This study aimed to assess the accuracy of tooth widths, intermolar widths, and arch lengths acquired through two intraoral scanners, including iTero Element Plus Series (Align Technology, Santa Clara, CA, USA) and Trios 4 (3Shape, Copenhagen, Denmark), specifically on mixed dentition. A total of 30 subjects were divided into 2 groups, each undergoing both alginate impressions and intraoral scanning using either the iTero or Trios scanner. The plaster models were measured with a caliper, while the digital models were measured virtually. In the iTero group, all tooth width measurements exhibited differences compared to the plaster values, except for maxillary left lateral incisors (p = 0.179), mandibular right (p = 0.285), and left (p = 0.073) central incisors. The Trios group did not display significant differences in any of the tooth width measurements. Intermolar width comparisons for both groups indicated differences, except for mandibular primary canine to primary canine values (p = 0.426) in the iTero group. Regarding arch length, the mandibular anterior, maxillary right, and left arch lengths in the iTero group demonstrated larger caliper values than those of iTero. Conversely, in the Trios group, all parameters showed smaller caliper values, especially in upper anterior, maxillary right, mandibular right, and mandibular left arch lengths with significance (p = 0.027, 0.007, 0.003, and 0.047, respectively). Despite the differences between the two groups, digital models might be clinically suitable alternatives for plaster models. Pediatric dentists should carefully assess these differences, as a comprehensive evaluation would result in precise orthodontic treatment planning and favorable outcomes for young patients with mixed dentition.

Background: The risk of device thrombosis and device-oriented clinical outcomes with bioresorbable vascular scaffold (BVS) was reported to be significantly higher than with contemporary drug-eluting stents (DESs). However, optimal device implantation may improve clinical outcomes in patients receiving BVS. The current study evaluated mid-term safety and efficacy of Absorb BVS with meticulous device optimization under intravascular imaging guidance. Methods: The SMART-REWARD and PERSPECTIVE-PCI registries in Korea prospectively enrolled 390 patients with BVS and 675 patients with DES, respectively. The primary endpoint was target vessel failure (TVF) at 2 years and the secondary major endpoint was patientoriented composite outcome (POCO) at 2 years. Results: Patient-level pooled analysis evaluated 1,003 patients (377 patients with BVS and 626 patients with DES). Mean scaffold diameter per lesion was 3.24 ± 0.30 mm in BVS group.Most BVSs were implanted with pre-dilatation (90.9%), intravascular imaging guidance (74.9%), and post-dilatation (73.1%) at proximal to mid segment (81.9%) in target vessel.Patients treated with BVS showed comparable risks of 2-year TVF (2.9% vs. 3.7%, adjusted hazard ratio [HR], 1.283, 95% confidence interval [CI], 0.487–3.378, P = 0.615) and 2-year POCO (4.5% vs. 5.9%, adjusted HR, 1.413, 95% CI, 0.663–3.012,P = 0.370) than those with DES. The rate of 2-year definite or probable device thrombosis (0.3% vs. 0.5%, P = 0.424) was also similar. The sensitivity analyses consistently showed comparable risk of TVF and POCO between the 2 groups. Conclusion With meticulous device optimization under imaging guidance and avoidance of implantation in small vessels, BVS showed comparable risks of 2-year TVF and device thrombosis with DES.

Background and Objectives: The outcome benefits of β-blockers in chronic coronary artery disease (CAD) have not been fully assessed. We evaluated the prognostic impact of β-blockers on patients with chronic CAD after percutaneous coronary intervention (PCI). Methods: A total of 3,075 patients with chronic CAD were included from the Grand DrugEluting Stent registry. We analyzed β-blocker prescriptions, including doses and types, in each patient at 3-month intervals from discharge. After propensity score matching, 1,170 pairs of patients (β-blockers vs. no β-blockers) were derived. Primary outcome was defined as a composite endpoint of all-cause death and myocardial infarction (MI). We further analyzed the outcome benefits of different doses (low-, medium-, and high-dose) and types (conventional or vasodilating) of β-blockers. Results: During a median (interquartile range) follow-up of 3.1 (3.0–3.1) years, 134 (5.7%) patients experienced primary outcome. Overall, β-blockers demonstrated no significant benefit in primary outcome (hazard ratio [HR], 0.88; 95% confidence interval [CI], 0.63–1.24), all-cause death (HR, 0.87; 95% CI, 0.60–1.25), and MI (HR, 1.25; 95% CI, 0.49–3.15). In subgroup analysis, β-blockers were associated with a lower risk of all-cause death in patients with previous MI and/ or revascularization (HR, 0.38; 95% CI, 0.14–0.99) (p for interaction=0.045). No significant associations were found for the clinical outcomes with different doses and types of β-blockers. Conclusions Overall, β-blocker therapy was not associated with better clinical outcomes in patients with chronic CAD undergoing PCI. Limited mortality benefit of β-blockers may exist for patients with previous MI and/or revascularization.

Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome is a rare autosomal recessive multisystemic disease that is associated with the liver, kidney, skin, and central nervous and musculoskeletal systems. ARC occurs as a result of mutations in the VPS33B (Vacuolar protein sorting 33 homolog B) or VIPAR (VPS33B interacting protein, apical-basolateral polarity regulator) genes. A female infant presented with neonatal cholestasis with a severe clinical outcome. She was diagnosed with ARC syndrome using targeted exome sequencing (TES). Exome sequencing revealed compound heterozygous mutations, c.707A>T and c.239+5G>A, in VPS33B, where c.707A>T was a novel variant; the resultant functional protein defects were predicted via in silico analysis. c.239+5G>A, a pathogenic mutation that affects splicing, is found in less than 0.1% of the general population. Invasive techniques, such as liver biopsies, did not contribute to a differential diagnosis of ARC syndrome; thus, early TES together with clinical presentations constituted an apparently accurate diagnostic procedure.
Biopsy ; Cholestasis ; Computer Simulation ; Diagnosis, Differential ; Exome ; Female ; Humans ; Infant ; Kidney ; Liver ; Musculoskeletal System ; Protein Transport ; Skin

No abstract available.
Camurati-Engelmann Syndrome*

BACKGROUND: Camurati-Engelmann disease (CED) is a rare genetic skeletal disorder characterized by limb pain, muscle emaciation and weakness, and cortical thickening of the diaphysis of long bones. It is caused by mutations in the transforming growth factor beta 1 (TGFB1) (type I) or other unknown gene(s) (type II). We present 8 consecutive patients with type I CED. METHODS: We retrospectively reviewed medical records and radiographs of type I CED patients with special reference to the mode of presentation, process of diagnostic work-up, and disease course. They were 4 sporadic patients, and two pairs of mother and son. RESULTS: We categorized the mode of presentation into three groups. Group I had 4 patients who mainly presented with motor disturbances in young age. They drew medical attention for waddling gait, awkward ambulation or running, difficulty in going upstairs, or a positive Gower's sign at age 4 to 6 years. Subsequent development of limb pain and radiographic abnormality led to the diagnosis of CED at age 6 to 29 years. Group II had 3 patients who mainly presented with limb pain at age 15, 20, and 54 years, respectively. Radiographic evaluation and molecular genetic test led to the diagnosis of CED. The remaining 1 patient (group III) was asymptomatic until age 9 years when bony lesions at the tibiae were found incidentally. For the last 10 years, he intermittently complained of leg pain in the morning or after sports activities, which did not interfere with daily life. All the patients in group I showed a body mass index in the underweight range (< 18.4 kg/m²). At the latest follow-up, 4 patients in groups I and II required medication for the limb pain. CONCLUSIONS: CED presents with a wide range of severity. Awareness of this rare disease entity may be the key to timely correct diagnosis. This disease entity should be considered in the differential diagnosis of limb pain or motor disturbance in children to avoid unnecessary diagnostic work-up.
Body Mass Index ; Camurati-Engelmann Syndrome* ; Child ; Diagnosis ; Diagnosis, Differential ; Diaphyses ; Emaciation ; Extremities ; Follow-Up Studies ; Gait ; Humans ; Leg ; Medical Records ; Molecular Biology ; Mothers ; Myalgia ; Orthopedics* ; Phenotype ; Rare Diseases ; Retrospective Studies ; Running ; Sports ; Thinness ; Tibia ; Transforming Growth Factor beta ; Walking

PURPOSE: The purpose of this study was to investigate the feasibility and survival benefits of combined treatment with radiotherapy and adjuvant temozolomide (TMZ) in a Korean sample. MATERIALS AND METHODS: A total of 750 Korean patients with histologically confirmed glioblastoma multiforme, who received concurrent chemoradiotherapy with TMZ (CCRT) and adjuvant TMZ from January 2006 until June 2011, were analyzed retrospectively. RESULTS: After the first operation, a gross total resection (GTR), subtotal resection (STR), partial resection (PR), biopsy alone were achieved in 388 (51.7%), 159 (21.2%), 96 (12.8%), and 107 (14.3%) patients, respectively. The methylation status of O6-methylguanine-DNA methyltransferase (MGMT) was reviewed retrospectively in 217 patients. The median follow-up period was 16.3 months and the median overall survival (OS) was 17.5 months. The actuarial survival rates at the 1-, 3-, and 5-year OS were 72.1%, 21.0%, and 9.0%, respectively. The median progression-free survival (PFS) was 10.1 months, and the actuarial PFS at 1-, 3-, and 5-year PFS were 42.2%, 13.0%, and 7.8%, respectively. The patients who received GTR showed a significantly longer OS and PFS than those who received STR, PR, or biopsy alone, regardless of the methylation status of the MGMT promoter. Patients with a methylated MGMT promoter also showed a significantly longer OS and PFS than those with an unmethylated MGMT promoter. Patients who received more than six cycles of adjuvant TMZ had a longer OS and PFS than those who received six or fewer cycles. Hematologic toxicity of grade 3 or 4 was observed in 8.4% of patients during the CCRT period and in 10.2% during the adjuvant TMZ period. CONCLUSION: Patients treated with CCRT followed by adjuvant TMZ had more favorable survival rates and tolerable toxicity than those who did not undergo this treatment.
Biopsy ; Chemoradiotherapy* ; Disease-Free Survival ; Follow-Up Studies ; Glioblastoma* ; Humans ; Korea* ; Methylation ; Radiotherapy ; Retrospective Studies* ; Survival Rate

BACKGROUND/AIMS: Although polypoid leiomyomas in the colon and rectum are rare, they are increasingly detected during colonoscopy. The aim of this study was to evaluate the efficacy and clinical outcomes of endoscopic removal for colorectal polypoid leiomyoma. METHODS: Data were retrospectively collected from 22 patients with polypoid leiomyoma arising from the muscularis mucosae in the colon and rectum who underwent endoscopic removal at single referral gastrointestinal endoscopy unit. Colonoscopic findings, endoscopic removal, success rates, complication rates (bleeding or perforation), pathologic characteristics, and recurrence rates were investigated. RESULTS: Most polypoid leiomyomas were small asymptomatic lesions less than 1 cm. The tumors were located predominantly in the left colon. Ten leiomyomas were removed using cold biopsy forceps, and 12 were resected by conventional polypectomy or endoscopic mucosal resection. All tumors arose from or involved the muscularis mucosa. There were no complications, such as bleeding or perforation. No local remnant lesions were found in 19 patients who underwent at least one follow-up colonoscopy. CONCLUSIONS: This case series represent cases of small colorectal polypoid leiomyoma that were safely removed endoscopically. An awareness of their endoscopic and clinic-pathological characteristics may provide safe treatment strategy for colonic leiomyomatous tumors of similar size in capable hands.
Biopsy ; Colon ; Colonic Polyps ; Colonoscopy ; Colorectal Neoplasms ; Endoscopy, Gastrointestinal ; Follow-Up Studies ; Hand ; Hemorrhage ; Humans ; Leiomyoma* ; Mucous Membrane ; Rectum ; Recurrence ; Referral and Consultation ; Retrospective Studies ; Surgical Instruments

BACKGROUND: Weaning from mechanical ventilation is difficult in the intensive care unit (ICU). Many controversial questions remain unanswered concerning the predictors of weaning failure. This study investigates patient characteristics and delayed weaning after lung transplantation. METHODS: This study retrospectively reviewed the medical records of 17 lung transplantation patients from October 2012 to December 2013. Patients able to be weaned from mechanical ventilation within 8 days after surgery were assigned to an early group (n = 9), and the rest of the patients were assigned to the delayed group (n=8). Patients' intraoperative and postoperative characteristics were collected and analyzed, and conventional weaning predictors, including rapid shallow breathing index (RSBI), were also assessed. RESULTS: The results of the early group showed a significantly shorter ICU stay in addition to a shorter hospitalization overall. Notably, the early group had a higher body mass index (BMI) than the delayed group (20.7 vs. 16.9, p = 0.004). In addition, reopening occurred more frequently in the delayed group (1/9 vs. 5/8, p = 0.05). During spontaneous breathing trials, tidal volume (TV) and arterial oxygen tension were significantly higher in the early group compared to the delayed weaning group, but differences in RSBI and respiratory rate (RR) between groups were not statistically significant. CONCLUSIONS: Low BMI might be associated with delayed ventilator weaning in lung transplantation patients. In addition, instead of the traditional weaning predictors of RSBI and RR, TV might be a better predictor for ventilator weaning after lung transplantation.
Body Mass Index* ; Hospitalization ; Humans ; Intensive Care Units ; Lung Transplantation* ; Medical Records ; Oxygen ; Respiration ; Respiration, Artificial ; Respiratory Rate ; Retrospective Studies ; Tidal Volume ; Ventilator Weaning* ; Weaning

BACKGROUND: Weaning from mechanical ventilation is difficult in the intensive care unit (ICU). Many controversial questions remain unanswered concerning the predictors of weaning failure. This study investigates patient characteristics and delayed weaning after lung transplantation. METHODS: This study retrospectively reviewed the medical records of 17 lung transplantation patients from October 2012 to December 2013. Patients able to be weaned from mechanical ventilation within 8 days after surgery were assigned to an early group (n = 9), and the rest of the patients were assigned to the delayed group (n=8). Patients' intraoperative and postoperative characteristics were collected and analyzed, and conventional weaning predictors, including rapid shallow breathing index (RSBI), were also assessed. RESULTS: The results of the early group showed a significantly shorter ICU stay in addition to a shorter hospitalization overall. Notably, the early group had a higher body mass index (BMI) than the delayed group (20.7 vs. 16.9, p = 0.004). In addition, reopening occurred more frequently in the delayed group (1/9 vs. 5/8, p = 0.05). During spontaneous breathing trials, tidal volume (TV) and arterial oxygen tension were significantly higher in the early group compared to the delayed weaning group, but differences in RSBI and respiratory rate (RR) between groups were not statistically significant. CONCLUSIONS: Low BMI might be associated with delayed ventilator weaning in lung transplantation patients. In addition, instead of the traditional weaning predictors of RSBI and RR, TV might be a better predictor for ventilator weaning after lung transplantation.
Body Mass Index ; Hospitalization ; Humans ; Intensive Care Units ; Lung Transplantation ; Medical Records ; Oxygen ; Respiration ; Respiration, Artificial ; Respiratory Rate ; Retrospective Studies ; Tidal Volume ; Ventilator Weaning ; Weaning