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Background/Aims@#Systemic lupus erythematosus (SLE) responder index (SRI)-4 response has been achieved with belimumab treatment in patients with moderate disease activity in cornerstone clinical trials and following studies. However, most studies involved patients treated with a mean prednisolone-equivalent dose of approximately 10 mg/d and focused on the steroid-sparing effect of belimumab. We aimed to identify the effect of belimumab in patients with mild-to-moderate SLE who were treated with low-dose or no corticosteroids. @*Methods@#We retrospectively reviewed the electronic medical records of patients treated with belimumab for at least 6 months between May 2021 and June 2022. The primary endpoint was SRI-4 response at 6 months. @*Results@#Thirty-one patients were included (13 low dose- and 18 steroid non-users). The mean age was 39.2 ± 11.4 years, and 90.3% of patients were female. The baseline Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) score was 6.0 (4.0–9.0). The primary endpoint was achieved in 32.3% (10/31) of patients. Significant improvements in anemia, C4 levels, and SELENA-SLEDAI score were observed during treatment. Univariate analysis showed that the baseline SELENA-SLEDAI and arthritis were significantly associated with SRI-4 response at 6 months, and only the SELENA-SLEDAI remained significant (p = 0.014) in multivariate analysis. @*Conclusions@#This cohort study is the first to report the efficacy of belimumab after minimizing the effect of corticosteroids. Belimumab showed efficacy in improving the SELENA-SLEDAI score, anemia, and low C4 in patients who did not receive corticosteroids or received only low doses.
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The integrity of the high cervical spine, the transition zone from the brainstem to the spinal cord, is crucial for survival and daily life. The region protects the enclosed neurovascular structure and allows a substantial portion of the head motion. Injuries of the high cervical spine are frequent, and the fractures of the C2 vertebra account for approximately 17–25% of acute cervical fractures. We review the two major types of C2 vertebral fractures, odontoid fracture and Hangman’s fracture. For both types of fractures, favorable outcomes could be obtained if the delicately selected conservative treatment is performed. In odontoid fractures, as the most common fracture on the C2 vertebrae, anterior screw fixation is considered first for type II fractures, and C1–2 fusion is suggested when nonunion is a concern or occurs. Hangman's fractures are the second most common fracture. Many stable extension type I and II fractures can be treated with external immobilization, whereas the predominant flexion type IIA and III fractures require surgical stabilization. No result proves that either anterior or posterior surgery is superior, and the surgeon should decide on the surgical method after careful consideration according to each clinical situation. This review will briefly describe the basic principles and current treatment concepts of C2 fractures.
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Purpose@#This study aimed to evaluate the effect of prophylactic abdominal drainage (AD) in laparoscopic hemicolectomy, focusing on assessing postoperative pain outcomes. @*Methods@#Patients were categorized into two groups: those with and without AD (AD group vs.no-AD group). A numerical rating scale (NRS) was used to assess postoperative pain on each postoperative day (POD). Further, the inverse probability of treatment weighting (IPTW) method was used to reduce intergroup bias. @*Results@#In total, 204 patients who underwent laparoscopic hemicolectomies by a single surgeon between June 2013 and September 2022 at a single institution were retrospectively reviewed. After adjusting for IPTW, NRS scores on POD 2 were significantly lower in the no-AD group (3.2 ± 0.8 vs. 3.4 ± 0.8, p = 0.043). Further examination of postoperative outcomes showed no statistically significant differences in complications between the AD (17.3%) and no-AD (12.4%) groups (p = 0.170). The postoperative length of hospital stay was 7.3 ± 2.8 days in the AD group and 6.9 ± 3.0 days in the no-AD group, with no significant difference (p = 0.298). Time to first flatus was 3.0 ± 0.9 days in the AD group and 2.7 ± 0.9 days in the no-AD group, with no significant difference (p = 0.078). Regarding readmission within 1 month, there were four cases each in the AD (2.3%) and no-AD (1.7%) groups, with no significant difference (p = 0.733). @*Conclusion@#Laparoscopic hemicolectomy without AD resulted in no significant differences in postoperative clinical outcomes, except for postoperative pain. This finding suggests that prophylactic AD may exacerbate postoperative pain.
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Purpose@#Stickler syndrome, a hereditary connective tissue disorder characterized by mutations in collagen genes, presents with progressive ophthalmopathy and diverse systemic manifestations. Here, we present three familial cases of Stickler syndrome, emphasizing the importance of early detection through clinical investigations and genetic testing.Case summary: Two generations of a family, a mother and her two daughters, were evaluated for Stickler syndrome. All three exhibited bilateral retinal lattice degeneration, perivascular retinal degeneration, and vitreous liquefaction. The daughters shared characteristic facial features, including a flattened face, broad nasal bridge, and micrognathia. Clinical symptoms and examination findings led to diagnoses of Stickler syndrome. Subsequent genetic testing in five family members confirmed a COL2A1 mutation in the three affected individuals. @*Conclusions@#Stickler syndrome carries a high risk of vision loss from ocular complications, necessitating early detection and intervention. In addition, the presence of systemic manifestations, such as musculoskeletal joint disorders, mitral valve prolapse, hearing loss, and cleft palate, emphasizes the importance of prompt detection through appropriate clinical investigations and genetic testing.
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Purpose@#To report treatment failure of multidrug-resistant Acinetobacter baumannii keratitis in a patient wearing contact lenses for a long time.Case summary: A 48-year-old man using daytime soft lenses for 20 years was transferred due to decreased visual acuity and eye pain. Slit-lamp biomicroscopy showed a large corneal epithelial defect and stromal infiltration, but no hypopyon. Treatment was initiated with moxifloxacin, polyhexamethylene biguanide eye drops, and oral antibiotics. Corneal infiltration worsened, corneal scrapings and culture were performed. Gram staining showed Gram-positive bacteria, potassium hydroxide (KOH) hyphae-positive, and culture yielded Bacillus. He was hospitalized and vancomycin, voriconazole, and amphotericin B eye drops were administered. Oral antifungal agents were also prescribed. The corneal epithelial defect was restored from temporal side for a while, and then deteriorated with an endothelial plaque. Therefore, voriconazole and amphotericin B were injected intracamerally. All four repeated culture tests were negative. Despite total conjunctival flap, the patient complained of severe pain and eventually underwent evisceration. In the culture of intraocular contents, Acinetobacter baumannii, which is resistant to all drugs except minocycline, was detected. @*Conclusions@#In patients wearing contact lens for a long time, not only mixed infection with acanthamoeba and fungi, but also gram-negative bacteria should be considered first, and multidrug-resistant Acinetobacter baumannii should also be considered.
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Purpose@#This report presents a case of repeated toxic conjunctival necrosis caused by amikacin eye drops in a patient with a corneal ulcer induced by Mycobacterium abscessus (M. abscessus).Case summary: During treatment for herpetic keratitis with complaints of conjunctival hyperemia and eye pain in the right eye for 1 month. Slit-lamp microscopy revealed a circular corneal epithelial defect and stromal infiltration of her right eye. Gram staining and culture were performed, and eye drops were prescribed for empirical treatment. The bacterial culture grew M. abscessus. Antibiotic susceptibility tests showed resistance to quinolone. The patient was prescribed amikacin eye drops and the corneal epithelial defects improved. Two weeks later, circular conjunctival epithelial defects and hyperemia were observed on the inferior conjunctiva. Conjunctival necrosis caused by amikacin was suspected. The amikacin eye drops were discontinued and the conjunctival lesion improved. However, the patient’s corneal ulcer recurred. The amikacin eye drops were restarted, but conjunctival toxicity recurred, so she was treated with topical clarithromycin and moxifloxacin. The eye drops were gradually reduced and the lesion healed leaving mild corneal opacity. @*Conclusions@#M. abscessus-induced keratitis resistant to quinolone antibiotics requires long-term combined therapy with multiple drugs. Amikacin eye drops should be used carefully as conjunctival toxicity may occur.
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Purpose@#The Korean Society of Coloproctology has been conducting Colorectal Cancer Awareness Campaign, also known as the Gold Ribbon Campaign, every September since 2007. The 2022 campaign was held through a metaverse platform targeting the younger age group under the slogan of raising awareness of early-onset colorectal cancer (CRC). This study aimed to analyze the impact of the 2022 campaign on a metaverse platform. @*Methods@#Anonymized survey data were collected from participants in the metaverse campaign from September 1 to 15, 2022. The satisfaction score of the participants was evaluated by sex, age group, and previous campaign participation status. @*Results@#During the campaign, 2,770 people visited the metaverse. Among them, 455 people participated in the survey (response rate, 16.4%). Approximately 95% of the participants reported being satisfied with the information provided by the campaign, understood the necessity of undergoing screening for and prevention of early-onset CRC, and were familiar with the structure of the metaverse. The satisfaction score for campaign information tended to decrease as the participants’ age increased. When the participants’ overall level of satisfaction with the metaverse platform was assessed, teenagers scored particularly lower than the other age groups. The satisfaction scores for CRC information provided in the metaverse, as well as the scores for recognizing the seriousness and necessity of screening for early-onset CRC, indicated a high positive tendency (P<0.001). @*Conclusion@#Most of the 2022 Gold Ribbon Campaign participants were satisfied with the metaverse platform. Medical society should pay attention to increasing participation in and satisfaction with future public campaigns.
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Complete mesocolic excision and central vascular ligation with D3 lymphadenectomy are important surgical principles for improving oncological outcomes in colon cancer. The cranial-first approach is a colonic mobilization–first approach to radical right hemicolectomy, which has several advantages, including early feasibility assessment, safe dissection from surrounding organs, preestablished inferior margin of lymph node dissection, and revelation of the tangible anatomy of the tributaries of the gastrocolic trunk. This video demonstrates the cranial-first approach to radical right hemicolectomy in a 66-year-old man with locally advanced cecal cancer.
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Purpose@#Anal wounds following hemorrhoidectomy can lead to severe pain and postoperative bleeding, impacting patient recovery and quality of life. Hyaluronic acid (HA) stimulates tissue regeneration and wound healing by accelerating cell migration and proliferation. This study aimed to investigate the differences in wound healing rate and completeness of recovery of perianal wounds topically treated with HA-soaked cotton in a murine model. @*Methods@#Forty-eight 8-week-old Sprague-Dawley rats with perianal wounds created using a biopsy punch were divided into 2 groups: simple dressing with gauze (control) and topical HA-soaked cotton. A single application of HA-soaked cotton was administered after surgery. Wound healing rate and completeness of recovery were evaluated by measuring the healed area and conducting histological analyses. @*Results@#The HA-cotton group exhibited a shorter complete wound healing duration compared to the control group (13.9 days vs. 16.4 days, P = 0.031). Differences in wound healing area between the 2 groups were greatest on postoperative day 2 (51.6% vs. 28.8%, P < 0.001). The HA-cotton group exhibited fewer cases of granulation tissue (2 vs. 5) or redness (0 vs.3) upon complete wound healing. Histologically, the HA-cotton group showed accelerated reepithelialization, rapid shift to lymphocyte-dominant inflammation, enhanced fibroblast proliferation, and increased collagen deposition compared to the control group. @*Conclusion@#Herein, topical application of HA-soaked cotton on perianal wounds in rats resulted in accelerated wound healing, particularly in the initial stages, and improved completeness of recovery, underscoring the potential of the topical application of HA-soaked cotton on hemorrhoidectomy wounds in human patients to improve wound healing.
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Purpose@#Managing recurrent inguinal hernias is complex, and choosing the right surgical approach (laparoscopic vs. open) is vital for patient outcomes. This study compared the outcomes of using the same vs. different surgical approaches for initial and subsequent hernia repairs. @*Methods@#We retrospectively analyzed patients who underwent recurrent inguinal hernia repair at Seoul National University Bundang Hospital between January 2014 and May 2023. Patients were divided into the “concordant” and “discordant” groups, comprising patients who underwent same and different approaches in both surgeries, respectively. Preoperative baseline characteristics, index surgery data, postoperative outcomes, and recurrence rates were analyzed and compared. @*Results@#In total, 131 patients were enrolled; the concordant and discordant groups comprised 31 (open, n = 19; laparoscopic, n = 12) and 100 patients (open to laparoscopic, n = 68; laparoscopic to open, n = 32), respectively. No significant differences were observed in the mean operation time (50.5 ± 21.7 minutes vs. 50.2 ± 20.0 minutes, P = 0.979), complication rates (6.5% vs. 14.0%, P = 0.356), or 36-month cumulative recurrence rates (9.8% vs. 9.8%; P = 0.865). The mean postoperative hospital stay was significantly shorter in the discordant than in the concordant group (1.8 ± 0.7 vs. 1.4 ± 0.6, P = 0.003). @*Conclusion@#Most recurrent inguinal hernia repairs were performed using the discordant surgical approach. Overall, concordance in the surgical approach did not significantly affect postoperative outcomes. Therefore, the selection of the surgical approach based on the patient’s condition and surgeon’s preference may be advisable.
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Background@#The guidelines of coronavirus disease 2019 (COVID-19) vaccination in patients with rheumatoid arthritis (RA) have been continuously updated, with extensive discussion on the effectiveness of the COVID-19 booster vaccines and antibody generation associated with the different types of vaccine. We investigated the effects of the third dose of the mRNA vaccine on antibody titer and the factors associated with antibody production in patients with RA who had previously received two doses of the ChAdOx1-S nCoV-19 vaccine. @*Methods@#Between October 14, 2021 and June 17, 2022, two patient groups diagnosed with RA were recruited prospectively: one with two doses of ChAdOx1-S nCoV-19 and the second group with the additional third mRNA vaccine. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antibody titers were determined through semiquantitative anti-SARS-CoV-2 spike (S) electrochemiluminescence immunoassay. Antibody titers were compared in both groups considering clinical features and medications. Multivariate logistic regression was performed to identify the factors associated with antibody production. Also, we followed up the antibody titers of whom completed the 3rd mRNA vaccination. @*Results@#Among 261 patients, all patients were over 60 years old except for 7 patients and the average age was 65 years; 153 had completed two doses of ChAdOx1-S nCoV-19, while 108 patients had also received the third mRNA vaccine. The positive rates of anti-SARS-CoV-2 anti-S1/receptor binding domain-specific antibody (titer > 0.8 U/mL) were 97% (149/153) and 99% (107/108) respectively. However, positive rates for high antibody titer (> 250 U/ mL) were found in only 31% (47/153) of group 1 but 94% (102/108) of group 2. Multivariate analysis revealed that corticosteroid use (odds ratio [OR], 0.35; 95% confidence interval [CI], 0.16–0.75), older age (OR, 0.91; 95% CI, 0.860–0.98), and male sex (OR, 0.23; 95% CI, 0.07–0.74) were associated with a lower rate of high antibody titer acquisition after two doses of ChAdOx1-S nCoV-19. Waning of antibody titers was observed in only two of 46 patients who followed up twice after the third mRNA vaccine inoculation. @*Conclusion@#Our findings suggest that the third dose of the mRNA vaccine could be beneficial in RA patients with risk factors including older age, male sex, and corticosteroid use after two doses of ChAdOx1-S nCoV-19.
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Background@#This study aimed to analyze pregnancy outcomes based on biologic agents use in women using the nationwide population-based database. @*Methods@#The study used the claims database to identify women of childbearing age with several rheumatic (rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, psoriatic arthritis) and inflammatory bowel diseases (Crohn’s disease and ulcerative colitis) who had pregnancy-related codes between January 2010 and December 2019. We analyzed live births and adverse pregnancy outcomes based on the previous use of biologics. We also stratified the patients according to duration of biologic agent exposure before pregnancy and the use of biologics during pregnancy to analyze the pregnancy outcomes by subgroups. @*Results@#We identified 4,787 patients with pregnancy events. Among them, 1,034 (21.6%) used biologics before pregnancy. Live birth rate was not different between the biologics group and biologics naïve group (75.0% vs. 75.2%). Multivariate analyses showed that biologics use was associated with higher risk of intrauterine growth retardation (odds ratio [OR], 1.780) and lower risk of gestational diabetes mellitus (OR, 0.776) compared with biologics naïve. Biologics use during pregnancy was associated with higher risk of preterm delivery (OR, 1.859), preeclampsia/eclampsia (OR, 1.762), intrauterine growth retardation (OR, 3.487), and cesarean section (OR, 1.831), but lower risk of fetal loss (OR, 0.274) compared with biologics naïve. @*Conclusions@#Although there was no difference in live birth rate between the biologics group and biologics naïve group, biologics use seems to be associated with several adverse pregnancy outcomes, especially in patients with biologics during pregnancy. Therefore, patients with biologics during pregnancy need to be carefully observed for adverse pregnancy outcomes.
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Objective@#Electroconvulsive seizure (ECS) is a potent treatment modality for various neuropsychiatric diseases, including Parkinson disease (PD). Recent animal studies showed that repeated ECS activates autophagy signaling, the impairment of which is known to be involved in PD. However, the effectiveness of ECS on PD and its therapeutic mechanisms have not yet been investigated in detail. @*Methods@#Systemic injection of a neurotoxin 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine hydrochloride (MPTP), which destroys dopaminergic neurons in the substantia nigra compacta (SNc), in mice was utilized to induce an animal model of PD. Mice were treated with ECS 3 times per week for 2 weeks. Behavioral changes were measured with a rotarod test. Molecular changes related to autophagy signaling in midbrain including SNc, striatum, and prefrontal cortex were analyzed with immunohistochemistry and immunoblot analyses. @*Results@#Repeated ECS treatments normalized the motor deficits and the loss of dopamiergic neurons in SNc of the MPTP PD mouse model. In the mouse model, LC3-II, an autophagy marker, was increased in midbrain while decreased in prefrontal cortex, both of which were reversed by repeated ECS treatments. In the prefrontal cortex, ECS-induced LC3-II increase was accompanied with AMP-activated protein kinase (AMPK)-Unc-51-like kinase 1-Beclin1 pathway activation and inhibition of mamalian target of rapamycin signaling which promotes autophagy initiation. @*Conclusion@#The findings revealed the therapeutic effects of repeated ECS treatments on PD, which could be attributed to the neuroprotective effect of ECS mediated by AMPK-autophagy signaling.
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Background/Aims@#The recent coronavirus disease 2019 (COVID-19) pandemic has been associated with changes in the epidemiology of not only infectious diseases but also several non-infectious conditions. This study investigated changes in the recorded incidence of various rheumatic diseases during the COVID-19 pandemic. @*Methods@#The number of patients for each disease from January 2016 to December 2020 was obtained from the Korean Health Insurance Review and Assessment Service database. We compared the incidence of nine rheumatic diseases (seropositive rheumatoid arthritis, systemic lupus erythematosus [SLE], idiopathic inflammatory myositis [IIM], ankylosing spondylitis [AS], systemic sclerosis, Sjögren’s syndrome, Behçet’s disease [BD], polymyalgia rheumatica, and gout) and hypertensive diseases to control for changes in healthcare utilisation before and after the COVID-19 outbreak. The disease incidence before and after the COVID-19 outbreak was compared using the autoregressive integrated moving average (ARIMA) and quasi- Poisson analyses. @*Results@#Compared with the predicted incidence in 2020 using the ARIMA model, the monthly incidence of SLE, BD, AS, and gout temporarily significantly decreased, whereas other rheumatic diseases and hypertensive diseases were within the 95% confidence interval (CI) of the predicted values in the first half of 2020. In age- and sex-adjusted quasi-Poisson regression analysis, the annual incidences of IIM (rate ratio [RR], 0.473; 95% CI, 0.307 to 0.697), SLE (RR, 0.845; 95% CI, 0.798 to 0.895), and BD (RR, 0.850; 95% CI, 0.796 to 0.906) were significantly decreased compared with those in the previous 4 years. @*Conclusions@#The recorded annual incidence of some rheumatic diseases, including IIM, SLE, and BD, decreased during the COVID-19 pandemic.
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Background and Objectives@#We evaluated the effect of diabetes on the relationship between body mass index (BMI) and clinical outcomes in patients following percutaneous coronary intervention (PCI) with drug-eluting stent implantation. @*Methods@#A total of 6,688 patients who underwent PCI were selected from five different registries led by Korean Multicenter Angioplasty Team. They were categorized according to their BMI into the following groups: underweight (<18.5 kg/m 2 ), normal weight (18.5–24.9 kg/m 2 ), overweight to obese (≥25.0 kg/m 2 ). Major adverse cardiac and cerebrovascular events (MACCE), defined as a composite of death, nonfatal myocardial infarction, stroke, and target-vessel revascularization, were compared according to the BMI categories (underweight, normal and overweight to obese group) and diabetic status. All subjects completed 1-year follow-up. @*Results@#Among the 6,688 patients, 2,561 (38%) had diabetes. The underweight group compared to normal weight group had higher 1-year MACCE rate in both non-diabetic (adjusted hazard ratio [HR], 2.24; 95% confidence interval [CI], 1.04–4.84; p=0.039) and diabetic patients (adjusted HR, 2.86; 95% CI, 1.61–5.07; p<0.001). The overweight to obese group had a lower MACCE rate than the normal weight group in diabetic patients (adjusted HR, 0.67 [0.49–0.93]) but not in non-diabetic patients (adjusted HR, 1.06 [0.77–1.46]), with a significant interaction (p-interaction=0.025). @*Conclusions@#Between the underweight and normal weight groups, the association between the BMI and clinical outcomes was consistent regardless of the presence of diabetes.However, better outcomes in overweight to obese over normal weight were observed only in diabetic patients. These results suggest that the association between BMI and clinical outcomes may differ according to the diabetic status.
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Objectives@#During the initial clozapine titration, it is crucial to monitor for inflammatory reactions to ensure safe and effective administration. Clozapine metabolism varies by ancestry, particularly among Asians, warranting lower dosage. Recently, Dr. De Leon introduced guidelines based on ancestral differences. We aimed to provide a Korean translation, focusing on illustrating the necessity through clinical cases. @*Methods@#The Korean translation of the guidelines, approved by Dr. De Leon, involved two psychiatrists who reviewed and revised each other’s work. An additional board-certified psychiatrist conducted an independent review. We examined two clinical cases from our institution’s database, where clozapine titration faced challenges due to fever and pneumonia, assessing guideline applicability. @*Results@#The guidelines recommend a target clozapine dose of 175-300 mg/day for Asians with average metabolism and a slower titration rate compared to other ancestries. In both cases, CRP elevation was detected either before or simultaneously with the onset of fever, with a concentration-to-dose ratio ranging from 3.06 to 6.97. @*Conclusion@#The initial clozapine titration process should consider metabolic differences by ancestry and monitor for inflammation. Further research is needed to optimize the titration process for Koreans, considering metabolic rates, usage patterns, and side effects.
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Objective@#Carotid artery stenting (CAS) is currently widely used for the treatment of carotid artery stenosis. The objective of this study was to analyze the outcomes of CAS performed in a single institution. @*Methods@#We retrospectively analyzed 313 CAS cases from January 2007 to December 2020, including 206 (66%) symptomatic and 107 (34%) asymptomatic cases. Procedure-related morbidity and mortality were assessed. Rates of periprocedural (≤30 days after CAS) and postprocedural ipsilateral strokes (>30 days after CAS) were also assessed. Logistic regression analysis was used to identify risk factors for the periprocedural complication, in-stent restenosis (ISR), and ipsilateral stroke. @*Results@#The success rate of CAS was 98%. Among 313 cases, 1 patient died due to hyperperfusion-related intracerebral hemorrhage (ICH). The CAS-related mortality rate was 0.31%. The overall incidence of periprocedural complications is 5.1%. A risk factor for periprocedural complication was a symptomatic carotid artery stenosis (7.3% vs. 0.9%, p=0.016). Twenty cases of ISR occurred during 63.7±42.1 months of follow-up. The overall incidence of ISR was 10.2% (20/196). A risk factors for ISR were diabetes mellitus (17.6% vs. 5.7%, p=0.008) and patients who used Open-cell stents (19.6% vs. 6.9%, p=0.010). The overall incidence of ipsilateral stroke is 5.6%. A risk factors for ipsilateral stroke was ISR (95% CI, p=0.002). @*Conclusions@#CAS is a safe and effective procedure for carotid artery stenosis. Although the incidence of complications is low, fatal complication such as hyperperfusion- related ICH can occur. To prevent hyperperfusion-related ICH, several methods such as strict blood pressure (BP) control, intentional less widening of stenotic segment should be used. To prevent ISR or stroke occurrence, special attention should be paid to patients who have ISR or ipsilateral stroke risk factors.
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Objective@#Renal involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can lead to severe renal dysfunction requiring dialysis at diagnosis. We aimed to study the clinical and pathologic characteristics of patients with AAV dependent on dialysis at presentation and the long-term renal outcomes of patients who recovered from dialysis. @*Methods@#This retrospective study analyzed data of patients diagnosed with AAV who were on dialysis from July 2005 to May 2021 at a single tertiary center in Korea. @*Results@#Thirty-four patients were included in the study (median age: 64.5 years, females: 61.8%), of which 13 discontinued and 21 continued dialysis. The proportion of normal glomeruli (p<0.001) and interstitial fibrosis (p=0.024) showed significant differences between both groups. Multivariable analysis showed that the proportion of normal glomeruli was associated with dialysis discontinuation (odds ratio=1.29, 95% confidence interval 0.99~1.68, p=0.063), although without statistical significance. Treatment modalities, including plasmapheresis, did not show significance with dialysis discontinuation. In the follow-up analysis of 13 patients who had discontinued dialysis for a median of 81 months, 12 did not require dialysis, and their glomerular filtration rate values significantly increased at follow-up time compared to when they stopped dialysis (37.5 [28.5~45.5] vs. 24.0 [18.5~30.0] mL/ min/1.73 m²; p=0.008). @*Conclusion@#Approximately 38% of AAV patients on dialysis discontinued dialysis, and the recovered patients had improved renal function without dialysis during longer follow-up. Patients with AAV on dialysis should be given the possibility of dialysis discontinuation and renal recovery, especially those with normal glomeruli in kidney pathology.
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Lesch–Nyhan syndrome (LNS) is a rare X-linked recessive disorder caused by a mutation in the hypoxanthine phosphoribosyltransferase 1 (HPRT1) gene. This syndrome is characterized by excessive production of uric acid, mental retardation, self-mutilation, choreoathetosis, and spasticity. The most distinctive symptom is compulsive self-mutilation. For patients with LNS, different methods have been tried to reduce self-biting behaviors including restraints, behavioral treatment, medications, deep brain stimulation, tooth extraction and botulinum toxin A injection. In this report, we present a case of LNS undergoing cheiloplasty due to self-mutilation and tooth extraction of the left deciduous maxillary canine.
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Objective@#Dysphagia is a common clinical condition characterized by difficulty in swallowing. It is sub-classified into oropharyngeal dysphagia, which refers to problems in the mouth and pharynx, and esophageal dysphagia, which refers to problems in the esophageal body and esophagogastric junction. Dysphagia can have a significant negative impact one’s physical health and quality of life as its severity increases. Therefore, proper assessment and management of dysphagia are critical for improving swallowing function and preventing complications. Thus a guideline was developed to provide evidence-based recommendations for assessment and management in patients with dysphagia. @*Methods@#Nineteen key questions on dysphagia were developed. These questions dealt with various aspects of problems related to dysphagia, including assessment, management, and complications. A literature search for relevant articles was conducted using Pubmed, Embase, the Cochrane Library, and one domestic database of KoreaMed, until April 2021. The level of evidence and recommendation grade were established according to the Grading of Recommendation Assessment, Development and Evaluation methodology. @*Results@#Early screening and assessment of videofluoroscopic swallowing were recommended for assessing the presence of dysphagia. Therapeutic methods, such as tongue and pharyngeal muscle strengthening exercises and neuromuscular electrical stimulation with swallowing therapy, were effective in improving swallowing function and quality of life in patients with dysphagia. Nutritional intervention and an oral care program were also recommended. @*Conclusion@#This guideline presents recommendations for the assessment and management of patients with oropharyngeal dysphagia, including rehabilitative strategies.