Severe Neonatal Presentation of Progressive Familial Intrahepatic Cholestasis Type 4 in an Omani Infant

Progressive familial intrahepatic cholestasis type 4 (PFIC4) is a relatively newly described autosomal recessive disorder caused by biallelic mutations in the gene encoding tight junction protein 2 (TJP2) which is located in chromosome 9q21. PFIC4 is characterized by cholestasis with or without other extrahepatic manifestations. Bleeding tendency due to vitamin k deficiency is a well-known complication of cholestasis. We present a neonate who presented with cholestasis and multiple intracranial bleeds. He was found to have severe coagulopathy and his genetic work up revealed a homozygous variant mutation in TJP2 gene causing PFIC4. He had persistent cholestasis that necessitated an internal biliary diversion with some clinical improvement. Keywords: Jaundice; Intracranial haemorrhage; Progressive Familial Intrahepatic Cholestasis type 4

Sentence Embedding Approach using LSTM Auto-encoder for Discussion Threads Summarization

Online discussion forums are repositories of valuable information where users interact and articulate their ideas and opinions, and share experiences about numerous topics. These online discussion forums are internet-based online communities where users can ask for help and find the solution to a problem. A new user of online discussion forums becomes exhausted from reading the significant number of irrelevant replies in a discussion. An automated discussion thread summarizing system (DTS) is necessary to create a candid view of the entire discussion of a query. Most of the previous approaches for automated DTS use the continuous bag of words (CBOW) model as a sentence embedding tool, which is poor at capturing the overall meaning of the sentence and is unable to grasp word dependency. To overcome these limitations, we introduce the LSTM Auto-encoder as a sentence embedding technique to improve the performance of DTS. The empirical result in the context of the proposed approach’s average precision, recall, and F-measure with respect to ROGUE-1 and ROUGE-2 of two standard experimental datasets demonstrates the effectiveness and efficiency of the proposed approach and outperforms the state-of-the-art CBOW model in sentence embedding tasks and boost the performance of the automated DTS model

Human Wharton's Jelly Stem Cell (hWJSC) Extracts Inhibit Ovarian Cancer Cell Lines OVCAR3 and SKOV3 in vitro by Inducing Cell Cycle Arrest and Apoptosis

Ovarian cancer is a highly lethal and the second highest in mortality among gynecological cancers. Stem cells either naïve or engineered are reported to inhibit various human cancers in both in-vitro and in-vivo. Herein we report the cancer inhibitory properties of human Wharton's jelly stem cell (hWJSC) extracts, namely its conditioned medium (hWJSC-CM) and cell lysate (hWJSC-CL) against two ovarian cancer cell lines (OVCAR3 and SKOV3) in-vitro. Cell metabolic activity assay of OVCAR3 and SKOV3 cells treated with hWJSC-CM (12.5, 25, 50, 75, 100%) and hWJSC-CL (5, 10, 15, 30, and 50 μg/ml) demonstrated concentration dependent inhibition at 24–72 h. Morphological analysis of OVCAR3 and SKOV3 cells treated with hWJSC-CM (50, 75, 100%) and hWJSC-CL (15, 30, and 50 μg/ml) for 24–72 h showed cell shrinkage, membrane damage/blebbings and cell death. Cell cycle assay demonstrated an increase in the sub-G1 and G2M phases of cell cycle following treatment with hWJSC-CM (50, 75, 100%) and hWJSC-CL (10, 15, and 30 μg/ml) at 48 h. Both OVCAR3 and SKOV3 cells demonstrated mild positive expression of activated caspase 3 following treatment with hWJSC-CM (50%) and hWJSC-CL (15 μg/ml) for 24 h. Cell migration of OVCAR3 and SKOV3 cells were inhibited following treatment with hWJSC-CM (50%) and hWJSC-CL (15 μg/ml) for 48 h. Tumor spheres (TS) of OVCAR3 and SKOV3 treated with hWJSC-CM (50, 75, 100%) and hWJSC-CL (10, 15, 30 μg/ml) for 48 h showed altered surface changes including vacuolations and reduction in size of TS. TS of OVCAR3 and SKOV3 also showed the presence of few ovarian cancer stem cells (CSCs) in minimal numbers following treatment with hWJSC-CM (50%) or hWJSC-CL (15 μg/ml) for 48 h. Real-time gene expression analysis of OVCAR3 and SKOV3 treated with hWJSC-CM (50%) or hWJSC-CL (15 μg/ml) for 48 h demonstrated decreased expression of cell cycle regulatory genes (cyclin A2, Cyclin E1), prostaglandin receptor signaling genes (EP2, EP4) and the pro-inflmmatory genes (IL-6, TNF-α) compared to untreated controls. The results indicate that hWJSC-CM and hWJSC-CL inhibit ovarian cancer cells at mild to moderate levels by inducing cellular changes, cell cycle arrest, apoptosis, decreasing the expression of CSC markers and related genes regulation. Therefore, the stem cell factors in hWJSCs extracts can be useful in cancer management

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000–2018)

BackgroundThe epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America.ObjectiveWe report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia.MethodsA national database of BA cases diagnosed between 2000 and 2018 was analyzed. We assessed clearance of jaundice (bilirubin <20 μmol/L) in all cases that underwent Kasai portoenterostomy (KPE). We then estimated survival using the Kaplan–Meier method with endpoints of liver transplantation (LT), death, or survival with native liver (SNL).ResultsBA was diagnosed in 204 infants (106 females; 10% pre-term). The incidence of BA was 1 in 44,365, or 2.254 in 100,000 live births (range, 0.5–4 in 100,000). Polysplenia was diagnosed in 22 cases (11%). The median age at referral was 65 days. A total of 146 children (71.5%) underwent KPE at a median age of 70 days. Clearance of jaundice was achieved in 66 of the 146 (45%) infants. The 10-year SNL after KPE was 25.5%, and the overall 10-year estimated survival was 72.5%. The Kaplan–Meier survival curves for patients undergoing KPE at the age of <60, 61–90, and >90 days showed a SNL rate at 51.6, 33, and 12.5%, respectively, at 5 years (P < 0.001). The 2-, 5-, and 10-year post-LT survival rates were 92.5, 90.6, and 90%, respectively. Undergoing an initial KPE did not impact negatively on the overall LT survival rate when compared to BA cases that underwent primary LT (P = 0.88).ConclusionThe incidence rate of BA in Saudi Arabia is lower than the incidence reported elsewhere. Late referral of BA cases remains a problem in Saudi Arabia; as a result, the SNL rate was lower than reported by other national registries. Hence, national policies devoted to timely referral and earlier age at KPE are needed

Reactive mercury in the troposphere: Model formation and results for Florida, the northeastern United States, and the Atlantic Ocean

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/95402/1/jgrd13669.pd

Long-term Atmospheric Mercury Wet Deposition at Underhill, Vermont

Section 112(m) of the 1990 Clean Air Act Amendments, referred to as the Great Waters Program, mandated an assessment of atmospheric deposition of hazardous air pollutants (HAPs) to Lake Champlain. Mercury (Hg) was listed as a priority HAP and has continued to be a high priority for a number of national and international programs. An assessment of the magnitude and seasonal variation of atmospheric Hg levels and deposition in the Lake Champlain basin was initiated in December 1992 which included event precipitation collection, as well as collection of vapor and particle phase Hg in ambient air. Sampling was performed at the Proctor Maple Research Center in Underhill Center, VT. The range in the annual volume-weighted mean concentration for Hg in precipitation was 7.8–10.5 ng/l for the 11-year sampling period and the average amount of Hg deposited with each precipitation event was 0.10 μg/m 2 . The average amount of Hg deposited through precipitation each year from 1993 to 2003 was 9.7 μg/m 2 /yr. A seasonal pattern for Hg in precipitation is clearly evident, with increased Hg concentrations and deposition observed during spring and summer months. While a clear trend in the 11-year event deposition record at Underhill was not observed, a significant decrease in the event max-to-monthly ratio was observed suggesting that a major source influence was controlled over time. Discrete precipitation events were responsible for significant fractions of the monthly and annual loading of Hg to the forested ecosystem in Vermont. Monthly-averaged temperatures were found to be moderately correlated with monthly volume-weighted mean Hg concentrations ( r 2 =0.61) and Hg deposition ( r 2 =0.67) recorded at the Vermont site. Meteorological analysis indicated the highest levels of Hg in precipitation were associated with regional transport from the west, southwest, and south during the warmer months.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/44444/1/10646_2004_Article_6260.pd

Burnout among surgeons before and during the SARS-CoV-2 pandemic: an international survey

Background: SARS-CoV-2 pandemic has had many significant impacts within the surgical realm, and surgeons have been obligated to reconsider almost every aspect of daily clinical practice. Methods: This is a cross-sectional study reported in compliance with the CHERRIES guidelines and conducted through an online platform from June 14th to July 15th, 2020. The primary outcome was the burden of burnout during the pandemic indicated by the validated Shirom-Melamed Burnout Measure. Results: Nine hundred fifty-four surgeons completed the survey. The median length of practice was 10 years; 78.2% included were male with a median age of 37 years old, 39.5% were consultants, 68.9% were general surgeons, and 55.7% were affiliated with an academic institution. Overall, there was a significant increase in the mean burnout score during the pandemic; longer years of practice and older age were significantly associated with less burnout. There were significant reductions in the median number of outpatient visits, operated cases, on-call hours, emergency visits, and research work, so, 48.2% of respondents felt that the training resources were insufficient. The majority (81.3%) of respondents reported that their hospitals were included in the management of COVID-19, 66.5% felt their roles had been minimized; 41% were asked to assist in non-surgical medical practices, and 37.6% of respondents were included in COVID-19 management. Conclusions: There was a significant burnout among trainees. Almost all aspects of clinical and research activities were affected with a significant reduction in the volume of research, outpatient clinic visits, surgical procedures, on-call hours, and emergency cases hindering the training. Trial registration: The study was registered on clicaltrials.gov "NCT04433286" on 16/06/2020

Sensitivity of urban photochemical models to upper wind measurements.

The 1992 Atlanta Field Intensive of the Southern Oxidants Research Program on Ozone Non-Attainment (SORP-ONA) provided a unique data set of urban meteorological measurements. The data were used to investigate the sensitivity of photochemical model results to the spatial and temporal resolution of upper-air meteorological measurements. Root Mean Square Differences (RMSD) and average absolute deviations for winds and model calculated \rm NO\sb{y} and \rm O\sb3 values were computed for a variety of measurement strategies which differed in the spatial and temporal resolution of the upper-air data. A Lagrangian particle dispersion model (LPDM) was used to study the movement of the plumes from the Atlanta urban core and an elevated power plant located northwest of Atlanta. Scenarios with different sets of wind measurements were performed. The results show that placement of upper-air measurements at or near the center of emissions density should be highest priority of field measurements campaigns. A prognostic model, the Regional Atmospheric Modeling System (RAMS), was used to generate the three dimensional winds to run the UAM. Results from the UAM runs also support the conclusion that upper-air measurements at or near the center of emissions density should be highest priority of field measurements campaigns. A number of Large Eddy Simulations (LES) were performed for urban forested and unforested areas with different soil moisture to examine the extent of the thermals that represent the height of the mixed layer. Results from the LES runs show that simulations with dry soil produce higher sensible heat flux and stronger thermals with a deeper mixed layer than simulations with moister soil. LES runs also showed that sensible heat fluxes, vertical motion, and mixing is weaker over forested areas compared to over urban areas. Three methods were used to calculate the height of the convective mixed layer. Differences of up to 200 m were found between the three methods.Ph.D.Atmospheric and Space SciencesUniversity of Michigan, Horace H. Rackham School of Graduate Studieshttp://deepblue.lib.umich.edu/bitstream/2027.42/104734/1/9610065.pdfDescription of 9610065.pdf : Restricted to UM users only

An Overlooked Complication of Vancomycin Induced Acute Flaccid Paralysis in a Child with Acute Leukemia: A Case Report

Vancomycin is a glycopeptide antibiotic which is commonly used to treat methicillin-resistant staphylococcal infections. It is commonly used in pediatric oncology wards for children with febrile neutropenia. We report a very rare side effect of vancomycin induced myopathy in a child with acute lymphoblastic leukemia. To the best of our knowledge, this is the first case reported from Oman