional studies must be conducted to develop an SDM model in collaboration with other academic organizations.
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Background@#In Fabry disease, the presence of globotriaosylceramide (GL3) deposits in various kidney cells leads to progressive renal dysfunction. However, kidney biopsy studies in patients with Fabry disease are limited. In the present study, the pathologic findings of patients with Fabry nephropathy receiving enzyme replacement therapy (ERT) and untreated patients without albuminuria were investigated. @*Methods@#The present study included 15 patients with Fabry disease who underwent renal biopsy while receiving ERT (group 1: n = 9, age 19–58 years, two males and seven females) or before ERT initiation (group 2: n = 6, age 11–66 years, one male and five females). All patients in group 2 were normoalbuminuric. @*Results@#Group 1 showed improved clinical symptoms, such as acroparesthesia. The ERT duration was 1.2 to 8 years and seven of the nine patients showed GL3 deposits in various kidney cells and segmental foot process effacement (FPE) of podocytes. GL3 deposits and FPE were not observed in the two remaining patients in group 1. Group 2 showed segmental FPE and podocyte GL3 deposits. Most patients in group 2 also showed GL3 deposits in the mesangium, endothelium, or tubular epithelium. @*Conclusion@#The study results showed that segmental FPE and GL3 deposits can persist in Fabry nephropathy despite ERT. In addition, segmental FPE and GL3 deposits were observed in various kidney cells in normoalbuminuric patients with Fabry disease. These findings indicated that kidney biopsies at baseline and follow-up evaluation of Fabry nephropathy are essential for timely ERT initiation and ERT response assessment.