Design and Implementation of Storage System Using Byte-index Chunking Scheme

In this paper, we present an enhanced storage system that supports Byte-index chunking algorithm. The storage system aims to provide efficient data deduplication with high performance and to be performed in rapid time. We describe the overall procedure of Byteindex chunking based storage system including read/write procedure and how the system works. The key idea of Byte-index chunking is to adapt fixed-size block chunk scheme which are distributed to “Index-table ” by chunk’s both side boundary values. We have found that Byte-index chunking in storage system provides high performance compared with other chunking schemes. Experiments result shows that the storage system with Byte-index chunking compresses overall data with high deduplication capability and reduce the speed of file processing

A 9-year-old Korean girl with Fontaine progeroid syndrome: a case report with further phenotypical delineation and description of clinical course during long-term follow-up

Background Gorlin–Chaudhry–Moss syndrome (GCMS) and Fontaine–Farriaux syndrome (FFS) are extremely rare genetic disorders that share similar clinical manifestations. Because a de novo missense mutation of the solute carrier family 25 member 24 (SLC25A24) gene was suggested to be the common genetic basis of both syndromes, it has been proposed recently that they be integrated into a single disorder under the name of Fontaine progeroid syndrome (FPS). Case presentation A 9-year-old Korean girl presented with typical clinical features of FPS. She had generalized loose skin with decreased subcutaneous fat, skin wrinkling on the forehead and limbs, skull deformities and a peculiar facial appearance with microphthalmia and midface hypoplasia, anomalies of the digits and nails, a large umbilical hernia and a nearly normal developmental outcome. She exhibited prenatal and postnatal growth retardation together with short stature, and records showed that her height and weight were invariably under − 2.0 SD from birth to the age of 10 years. SLC25A24 analysis revealed a heterozygous mutation reported previously, NM_013386:c.650G > A, p.[Arg217His]. After screening her family for the identified mutation, she was confirmed as being a de novo case of FPS caused by an SLC25A24 mutation. Conclusion We describe a Korean girl with typical clinical findings of FPS and a de novo mutation in SLC25A24, as well as 10 years of clinical follow-up, including growth and developmental achievements.This study was supported by grant no. 05–2018-0010 from the SNUH Research Fund. The funder had no role in study design, data collection and analysis, decision to publish or preparation of the manuscript

Elastography Can Effectively Decrease the Number of Fine-Needle Aspiration Biopsies in Patients with Calcified Thyroid Nodules

AbstractWhen calcification, frequently found in both benign and malignant nodules, is present in thyroid nodules, non-invasive differentiation with ultrasound becomes challenging. The goal of this study was to evaluate the utility of elastography in differentiating calcified thyroid nodules. Consecutive patients (165 patients with 196 nodules) referred for fine-needle aspiration who had undergone both ultrasound elastography and B-mode examinations were analyzed retrospectively. Calcification was present in 45 benign and 20 malignant nodules. On 65 calcified nodules, elastography had 95% sensitivity, 51.1% specificity, 46.3% positive predictive value and 95.8% negative predictive value in detecting malignancy. Twenty-three of 45 benign calcified nodules were correctly diagnosed with elastography compared with 4 of 45 by B-mode ultrasound. Although it is difficult to differentiate benign and malignant calcified thyroid nodules solely with B-mode ultrasound, elastography has the potential to reduce the number of fine-needle aspiration biopsies performed on calcified nodules

Relations of Pulse Wave Velocity to Waist Circumference Independent of Hip Circumference

- OBJECTIVES: Little is known about the effect of waist circumference (WC) on brachial artery pulse wave velocity (baPWV) independent of hip circumference (HC). Therefore, this study aimed to dissociate specific effect of WC on baPWV independent of HC. - METHODS: Of 1,053 rural residents (2004-2005), 777 subjects with no known history of coronary artery diseases or diabetes mellitus over 40 yr were included. To reduce collinearity, we assessed the independent effect of WC with HC on PWV by residual method (WC [RM]). - RESULTS: In women, most correlation coefficients were significant between measures of abdominal obesity and baPWV, with the highest (0.32) in waist to hip ratio (WHR), whereas no significance was found in men. All mean values of baPWV among the abdominally obese were higher than those of normal group in women, which were in the order of WHR, WC (RM), and WC. Adjusted OR with 95% CI for baPWV was significantly elevated by increase of WC (RM) upto 4.8 (95% CI: 2.1-11.2), and as 4.3 by WHR (95% CI: 1.6-11.4). - CONCLUSION: Considering the difficulty in biologically interpreting WHR, WC (RM) may be a useful indicator of abdominal obesity among females in that it reflects the risk of pulse wave velocity

Design of the VISTA-ITL Test Facility for an Integral Type Reactor of SMART and a Post-Test Simulation of a SBLOCA Test

To validate the performance and safety of an integral type reactor of SMART, a thermal-hydraulic integral effect test facility, VISTA-ITL, is introduced with a discussion of its scientific design characteristics. The VISTA-ITL was used extensively to assess the safety and performance of the SMART design, especially for its passive safety system such as a passive residual heat removal system, and to validate various thermal-hydraulic analysis codes. The VISTA-ITL program includes several tests on the SBLOCA, CLOF, and PRHRS performances to support a verification of the SMART design and contribute to the SMART design licensing by providing proper test data for validating the system analysis codes. A typical scenario of SBLOCA was analyzed using the MARS-KS code to assess the thermal-hydraulic similarity between the SMART design and the VISTA-ITL facility, and a posttest simulation on a SBLOCA test for the shutdown cooling system line break has been performed with the MARS-KS code to assess its simulation capability for the SBLOCA scenario of the SMART design. The SBLOCA scenario in the SMART design was well reproduced using the VISTA-ITL facility, and the measured thermal-hydraulic data were properly simulated with the MARS-KS code

The Effects of Glyburide on Apoptosis and Endoplasmic Reticulum Stress in INS-1 Cells in a Glucolipotoxic Condition

Backgroundβ-cell death due to endoplasmic reticulum (ER) stress has been regarded as an important pathogenic component of type 2 diabetes. The possibility has been suggested that sulfonylurea, currently being used as one of the main oral hypoglycemic agents of type 2 diabetes, increases ER stress, which could lead to sulfonylurea failure. The authors of the present study examined ER stress of β-cells in a glucolipotoxic condition using glyburide (GB) in an environment mimicking type 2 diabetes.MethodsApoptosis was induced by adding various concentrations of GB (0.001 to 200 µM) to a glucolipotoxic condition using 33 mM glucose, and the effects of varied concentrations of palmitate were evaluated via annexin V staining. The markers of ER stress and pro-apoptotic markers were assessed by Western blotting and semi-quantitative reverse transcription-polymerase chain reaction. Additionally, the anti-apoptotic markers were evaluated.ResultsAddition of any concentration of GB in 150 µM palmitate and 33 mM glucose did not increase apoptosis. The expression of phosphorylated eukaryotic initiation factor (eIF-2α) was increased and cleaved caspase 3 was decreased by adding GB to a glucolipotoxic condition. However, other ER stress-associated markers such as Bip-1, X-box binding protein-1, ATF-4 and C/EBP-homologous protein transcription factor and anti-apoptotic markers phosphor-p85 phosphatidylinositol 3-kinase and phosphorylation of Akt did not change significantly.ConclusionGB did not show further deleterious effects on the degree of apoptosis or ER stress of INS-1 cells in a glucolipotoxic condition. Increased phosphorylation of eIF-2α may attenuate ER stress for adaptation to increased ER protein load

Delta neutrophil index as a predictor of vesicoureteral reflux in children with febrile urinary tract infection

Purpose Delta neutrophil index (DNI) indicates immature granulocytes in peripheral blood and has been confirmed to be effective as a prognostic factor for neonatal sepsis. Also, it has been reported to have diagnostic value in acute pyelonephritis and in predicting vesicoureteral reflux (VUR) in the infant. We conducted the study to verify whether DNI is also helpful in the entire pediatric age group with febrile urinary tract infection (UTI). Methods Medical records of children hospitalized for febrile UTIs were analyzed retrospectively. All subjects underwent kidney ultrasound and voiding cystourethrography. In the group with and without VUR, we compared sex and age, and the following laboratory values: the white blood cell count, neutrophil, polymorphonuclear leucocyte, eosinophil, hemoglobin, platelet count, C-reactive protein, DNI value, and the finding of ultrasound. Results A total of 315 patients (163 males and 152 females; range, 0–127 months) were eligible, and 41 patients (13%) had VUR. As a result of univariate analysis, the white blood cell count, neutrophil, DNI, and ultrasonic abnormalities were high in the reflux group, and the hemoglobin and lymphocyte fraction values were low. The value of DNI and the abnormal ultrasound were significantly higher in the reflux group on the multivariate analysis. The area under the curve value of the receiver operating curve was higher in DNI (0.640; 95% confidence interval, 0.536–0.744; P=0.004), and the DNI cutoff value for VUR prediction was 1.85%. Conclusions We identified that ultrasound findings and DNI values were helpful predictors of VUR in pediatric febrile UTIs