Objective: This study evaluated the accuracy of the pre-hospital shock index multiplied by the AVPU scale (PSIAVPU) as a predictor of massive transfusion (MT) and traumatic coagulopathy. Methods: This research was a retrospective single-center study that included patients consecutively presenting to a trauma center between 2017 and 2020. The predictive value of the PSIAVPU for MT, in-hospital mortality, and traumatic coagulopathy was measured using the area under the curve (AUC) of the receiver operating characteristic curve. The AUC of the PSIAVPU was compared with the Reverse Shock Index multiplied by the Glasgow Coma Scale (rSIG) measured at the trauma center presentation. Results: One thousand seven hundred and ninety-two patients were included, of which 163 patients (9.09%) received MT and 195 patients (10.88%) died during their hospital stay. Traumatic coagulopathy was observed in 245 patients. The AUC values for the PSIAVPU in terms of predicting MT, hospital mortality, and traumatic coagulopathy were 0.755, 0.752, and 0.736, respectively. Conclusion In patients with trauma, the predictive power of the PSIAVPU was higher than that of the prehospital shock index and was comparable to that of the rSIG. The PSIAVPU is a useful indicator that can be used easily and quickly for trauma patients at the prehospital stage.

Purpose: For the treatment of locally advanced rectal cancer (LARC), research on primary lesions with mesorectal fascia (MRF) involvement is lacking. This study analyzed the clinical outcomes and efficacy of dose-escalated neoadjuvant concurrent chemoradiotherapy (NCRT) to patients with LARC involving MRF. Materials and Methods: We retrospectively reviewed 301 patients who were diagnosed with LARC involving MRF and underwent NCRT followed by total mesorectal excision (TME). Patients who received radiotherapy (RT) doses of ≤50.4 Gy were defined as the non-boost group, while ≥54.0 Gy as the boost group. Pathological tumor response and survival outcomes, including intrapelvic recurrence-free survival (IPRFS), distant metastases-free survival (DMFS) and overall survival (OS), were analyzed. Results: A total of 269 patients (89.4%) achieved a negative pathological circumferential resection margin and 104 (34.6%) had good pathological tumor regression grades. With a median follow-up of 32.4 months, IPRFS, DMFS, and OS rates at 5-years were 88.6%, 78.0%, and 91.2%, respectively. In the subgroup analysis by RT dose, the boost group included more advanced clinical stages of patients. For the non-boost group and boost group, 5-year IPRFS rates were 90.3% and 87.0% (p = 0.242), 5-year DMFS rates were 82.0% and 71.3% (p = 0.105), and 5-year OS rates were 93.0% and 80.6% (p = 0.439), respectively. Treatment related toxicity was comparable between the two groups (p = 0.211). Conclusion Although this retrospective study failed to confirm the efficacy of dose-escalated NCRT, favorable IPRFS and pathological complete response was achieved with NCRT followed by TME. Further studies combining patient customized RT dose with systemic therapies are needed.

Purpose: The purpose of this phase II trial was to evaluate whether the addition of simvastatin, a synthetic 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor, to preoperative chemoradiotherapy (CRT) with capecitabine confers a clinical benefit to patients with locally advanced rectal cancer (LARC). Materials and Methods: Patients with LARC (defined by clinical stage T3/4 and/or lymph node positivity) received preoperative radiation (45-50.4 Gy in 25-28 daily fractions) with concomitant capecitabine (825 mg/m2 twice per day) and simvastatin (80 mg, daily). Curative surgery was planned 4-8 weeks after completion of the CRT regimen. The primary endpoint was pathologic complete response (pCR). The secondary endpoints included sphincter-sparing surgery, R0 resection, disease-free survival, overall survival, the pattern of failure, and toxicity. Results: Between October 2014 and July 2017, 61 patients were enrolled; 53 patients completed CRT regimen and underwent total mesorectal excision. The pCR rate was 18.9% (n=10) by per-protocol analysis. Sphincter-sparing surgery was performed in 51 patients (96.2%). R0 resection was achieved in 51 patients (96.2%). One patient experienced grade 3 liver enzyme elevation. No patient experienced additional toxicity caused by simvastatin. Conclusion The combination of 80 mg simvastatin with CRT and capecitabine did not improve pCR in patients with LARC, although it did not increase toxicity.

PURPOSE: The purpose of this study was to investigate changes in retention and wear pattern of Locator® and ADD-TOC attachments on a digital milled bar by performing chewing simulation and repeated insertion/removal of prostheses in fully edentulous models. MATERIALS AND METHODS: Locator (Locator®; Zest Anchors Inc., Escondido, CA, USA) was selected as the control group and ADD-TOC (ADDTOC; PNUAdd Co., Ltd., Busan, Republic of Korea) as the experimental group. A CAD-CAM milled bar was mounted on a master model and 3 threaded holes for connecting a bar attachment was formed using a tap. Locator and ADD-TOC attachments were then attached to the milled bar. Simulated mastication and repeated insertion/removal were performed over 400,000 cyclic loadings and 1,080insertions/removals, respectively. Wear patterns on deformed attachment were investigated by field emission scanning electron microscopy. RESULTS: For the ADD-TOC attachments, chewing simulation and repeated insertion/removal resulted in a mean initial retentive force of 24.43 ± 4.89 N, which were significantly lower than that of the Locator attachment, 34.33 ± 8.25 N (P < .05). Amounts of retention loss relative to baseline for the Locator and ADD-TOC attachments were 21.74 ± 7.07 and 8.98 ± 5.76 N (P < .05). CONCLUSION CAD-CAM milled bar with the ADD-TOC attachment had a lower initial retentive force than the Locator attachment. However, the ADD-TOC attachment might be suitable for long-term use as it showed less deformation and had a higher retentive force after simulated mastication and insertion/removal repetitions.

Background: Pediatric alopecia areata (AA) can affect the quality of life (QoL) of patients and their family members. Research on the QoL and burden on family members in pediatric AA is limited. Objective: This nationwide multicenter questionnaire study described the QoL and burden of the family members of patients with pediatric AA. Methods: This nationwide multicenter questionnaire study enrolled AA patients between the ages of 5 and 18 years from March 1, 2017 to February 28, 2018. Enrolled patients and their parents completed the modified Children’s Dermatology Life Quality Index (CDLQI) and the modified Dermatitis Family Impact (mDFI). The disease severity was measured using the Severity of Alopecia Tool (SALT) survey scores. Results: A total of 268 patients with AA from 22 hospitals participated in this study. Our study found that the efficacy and satisfaction of previous treatments of AA decreased as the severity of the disease increased. The use of home-based therapies and traditional medicines increased with the increasing severity of the disease, but the efficacy felt by patients was limited. CDLQI and mDFI scores were higher in patients with extensive AA than those with mild to moderate AA. The economic and time burden of the family members also increased as the severity of the disease increased. Conclusion The severity of the AA is indirectly proportional to the QoL of patients and their family members and directly proportional to the burden. Physicians need to understand these characteristics of pediatric AA and provide appropriate intervention to patients and their family members.

Purpose: Nontuberculous mycobacteria (NTM) is ubiquitous in the environment, but NTM lung disease (NTM-LD) is uncommon. Since exposure to NTM is inevitable, patients who develop NTM-LD are likely to have specific susceptibility factors, such as primary ciliary dyskinesia (PCD). PCD is a genetically heterogeneous disorder of motile cilia and is characterized by chronic respiratory tract infection, organ laterality defect, and infertility. In this study, we performed whole exome sequencing (WES) and investigated the genetic characteristics of adult NTM patients with suspected PCD. Materials and Methods: WES was performed in 13 NTM-LD patients who were suspected of having PCD by clinical symptoms and/or ultrastructural ciliary defect observed by transmission electron microscopy. A total of 45 PCD-causing genes, 23 PCDcandidate genes, and 990 ciliome genes were analyzed. Results: Four patients were found to have biallelic loss-of-function (LoF) variants in the following PCD-causing genes: CCDC114, DNAH5, HYDIN, and NME5. In four other patients, only one LoF variant was identified, while the remaining five patients did not have any LoF variants. Conclusion At least 30.8% of NTM-LD patients who were suspected of having PCD had biallelic LoF variants, and an additional 30.8% of patients had one LoF variant. Therefore, PCD should be considered in patients with NTM-LD with symptoms or signs suspicious of PCD.

Purpose: The purpose of this study was to evaluate the retentive characteristics of the additional attachments used with implant bar attachment under repeated insertion/removal cycles. Materials and methods: The newly developed attachment and the commercially available attachment were investigated: ADD-Lock (AL), Locator blue (LB). Two fixtures were placed parallel to each other on the custom lower mounting, and patrix of each attachment was fixed to the fixture. Also, the matrix of each attachment was placed on the opposing upper mounting. A universal testing machine was used to measure the retentive force during initial, 100, 250, 500, 1000, 2000, and 2500 repeated insertion/removal cycles. Wear and deformation of the attachment s were observed by scanning electron microscopy (SEM).Mann-Whitney U test (α=.05) and wilcoxon signed-rank test (α=.05) were performed to compare retentive force between each group and before and after 2500 repeated insertion/removal cycles. Results: In terms of initial retentive force and retentive force after 2500 repeated insertion/removal cycles, the AL group (15.24 ± 1.46 N and 9.74 ± 1.16 N) showed significantly smaller values than the LB group (43.53 ± 12.39 N and 22.99 ± 4.77 N) (P <.05). Also, in the loss of retentive force, the AL group (5.50 ± 1.08 N, 36.08%) showed a smaller value than the LB group (20.54 ± 11.89 N, 47.19%) (P <.05). Based on SEM analysis, The AL group showed noticeable wear and deformation in the patrix and the LB group in the matrix. Conclusion Locator showed a higher initial retentive force than newly developed attachment, while the loss of retentive force was also higher. Both additional attachments are considered to have sufficient retentive force after repeated insertion/removal cycles.

Purpose: The purpose of this study was to evaluate the retentive characteristics of the additional attachments used with implant bar attachment under repeated insertion/removal cycles. Materials and methods: The newly developed attachment and the commercially available attachment were investigated: ADD-Lock (AL), Locator blue (LB). Two fixtures were placed parallel to each other on the custom lower mounting, and patrix of each attachment was fixed to the fixture. Also, the matrix of each attachment was placed on the opposing upper mounting. A universal testing machine was used to measure the retentive force during initial, 100, 250, 500, 1000, 2000, and 2500 repeated insertion/removal cycles. Wear and deformation of the attachment s were observed by scanning electron microscopy (SEM).Mann-Whitney U test (α=.05) and wilcoxon signed-rank test (α=.05) were performed to compare retentive force between each group and before and after 2500 repeated insertion/removal cycles. Results: In terms of initial retentive force and retentive force after 2500 repeated insertion/removal cycles, the AL group (15.24 ± 1.46 N and 9.74 ± 1.16 N) showed significantly smaller values than the LB group (43.53 ± 12.39 N and 22.99 ± 4.77 N) (P <.05). Also, in the loss of retentive force, the AL group (5.50 ± 1.08 N, 36.08%) showed a smaller value than the LB group (20.54 ± 11.89 N, 47.19%) (P <.05). Based on SEM analysis, The AL group showed noticeable wear and deformation in the patrix and the LB group in the matrix. Conclusion Locator showed a higher initial retentive force than newly developed attachment, while the loss of retentive force was also higher. Both additional attachments are considered to have sufficient retentive force after repeated insertion/removal cycles.

PURPOSE: While there is an urgent need for diagnosis and therapeutic intervention in patients with primary immunodeficiency diseases (PIDs), current genetic tests have drawbacks. We retrospectively reviewed the usefulness of flow cytometry (FCM) as a quick tool for immunophenotyping and functional assays in patients suspected to have PIDs at a single tertiary care institute.METHODS: Between January 2001 and June 2018, patients suspected of having PIDs were subjected to FCM tests, including lymphocyte subset analysis, detection of surface- or intracellular-target proteins, and functional analysis of immune cells, at Samsung Medical Center, Seoul, Korea. The genetic diagnosis was performed using Sanger or diagnostic exome sequencing.RESULTS: Of 60 patients diagnosed with definite or probable PID according to the European Society of Immune Deficiencies criteria, 24 patients were provided with useful information about immunological dysfunction after initial FCM testing. In 10 patients, the PID diagnosis was based on abnormal findings in FCM testing without genetic tests. The FCM findings provided strong evidence for the diagnosis of severe combined immunodeficiency (n = 6), X-linked chronic granulomatous diseases (CGD) (n = 6), leukocyte adhesion deficiency type 1 (n = 3), X-linked agammaglobulinemia (n = 11), autoimmune lymphoproliferative syndrome-FASLG (n = 1), and familial hemophagocytic lymphohistiocytosis type 2 (n = 1), and probable evidence for autosomal recessive-CGD (n = 2), autosomal dominant-hyper-immunoglobulin E (IgE)-syndrome (n = 1), and STAT1 gain-of-function mutation (n = 1). In PIDs derived from PIK3CD (n = 2), LRBA (n = 2), and CTLA4 mutations (n = 3), the FCM test provided useful evidence of immune abnormalities and a tool for treatment monitoring.CONCLUSIONS: The initial application of FCM, particularly with known protein targets on immune cells, would facilitate the timely diagnosis of PIDs and thus would support clinical decisions and improve the clinical outcome.
Agammaglobulinemia ; Diagnosis ; Exome ; Flow Cytometry ; Genetic Testing ; Granulomatous Disease, Chronic ; Humans ; Immunophenotyping ; Korea ; Leukocytes ; Lymphocyte Subsets ; Lymphohistiocytosis, Hemophagocytic ; Phenotype ; Retrospective Studies ; Seoul ; Severe Combined Immunodeficiency ; Tertiary Healthcare