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Andersen-Tawil syndrome (ATS) is a rare genetic disease characterized by a triad of episodic flaccid muscle weakness, ventricular arrhythmias, and physical anomalies. ATS patients have various cardiac arrhythmias that can cause sudden death. Implantation of an implantable cardioverter-defibrillator (ICD) is required when life-threatening cardiac arrhythmias do not respond to medical treatment. An 11-year-old girl underwent surgery for an ICD implantation. For general anesthesia in ATS patients, anesthesiologists should focus on the potentially difficult airway, serious cardiac arrhythmias, such as ventricular tachycardia (VT), and delayed recovery from neuromuscular blockade. We followed the difficult airway algorithm, avoided drugs that can precipitate QT prolongation and fatal cardiac arrhythmias, and tried to maintain normoxia, normocarbia, normothermia, normoglycemia, and pain control for prevention of sympathetic stimulation. We report the successful application of general anesthesia for ICD implantation in a pediatric patient with ATS and recurrent VT.
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Osteoporosis and osteoporotic fractures cause socioeconomic concerns, and medical system and policies appear insufficient to prepare for these issues in Korea, where the older adult population is rapidly increasing. Many countries around the world are already responding to osteoporosis and osteoporotic fractures by adopting fracture liaison service (FLS), and such an attempt has only begun in Korea. In this article, we introduce the operation methods for institutions implementing FLS and characteristics of services, and activities of the FLS Committee for FLS implementation in the Korean Society for Bone and Mineral Research. In addition, we hope that the current position statement will contribute to the implementation of FLS in Korea and impel policy changes to enable a multidisciplinary and integrated FLS operated under the medical system.
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Background/Aims@#The fecal microbiota of Korean patients with inflammatory bowel disease (IBD) was investigated with respect to disease phenotypes and taxonomic biomarkers for diagnosis and prognosis of IBD. @*Methods@#Fecal samples from 70 ulcerative colitis (UC) patients, 39 Crohn’s disease (CD) patients, and 100 healthy control individuals (HC) were collected. The fecal samples were amplified via polymerase chain reaction and sequenced using Illumina MiSeq. The relationships between fecal bacteria and clinical phenotypes were analyzed using the EzBioCloud database and 16S microbiome pipeline. @*Results@#The alpha-diversity of fecal bacteria was significantly lower in UC and CD (P<0.05) compared to that in HC. Bacterial community compositions in UC and CD were significantly different from that of HC according to Bray-Curtis dissimilarities, and there was also a difference between community composition in UC and CD (P=0.01). In UC, alpha-diversity was further decreased when the disease was more severe and the extent of disease was greater, and community composition significantly differed depending on the extent of the disease. We identified 9 biomarkers of severity and 6 biomarkers of the extent of UC. We also identified 5 biomarkers of active disease and 3 biomarkers of ileocolonic involvement in CD. Lachnospiraceae and Ruminococcus gnavus were biomarkers for better prognosis in CD. @*Conclusions@#The fecal microbiota profiles of IBD patients were different from those of HC, and several bacterial taxa may be used as biomarkers to determine disease phenotypes and prognosis. These data may also help discover new therapeutic targets for IBD.
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Colonoscopic polypectomy is effective in decreasing the incidence and mortality of colorectal cancer (CRC). Premalignant polyps discovered during colonoscopy are associated with the risk of metachronous advanced neoplasia. Postpolypectomy surveillance is the most important method for managing advanced metachronous neoplasia. A more efficient and evidence-based guideline for postpolypectomy surveillance is required because of the limited medical resources and concerns regarding colonoscopy complications. In these consensus guidelines, an analytic approach was used to address all reliable evidence to interpret the predictors of CRC or advanced neoplasia during surveillance colonoscopy. The key recommendations state that the high-risk findings for metachronous CRC following polypectomy are as follows: adenoma ≥10 mm in size; 3 to 5 (or more) adenomas; tubulovillous or villous adenoma; adenoma containing high-grade dysplasia; traditional serrated adenoma; sessile serrated lesion containing any grade of dysplasia; serrated polyp of at least 10 mm in size; and 3 to 5 (or more) sessile serrated lesions. More studies are needed to fully comprehend the patients who are most likely to benefit from surveillance colonoscopy and the ideal surveillance interval to prevent metachronous CRC.
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Background/Aims@#Efficacy of proton pump inhibitors is limited in patients with nonerosive reflux disease (NERD). The aim of this study was to comparatively evaluate the efficacy and safety of esomeprazole with sodium bicarbonate and esomeprazole alone. @*Methods@#This was a multicenter, randomized, double-blind, active-controlled, noninferiority comparative study. A total of 379 patients with NERD were randomly allocated to receive either EsoduoⓇ/sup> (esomeprazole 20 mg with sodium bicarbonate 800 mg) or NexiumⓇ/sup> (esomeprazole 20 mg) once daily for 4 weeks from January 2019 to December 2019. The patients had a history of heartburn for at least 2 days in the week before randomization as well as in the last 3 months and no esophageal mucosal breaks on endoscopy. The primary endpoint was a complete cure of heartburn at week 4. The secondary and exploratory endpoints as well as the safety profiles were compared in the groups at weeks 2 and 4. @*Results@#A total of 355 patients completed the study (180 in the EsoduoⓇ/sup> group and 175 in the NexiumⓇ/sup> group). The proportions of patients without heartburn in the entire 4th week of treatment were not different between the two groups (33.33% in the EsoduoⓇ/sup> group and 35% in the NexiumⓇ/sup> group, p=0.737). There were no significant differences in most of the secondary and exploratory endpoints as well as the safety profiles. @*Conclusions@#EsoduoⓇ/sup> is as effective and safe as NexiumⓇ/sup> for managing typical symptoms in patients with NERD (ClinicalTrial.gov identifier: NCT03928470).
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Background/Aims@#We investigated the factors related to spleen length and the diagnostic accuracy of a model using spleen length corrected by related factors, for the prediction of varices needing treatment (VNT). @*Methods@#Various prediction models for VNT including spleen length were analyzed in the cohort of compensated advanced chronic liver disease (cACLD), defined as liver stiffness (LS) ≥10 kPa in a recent study. The associated factors for spleen length were identified in healthy subjects to improve the prediction of VNT. @*Results@#Among 1,218 cACLD patients, VNT was noted in 249 patients (20.4%). On multivariate analysis, longer spleen length, lower platelet count, and higher LS value were independent predictors for VNT (all p<0.001). In multivariate analysis of 1,041 healthy subjects, age (β=–0.027), sex (β=0.762), and body mass index (β=0.097) were found to be significant factors for spleen length (all p<0.001). Using the β values, the estimated spleen length was calculated. To improve the prediction of VNT, the ratio of measured and estimated spleen length was calculated. Based on binary regression analysis results, the LS value-spleen ratio to platelet score (LSRPS) was calcu-lated as follows: 0.027×LS value (kPa)+2.690×measured/estimated spleen ratio–0.011×platelet count (cells×10 9 /L)–4.215. The area under the receiver operating characteristic of the LSRPS for VNT was 0.820, which was significantly higher than 0.797 of LS value-spleen diameter to plateletratio score (LSPS) (p=0.006). @*Conclusions@#Spleen length is influenced by age, sex, and body mass index in the Asian population. The LSRPS using the measured/estimated spleen ratio had higher diagnostic accuracy than LSPS in predicting VNT in patients with cACLD.
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We compared the glycemic efficacy of treatment intensification between quadruple oral antidiabetic drug therapy and once-weekly glucagon-like peptide-1 receptor agonist (GLP-1RA)-based triple therapy in patients with poorly controlled type 2 diabetes mellitus refractory to triple oral therapy. For 24 weeks, changes in glycosylated hemoglobin (HbA1c) from baseline were compared between the two treatment groups. Of all 96 patients, 50 patients were treated with quadruple therapy, and 46 were treated with GLP-1RA therapy. Reductions in HbA1c for 24 weeks were comparable (in both, 1.1% reduction from baseline; P=0.59). Meanwhile, lower C-peptide level was associated with a lower glucose-lowering response of GLP-1RA therapy (R=0.3, P=0.04) but not with quadruple therapy (R=–0.13, P=0.40). HbA1c reduction by GLP-1RA therapy was inferior to that by quadruple therapy in the low C-peptide subgroup (mean, –0.1% vs. –1.3%; P=0.04). Treatment intensification by switching to quadruple oral therapy showed similar glucose-lowering efficacy to weekly GLP-1RA-based triple therapy. Meanwhile, the therapeutic response was affected by C-peptide levels in the GLP-1RA therapy group but not in the quadruple therapy group.
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Non-alcoholic fatty liver disease (NAFLD) is one of the most common liver diseases worldwide, with a global prevalence of approximately 30%. However, the prevalence of NAFLD has been variously reported depending on the comorbidities. The rising prevalence of obesity in both the adult and pediatric populations is projected to consequently continue increasing NAFLD prevalence. It is a major cause of chronic liver disease worldwide, including cirrhosis and hepatocellular carcinoma (HCC). NAFLD has a variety of clinical phenotypes and heterogeneity due to the complexity of pathogenesis and clinical conditions of its occurrence, resulting in various clinical prognoses. In this article, we briefly described the basic definition of NAFLD and classified the subtypes based on current knowledge in this field.
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Purpose@#Plasma circulating tumor DNA (ctDNA) could reflect the genetic alterations present in tumor tissues. However, there is little information about the clinical relevance of cell-free DNA genotyping in peripheral T-cell lymphoma (PTCL). @*Materials and Methods@#After targeted sequencing plasma cell-free DNA of patients with various subtypes of PTCL (n=94), we analyzed the mutation profiles of plasma ctDNA samples and their predictive value of dynamic ctDNA monitoring for treatment outcomes. @*Results@#Plasma ctDNA mutations were detected in 53 patients (56%, 53/94), and the detection rate of somatic mutations was highest in angioimmunoblastic T-cell lymphoma (24/31, 77%) and PTCL, not otherwise specified (18/29, 62.1%). Somatic mutations were detected in 51 of 66 genes that were sequenced, including the following top 10 ranked genes: RHOA, CREBBP, KMT2D, TP53, IDH2, ALK, MEF2B, SOCS1, CARD11, and KRAS. In the longitudinal assessment of ctDNA mutation, the difference in ctDNA mutation volume after treatment showed a significant correlation with disease relapse or progression. Thus, a ≥ 1.5-log decrease in genome equivalent (GE) between baseline and the end of treatment showed a significant association with better survival outcomes than a < 1.5-log decrease in GE. @*Conclusion@#Our results suggest the clinical relevance of plasma ctDNA analysis in patients with PTCL. However, our findings should be validated by a subsequent study with a larger study population and using a broader gene panel.
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Purpose@#This multicenter retrospective study aimed to investigate clinical, radiologic, and treatment-related factors affecting survival in patients with newly diagnosed diffuse intrinsic pontine glioma (DIPG) treated with radiotherapy. @*Materials and Methods@#Patients aged <30 years who underwent radiotherapy as an initial treatment for DIPG between 2000 and 2018 were included; patients who did not undergo magnetic resonance imaging at diagnosis and those with pathologically diagnosed grade I glioma were excluded. We examined medical records of 162 patients collected from 10 participating centers in Korea. The patients’ clinical, radiological, molecular, and histopathologic characteristics, and treatment responses were evaluated to identify the prognosticators for DIPG and estimate survival outcomes. @*Results@#The median follow-up period was 10.8 months (interquartile range, 7.5 to 18.1). The 1- and 2-year overall survival (OS) rates were 53.5% and 19.0%, respectively, with a median OS of 13.1 months. Long-term survival rate (≥ 2 years) was 16.7%, and median OS was 43.6 months. Age (< 10 years), poor performance status, treatment before 2010, and post-radiotherapy necrosis were independently associated with poor OS in multivariate analysis. In patients with increased post-radiotherapy necrosis, the median OS estimates were 13.3 months and 11.4 months with and without bevacizumab, respectively (p=0.138). @*Conclusion@#Therapeutic strategy for DIPG has remained unchanged over time, and the associated prognosis remains poor. Our findings suggest that appropriate efforts are needed to reduce the occurrence of post-radiotherapy necrosis. Further well-designed clinical trials are recommended to improve the poor prognosis observed in DIPG patients.
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Purpose@#Although osimertinib is the standard-of-care treatment of epidermal growth factor receptor (EGFR) T790M mutation–positive non–small cell lung cancer, real-world evidence on the efficacy of osimertinib is not enough to reflect the complexity of the entire course of treatment. Herein, we report on the use of osimertinib in patients with EGFR T790M mutation–positive non–small cell lung cancer who had previously received EGFR tyrosine kinase inhibitor (TKI) treatment in Korea. @*Materials and Methods@#Patients with confirmed EGFR T790M after disease progression of prior EGFR-TKI were enrolled and administered osimertinib 80 mg daily. The primary effectiveness outcome was progression-free survival, with time-to-treatment discontinuation, treatment and adverse effects leading to treatment discontinuation, and overall survival being the secondary endpoints. @*Results@#A total of 558 individuals were enrolled, and 55.2% had investigator-assessed responses. The median progression-free survival was 14.2 months (95% confidence interval [CI], 13.0 to 16.4), and the median time-to-treatment discontinuation was 15.0 months (95% CI, 14.1 to 15.9). The median overall survival was 36.7 months (95% CI, 30.9 to not reached). The benefit with osimertinib was consistent regardless of the age, sex, smoking history, and primary EGFR mutation subtype. However, hepatic metastases at the time of diagnosis, the presence of plasma EGFR T790M, and the shorter duration of prior EGFR-TKI treatment were poor predictors of osimertinib treatment. Ten patients (1.8%), including three with pneumonitis, had to discontinue osimertinib due to severe adverse effects. @*Conclusion@#Osimertinib demonstrated its clinical effectiveness and survival benefit for EGFR T790M mutation–positive in Korean patients with no new safety signals.
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The superficial temporal artery (STA), the terminal branch of the external carotid artery, is divided into the frontal (anterior) and parietal (posterior) branches. The frontal branch of the STA is located superficially on the anterior region of the scalp, making it especially susceptible to trauma. Here, we report a traumatic pseudoaneurysm of the STA in a 7-year-old boy who was injured in a minor car accident. A physical examination showed only a small bruise on the patient’s forehead, and all vital signs were stable at the emergency room of our medical center. A facial computed tomography scan showed no significant findings. However, the boy later re-visited the hospital with slight swelling on the right forehead, and an ultrasonography scan revealed a hematoma near the right temporal artery. The resected hematoma (approximately 2 cm) was diagnosed as a traumatic pseudoaneurysm. Awareness of the possibility of a traumatic pseudoaneurysm in the STA may prevent a circumspect diagnosis in the future.
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Background@#Proper nasal tip projection and rotation are essential for an aesthetically pleasing nose. However, East Asians usually have thicker skin and underdeveloped nasal cartilage compared to Caucasians. Thus, techniques such as the septal extension graft, columellar strut graft, and L-shaped alloplastic implants have been introduced for additional nasal tip support and projection. However, no consensus exists regarding the optimal method. @*Methods@#A hybrid columellar strut made of an osteochondral graft recombined with the nasal crest of the maxilla and conchal cartilage was devised. In a cadaveric study, changes due to an external bending force of the hybrid strut and costal cartilage in the same dimension were measured. In a comparative study, we divided 20 subjects into two groups according to the material used for the columellar strut graft: a hybrid columellar strut (experimental group) and a double-layered conchal columellar strut (control group). Preoperative, 1-month postoperative, and 1-year postoperative anthropometric measurements were compared between groups. @*Results@#In the cadaveric study, the hybrid columellar strut showed superior mechanical strength. In the comparative study, the average 1-year postoperative change of the columellar labial angle was significantly smaller in the experimental group (–0.7°±1.16° vs. 2.2°±1.32°, P=0.015). The hybrid columellar strut showed superior postoperative nasal tip stability. @*Conclusions@#We devised a new hybrid columellar strut and confirmed its superior mechanical firmness and stability. Considering the suggested indications and contraindications, the hybrid columellar strut graft can be a new modality for tip plasty in East Asian rhinoplasty that can provide sufficient long-term nasal tip stability.
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Skin avulsions are severe traumatic injuries, in which sections of skin and subcutaneous tissue are torn off from the body, and the surgical management and salvage of these injuries are quite challenging due to their high morbidity and mortality. The entire or partial loss of an avulsed flap is prone to occur. If this happens, scars can be particularly conspicuous, and additional surgery, such as skin grafts or local flaps or even composite grafts, might be required. A 24-year-old male patient presented to the emergency room with a severe traumatic avulsion injury on his nose. We used a combination of three therapies to minimize the loss of the distal portion of the avulsed flap: polydeoxyribonucleotide injection, continuous non-rebreather mask oxygen therapy, and chemical leeching. We achieved complete flap salvage of the avulsed wound, and the patient showed full satisfaction in both aesthetic and functional aspects. Although this report is confined to a single case of severe avulsed injury, we suggest this triple-combination therapy as a good combined modality for maximizing the salvage of an avulsed flap on the basis of this case and a literature review.
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Purpose@#Appendectomy is one of the most common surgeries in children. Although various radiological examinations are performed, they do not always reveal a definitive diagnosis of appendicitis. This study aimed to investigate the clinical course of equivocal appendicitis, identify the factors associated with appendectomy, and suggest appropriate management for these patients. @*Methods@#Patients younger than 19 years who visited Seoul National University Bundang Hospital with a differential diagnosis of appendicitis from January 2013 to December 2017 were included. All participants conducted ‘appendiceal CT’ with a scoring scale of 1–5. The higher the score, the higher the likelihood of a radiologic diagnosis of appendicitis. We defined the appendicitis CT score of 2–4 as equivocal appendicitis (n = 143). Medical records were reviewed retrospectively for demographics, further examination as abdominal ultrasonography, and appendectomy status (yes or no). The mean follow-up period was 15.6 ± 71 days. @*Results@#Equivocal appendicitis accounted for 16.7%. Additional ultrasonography test was performed in 24.5% (35 of 143). In total, 34 patients (23.8%) underwent appendectomy. Among the patients with appendiceal CT scores 2, 3, and 4, 4.9%, 50.0%, and 87.5% underwent appendectomy, respectively. Higher WBC count, higher appendicitis CT score, and readmission were significantly associated with appendectomy in patients with equivocal appendicitis. @*Conclusion@#Higher appendicitis CT score and WBC level were positively associated with appendectomy. Careful observation can be a treatment option in appendicitis CT score 2 or 3 groups. Appendectomy is the first-line treatment for patients with appendicitis score 4. Additional ultrasonography test is advisable to determine treatment modality for equivocal appendicitis.
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Purpose@#The aim of this study was to examine whether gonadotropin-releasing hormone (GnRH) agonist treatment is effective in preserving final height in patients with central precocious puberty (CPP) or early puberty (EP). @*Methods@#The medical records of 40 patients with CPP and 206 patients with EP who completed GnRH agonist treatment following diagnosis were analyzed retrospectively. Height and height standard deviation (height SDS) scores based on bone age (BA) were measured and calculated at baseline, after treatment completion, and at final follow-up to compare changes within and between groups. Predicted adult height (PAH) was estimated by the height corresponding to height SDS for BA in girls at 18 years 11 months of age based on the growth chart. @*Results@#PAH at baseline did not differ significantly between the CPP group (153.67±4.95) and the EP group (154.77±3.72). In the CPP group, PAH significantly increased at treatment completion (156.01±4.61) and at final follow-up (158.52±6.04) compared to baseline. In the EP group, PAH significantly increased at treatment completion (157.7±3.60) and at final follow-up (159.31±4.26) compared to baseline. The increase in PAH at all timepoints compared to baseline did not significantly differ between the CPP and EP groups. @*Conclusion@#Both CPP and EP groups had significantly greater PAH after treatment, with no difference in the amount of increase between groups. These results show that GnRH agonist treatment can help increase final height even in patients diagnosed with EP after the age of 8 years.
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Background/Aims@#For patients hospitalized with coronavirus disease 2019 (COVID-19) who require supplemental oxygen, the evidence of the optimal duration of corticosteroid is limited. This study aims to identify whether long-term use of corticosteroids is associated with decreased mortality. @*Methods@#Between February 10, 2020 and October 31, 2021, we analyzed consecutive hospitalized patients with COVID-19 with severe hypoxemia. The patients were divided into short-term (≤ 14 days) and long-term (> 14 days) corticosteroid users. The primary outcome was 60-day mortality. We performed propensity score (PS) analysis to mitigate the effect of confounders and conducted Kaplan-Meier curve analysis. @*Results@#There were 141 (52%) short-term users and 130 (48%) long-term corticosteroid users. The median age was 68 years and the median PaO2/FiO2 at admission was 158. Of the patients, 40.6% required high-flow nasal cannula, 48.3% required mechanical ventilation, and 11.1% required extracorporeal membrane oxygenation. The overall 60-day mortality rate was 23.2%, and that of patients with hospital-acquired pneumonia (HAP) was 22.9%. The Kaplan-Meier curve for 60- day survival in the PS-matched cohort showed that corticosteroid for > 14 days was associated with decreased mortality (p = 0.0033). There were no significant differences in bacteremia and HAP between the groups. An adjusted odds ratio for the risk of 60-day mortality in short-term users was 5.53 (95% confidence interval, 1.90–18.26; p = 0.003). @*Conclusions@#For patients with severe COVID-19, long-term use of corticosteroids was associated with decreased mortality, with no increase in nosocomial complications. Corticosteroid use for > 14 days can benefit patients with severe COVID-19.
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Although tooth wear is a normal process due to aging, severe tooth wear causes various complications such as increased tooth sensitivity, loss of tooth structure, and pulp complications. In the treatment of patients with excessive tooth wear, the evaluation of loss of vertical occlusal dimension should be prioritized. If it is necessary to increase the vertical dimension to secure the restoration space, it is important to establish a treatment plan with the comprehensive analysis and determine the minimum vertical dimension elevation. In this case, 66-year-old male patient with severe worn dentition wanted to restore masticatory function and improve esthetic restoration. In order to determine the appropriate vertical dimension of the patient, we evaluated oral examination, radiographic examination, and diagnostic cast examination, and performed rehabilitation with minimum vertical dimension elevation. As a result of observation for 8 months, the definitive prosthesis was completed with contact of all teeth in centric occlusion, and proper anterior/posterior guidance. Through the above process, satisfactory aesthetic and functional outcomes were obtained.
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Black larder beetles prefer to feed on decomposing animal bodies, particularly those that are dried. When conditions are dry and warm, these beetles often appear in large numbers on dried animal carcasses. In our case, the dried carcasses of several cats were found nearly skeletonized at a villa in Daegu Metropolitan City on July 13, 2022. There were very few empty pupae of Lucilia sericata (Meigen) in the villa, but many adult black larder beetles, larvae, and larval cast skin (exuviae) (Dermestes haemorrhoidalis Küster) belonging to the family of larder beetles (Dermestidae) were found. We estimated the minimum post-mortem interval to be 44.5 days using temperature data from the nearest meteorological observatory and reported animal carcass decomposition and Dermestidae developmental rates. Police investigation confirmed that the cats were alive at least 3 months ago. Consequently, the neglected cats could not have been deceased in the villa for more than 3 months. As a result, the estimate closely matched the statement from the suspect.
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Objective@#Acute renal infarction is a rare and easily misdiagnosed disease. Scarce research has been conducted on the predictive factors and prognosis of acute renal infarction due to its rarity. In this study, we analyzed the clinical and laboratory findings of a patient diagnosed with renal infarction to predict clinical outcomes. @*Methods@#In this retrospective clinical study, we collected and analyzed the medical records data of 61 acute renal infarction patients diagnosed in the emergency department (ED) of Konkuk University Hospital, Seoul, South Korea from January 2007 to December 2020. @*Results@#Lactate dehydrogenase (LDH; 93.3%, 28 of 30 patients) and D-dimer (77.8%, 28 of 36 patients) levels of the acute renal infarction patients were higher than the normal. Proteinuria was found in 26 of 47 patients. The only significant prognostic factor for the occurrence of acute kidney injury (AKI) in acute renal infarction is the ratio of the infarction volume to the total renal volume. Age, occurrence of AKI, and proteinuria were correlated with the progression of chronic kidney disease (CKD). @*Conclusion@#The ratio of the infarction volume to the total renal correlated with occurance of AKI. Age, AKI, and proteinuria were correlated with the progression of CKD.