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Purpose: Association between hyponatremia and the severity of respiratory symptoms in infants with respiratory syncytial virus (RSV) infection has not yet been studied. This study aimed to compare respiratory symptoms, assessed using the Pediatric Respiratory Score (PRS), in infants with RSV infection, with or without hyponatremia. Methods: RSV-positive patients aged <12 months who were admitted with respiratory symptoms within 7 days of onset at Jeonbuk National University Children's Hospital from January 2016 to December 2019 were retrospectively analyzed. Each patient was categorized into those with or without hyponatremia (serum sodium concentration of <136 mmol/L). Clinical findings included PRS on the day of admission. Results: The mean±standard deviation age of the 125 patients included in the study was 2.7±3.3 months, and, 20 patients (16.0%) showed hyponatremia. Infants with RSV infection and hyponatremia had lower birth weights, longer hospital stays, and higher blood urea nitrogen level. The C-reactive protein level was significantly higher in the hyponatremic infants, who had higher PRSs. The non-hyponatremia group had more normal PRSs than the hyponatremia group, which had more severe PRSs. After adjustment for age at admission, blood urea nitrogen level (OR, 1.218; 95% CI, 1.023 to 1.451; P<0.05), and PRS grade (OR, 2.885; 95% CI, 1.158 to 7.187; P< 0.05) were identified as independent risk factors. Conclusion: Hyponatremia was strongly associated with respiratory severity in infants with RSV. Therefore, infants admitted with RSV infection who show higher PRS grade need to be evaluated and treated for hyponatremia. [ABSTRACT FROM AUTHOR]

Abstract Preterm birth prediction is essential for improving neonatal outcomes. While many machine learning techniques have been applied to predict preterm birth using health records, inflammatory markers, and vaginal microbiome data, the role of prenatal oral microbiome remains unclear. This study aimed to compare oral microbiome compositions between a preterm and a full-term birth group, identify oral microbiome associated with preterm birth, and develop a preterm birth prediction model using machine learning of oral microbiome compositions. Participants included singleton pregnant women admitted to Jeonbuk National University Hospital between 2019 and 2021. Subjects were divided into a preterm and a full-term birth group based on pregnancy outcomes. Oral microbiome samples were collected using mouthwash within 24 h before delivery and 16S ribosomal RNA sequencing was performed to analyze taxonomy. Differentially abundant taxa were identified using DESeq2. A random forest classifier was applied to predict preterm birth based on the oral microbiome. A total of 59 women participated in this study, with 30 in the preterm birth group and 29 in the full-term birth group. There was no significant difference in maternal clinical characteristics between the preterm and the full-birth group. Twenty-five differentially abundant taxa were identified, including 22 full-term birth-enriched taxa and 3 preterm birth-enriched taxa. The random forest classifier achieved high balanced accuracies (0.765 ± 0.071) using the 9 most important taxa. Our study identified 25 differentially abundant taxa that could differentiate preterm and full-term birth groups. A preterm birth prediction model was developed using machine learning of oral microbiome compositions in mouthwash samples. Findings of this study suggest the potential of using oral microbiome for predicting preterm birth. Further multi-center and larger studies are required to validate our results before clinical applications.

Abstract Iron deficiency (ID) in very preterm infants born at 28–32 weeks of gestational age (GA) can lower mental and motor test scores. This study aimed to determine whether the rapid growth of very preterm infants might be associated with ID. Among 134 very preterm born between January 2014 and December 2020 at Jeonbuk National University Hospital and discharged home, 93 were included in this study. Rapid BMI increase (RBI) was defined as a z-score difference of > 1 standard deviation between birth and 8 months. ID occurred in 23 of 93 (24.7%) infants at 8 months of corrected age (CA). ID was more common in the RBI group (50%) than in the non-RBI group (18.7%). In the multivariate logistic regression corrected for GA, infants small for gestational age (SGA) (odds ratio [OR] 6.06, 95% confidence interval [CI] 1.34–30.21) and RBI by z-score (OR 4.26, 95% CI 1.28–14.65) were identified as independent risk factors for ID at 8 months of CA. Conclusively, both SGA and RBI in the early life of very preterm were risk factors for ID at 8 months of CA.

ABO and Rh incompatibility are the leading causes of hemolytic disease of the fetus and newborn (HFDN). Due to the high antigenicity of the D antigen, hemolysis and symptoms progress rapidly. However, minor blood group incompatibility manifests varying clinical symptoms, from asymptomatic cases to neonatal jaundice and fetal hydrops. This study presents a case of HFDN in which anti-C and anti-e antibodies were identified and treated with intensive phototherapy. A full-term infant weighing 3,100 g at birth, with no complications during delivery, presented with jaundice and was admitted for intensive phototherapy. Antibody testing detected anti-C and anti-e antibodies in the neonate and the mother. The patient responded well to phototherapy, and intravenous immunoglobulin was administered. The total bilirubin levels decreased, and the infant was discharged after 5 days. At the age of 12 months, the infant exhibited normal neurodevelopment. In conclusion, neonates with HFDN, due to rare minor blood incompatibility, specifically anti-C and anti-e antibodies, can mitigate hyperbilirubinemia using phototherapy. Future research should also consider the severity of the minor blood group incompatibility.

Abstract Background As the survival rates of very low birth weight (VLBW) infants have increased, their neurodevelopmental outcomes are of concern. This study aims to determine the demographic and perinatal characteristics of premature infant according to head growth, identify clinical factors affecting growth catch-up, and explore differences in developmental outcomes according to catch-up states. Methods This nationwide prospective cohort study of Korean Neonatal Network data analyzed premature infants with very low birth weight (

INTRODUCTION: Herein, we compared surgical outcome of dorsal shortening (DS) vs. ventral lengthening (VL) for correcting congenital ventral curvatures. METHODS: A systematic literature search was performed in September 2021 using the PubMed, EMBASE, Scopus, CENTRAL, ProQuest, and Clinicaltrials.gov databases. Comparative studies were identified and evaluated according to Cochrane Collaboration recommendations. Assessed outcomes included success and complication rates, which were extrapolated for the respective odds ratios (OR) with 95% confidence intervals (CIs). Subgroup analyses were performed according to congenital curvature, with or without severe hypospadias or recurrent curvatures (PROSPERO: CRD42021276193). RESULTS: Based on pooled effect estimates from 12 studies with 430 (DS 253, VL 177) cases of ventral curvature repair, VL rendered a better success rate for curvature correction (OR 4.20, 95% CI 2.11, 8.33) than DS, with comparable composite surgical complication rates (OR 0.77, 95% CI 0.27, 2.18). Subgroup analysis showed that the success rate remained significantly better for VL among patients with associated severe hypospadias (OR 3.59, 95% CI 1.25, 10.26) and recurrent penile curvatures (OR 5.70, 95% CI 1.69, 19.21) but not among those with congenital curvature without hypospadias or those with mild hypospadias (OR 2.99, 95% CI 0.32, 27.57). CONCLUSIONS: For congenital curvature associated with severe hypospadias and recurrent curvatures, VL renders a modestly better success rate; however, careful selection of patients is key for best outcome. [ABSTRACT FROM AUTHOR]

Abstract This study investigated the role of cesarean section (CS) in mortality and morbidity of very-low-birth-weight infants (VLBWIs) weighing less than 1500 g. This nationwide prospective cohort study of the Korean Neonatal Network consisted of 9,286 VLBWIs at 23–34 gestational weeks (GW) of age between 2013 and 2017. The VLBWIs were stratified into 23–24, 25–26, 27–28 and 29–34 GW, and the mortality and morbidity were compared according to the mode of delivery. The total CS rate was 78%, and was directly proportional to gestational age. The CS rate was the lowest at 61% in case of infants born at 23–24 GW and the highest at 84% in VLBWIs delivered at 29–34 GW. Contrary to the significantly lower total mortality (12%) and morbidities including sepsis (21%) associated with CS than vaginal delivery (VD) (16% and 24%, respectively), the mortality in the 25–26 GW (26%) and sepsis in the 27–28 GW (25%) and 29–34 GW (12%) groups were significantly higher in CS than in VD (21%, 20% and 8%, respectively). In multivariate analyses, the adjusted odds ratios (ORs) for mortality (OR 1.06, 95% CI 0.89–1.25) and morbidity including sepsis (OR 1.12, 95% CI 0.98–1.27) were not significantly reduced with CS compared with VD. The adjusted ORs for respiratory distress syndrome (1.89, 95% CI 1.59–2.23) and symptomatic patent ductus arteriosus (1.21, 95% CI 1.08–1.37) were significantly increased with CS than VD. In summary, CS was not associated with any survival or morbidity advantage in VLBWIs. These findings indicate that routine CS in VLBWIs without obstetric indications is contraindicated.

Three-dimensional (3D) poly(3,4-ethylenedioxythiophene) (PEDOT) films were demonstrated as an efficient Pt-free catalyst in dye-sensitized solar cells (DSSCs). The 3D PEDOT films were fabricated by the deposition of a polystyrene (PS) bead (diameter=1µm) monolayer on fluorine-doped tin oxide (FTO) glass, followed by electrochemical polymerization (EP) of ethylenedioxythiophene (EDOT) monomer. For comparison, a flat PEDOT film and Pt counter electrodes were additionally prepared by solution casting polymerization (SCP) and the thermal reduction of a spin-coated H2PtCl6solution, respectively. When these films were implemented as counter electrodes in quasi-solid-state DSSCs with a nanogel electrolyte, the cell efficiency of the 3D PEDOT film prepared by EP for 30sec reached 5.05%, which is higher than those of the flat PEDOT (4.11%) and Pt counter electrode (4.59%). The improved efficiency of the 3D PEDOT-based cell is attributed to its higher electrocatalytic performance and improved light reflectance, as determined by cyclic voltammogram (CV), incident photon-to-current efficiency (IPCE), and electrochemical impedance spectroscopy (EIS) analyses. [ABSTRACT FROM AUTHOR]

Viscum album var. coloratum (Kom.) Ohwi is a traditional herbal medicine used in East Asia to treat hypertension, skeletal muscle disorders, and cancer. The inhibitory effects of Viscum album (VA) extract on chemokines and its therapeutic potential in erlotinib-induced skin rash were investigated in this study. ELISA was used to measure the levels of chemokines, MCP-1 and RANTES, which are thought to be mediators of erlotinib-induced skin rash in RAW264.7 cells. Western blot analysis was used to look into the activation of signaling pathways like AKT, MAPK, and EGF. In order to investigate the active compounds in VA extract, solvent fractionation and preparative HPLC were performed sequentially. VA extract significantly reduced the production of TNF-α, MCP-1, and RANTES but not IL-1. Furthermore, macrophage transmigration was inhibited without causing cell toxicity. VA extract had no effect on the phosphorylation of EGF receptors stimulated by EGF or suppressed by erlotinib in both A549, a non-small cell lung cancer cells, and Hacat, a human skin keratinocyte. The isolated viscumneoside III and viscumneoside V from VA extract significantly suppressed the expression of MCP-1, according to activity guided fractionation with organic solvent fractionation and preparative HPLC. These findings suggest that VA extract and its active compounds, viscumneoside III and viscumneoside V, regulate MCP-1 production and may have the potential to suppress erlotinib-induced skin toxicity by modulating macrophage activity without neutralizing anti-cancer efficacy.

There are more than two hundred fifty different types of cancers, that are diagnosed around the world. Prostate cancer is one of the suspicious type of cancer spreading very fast around the world, it is reported that in 2018, 29430 patients died of prostate cancer in the United State of America (USA), and hence it is expected that one out of nine men diagnosed with this severe disease during their lives. Medical science has identified cancer at several stages and indicated genes mutations involved in the cancer cell progressions. Genetic implications have been studied extensively in cancer cell growth. So most efficacious drug for prostate cancer is highly required just like other severe diseases for men. So nutraceutical companies are playing major role to manage cancer disease by the recommendation of best natural products around the world, most of these natural products are isolated from plant and mushrooms because they contain several chemoprotective agents, which could reduce the chances of development of cancer and protect the cells for further progression. Some nutraceutical supplements might activate the cytotoxic chemotherapeutic effects by the mechanism of cell cycle arrest, cell differentiation procedures and changes in the redox states, but in other, it also elevate the levels of effectiveness of chemotherapeutic mechanism and in results, cancer cell becomes less reactive to chemotherapy. In this review, we have highlighted the prostate cancer and importance of nutraceuticals for the control and management of prostate cancer, and the significance of nutraceuticals to cancer patients during chemotherapy.

Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal dysplastic disease associated with mutations in the gene encoding the Runt-related transcription factor 2 (RUNX2). CCD is characterized by hypoplastic clavicles and patent cranial sutures. Management is essential to prevent complications during growth of the patient. Herein, we report a sporadic case of an infant with hypoplastic skull and clavicles at birth, which correlated with clinical findings of CCD. A heterozygous mutation was identified in the RUNX2 gene, which confirmed the diagnosis of CCD.

Iatrogenic esophageal perforation is a rare condition in children, but occasionally occurs in premature infants due to repeated intubation or nasogastric tube insertion. Esophageal perforation is common in pneumothorax and interstitial emphysema, but rarely occurs in the absence of pneumothorax. Although complications, such as mediastinitis, after esophageal perforation are rare, they can be fatal. Therefore, rapid and accurate diagnosis and appropriate treatment are important. The authors report a case of huge retrocardiac pneumomediastinum and subcutaneous emphysema suspected to be caused by esophageal perforation after repeated intubation.

Diabetes mellitus is a life-threatening disorder affecting people of all ages and adversely disrupts their daily functions. Despite the availability of numerous synthetic-antidiabetic medications and insulin, the demand for the development of novel antidiabetic medications is increasing due to the adverse effects and growth of resistance to commercial drugs in the long-term usage. Hence, antidiabetic phytochemicals isolated from fruit plants can be a very nifty option to develop life-saving novel antidiabetic therapeutics, employing several pathways and MoAs (mechanism of actions). This review focuses on the antidiabetic potential of commonly available Bangladeshi fruits and other plant parts, such as seeds, fruit peals, leaves, and roots, along with isolated phytochemicals from these phytosources based on lab findings and mechanism of actions. Several fruits, such as orange, lemon, amla, tamarind, and others, can produce remarkable antidiabetic actions and can be dietary alternatives to antidiabetic therapies. Besides, isolated phytochemicals from these plants, such as swertisin, quercetin, rutin, naringenin, and other prospective phytochemicals, also demonstrated their candidacy for further exploration to be established as antidiabetic leads. Thus, it can be considered that fruits are one of the most valuable gifts of plants packed with a wide spectrum of bioactive phytochemicals and are widely consumed as dietary items and medicinal therapies in different civilizations and cultures. This review will provide a better understanding of diabetes management by consuming fruits and other plant parts as well as deliver innovative hints for the researchers to develop novel drugs from these plant parts and/or their phytochemicals.

Although the surgical management of pediatric urolithiasis does not differ significantly from that of adults, there are anatomical and physiological differences that necessitate special considerations. This review aims to summarize the existing high-level evidence (systematic review, meta-analysis, randomized controlled trials) for surgical management of pediatric urolithiasis, with a discussion on different technical tips to make these surgical approaches achievable. A PubMed web-based medical literature search was performed on May 26, 2020, using the search strategy (Pediatric or children) and (urolithiasis or stone) and Urology. The search was limited to meta-analysis, systematic reviews, and randomized controlled trials published in the past 10 years. Only studies that focused on surgical management of pediatric urolithiasis were included. Fifty-seven records were identified and 47 were excluded as these records were duplicates, did not assess surgical management, or were trials that were included in systematic reviews/meta-analyses that were included in this study. Ten studies were included in this review. Depending on the review/trial, the stone-free rate (SFR) had wide ranges for all of the surgical management options assessed (shock wave lithotripsy, retrograde intrarenal surgery, and percutaneous nephrolithotomy). There are many considerations in choosing the appropriate surgical management for a patient presenting with pediatric nephrolithiasis–SFRs, radiation exposure, and adverse events being some of these features. All three procedures assessed had different characteristics, with advantages and disadvantages unique to each procedure. As each surgical technique for pediatric urolithiasis provides its advantages and disadvantages, surgeons should discuss all options to provide the best-informed decision-making process to a patient or family who may require surgical management of pediatric nephrolithiasis.

Merkel cell carcinoma (MCC) is a rare form of aggressive skin cancer mainly caused by Merkel cell polyomavirus (MCPyV). Most MCC tumors express MCPyV large T (LT) antigens and play an important role in the growth-promoting activities of oncoproteins. Truncated LT promotes tumorigenicity as well as host cell proliferation by activating the viral replication machinery, and inhibition of this protein in humans drastically lowers cellular growth linked to the corresponding cancer. Our study was designed with the aim of identifying small molecular-like natural antiviral candidates that are able to inhibit the proliferation of malignant tumors, especially those that are aggressive, by blocking the activity of viral LT protein. To identify potential compounds against the target protein, a computational drug design including molecular docking, ADME (absorption, distribution, metabolism, and excretion), toxicity, molecular dynamics (MD) simulation, and molecular mechanics generalized Born surface area (MM-GBSA) approaches were applied in this study. Initially, a total of 2190 phytochemicals isolated from 104 medicinal plants were screened using the molecular docking simulation method, resulting in the identification of the top five compounds having the highest binding energy, ranging between −6.5 and −7.6 kcal/mol. The effectiveness and safety of the selected compounds were evaluated based on ADME and toxicity features. A 250 ns MD simulation confirmed the stability of the selected compounds bind to the active site (AS) of the target protein. Additionally, MM-GBSA analysis was used to determine the high values of binding free energy (ΔG bind) of the compounds binding to the target protein. The five compounds identified by computational approaches, Paulownin (CID: 3084131), Actaealactone (CID: 11537736), Epigallocatechin 3-O-cinnamate (CID: 21629801), Cirsilineol (CID: 162464), and Lycoricidine (CID: 73065), can be used in therapy as lead compounds to combat MCPyV-related cancer. However, further wet laboratory investigations are required to evaluate the activity of the drugs against the virus.

The activation of NLRP3 results in the assembly of inflammasome that regulates caspase-1 activation and the subsequent secretion of bioactive interleukin (IL)-1β. Excessive activation of the NLRP3 inflammasome is mechanistically linked to diverse pathophysiological conditions, including airway inflammation. Here, we discovered that Curcuma phaeocaulis can suppress caspase-1 activation and processing of pro-IL-1β into mature cytokine in macrophages stimulated with NLRP3 inflammasome activators, such as SiO2 or TiO2 nanoparticles. Furthermore, in the bronchoalveolar lavage fluids of animals administered the nanoparticles, the in vitro effects of C. phaeocaulis translated into a decrease in IL-1β levels and cell infiltration. Demethoxycurcumin (DMC) and curcumin were found to be responsible for the inflammasome inhibitory activity of C. phaeocaulis. Interestingly, in contrast to the previously reported higher antioxidant- and NFκB-inhibitory activities of curcumin, DMC exhibited approximately two-fold stronger potency than curcumin against nanoparticle induced activation of NLRP3 inflammasome. In the light of these results, both compounds seem to act independently of their antioxidant- and NFκB-inhibitory properties. Although how C. phaeocaulis inhibits nanoparticle-activated NLRP3 inflammasome remains to be elucidated, our results provide a basis for further research on C. phaeocaulis extract as an anti-inflammatory agent for the treatment of disorders associated with excessive activation of NLRP3 inflammasome.

Purpose Subgaleal hemorrhage (SGH) is a rare but potentially fatal condition in newborns; however, few studies have reported on this condition. We aimed to identify the clinical characteristics and prognostic factors of SGH. Methods We retrospectively reviewed the medical records of 20 neonates diagnosed with SGH between January 2000 and June 2017. Enrolled neonates were clinically diagnosed when they had tender fluctuant scalp swelling that crossed the suture lines. Results Among 20 neonates with SGH, 12 were boys and 7 were girls; median hospitalization duration was 9.7±6.9 days. Fourteen neonates (70%) were born via vacuum-assisted vaginal delivery, and 4 via vacuum-assisted cesarean section. Of the neonates enrolled, half of them initially showed unstable vital signs, including apnea, desaturation, and cyanosis. Ten neonates had acidosis and 3 had asphyxia (pH

Abstract Background Rotavirus (RV) infection in neonates can be mild or even asymptomatic. In RV infection, jaundice is often reported, but the relationship between jaundice and RV infection has not been studied. This study aimed to determine the importance of asymptomatic RV screening in neonates with jaundice. Methods Neonates from the neonatal intensive care unit (NICU) of Chonbuk National University Hospital, those transferred from local obstetrics and gynecology hospitals and outpatient clinics were selected from 2014 to 2017. The study included only infants aged between 3 and 28 days. Jaundice was defined according to gestational age and birth age, in accordance with the American Academy of Pediatrics guidelines criteria. RV infection was confirmed by a stool test, and RV screening and laboratory tests were performed at admission. Results Among 596 patients, 166 patients had jaundice. RV infection was observed in 70 (42%) jaundice patients. There were 36 (22%) jaundice patients with asymptomatic RV infection. Patients with onset of jaundice 3–7 days after birth had a high incidence of RV infection. When the RV test was positive, the risk of jaundice was significantly high [odds ratio (OR) 1.89; 95% confidence interval (CI), 1.20–2.98; p = 0.006]. Conclusions Infants with the onset of jaundice > 3 days after birth were likely to have RV infection. Therefore, we suggest that screening tests for RV infection be included as part of the evaluation of jaundiced infants presenting to NICU.

Abstract Background To investigate the trends in the incidence and associated factors of late-onset sepsis (LOS) associated with improved survival in extremely preterm infants. Methods Medical records of 364 infants who were born at 23–26 weeks’ gestation from 2000 to 2005 (period I, n = 124) and from 2006 to 2011 (period II, n = 240) were retrospectively reviewed. The infants were stratified into subgroups of 23–24 and 25–26 weeks’ gestation within each period, and survival, LOS rate, and clinical characteristics were analyzed. Multivariate logistic regression analyses were completed to identify the clinical factors associated with LOS. Results The survival rate of 75.8% during period I significantly improved to 85.4% during period II, especially in infants at 23–24 weeks’ gestation (55.1% vs. 78.1%, respectively). The LOS rate of 33.1% during period I significantly reduced to 15.8% during period II, especially in infants at 25–26 weeks’ gestation (32.0% vs. 8.9%, respectively). The LOS rate per 1000 hospital days of 4.0 during period I significantly reduced to 1.8 during period II. Candida presence reduced from 21.3% during period I to 4.7% during period II. In multivariate analyses, during period I, prolonged intubation, especially in infants at 25–26 weeks’ gestation, and necrotizing enterocolitis, especially in infants at 23–24 weeks’ gestation, were significantly associated with LOS. Conclusions Improved survival of infants at 23–24 weeks’ gestation was associated with a simultaneous reduction of LOS incidence in infants at 25–26 weeks’ gestation. Less-invasive assisted ventilation may be one of the details of improved perinatal and neonatal care that has contributed to lowering risk of infection or death among periviable infants.

Antenatal corticosteroid (ACS) administration has been known as one of the most effective treatment in perinatal medicine, but the beneficial effects of ACS may vary not only gestational age, but also the quality of perinatal and neonatal care of the institution. This nationwide cohort study of the Korean Neonatal Network (KNN) data was consisted of

Hemophagocytic lymphohistiocytosis (HLH) occurs in the primary form (genetic or familial) or secondary form (acquired). The familial form of HLH (FHL) is a potentially fatal autosomal recessive disorder that occurs because of constitutional defects in cell-mediated cytotoxicity. Here, we report a fatal neonatal case of type 2 FHL (FHL2) that involved a novel frameshift mutation. Clinically, the newborn presented with severe sepsis-like features and required mechanical ventilation and continuous venovenous hemodiafiltration. Flow cytometry analysis showed marked HLH and complete absence of intracytoplasmic perforin expression in cytotoxic cells; therefore, we performed molecular genetic analyses for PRF1 mutations, which showed that the patient had a compound heterozygous mutation in PRF1, that is, c.65delC (p.Pro22Argfs*2) and c.1090_1091delCT (p.Leu364Glufs*93). Clinical and genetic assessments for FHL are required for neonates with refractory fever and progressive multiple organ failure, particularly when there is no evidence of microbiological or metabolic cause.

A retropharyngeal abscess is a rare disease entity in young infants but can develop after nasopharyngeal viral infection. Group B Streptococcus and Staphylococcus aureus are the most common pathogens in young infants, however, Mycobacterium tuberculosis is very rare. We report the case of retropharyngeal abscess and coinfection with S. aureus and M. tuberculosis in a very young infant presenting with respiratory symptoms due to upper airway obstruction. Usually tuberculous retropharyngeal abscesses are caused by the direct invasion of the bacteria from the spine via anterior longitudinal ligament of the spine. However, in this case, no associated spinal disease was observed.

Nonketotic hyperglycinemia (NKH) is a rare inborn error of amino acid metabolism. A defect in the glycine cleavage enzyme system results in highly elevated concentrations of glycine in the plasma, urine, cerebrospinal fluid, and brain, resulting in glycine-induced encephalopathy and neuropathy. The prevalence of NKH in Korea is very low, and no reports of surviving patients are available, given the scarcity and poor prognosis of this disease. In the current study, we present a patient with NKH diagnosed on the basis of clinical features, biochemical profiles, and genetic analysis. Magnetic resonance spectroscopy (MRS) allowed the measurement of absolute glycine concentrations in different parts of the brain that showed a significantly increased glycine peak, consolidating the diagnosis of NKH. In additional, serial MRS follow-up showed changes in the glycine/creatinine ratios in different parts of the brain. In conclusion, MRS is an effective, noninvasive diagnostic tool for NKH that can be used to distinguish this disease from other glycine metabolism disorders. It may also be useful for monitoring NKH treatment.

Purpose : With improved survival of extremely low birth weight infants (ELBWI), there is an increase in the incidence of necrotizing enterocolitis (NEC) requiring laparotomy, and the risk of morbidity and mortality in these ELBWI is increased. Thus, we determined the prognostic factors in ELBWI who underwent laparotomy for NEC. Methods : We retrospectively reviewed the medical records of 35 ELBWI who underwent laparotomy for NEC from January 2001 to December 2008 at Samsung Medical Center. Results : Of 480 ELBWI, 35 required laparotomy for NEC; the mortality rate was 20% (Alive group n=28, Dead group n= 7). The values of preoperative score for neonatal acute physiology-II (P=0.022) and fraction of inspired oxygen (P

The molecular function of the cellular prion protein (PrPC) and the mechanism by which it may contribute to neurotoxicity in prion diseases and Alzheimer's disease are only partially understood. Mouse neuroblastoma Neuro2a cells and, more recently, C2C12 myocytes and myotubes have emerged as popular models for investigating the cellular biology of PrP. Mouse epithelial NMuMG cells might become attractive models for studying the possible involvement of PrP in a morphogenetic program underlying epithelial-to-mesenchymal transitions. Here we describe the generation of PrP knockout clones from these cell lines using CRISPR-Cas9 knockout technology. More specifically, knockout clones were generated with two separate guide RNAs targeting recognition sites on opposite strands within the first hundred nucleotides of the Prnp coding sequence. Several PrP knockout clones were isolated and genomic insertions and deletions near the CRISPR-target sites were characterized. Subsequently, deep quantitative global proteome analyses that recorded the relative abundance of>3000 proteins (data deposited to ProteomeXchange Consortium) were undertaken to begin to characterize the molecular consequences of PrP deficiency. The levels of ∼ 120 proteins were shown to reproducibly correlate with the presence or absence of PrP, with most of these proteins belonging to extracellular components, cell junctions or the cytoskeleton.

Ni-rich mixed metal oxides (LiNi0.80Co0.15Mn0.05O2) as a high capacity cathode material for lithium ion battery are synthesized in two different heat-treatment atmospheres of air and oxygen, respectively. A cell with LiNi0.80Co0.15Mn0.05O2 heat-treated in the oxygen atmosphere shows better electrochemical performance of initial (193.7 mAh/g) and retention (185.2 mAh/g) capacities than a cell with the cathode material heat-treated in the air atmosphere (initial capacity: 185.2 mAh/g and retention capacity: 172.0 mAh/g). In order to find the reasons of the different electrochemical performance, the samples have been characterized in detail by X-ray diffraction and scanning electron microscopy. The structural differences are revealed at the nanometer scale from the experimental works of scanning transmission electron microscopy images mapped with orientation and reliability indices and electron energy loss spectroscopy. The NiO phase of the rock salt structure is considerably developed on the surfaces of the materials in the less oxidative environment of the air atmosphere; this is related to the poor electrochemical performances. [ABSTRACT FROM AUTHOR]

SPIN90 is a widely expressed Nck-binding protein that contains one Src homology 3 (SH3) domain, three Pro-rich motifs, and a serine/threonine-rich region, and is known to participate in sarcomere assembly during cardiac myocyte differentiation. We used in vitro binding assays and yeast two-hybrid screening analysis to identify Nck, βPIX, Wiscott-Aldrich syndrome protein (WASP), and ERK1 as SPIN90-binding proteins. It appears that βPIX, WASP, and SPIN90 form a complex that interacts with Nck in a manner dependent upon cell adhesion to extracellular matrix. The βPIX·WASP·SPIN9·Nck interaction was abolished in suspended and cytochalasin D-treated cells, but was recovered when cells were replated on fibronectin-coated dishes. The SPIN90·βPIX·WASP complex was stable, even in suspended cells, suggesting SPIN90 serves as an adaptor molecule to recruit other proteins to Nck at focal adhesions. In addition, we found that overexpression of the SPIN90 SH3 domain or Pro-rich region, respectively, abolished SPIN90·Nck and SPIN90·βPIX interactions, resulting in detachment of cells from extracellular matrix. SPIN90 was phosphorylated by ERK1, which was, itself, activated by cell adhesion and platelet-derived growth factor. Such phosphorylation of SPIN90 likely promotes the interaction of the SPIN90·βPIX·WASP complex and Nck. It thus appears that the interaction of the βPIX·WASP·SPIN90 complex with Nck is crucial for stable cell adhesion and can be dynamically modulated by SPIN90 phosphorylation that is dependent on cell adhesion and ERK activation. [ABSTRACT FROM AUTHOR]