Σάββατο, 14 Απριλίου 2018

EMS inventions: Where did they come from?

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Hard to believe, but there was a time we were without the most necessary EMS tools. That all changed, thanks to these creative minds.

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MedStar slalom

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MedStar replaced training diagrams with drone flyovers of the driving course to better demonstrate how to navigate a complicated driving scenario.

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MedStar AJody Stevens Banana Microphone

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To keep the students' attention, MedStar incorporated some entertaining videos.

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MedStar: Clearing Intersection, OV does not stop

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MedStar has traditionally used the CEVO program. They began augmenting this training with several real-life scenarios captured on camera to show true outcomes of both bad and good driving habits, like this crew clearing an intersection.

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EMS inventions: Where did they come from?

ems.jpg

Hard to believe, but there was a time we were without the most necessary EMS tools. That all changed, thanks to these creative minds.

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MedStar slalom

hqdefault.jpg

MedStar replaced training diagrams with drone flyovers of the driving course to better demonstrate how to navigate a complicated driving scenario.

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MedStar AJody Stevens Banana Microphone

hqdefault.jpg

To keep the students' attention, MedStar incorporated some entertaining videos.

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MedStar: Clearing Intersection, OV does not stop

hqdefault.jpg

MedStar has traditionally used the CEVO program. They began augmenting this training with several real-life scenarios captured on camera to show true outcomes of both bad and good driving habits, like this crew clearing an intersection.

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Q&A: Addressing EMS challenges through collaboration at Pinnacle 2018

By EMS1 Staff The Pinnacle EMS Leadership Forum brings together EMS leaders from every service model and every part of the nation for an exploration of the trends and challenges that face our profession. EMS1 is proud to be the premier media partner for this important event. Jay Fitch, PhD, founding partner of Fitch & Associates, which produces Pinnacle, tells us what to expect this year. Paramedic ...

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Inside EMS Podcast: Is 'Alexa' the future of patient care?

Download this podcast on iTunes, SoundCloud or via RSS feed In this Inside EMS Podcast episode, co-hosts Chris Cebollero and Kelly Grayson discuss the recording of their 200th episode and reflect on the past four years of co-hosting the show. They also talk about a recent article about Brewster Ambulance Services in Quincy, Ma., installing Amazon Echos in its ambulances to assist their workforce. Learn ...

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Paramedic spends birthday saving life for the second time

By EMS1 Staff PLYMOUTH, Mass. — A paramedic spent her last two birthdays saving the lives of two different patients. FOX25 reported that Brewster Ambulance paramedic Erika Apicella chose to work on her birthday for the past two years, and was able to save two cardiac arrest patients. Last year on her birthday, Apicella and her partner, Chris King, responded to the home of John Sullivan and transported ...

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Texas paramedics honored at recognition ceremony

By Mike Parker Austin American Statesman WILLIAMSON COUNTY, Texas —The Williamson County EMS department held its annual Recognition Ceremony on March 29 to celebrate and honor exemplary service paramedics perform on a daily basis. During the ceremony, the department recognized paramedics that were involved in caring for and resuscitating patients going into cardiac arrest, patients going into ...

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EMS inventions: Where did they come from?

ems.jpg

Hard to believe, but there was a time we were without the most necessary EMS tools. That all changed, thanks to these creative minds.

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MedStar slalom

hqdefault.jpg

MedStar replaced training diagrams with drone flyovers of the driving course to better demonstrate how to navigate a complicated driving scenario.

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MedStar AJody Stevens Banana Microphone

hqdefault.jpg

To keep the students' attention, MedStar incorporated some entertaining videos.

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MedStar: Clearing Intersection, OV does not stop

hqdefault.jpg

MedStar has traditionally used the CEVO program. They began augmenting this training with several real-life scenarios captured on camera to show true outcomes of both bad and good driving habits, like this crew clearing an intersection.

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Crestline welcomes StarTrans Bus to the Crestline family of products

Saskatoon, SK, Canada — Crestline Coach, Canadian distributor of small to mid-sized buses and a global leader in ambulance and specialty vehicle manufacturing, announced the addition of StarTrans Bus to its portfolio of bus solutions and products. StarTrans Bus is a major builder of shuttle buses for both the public and private transportation markets. As a division of Forest River Inc., and being ...

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ESO Expands TrackEMS Capabilities and User Experience

Key enhancements include Health Data Exchange integration, status tracking and customizable patient conditions AUSTIN — ESO Solutions, Inc., the leading data and software company serving emergency medical services (EMS), hospitals and fire departments, today announced significant enhancements to the powerful TrackEMS platform. Key updates include integration into ESO's Health Data Exchange ...

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Video teaches CPR to the tune of ‘500 miles’

Save a Life for Scotland, a campaign by the Scottish government to raise CPR awareness, created the video in an effort to teach 500,000 people CPR

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Mo. county 911 dispatchers to view school cameras in emergencies

Marion County 911 and the Hannibal public school district are teaming up to access any of the security cameras in operation throughout the school district

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First-Trimester Maternal Thyroid Function Is Not Associated with Child Scores on Standardized Educational Tests

Clinical Thyroidology, Volume 30, Issue 4, Page 179-181, April 2018.


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During Active Surveillance of Papillary Thyroid Microcarcinomas Higher Serum TSH Is Associated with Nodule Growth

Clinical Thyroidology, Volume 30, Issue 4, Page 156-158, April 2018.


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Circulating BRAF V600E Levels Correlate with Treatment State in Thyroid Cancer

Clinical Thyroidology, Volume 30, Issue 4, Page 152-155, April 2018.


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Patients with Advanced Papillary Thyroid Cancer Have Fewer Recurrences after Undergoing Surgery by High-Volume Surgeons

Clinical Thyroidology, Volume 30, Issue 4, Page 159-161, April 2018.


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Should the TBG Level Be Included Routinely When Assessing Newborns for Congenital Hypothyroidism?

Clinical Thyroidology, Volume 30, Issue 4, Page 186-189, April 2018.


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Extent of Initial Surgery May Impact Overall Survival, Even for Low-Risk Papillary Thyroid Cancers

Clinical Thyroidology, Volume 30, Issue 4, Page 162-164, April 2018.


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A Patient Survey of Hypothyroid Individuals Demonstrates Dissatisfaction with Treatment and with Managing Physicians

Clinical Thyroidology, Volume 30, Issue 4, Page 175-178, April 2018.


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Protecting Parathyroid Glands During Thyroidectomy — The Challenges Remain

Clinical Thyroidology, Volume 30, Issue 4, Page 165-170, April 2018.


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Stable Isotope Methodology Confirms Body Weight to Be the Main Levothyroxine Dose Adjustment Variable

Clinical Thyroidology, Volume 30, Issue 4, Page 182-185, April 2018.


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Punctate Echogenic Foci with Comet-Tail Artifacts May Be Associated with Malignancy When Occurring in Solid Portions of a Thyroid Nodule

Clinical Thyroidology, Volume 30, Issue 4, Page 171-174, April 2018.


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Exposure to Per- and Polyfluoroalkyl Substances Is Associated with Lower Thyroid Hormone Availability during Pregnancy

Clinical Thyroidology, Volume 30, Issue 4, Page 190-192, April 2018.


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Hepatic Encephalopathy and Helicobacter pylori



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Fecal Calprotectin in Assessing Endoscopic and Histological Remission in Patients with Ulcerative Colitis

Abstract

Background

Persistent active endoscopic and histological inflammation is associated with poorer outcomes in ulcerative colitis (UC). Fecal calprotectin is a surrogate marker of endoscopic and histological remission.

Aims

To confirm the correlation between fecal calprotectin and endoscopic or histological disease activity and to define the optimal cutoff value to detect endoscopic and histological remission.

Methods

From a prospectively maintained database, we analyzed 61 UC patients who had fecal calprotectin measurement and endoscopy performed within 1 month. Endoscopic activity was graded using the Mayo endoscopic subscore (MES). Histological remission was defined as normal histology or quiescent histological activity.

Results

Eighteen patients (29.5%) and five patients (8.1%) had endoscopic remission defined as MES ≤ 1 or MES = 0, respectively. We observed a significantly lower median level of fecal calprotectin in patients with endoscopic remission than those with endoscopic activity for both definition of endoscopic remission, i.e., MES ≤ 1 (158 vs 490 µg/g, p = 0.0005) or MES = 0 (94 vs 414 µg/g, p = 0.013). Seven patients (11.5%) were in histological remission. They had a lower median level of fecal calprotectin than those with active histological inflammation (107 vs 416 µg/g, p = 0.016). Using a ROC curve, fecal calprotectin < 250 µg/g predicted endoscopic remission (MES ≤ 1) with a sensitivity of 67% and specificity of 77%, while fecal calprotectin < 200 µg/g predicted histological remission with a sensitivity of 71% and specificity of 76%.

Conclusion

Fecal calprotectin level correlated with both endoscopic activity and histological activity and is a reliable biomarker in assessing mucosal healing in UC.



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Adenoma Detection Rate in Asymptomatic Patients with Positive Fecal Immunochemical Tests

Abstract

Background and Aims

The adenoma detection rate (ADR) is a powerful measure of screening colonoscopy quality. Patients who undergo colonoscopy for the evaluation of a positive fecal immunochemical test (FIT) have increased prevalence of colorectal neoplasia, but it is not known whether separate quality benchmarks are required. The aim of this study was to compare the conventional ADR to the ADR of colonoscopies performed for the evaluation of positive FIT, in asymptomatic average-risk patients.

Methods

Patients ≥ 50 years old who underwent colonoscopy for the evaluation of a positive FIT between January 1, 2013, and July 31, 2014, at a tertiary Veterans Affairs Medical Center were identified. FIT performed for any indication other than average-risk screening was excluded. The comparison group included average-risk patients ≥ 50 years old undergoing screening colonoscopy during the same time frame. The two groups were compared for ADR, advanced neoplasm [adenoma ≥ 10 mm, tubulovillous, high-grade dysplasia, CRC, sessile serrated polyp (SSP) ≥ 10 mm], CRC, and SSP detection after propensity score adjustment using a logistic regression model adjusted for endoscopist.

Results

There were 207 patients in the FIT group and 601 in the screening colonoscopy comparison group. After propensity score adjustment, ADR (72.9 vs. 50.0%, p = 0.003), number of adenomas per colonoscopy (3.3 ± 3.6 vs. 1.4 ± 2.3, p = 0.033), and advanced neoplasm detection rate (32.4 vs. 11.0%, p < 0.0001) were significantly higher in the FIT group. There were no significant differences in the number of CRC and the SSP detection rate.

Conclusions

In this cohort of average-risk Veterans, the ADR of colonoscopies performed for the evaluation of a positive FIT was higher than the ADR of screening colonoscopies. Patients with a positive FIT also had significantly more adenomas per colonoscopy and advanced neoplasms. These findings suggest that the quality of colonoscopies performed for a positive FIT is insufficiently assessed by the conventional ADR and requires additional quality metrics.



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Severe Intestinal Inflammation in the Small Intestine of Mice Induced by Controllable Deletion of Claudin-7

Abstract

Background

As a potential tumor suppressor gene, Claudin-7 (Cldn7), which is a component of tight junctions, may play an important role in colorectal cancer occurrence and development.

Aims

To generate a knockout mouse model of inducible conditional Cldn7 in the intestine and analyze the phenotype of the mice after induction with tamoxifen.

Methods

We constructed Cldn7-flox transgenic mice and crossed them with Villin-CreERT2 mice. The Cldn7 inducible conditional knockout mice appeared normal and were well developed at birth. We induced Cldn7 gene deletion by injecting different dosages of tamoxifen into the mice and then conducted a further phenotypic analysis.

Results

After induction for 5 days in succession at a dose of 200 µl tamoxifen in sunflower oil at 10 mg/ml per mouse every time, the mice appeared dehydrated, had a lower temperature, and displayed inactivity or death. The results of hematoxylin–eosin staining showed that the intestines of the Cldn7 inducible conditional knockout mice had severe intestinal defects that included epithelial cell sloughing, necrosis, inflammation and hyperplasia. Owing to the death of ICKO mice, we adjusted the dose of tamoxifen to a dose of 100 µl in sunflower oil at 10 mg/ml per mouse (aged more than 8 weeks old) every 4 days. And we could induce atypical hyperplasia and adenoma in the intestine. Immunofluorescent staining indicated that the intestinal epithelial structure was destroyed. Electron microscopy experimental analysis indicated that the intercellular gap along the basolateral membrane of Cldn7 inducible conditional knockout mice in the intestine was increased and that contact between the cells and matrix was loosened.

Conclusions

We generated a model of intestinal Cldn7 inducible conditional knockout mice. Intestinal Cldn7 deletion induced by tamoxifen initiated inflammation and hyperplasia in mice.



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Co-expression Network Analysis Identified COL8A1 Is Associated with the Progression and Prognosis in Human Colon Adenocarcinoma

Abstract

Backgrounds and Aims

Human colon adenocarcinoma is one of the major causes of tumor-induced death worldwide. A complicated gene interconnection network significantly regulates its progression and prognosis. The aim of our study was to find hub genes associated with the progression and prognosis of colon adenocarcinoma and to illustrate the underlying mechanisms.

Methods

A weighted gene co-expression network analysis was performed in our study to identify significant gene modules and hub genes associated with the TNM stage of colon adenocarcinoma (n = 441).

Results

In the turquoise module of interest, 23 hub genes were initially selected, and 10 of them were identified as "real" hub genes with high connectivity in the protein–protein interaction network. In the terms of validation, COL8A1 had the highest correlation with clinical traits among all of the hub genes. Data obtained from the Oncomine and GEPIA databases showed a higher expression of COL8A1 in colon adenocarcinoma tissues compared with normal colon tissues. Kaplan–Meier survival curves showed that higher expression of COL8A1 resulted in a shorter overall survival time and disease-free survival time. Univariate and multivariate Cox proportional hazards analyses indicated that the COL8A1 expression was an independent prognostic factor for survival in colon adenocarcinoma patients. Finally, gene set enrichment analysis indicated that the gene sets associated with focal adhesion were significantly enriched in colon adenocarcinoma samples with COL8A1 highly expressed.

Conclusions

COL8A1 was identified and proved to be correlated with the progression and prognosis of human colon adenocarcinoma, probably through regulating focal adhesion-related pathways.



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Genetic and Structural Analysis of a SKIV2L Mutation Causing Tricho-hepato-enteric Syndrome

Abstract

Background

Advances in genomics have facilitated the discovery of monogenic disorders in patients with unique gastro-intestinal phenotypes. Syndromic diarrhea, also called tricho-hepato-enteric (THE) syndrome, results from deleterious mutations in SKIV2L or TTC37 genes. The main features of this disorder are intractable diarrhea, abnormal hair, facial dysmorphism, immunodeficiency and liver disease.

Aim

To report on a patient with THE syndrome and present the genetic analysis that facilitated diagnosis.

Methods

Whole-exome sequencing (WES) was performed in a 4-month-old female with history of congenital diarrhea and severe failure to thrive but without hair anomalies or dysmorphism. Since the parents were first-degree cousins, the analysis focused on an autosomal recessive model. Sanger sequencing was used to validate suspected variants. Mutated protein structure was modeled to assess the effect of the mutation on protein function.

Results

We identified an autosomal recessive C.1891G > A missense mutation (NM_006929) in SKIV2L gene that was previously described only in a compound heterozygous state as causing THE syndrome. The mutation was determined to be deleterious in multiple prediction models. Protein modeling suggested that the mutation has the potential to cause structural destabilization of SKIV2L, either through conformational changes, interference with the protein's packing, or changes at the protein's interface.

Conclusions

THE syndrome can present with a broad range of clinical features in the neonatal period. WES is an important diagnostic tool in patients with congenital diarrhea and can facilitate diagnosis of various diseases presenting with atypical features.



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Infliximab Versus Adalimumab in Patients with Biologic-Naïve Crohn’s Disease: Is the Difference Real?



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Mortality from Spontaneous Bacterial Peritonitis Among Hospitalized Patients in the USA

Abstract

Background and Aim

Spontaneous bacterial peritonitis (SBP) is a serious complication of cirrhosis and is associated with significant morbidity and mortality. In this study, we examined the clinical characteristics and risk factors associated with mortality in hospitalized patients presenting with SBP.

Methods

The Nationwide Inpatient Sample was queried for all hospitalizations involving SBP from 2006 to 2014 using the International Classification of Disease-9-CM Code. Logistic regression was performed to evaluate the association between SBP mortality and factors such as age, gender, race/ethnicity, and concomitant medical conditions at presentation (e.g., variceal hemorrhage, hepatic encephalopathy, acute renal failure, coagulopathy, and other infections including pneumonia). The lengths of stay (LOS) and total charges were also examined.

Results

From 2006 to 2014, there were 88,167 SBP hospitalizations with 29,963 deaths (17.6% in-hospital mortality). The mean age of patients who died in the hospital was higher (58.2 years vs. 55.8, p < 0.01) than those who survived the admission. Acute alcoholic hepatitis was noted among a higher proportion of patients who died (7.0 vs. 5.9%, p < 0.01), who were also likely to have more medical comorbidities. In multivariable analysis, older age, female gender, hepatic encephalopathy, coagulopathy, variceal hemorrhage, sepsis, pneumonia, and acute kidney injury were associated with increased in-hospital mortality. This group also had longer LOS (11.6 days vs. 9.1, p < 0.01) and higher total charges ($138,273 vs. $73,533, p < 0.01).

Conclusion

SBP is associated with significant in-hospital mortality, especially in patients with concurrent risk factors. SBP remains a significant burden to the healthcare system.



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Early gastric cancer with lymphoid stroma presenting as a subepithelial lesion diagnosed by endoscopic submucosal dissection

Abstract

A 53-year-old man underwent an esophagogastroduodenoscopy that showed a 20-mm subepithelial lesion in the middle gastric body. Endoscopic ultrasound revealed a hypoechoic mass located in the submucosa. Biopsy specimens revealed a benign gastric mucosa with severe lymphocytic infiltration in the submucosa. Malignant lymphoma or gastric cancer with lymphoid stroma was suspected. We performed endoscopic submucosal dissection for definitive diagnosis. Histological examination showed undifferentiated adenocarcinoma, which showed positive Epstein–Barr virus-encoded RNA in situ hybridization results, invading the submucosa mixed with dense lymphocytic infiltration. Thus, Epstein–Barr virus-positive gastric cancer with lymphoid stroma was diagnosed. Gastric cancer with lymphoid stroma is a rare subtype of gastric cancer, which is associated with Epstein–Barr virus infection; it sometimes appears as a subepithelial lesion, which makes it difficult to diagnose using standard biopsy. Endoscopic submucosal dissection was useful in obtaining a sufficient tissue for full histological assessment, including immunostaining.



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Contrast-enhanced intraoperative ultrasound in the resection of colorectal liver metastases with intrabiliary growth

Abstract

A 68-year-old male who had undergone low anterior resection for primary rectal cancer 19 months ago presented with multiple CLM at Couinaud's segments IV, V, and VIII. There was no apparent macroscopic intrabiliary growth on preoperative computed tomography and gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI). However, the hepatobiliary phase of EOB-MRI revealed peritumoral low signal intensity in lesions in segments V and VIII, which indicates vascular invasion around hepatocellular carcinoma. Contrast-enhanced intraoperative ultrasound (CE-IOUS) clearly determined the extent of macroscopic glissonean growth from lesions in segments V and VIII, and more extensive resection was performed than was planned. Analysis of the resected specimens from segments V and VIII confirmed the presence of macroscopic intrabiliary growth with microscopic portal vein invasion. All three CLM were histopathologically diagnosed as well-to-moderately differentiated adenocarcinoma, and R0 resection was verified. Postoperative recovery was uneventful, and the patient was alive without evidence of recurrence 12 months after hepatic resection. CE-IOUS should be considered at the time of CLM resection, as it might enable more accurate detection of macroscopic intrabiliary growth of CLM, and enable resection with safer margins.



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Intestinal Microbiota in Hirschsprung Disease

Objectives: To characterize the microbiota profiles of patients with Hirschsprung's disease (HD), and to evaluate this in relation to postoperative bowel function and the incidence of Hirschsprung-associated enterocolitis (HAEC). Methods: All patients operated on for HD at our center between 1987–2011 were invited to answer questionnaires on bowel function and to participate in a clinical follow-up for laboratory investigations, including fecal DNA extraction, fecal calprotectin (FC), and brush border lactase (LCT) genotyping. The microbiota compositions of HD patients were compared to those of healthy controls aged 2–7 years. Results: The microbiota composition of eligible HD patients (n = 34; median age 12 (range, 3–25) years) differed from the healthy controls (n = 141), showing decreased overall microbial richness (p 

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Anorectal Manometry May Reduce the Number of Rectal Suction Biopsy Procedures Needed to Diagnose Hirschsprung's Disease

Objectives: To evaluate whether anorectal manometry (ARM), which is used to test the rectoanal inhibitory reflex (RAIR), is a safe alternative for reducing the number of invasive rectal suction biopsy (RSB) procedures needed to diagnose Hirschsprung's disease (HD). Methods: Between 2010 and 2017, we prospectively collected the ARM results of 105 patients suspected of having HD. Following the outcome, the patients either underwent additional tests to confirm HD or they were treated conservatively. Primary ARM-based diagnoses were compared with the definitive diagnoses based on the pathology reports and/or clinical follow-ups. Additionally, we analyzed whether modifications to our ARM protocol improved diagnostic accuracy. Results: The sensitivity of ARM and RSB was comparable (97% versus 97%). The specificity of ARM, performed according to our initial protocol, was significantly lower than that of RSB. After we modified the protocol the difference between the specificity of ARM and RSB was no longer statistically significant (74% versus 84%, respectively, P = .260). The negative predictive value of ARM was 100%, while their positive predictive value was significantly lower than that of RSB (56% versus 97%, P 

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Birth Month as a Risk Factor for the Diagnosis of Celiac Disease Later in Life: A Population-based Study

Various perinatal factors have been implicated in association with the risk of developing celiac disease (CD) in genetically susceptible individuals. Our aim was to investigate the association of month and season of birth with the development of CD later in life in a large National cohort in Israel. Data were retrieved from a National database of more than 2 million Israeli Jewish adolescents born between 1971 and 1998. Overall, 10,566 CD cases out of 2,001,353 subjects (0.53%) were identified and analyzed. CD risk was significantly higher for subjects born in May (odds ratio [OR] 1.07, P = 0.04) and June (OR 1.09, P = 0.008). Birth during the winter season (December to February) showed a marginal significance toward reduced risk (OR 0.97, P = 0.05). In conclusion, children born in May and June are at increased risk for CD. This increased risk provides further evidence that perinatal environmental factors increase the risk of developing CD. Address correspondence and reprint requests to Amit Assa, MD, MHA, Institute of Gastroenterology, Nutrition and Liver Disease, Schneider Children's Medical Center of Israel, Sackler Faculty of Medicine, Tel- Aviv University, 14 Kaplan St. Petach-Tikva, 4920235, Israel (e-mail: dr.amit.assa@gmail.com). Received 27 November, 2017 Accepted 31 March, 2018 The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Pediatric Liver Transplant Teams’ Coping with Patient Death

Objectives: Coping with patient death among pediatric liver transplant teams has received little attention despite general recognition of the potentially negative emotional consequences associated with such loss. The purpose of this study was to investigate 1) the ways in which members of pediatric liver transplant teams cope with the death of patients on the waitlist and post-transplant and 2) the institutional resources available to facilitate this coping. Methods: Participants included 120 physicians, nurses, and mental health professionals from multiple transplant centers across the United States. Participants completed an online questionnaire that assessed the availability of formal coping resources at their institutions, informal sources of support used to cope with patient death, and as indices of coping, bereavement and emotional exhaustion symptoms experienced. Results: Debriefing, the most commonly offered support, was available to about half (55.8%) of the sample; yet, nearly all respondents (98.3%) indicated that debriefing would be useful. On average, bereavement and emotional exhaustion levels were comparable to normative data, but patterns of coping varied based on participants' position within the transplant team. For participants who reported that debriefing was available at their institutions, emotional exhaustion was lower. Conclusions: Overall, formal supports were inconsistently offered to pediatric transplant team members. Team members expressed high acceptability for debriefing, which has been associated with benefits in other populations, and findings indicated better coping in the transplant setting when it was offered. Address correspondence and reprint requests to Sarah E. Duncan, Department of Psychology, Fordham University, 441 E. Fordham Road, Bronx, NY 10458 (e-mail: sduncan6@fordham.edu). Received 20 July, 2017 Accepted 17 January, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Authorship Statements Sarah E Duncan: Concept/design, statistics, data analysis/interpretation, drafting article, critical revision of article, approval of article Ronen Arnon: Concept/design, data analysis/interpretation, critical revision of article, approval of article Christie DiPietrantonio: Concept/design, data collection, critical revision of article, approval of article Katie Ehrlich: Concept/design, data collection, critical revision of article, approval of article Christopher S Knight: Concept/design, data collection, critical revision of article, approval of article Jaime Chu: Data analysis/interpretation, critical revision of article, approval of article Rachel A Annunziato: Concept/design, data collection, statistics, data analysis/interpretation, drafting article, critical revision of article, approval of article Conflict of Interest and Sources of Funding: The authors of this manuscript have no conflicts of interest or sources of funding to disclose. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Pulmonary Function Test Abnormalities in Children With Inflammatory Bowel Disease: Is It Common?

Objective: The aim of the study was to evaluate the frequency and type of pulmonary dysfunction in newly diagnosed children with inflammatory bowel disease (IBD) and the correlation between pulmonary function tests (PFTs) and IBD activity. Methods: It is an observational case-control study. One hundred newly diagnosed children with IBD were enrolled as the patient group, which was subdivided into 52 with Crohn disease (CD) and 48 with ulcerative colitis (UC). Fifty healthy children matched for age, sex, height, and body mass index (BMI) served as the control group. PFTs in the form of forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC, residual volume (RV), total lung capacity (TLC), mid-forced expiratory flow of 25% to 75% (FEF 25%–75%) and diffusing capacity of the lung for carbon monoxide (DLCO) were evaluated in all studied children. PFTs were measured at diagnosis, every 6 months for a period of 3 years, during remission and at least once during activity in patient group. Results: There was significant progressive deterioration in all PFTs in IBD patients compared with their PFTs at the start of the study (P  0.05). There was significant deterioration during disease activity compared with remission state regarding FEV1, FVC, FEV 25% to 75%, and DLCO (P 

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Celiac Disease 30 Years after Diagnosis: Struggling With Gluten-free Adherence or Gaining Gluten Tolerance?

Objectives: Studies investigating patients with coeliac disease (CD) on very long-term follow-up are limited. We aimed to evaluate the characteristics of patients with CD diagnosed more than 30 years ago. Methods: Clinical, histologic, genetic and demographic data of patients with CD diagnosis made before 1985 were collected and their standardised mortality ratio (SMR) calculated. According to the gluten free diet (GFD) status, CD patients were divided into three groups and a specific questionnaire on GFD awareness and gluten-free products was administered to patients and caregivers. Results: 337 CD patients were included in the study. The SMR was 0.37 (CI 0.10 to 0.94) compared to a matched population. A total of 197 patients were grouped according to GFD compliance, with 35 CD patients reporting chronic voluntary gluten ingestion. No significant differences were found between groups regarding family history of CD, symptoms and histology at diagnosis, autoimmune disorders. Follow-up histology was performed in 63 patients. Twenty patients had normal histology on gluten containing diet (GCD). Questionnaire scores were lower in patients on GCD. Caregivers scores were not correlated with patients' gluten consumption. Conclusions: Although poor adherence to GFD is the major predictor of persistence of mucosal lesions at follow-up histology, a proportion of patients did not show a relapse of villous atrophy in spite chronic voluntary gluten ingestion, nor increase in mortality. Moreover, GFD knowledge and adherence could be partly lost during the transition between childhood and adulthood. Address correspondence and reprint requests to Luca Elli, MD PhD, Center for the Prevention and Diagnosis of Celiac Disease, Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza, 28, 20122 Milan, Italy (e-mail: dottorlucaelli@gmail.com). Received 3 October, 2017 Accepted 22 March, 2018 Lorenzo Norsa and Federica Branchi, Equal Contributors. Sources of Funding: Research support for this study was provided in part by grants from the Italian Ministry of Health and Lumbardy's Regional Government Authority (Ministero della Salute e Regione Lombardia call no. R.F.GR 2011–02348234). Conflicts of interest: none declared. Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Early Serum Gut Hormone Concentrations Associated with Time to Full Enteral Feedings in Preterm Infants

Objectives: The primary objective of this study was to evaluate early postnatal serum gut hormone concentrations in preterm infants as predictors of time to full enteral feedings. The secondary objective was to identify infant characteristics and nutritional factors that modulate serum gut hormone concentrations and time to full enteral feedings. Methods: Sixty-four preterm infants less than 30 weeks of gestation were included in this retrospective cohort study. Serum gut hormone concentrations at postnatal days 0 and 7 were measured using enzyme-linked immunosorbent assays. Linear regression and mediation analyses were performed. Results: Median (IQR) serum concentrations of glucose-dependent insulinotropic peptide (GIP) and peptide YY (PYY) on postnatal day 7 were 31.3 pg/mL (18.2, 52.3) and 1181.7 pg/mL (859.0, 1650.2), respectively. GIP and PYY concentrations on day 7 were associated with days to full enteral feedings after adjustment for confounders (β = −1.1, p = 0.03; and β = −0.002, p = 0.02, respectively). Nutritional intake was correlated with serum concentrations of GIP and PYY on postnatal day 7 and time to full enteral feedings. Mediation analysis revealed that the effect of serum gut hormone concentrations on time to full enteral feedings was not fully explained by nutritional intake. Intrauterine growth restriction (IUGR), mechanical ventilation on postnatal day 7, and patent ductus arteriosus (PDA) treated with indomethacin were associated with longer time to full enteral feedings. Conclusions: Serum concentrations of GIP and PYY on postnatal 7 are independently associated with time to full enteral feedings. The link between serum gut hormone concentrations and time to full enteral feedings is not fully mediated by nutritional factors, suggesting an independent mechanism underlying the influence of gut hormones on feeding tolerance and time to full enteral feedings. Address correspondence and reprint requests to: Camilia R. Martin, MD, MS, Beth Israel Deaconess Medical Center, Department of Neonatology, 330 Brookline Avenue, Rose-318, Boston, MA 02215 (e-mail: cmartin1@bidmc.harvard.edu). Received 29 October, 2017 Accepted 16 March, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Author Contributions: Kristen H. Shanahan: conception and design of the study, acquisition, analysis, and interpretation of the data, drafting and revision of manuscript, final approval of manuscript for submission Xinting Yu: analysis and interpretation of data, final approval of manuscript for submission Laura G. Miller: acquisition of data, final approval of manuscript for submission Steven D. Freedman: conception and design of the study, analysis and interpretation of the data, drafting and revision of manuscript, final approval of manuscript for submission Camilia R. Martin: conception and design of the study, analysis and interpretation of the data, drafting and revision of manuscript, final approval of manuscript for submission Conflicts of Interest and Sources of Funding: CRM receives research funding from Abbott Nutrition and Alcresta Therapeutics. The other authors have no conflicts of interest. KHS was supported by the Alpha Omega Alpha Honor Medical Society (Carolyn L. Kuckein Student Research Fellowship). CRM and SDF were supported by philanthropic contributions to the Charles and Judy Hood Family Infant Health Repository Program, Abbott Nutrition, and Alcresta Therapeutics. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Therapeutic Plasma-Albumin Exchange for Hyperlipidemia Induced Acute Pancreatitis

No abstract available

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Small-Fiber Neuropathy in a Pediatric Patient Following Anti-TNF-α Therapy for Ulcerative Colitis

No abstract available

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A Rare Case of Gallbladder and Common Bile Duct Benign Capillary Hemangioma in a Toddler

No abstract available

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High serum procalcitonin concentration and dynamics of its changes as a prognostic factor of mortality



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Tips and troubleshooting during intubation with AirTraq videolaryngoscope



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Always check anaesthetic equipment



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Autoimmune anti-N-methyl-D-aspartate receptor encephalitis — the current state of knowledge based on a clinical case

The aim of this article is to conduct an overview of the current state of knowledge about patients presenting anti-Nmethyl- D-aspartate receptor encephalitis associated with neoplastic process, as well as diagnosis and treatment. This disease concerns mainly young women and correlates with ovarian teratoma. Most important problems seems to be the difficulties in making a proper diagnosis ensuing from the rarity of this syndrome, the period from the appearance the first symptoms to starting treatment and the correct handling of intensive care complications. There are only a few articles describing severe, complicated cases of this type of encephalitis, requiring treatment in an intensive care unit.

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Day and time of admissions to intensive care units — does it matter?

Background: The literature data pertaining to the significance of day and time of ICU admission for outcomes of patients are inconsistent. The issue has not been analysed in Poland to date. The aim of the study was to gather information about differences between patients admitted to ICU outside regular working hours (off-hours) and those admitted during working hours (on-hours). Methods: Analysis involved 20,651 patients from the Silesian Registry of Intensive Care Units carried out since 2010. The findings demonstrated that 34.8% of patients were admitted to ICUs during on-hours (between 8.00 a.m. and 3 p.m. on weekdays) and 65.2% were admitted during off-hours (outside regular working hours). The incidence of admissions and data of patients in both groups were compared in terms of the population characteristics and treatment outcomes. Results: The incidence of admissions (calculated per each 24 hours of treatment) was found to be almost twice as high during on-hours, as compared to off-hours (14.5 vs. 6.9 patients/day). Patients admitted to the ICU during on-hours were less likely to be admitted from the surgical department (19.1% vs. 31.0%, P Conclusions: Patients admitted to ICUs during off-hours are in more severe general condition and their treatment outcomes are worse, as compared to patients admitted to ICU during on-hours.

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The impact of the blood glucose levels of non-diabetic critically ill patients on their clinical outcome

Background: Stress hyperglycaemia is thought to result from a hormonal response (release of catecholamines,
glucocorticoids, glucagon, etc.) following stress, sepsis or trauma. Although stress hyperglycaemia is a very common
finding in critically ill populations, there are many non-diabetic critically ill patients who do not develop a hyperglycaemic
stress response to trauma or acute illness. We suggest that the lack of a hyperglycaemic stress response
during the acute phase of a critical illness may correlate significantly with the clinical outcome of these critically ill
non-diabetic patients.

Methods: This was a retrospective study of 700 non-diabetic critically ill patients admitted to the general intensive
care unit (ICU) at Soroka Medical Center, Beer Sheva, Israel. We analyzed the clinical impact of the blood glucose levels
of these patients measured during their first week of ICU hospitalization on their clinical outcome.


Results: Age, male gender, and the Acute Physiology and Chronic Health Evaluation (APACHE) score were found to
be independent risk factors for new episodes of infection during the patients' stay in the ICU. Age and the APACHE
and Sequential Organ Failure Assessment scores were found to be independent risk factors for intra-ICU mortality. In
contrast, blood glucose analysis performed during the patients' stay in the ICU was not found to be an independent
predictor for new infectious events or for mortality during the ICU stay.

Conclusion: Our study did not demonstrate an association between blood glucose levels and clinical outcomes in
non-diabetic critically ill patients.



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Impact of anaemia on outcome in burn patients

Background: Currently, most critical care physicians maintain a patient's haemoglobin levels at 7 to 8 g dL-1. However,
little data have been available on haemoglobin-related outcomes in burn patients. The purpose of this study was
to evaluate inpatients with greater than 20% total body surface area burns and the effects of haemoglobin below
8 g dL-1 on clinical outcomes.

Methods: This study included 70 patients with burns amounting to greater than 20% of total body surface area. Data
were retrospectively evaluated and included age, gender, adult respiratory distress syndrome presence, length of
intensive care unit stay, length of mechanical ventilation, days requiring vasopressors, renal insufficiency, positive
cultures/infections, cardiovascular complications, number of operations, inhalation injury, and mortality. Logistic
regression analyses that were adjusted for age, sex, and percent total body surface area were used to assess the
relationships between haemoglobin and multiple clinical outcomes. Odds ratios (OR) were estimated with 99%
confidence intervals (99% CI).

Results: Haemoglobin below 8 g dL-1 was associated with a need for vasopressors (OR = 2.17; 99% CI = 1.03–8.22).
Furthermore, haemoglobin below 8 g dL-1 was associated with higher positive wound (OR = 2.86; 99% CI = 1.00–34.40),
urine (OR = 4.63; 99% CI = 1.15–67.00), and lung cultures (OR = 2.24; 99% CI = 1.06–5.47). These associations largely
remained after controlling for blood transfusions.

Conclusions: Contrary to most other patient groups, burn patients with burns amounting to greater than 20% of
total body surface area and low haemoglobin levels were more likely to develop positive cultures in urine, wounds,
and the lung and require vasopressor treatment.



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Gasping for a Diagnosis: Pediatric Vocal Cord Dysfunction

Vocal cord dysfunction is an obstruction of the upper airway, primarily on inspiration, due to the paradoxical adduction of the vocal cords. Vocal cord dysfunction continues to be underdiagnosed as its own entity. The lack of diagnosis can be attributed to the overlap of symptoms between asthma and exercise-induced bronchospasm. It is possible for patients diagnosed with asthma and/or exercise-induced bronchospasm to have underlying vocal cord dysfunction, which needs to be considered when prescribing asthma medications.

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Caring for Military Children: Implications for Nurse Practitioners

Nearly two million American children belong to a military family. Armed Forces families are located in almost every state and around the globe. Military children are a unique and at times vulnerable population. Military children and adolescents may face significant stressors throughout their lives compared to their civilian counterparts. Military families encounter frequent moves and over half of these children have encountered at least one parental deployment since September 11th, 2001. Civilian health care providers often care for military children and adolescents.

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Feasibility of Providing Pediatric Neurology Telemedicine Care to Youth with Headache

Telemedicine has been described as use of electronic communication between two sites to deliver medical information to improve a patient's clinical health status (American Telemedicine Association, 2010). It has been widely used since the 1990s in remote areas such as Alaska and the upper reaches of Canada in addition to other rural parts of the United States (Gifford, Niles, Rivkin, Koverola, & Polaha, 2012). The American Academy of Pediatrics (2015) has supported telemedicine as a way to deliver both primary care and certain specialty areas with provider shortages.

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Clinical impact of intratumoral HER2 heterogeneity on trastuzumab efficacy in patients with HER2-positive gastric cancer

Abstract

Background

There is growing interest in the clinical significance of intratumoral HER2 heterogeneity. Its prognostic and predictive impacts on trastuzumab efficacy were demonstrated in breast cancer. However, its clinical significance in gastric cancer is still unclear.

Methods

Twenty-eight HER2-positive gastric cancer patients who had gastrectomy prior to trastuzumab-based chemotherapy were consecutively enrolled. Intratumoral HER heterogeneity was evaluated using whole-tissue sections by immunohistochemistry. When all tumor cells overexpressed HER2 protein, the tumor was defined as homogeneously HER2 (Homo-HER2)-positive group. The others were defined as heterogeneously HER2 (Hetero-HER2)-positive group.

Results

There was no significant difference in clinicopathological features between the two groups. The median progression-free survival (PFS) and overall survival (OS) in the Homo-HER2-positive group were significantly longer than those in the Hetero-HER2-positive group (PFS; 20.0 months [95% CI 17.8–22.2] vs. 6.0 months [95% CI 2.3–9.7]; HR 0.11; 95% CI 0.03–0.41; p < 0.001, OS; not reached vs. 14.0 months [95% CI 11.9–16.1]; HR 0.18; 95% CI 0.06–0.61; p = 0.003). In the multivariate analysis, these associations remained significant both in PFS (HR 0.12; 95% CI 0.03–0.46, p = 0.002) and OS (HR 0.21; 95% CI 0.06–0.72, p = 0.013). With respect to response rate, no statistical difference was found between two groups. However, deeper tumor shrinkage was obtained in the Homo-HER2-positive group compared with the Hetero-HER2-positive group (p = 0.046).

Conclusions

Intratumoral HER2 heterogeneity may have robust clinical impact on trastuzumab efficacy in patients with HER2-positive gastric cancer. These findings should be validated by larger independent cohorts and further molecular correlative analyses are warranted.



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Real-world efficacy and safety of ledipasvir and sofosbuvir in patients with hepatitis C virus genotype 1 infection: a nationwide multicenter study by the Japanese Red Cross Liver Study Group

Abstract

Background

We aimed to describe the real-world efficacy and safety of combination therapy with ledipasvir and sofosbuvir (LDV/SOF) for chronic hepatitis C virus (HCV) genotype 1 (GT1) infection.

Methods

This retrospective analysis of a prospective, nationwide, multicenter registry included GT1-infected patients treated with LDV/SOF for 12 weeks. We assessed the rate of sustained virological response at 12 weeks post-treatment (SVR12), incidence of adverse events, and serum markers of hepatocellular carcinoma (HCC).

Results

Among the 1461 patients included (mean age, 69 years; 29.5% aged > 75 years; cirrhosis, 23.8%; history of treatment for HCC, 10.9%), the overall SVR12 rate was 98.4% (1438/1461). Factors associated with treatment failure were cirrhosis (odds ratio, 4.19; p = 0.014) and resistance-associated substitutions (RASs) in NS5A at baseline (odds ratio, 7.78; p = 0.0004). The SVR12 rate in patients with cirrhosis and NS5A RASs was 93.0% compared to 100% in patients without cirrhosis or NS5A RASs. In patients with SVR, the levels of alpha-fetoprotein (AFP), AFP-L3, and Mac-2 binding protein glycosylation isomer (M2BPGi) decreased from baseline to end of treatment (from 13.4 ± 37.6 to 6.0 ± 10.6 ng/mL, p < 0.0001; from 2.2 ± 4.9 to 1.5 ± 6.3%, p < 0.005; and from 3.6 ± 3.7 to 2.0 ± 3.5 cut-off index, p < 0.0001; respectively). Adverse events were rare and not associated with age. No decrease in estimated glomerular filtration rate was observed in patients with baseline chronic kidney disease stage 3.

Conclusions

LDV/SOF therapy is highly effective and safe in elderly Japanese patients with HCV GT1, even in the presence of cirrhosis or NS5A RASs. Patients with SVR may have a lower risk of HCC.



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Reply to “Letter to Editor submitted by Rui Huang et al. entitled potential clinical application of strain elastography in chronic liver diseases”



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Potential clinical application of strain elastography in chronic liver diseases



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Author’s reply: can budesonide form be alterative treatment for ulcerative proctosigmoiditis?



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A proposed treatment algorithm for mild to moderate ulcerative colitis—with an emphasis on budesonide foam and mucosal healing



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Lymph node metastasis of adenocarcinoma and different definitions of sm1 cancer in the esophagus



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Reply to the letter to the editor: Lymph node metastasis of adenocarcinoma and different definitions of sm1 cancer in the esophagus



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Applications of Colon Capsule Endoscopy

Abstract

Purpose of Review

This is a review of colon capsule endoscopy (CCE) with a focus on its recent developments, technological improvements, and current and potential future indications.

Recent Findings

Based on the current literature, CCE II demonstrates comparable polyp detection rates as optical colonoscopy and CT colonography, and improved cost-effectiveness. The main limitation to patient acceptance is the requirement of a rigorous bowel preparation. Preliminary studies show good correlation between CCE and optical colonoscopy for assessment of colonic disease activity in inflammatory bowel disease (IBD).

Summary

CCE II is currently FDA, approved as an adjunctive test in patients with prior incomplete colonoscopy, and in the evaluation of patients with suspected lower gastrointestinal bleeding. The test is approved in Europe as one of the options for average-risk colorectal cancer screening, and high-risk screening in patients with contraindications or unwilling to undergo colonoscopy. CCE has a potential role in the evaluation and monitoring of colonic disease activity in IBD. Future technological advances should focus on minimizing bowel preparation, improvement in reading times, and development of therapeutic capabilities.

• With technological improvements, the second-generation colon capsule has a significantly higher sensitivity than the first-generation capsule for detection of colon polyps.

• Colon capsule endoscopy has been approved in Europe as an option for average-risk colorectal cancer screening, and high-risk screening in patients with contraindications or unwilling to undergo colonoscopy.

• Colon capsule endoscopy has received FDA approval as an option for colorectal cancer screening in patients with prior incomplete colonoscopy, and in evaluation of patients with suspected lower gastrointestinal bleeding.

• Colon capsule endoscopy may have a role in evaluation and monitoring of inflammatory bowel disease.

• Colon capsule endoscopy currently requires a bowel preparation that is more rigorous than colonoscopy.



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Update on the Therapeutic Management of Hepatic Encephalopathy

Abstract

Purpose of Review

Hepatic encephalopathy (HE) is a common and devastating complication to chronic liver disease. In this paper, we summarize the latest research and evidence of both conventional and up-coming treatments.

Recent Findings

Meta-analyses report beneficial effects of lactulose, branched-chain amino acids, rifaximin, and to some degree l-ornithine l-aspartate on the manifestations of HE in patients with cirrhosis, and generally the numbers needed to treat are low. Recent studies on newer HE treatments including ornithine phenylacetate, spherical carbon, and fecal microbiota transplant also report potentially beneficial effects on HE manifestations.

Summary

The conventional treatments benefit patients with HE. Newer treatments are under study and more research is needed for their validation.



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Considerations When Treating Hepatitis C in a Cirrhotic Transplant Candidate

Abstract

Purpose of Review

This review examines the issues in determining the decision to treat a HCV-positive patient who is a liver transplant (LT) candidate with highly effective and well-tolerated direct-acting antiviral (DAA) therapies.

Recent Findings

Cure of HCV with DAA can improve liver function and allow delisting in some patients. Beyond a threshold of hepatic impairment (likely MELD score > 16 to 20), patients may experience a decline in MELD score with HCV cure without improvement in liver-related complications resulting in decreased opportunity to receive a LT. Eradicating HCV from patients who need LT regardless also deprives them of the option of receiving HCV-positive donor organs. Patients with MELD > 16 or Child-Pugh B/C may also have reduced cure rates of HCV, increased risk of hepatic decompensation, and adverse events with DAA pre-LT compared to post-LT DAA therapy. Preliminary data demonstrates increase risk of hepatocellular carcinoma (HCC) recurrence after treatment with DAA with subsequent studies raising doubts about this association.

Summary

Patients with HCV cirrhosis on the LT waiting list with MELD score > 16, CTP-B/C, and HCC are best treated after LT with better response, tolerability, and the ability to receive organs from a larger donor pool that includes HCV-positive donors. Larger, prospective studies are needed to assess whether increased HCC recurrence after DAA is a true effect.



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Therapy for Crohn’s Disease: a Review of Recent Developments

Abstract

Purpose of Review

To review recent advances in the treatment of Crohn's disease.

Recent Findings

Several key advances are highlighted, including the increasing role of treatment algorithms and where new therapies can be used most effectively, the appropriate use of therapeutic drug monitoring, optimal management of post-surgical patients, and the role of multi-disciplinary clinics.

Summary

The last several years have seen a number of exciting developments in the field of Crohn's therapy. This review covers research advances including updated treatment algorithms focusing on identifying patient risk as well as the role of drug monitoring in managing the disease. We also review the optimal management of post-surgical patients as well as new biologics and biosimilars. Finally, we describe innovations in care delivery including multi-disciplinary clinics and emerging evidence from developing therapeutics.



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Food Allergy

Abstract

Purpose of Review

The goal of this review is to present an updated summary of the natural history of major childhood and adult food allergies and report recent advances in potential treatments for food allergy.

Recent Findings

The most common childhood food allergies are typically outgrown by adolescence or adulthood. However, peanut/tree nut allergies appear to more commonly persist into adulthood. Adults can develop new IgE-mediated food allergies; the most common is oral allergy syndrome. There are multiple different approaches being tried as possible treatments for food allergy.

Summary

The prevalence of food allergy appears to be increasing but the varied approaches to treatment are being actively pursued such that an approved modality may not be too far in the future.



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Therapeutic Drug Monitoring in Pediatric Inflammatory Bowel Disease

Abstract

Purpose of review

Therapeutic drug monitoring (TDM) has emerged as a useful tool to optimize the use of drug therapies in adults with inflammatory bowel disease (IBD), including both Crohn's disease (CD) and ulcerative colitis (UC), especially during the use of biological therapies, for which the pharmacokinetics and pharmacodynamics are highly variable among patients. Fewer data exist in children. This review examines the current literature on TDM in pediatric IBD.

Recent findings

Drug clearance is affected by a number of patient and disease factors. For thiopurines, adjusting dosing by monitoring 6-thioguanine (6TGN) and 6-methylmercaptopurine ((6MMP) levels is demonstrated to maximize response and minimize toxicity, while monitoring metabolite levels when treating with anti-tumor necrosis factor (anti-TNF) remain controversial. While in adults the use of TDM in the setting of loss of response to anti-TNF therapy is established, in children, only a small number of studies exist, but these too have encouraging results. There are however, conflicting data regarding the optimal timing of TDM, comparing "reactive" monitoring and "proactive" monitoring. No such data exist in pediatrics. TDM is cost-effective, and dose reduction may represent a safety benefit. There are limited adult data for use of TDM for the newer biologics, vedolizumab and ustekinumab, but early results suggest similarly promising utility.

Summary

The use of TDM in pediatric IBD is increasing in clinical practice, with similar efficacy to adults demonstrated in children with loss of response to anti-TNF therapy. More prospective studies are needed in children to examine proactive monitoring and utility of TDM with newer biologics.



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Colorectal Cancer in the Young

Abstract

Purpose of Review

Colorectal cancer incidence has been rapidly rising in those under the age of 50 over the last 20 years. This paper will review the epidemiology, clinicopathologic, molecular features, proposed risk factors, and prevention/treatment approach for early onset CRC (EOCRC) patients.

Recent Findings

EOCRC appears to have a different spectrum of clinical, pathologic, and molecular presentation compared to CRC diagnosed in older individuals. EOCRCs are disproportionately located in the distal colon; these patients tend to present with symptoms, and there is a longer interval between symptoms and diagnosis. There may be a distinct molecular signature, including progression through the microsatellite and chromosomal stable (MACS) pathway and LINE-1 hypomethylation for a subset of EOCRCs. The majority of EOCRCs are sporadic without clear risk factors that would have made the patient eligible for earlier screening.

Summary

There is an acute need for educational efforts aimed at both providers and patients to raise awareness about CRC in the young. Improving adherence to screening in young patients eligible for screening and emphasizing early evaluation of symptoms are important steps to decreasing the burden of CRC in younger patients. Modeling and empiric data are needed to determine whether our current screening approach should be modified and whether causation and treatment options may be different in a molecular subset EOCRCs.



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Editorial Board



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The Value and Significance of 25(OH) and 1,25(OH) vitamin D serum levels in adult coeliac patients: A review of the literature

Within the wide spectrum of symptoms and alteration of systems that characterizes CeD, several studies indicate a low-level of vitamin D, therefore recent guidelines suggest its evaluation at the time of diagnosis. This review examines the data from existing studies in which vitamin D has been assessed in CeD patients. Our review indicates that most of the studies on vitamin D in adult CeD report a 25 (OH) vitamin D deficiency at diagnosis that disappears when the patient goes on a gluten-free diet, independently of any supplementation.

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Entecavir Or Tenofovir Monotherapy Prevents HBV Recurrence In Liver Transplant Recipients: A 5-Year Follow-Up Study After Hepatitis B Immunoglobulin Withdrawal

Recent data suggest that oral third-generation nucleos(t)ide analogs (NA) monoprophylaxis following hepatitis B immunoglobulin (HBIg) withdrawal may be effective to prevent HBV reinfection after liver transplantation (LT).

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THE EPIDEMIOLOGY OF BUDD-CHIARI SYNDROME IN FRANCE

Epidemiological data is lacking on primary Budd-Chiari syndrome (BCS) in France.

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Acomparison study between Magniview high definition white light endoscopy in detecting villous atrophy coeliac disease: A single centre pilot study

Coeliac disease may be missed at gastroscopy. We aimed to assess the sensitivity of Pentax optical zoom technology endoscopes in detecting duodenal villous atrophy and the ease of image interpretation by non-coeliac specialists.

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Laparoscopic right hemicolectomy for a colon cancer patient with an ileal conduit

Asian Journal of Endoscopic Surgery, EarlyView.


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Outcomes of early versus delayed laparoscopic cholecystectomy for acute cholecystitis performed at a single institution

Asian Journal of Endoscopic Surgery, EarlyView.


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Older E-bike Users: Demographic, Health, Mobility Characteristics, and Cycling Levels

Purpose Electric bicycles (e-bikes) may offer an opportunity to stimulate physical activity among older adults. The current study compared Flemish (Belgian) older e-bike users against those not using an e-bike on socio-demographics, health characteristics and access to motorized transport. Additionally, it examined the association between e-bike use and levels of cycling and the moderating effects of sex, BMI and cycling limitations. Methods An online- or interview-version of the same questionnaire were completed by 1146 participants. Data were analysed using logistic regression and hurdle models. Results Women, those with a higher BMI and those with one (compared to no) motorized vehicle in the household had higher odds of being an e-bike user. E-bike use was related to higher odds of having cycled for transport in the past week and this relationship was stronger among those with a higher BMI (low BMI: OR= 1.89, 95% CI= 1.24-2.91; high BMI: OR= 3.34, 95% CI= 2.26-5.00). Among those who cycled for transport in the last week, e-bike use was associated with 35% more minutes of cycling for transport (95% CI= 17-56%). E-bike use was related to 183% higher odds of having biked for recreation (95% CI= 115-274%). Among women and those with cycling limitations who cycled for recreation in the last week, e-bike use was also related to 57% (95% CI= 18-109%) and 180% (95% CI= 63-381%) more minutes of cycling for recreation, respectively. Conclusion E-bikes may provide an opportunity to promote cycling among older adults, particularly among subgroups at risk for physical inactivity. Corresponding author: Jelle Van Cauwenberg, Department of Public Health, Faculty of Medicine and Health Sciences, Ghent University, De Pintelaan 185, 9000 Ghent, Belgium. +32 9 332 83 72. jelle.vancauwenberg@ugent.be JVC was supported by a postdoctoral fellowship of the Research Foundation Flanders (FWO, 12I1117N). The authors declare that they do not have professional relationships with companies or manufacturers who will benefit from the results of the present study. The results of the present study do not constitute endorsement by ACSM. The authors declare that the results of the study are presented clearly, honestly, and without fabrication, falsification, or inappropriate data manipulation. © 2018 American College of Sports Medicine

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Bone and Inflammatory Responses to Training in Female Rowers over an Olympic Year

Introduction/Purpose To examine whether fluctuations in training load during an Olympic year lead to changes in bone mineral densities and factors that regulate bone (sclerostin, osteoprotegerin [OPG] and receptor activator of nuclear factor kappa-B ligand [RANKL]), energy metabolism (insulin-like growth factor-1 [IGF-1] and leptin), and inflammation (tumor necrosis factor-α [TNF-α] and interleukin-6 [IL-6]) in elite heavyweight female rowers. Methods Blood samples were drawn from 15 female heavyweight rowers (27.0±0.8y, 80.9±1.3 kg, 179.4±1.4 cm) at baseline (T1 – 45 weeks pre-Olympic Games) and following 7, 9, 20, 25 and 42 weeks (T1-6, respectively). Ongoing nutritional counselling was provided. Total weekly training load was recorded over the weeks prior to each time point. BMD was measured by dual energy X-ray absorptiometry at T1 and T6. Results Total BMD increased significantly pre- to post-training (+0.02 g[BULLET OPERATOR]cm−2), however was below the least significant change (±0.04 g[BULLET OPERATOR]cm−2). OPG, IGF-1, and leptin remained stable across all time points. Fluctuations in training load (high vs. low) were accompanied by parallel changes in TNF-α (2.1±0.2 vs. 1.5±0.2 pg[BULLET OPERATOR]ml−1), IL-6 (1.2±0.08 vs. 0.8±0.09 pg[BULLET OPERATOR]ml−1), and sclerostin (high=993±109 vs. low=741±104 pg[BULLET OPERATOR]ml−1). Conclusion In this population of young female athletes with suitable energy availability, sclerostin and inflammation markers responded to fluctuations in training load while BMD and BMC were stable during the season, suggesting that training load periodization is not harmful for the bone health in athletes. Corresponding Author: Panagiota Klentrou, Department of Kinesiology, Brock University, 1812 Sir Isaac Brock Way, St. Catharines, Ontario, Canada, L2S 3A1. Tel: (905) 688-5550 x.4538. Fax: (905) 688-8364. nklentrou@brocku.ca The study was funded by the Canadian Sports Institute Ontario, and partially funded by Natural Sciences of Engineering Research Council of Canada. Nigel Kurgan holds an Ontario Graduate Scholarship. The authors report no conflict of interest, and the results of this study do not constitute endorsement by ACSM and they are presented clearly, honestly, and without fabrication, falsification or inappropriate data manipulation. © 2018 American College of Sports Medicine

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Temporal Changes in Cardiovascular Remodeling Associated with Football Participation

Purpose ASF participation rates in the United States are highest among high school (HS) athletes. This study sought to compare the cardiovascular response to HS versus collegiate ASF participation. Methods ASF participants (HS, N=61; Collegiate, N=87) were studied at pre- and post-season time points with echocardiography and applanation tonometry. Primary outcome variables included: left ventricular (LV) mass index, LV diastolic function (early relaxation velocity [E′]), and arterial stiffness (pulse wave velocity [PWV]). Results HS (17.1±0.4 years old) and collegiate ASF participants (18±0.4 years old) experienced similar LV hypertrophy (ΔLV mass HS=10.5±10 vs. Collegiate=11.2±13.6 g/m2, P=0.97). Among HS participants, increases in LV mass were associated with stable diastolic tissue velocities (ΔE′=-0.3±2.9 cm/s, P=0.40) and vascular function (ΔPWV=-0.1±0.6 m/s, P = 0.13). In contrast, collegiate participants demonstrated a higher burden of concentric LV hypertrophy (21/87, 24% vs. 7/61, 11%, P=0.026) with concomitant reductions in diastolic tissue velocities (ΔE′: -2.0±2.7 cm/s, P

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Is Exercise the Right Medicine for Dystrophic Muscle?

Introduction Duchenne muscular dystrophy (DMD) is a neuromuscular disease caused by a dystrophin protein deficiency. Dystrophin functions to stabilize and protect the muscle fiber during muscle contraction, thus the absence of functional dystrophin protein leads to muscle injury. DMD patients experience progressive muscle necrosis, loss of function, and ultimately succumb to respiratory failure or cardiomyopathy. Exercise is known to improve muscle health and strength in healthy individuals as well as positively impact other systems. Because of this, exercise has been investigated as a potential therapeutic approach for DMD. Methods This review aims to provide a concise presentation of the exercise literature with a focus on dystrophin deficient muscle. Our intent was to identify trends and gaps in knowledge with an appreciation of exercise modality. Results After compiling data from mouse and human studies it became apparent that endurance exercises such as a swimming and voluntary wheel running have therapeutic potential in limb muscles of mice and respiratory training was beneficial in humans. However, in the comparatively few long-term investigations the effect of low intensity training on cardiac and respiratory muscles was contradictory. In addition, the effect of exercise on other systems is largely unknown. Conclusion In order to safely prescribe exercise as a therapy to DMD patients, multi-systemic investigations are needed including the evaluation of respiratory and cardiac muscle. Address for Correspondence: Joshua Selsby, Ph.D., 2356 Kildee Hall, Ames, IA 50011. Office: 515.294.7227. Fax: 515.294.4471. email: jselsby@iastate.edu The authors have no conflicts of interest. Partial support for HRS provided by Project Parent Muscular Dystrophy (PPMD), Ryan's Quest and Michael's Cause. The results of the study do not constitute and endorsement by the American College of Sports Medicine. © 2018 American College of Sports Medicine

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Effects of Neuromuscular Electrical Stimulation in People with Spinal Cord Injury

Introduction Muscle force production is usually impaired in people with spinal cord injury (SCI). The use of high-intensity neuromuscular electrical stimulation (NMES) strength training can help promote metabolically active lean muscle mass and thus, increase muscle mass and improve physical health and quality of life (QoL). Nonetheless, NMES is usually used at low-stimulation intensities and there is limited evidence on the effects of high-intensity NMES strength training into improving muscle force and mass, symptoms of spasticity or physical health and quality of life (QoL) in people with SCI. Methods Five individuals with chronic SCI completed five 10-repetition sets of high-intensity knee extension NMES strength training sessions for 12 weeks in both quadriceps muscles. Quadriceps femoris (QF) knee extensor torque was measured on a dynamometer and cross-sectional area (CSAQF) was measured with extended-field-of-view ultrasonography. Venous blood samples were collected for blood lipid profiling and c-reactive protein (CRP) analyses. The Spinal Cord Injury Spasticity Evaluation Tool (SCI-SET) was used to assess symptoms of spasticity and the quality of life index (QLI) SCI version III was used for QoL measures. Results QF tetanic knee extensor torque increased on average by 35% (2 - 92%) and CSAQF increased by 47% (14 - 145%). A significant increase in the HDL/LDL cholesterol ratio (p

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The Role of Task Difficulty in Learning a Visuomotor Skill

Introduction Task difficulty affects the amount of interpretable information from a task, which is thought to interfere with motor learning. However, it is unclear whether task difficulty in itself is a stimulus for motor learning because the experimental evidence is mixed in support of the optimal challenge point framework that predicts one specific level of task difficulty to produce the greatest magnitude of motor learning. Purpose We determined the effects of functional task difficulty on motor skill acquisition, retention, and transfer. Methods Healthy young participants (N = 36) learned a mirror star-tracing task at a low, medium or hard difficulty level defined by the bandwidth of the star. We measured skill acquisition, retention and transfer to untrained difficulty levels, as well as the perceived mental workload during the task. Results Task difficulty affected motor performance, but did not affect motor learning and transfer. For the groups that practiced the task at the medium and hard but not at the low difficulty level, initial skill level correlated with the magnitude of learning. Conclusion The optimal challenge point framework does not capture the complex relationship between task difficulty and motor learning. Previously reported effects of task difficulty on the magnitude of motor learning are probably mediated by perceived mental workload. Task difficulty did not affect the magnitude of visuomotor skill learning but it affected how learning occurred. The data have implications on how athletes learn new motor skills and patients re-learn injury-impaired motor skills during rehabilitation. Corresponding author at: Josje M. Bootsma, University Medical Center Groningen, Antonius Deusinglaan 1 9713 AV FA23, Groningen, The Netherlands. Email address: j.m.bootsma@student.rug.nl, Phone number: +31618668396 This work was supported by start-up funds from the University Medical Center Groningen. All authors state that there are no financial and personal relationships with third parties that could have inappropriately influenced the present work. The results of the current study do not constitute endorsement by the American College of Sports Medicine and are presented clearly, honestly and without fabrication, falsification or inappropriate data manipulation. © 2018 American College of Sports Medicine

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Benefits of 8-Week MBSR or Aerobic Training on Seasonal Declines in Physical Activity

Mindfulness-based stress reduction (MBSR) and aerobic exercise training (AET) programs improve health and wellbeing. Exercise participation has been related to mindfulness and may be altered by MBSR training. PURPOSE Compare 8-weeks of MBSR, AET and no-treatment control during the fall season on objectively-measured physical activity in healthy adults. METHODS Participants (n=66) wore an Actigraph GT3X+ accelerometer for seven days pre-randomization, and following 8-week MBSR or AET interventions, or neither (control). Mean daily minutes (min) of moderate-to-vigorous physical activities (MVPA) were calculated along with weekly time spent in bouts of MVPA ≥10 min (MVPABouts) to assess physical activity sufficient to meet national guidelines. Groups were compared on pairwise changes in outcomes across time. Effect sizes were calculated using Cohen's d. RESULTS Sufficient data (≥3 weekdays, ≥1 weekend day, ≥10 hours/day) were obtained from 49 participants (18 MBSR, 14 AET, 17 control). Daily MVPA decreased in all groups from pre-randomization to post-intervention (Aug-Nov); control decreased 17.9±25.7 min/day, MBSR decreased 5.7±7.5 min/day, and AET decreased 7.4±14.3 min/day (mean±SD), without significant differences among the groups (all p>0.05). MVPABouts decreased 77.3±106.6 min/week in control and 15.5±37.0 min/week in MBSR (between-group difference: p=0.08; d=0.86), while it increased by 5.7±64.1 min/week in AET (compared to control: p=0.029; d=0.97; compared to MBSR; p=0.564; d=0.29). CONCLUSION Data from participants in a randomized controlled trial showed that while AET increases moderate-to-vigorous physical activity bouts compared to no treatment, MBSR training may also mitigate the influence of shorter day length and cooler weather on participation in physical activities. Future research is needed to determine how MBSR affects exercise to inform interventions. Interventions combining MBSR and exercise may be particularly successful at increasing physical activity participation. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Corresponding author: Jacob D. Meyer, Department of Kinesiology, 111E Barbara E Forker Building, 834 Wallace Rd, Ames, IA 50011-1160, jdmeyer3@iastate.edu, Tel: (515) 294-1386 Funding for the MEPARI2 trial came from National Institutes of Health, National Center for Complementary and Integrative Health (NCCIH; R01AT006970). JM was supported by the Health Resources and Services Administration (HRSA; T32HP10010) during his time on this project while MG was supported by NCCIH (T32AT006956). Bruce Barrett was supported by a mid-career research and mentoring grant from NCCIH (K24AT006543). AEZ's work was supported by the K23AA017508 award from the National Institutes of Health National Institute on Alcohol Abuse and Alcoholism. ET was supported by the University of Wisconsin-Madison Office of the Vice Chancellor for Research and Graduate Education Fall Competition Award, the UW Institute for Clinical and Translation Research (ICTR) Scholars Program, and the Clinical and Translational Science Award program through the NIH National Center for Advancing Translational Sciences (UL1TR000427 & KL2TR000428, PI: Drezner). The trial was registered at ClinicalTrials.gov before the first participant was enrolled (NCT01654289). The authors report no conflicts of interest. The results of the present study do not constitute endorsement by ACSM. The results of the study are presented clearly, honestly, and without fabrication, falsification, or inappropriate data manipulation. © 2018 American College of Sports Medicine

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Black Carbon Reduces the Beneficial Effect of Physical Activity on Lung Function

Introduction When physical activity is promoted in urban outdoor settings (e.g. walking and cycling), individuals are also exposed to air pollution. It has been reported that short-term lung function increases as a response to physical activity, but this beneficial effect is hampered when elevated air pollution concentrations are observed. Our study assessed the long-term impact of air pollution on the pulmonary health benefit of physical activity. Methods Wearable sensors were used to monitor physical activity levels (SenseWear) and exposure to black carbon (microAeth) of 115 healthy adults during one week in three European cities (Antwerp, Barcelona, London). The experiment was repeated in three different seasons to approximate long-term behavior. Spirometry tests were performed at the beginning and end of each measurement week. All results were averaged on a participant level as a proxy for long-term lung function. Mixed effect regression models were used to analyze the long-term impact of physical activity, black carbon and their interaction on lung function parameters FEV1, FVC, FEV1/FVC, FEF25-75 and PEF. Interaction plots were used to interpret the significant interaction effects. Results Negative interaction effects of physical activity and black carbon exposure on FEV1 (p=0.07), FEV1/FVC (p=0.03) and FEF25-75 (p=0.03) were observed. For black carbon concentrations up to approximately 1 μg/m3, an additional METhour per week resulted in a trend towards lung function increases (FEV1, FEV1/FVC and FEF25-75 increased 5.6 mL, 0.1% and 14.5 mL/s, respectively). Conclusion We found that lung function improved with physical activity at low black carbon levels. This beneficial effect decreased in higher air pollution concentrations. Our results suggest a greater need to reduce air pollution exposures during physical activity. Correspondence to: Prof dr Luc Int Panis, Flemish Institute for Technological Research (VITO), Boeretang 200, 2400 Mol, Belgium, e-mail: luc.intpanis@vito.be This research received funding from the European Union's Seventh Framework Program to the PASTA project under grant agreement No. 602624 (FP7-HEALTH-2013-INNOVATION-1) (Physical Activity through Sustainable Transport Approaches). Duration 1 November 2013 to 31 October 2017. Michelle Laeremans is supported by a VITO PhD scholarship. Evi Dons is supported by a postdoctoral scholarship from FWO-Research Foundation Flanders. Juan Pablo Orjuela-Mendoza received a beneficiary grant from Colciencias (Administrative Department of Science, Technology and Innovation of the Colombian Government) (grant number 646). All authors declare that they have no conflicts of interest and that the results of the study are presented clearly, honestly, and without fabrication, falsification, or inappropriate data manipulation. The results of the present study do not constitute endorsement by ACSM. © 2018 American College of Sports Medicine

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Role of fungal laccase in iodide oxidation in soils

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Publication date: September 2018
Source:Journal of Environmental Radioactivity, Volume 189
Author(s): Reiko Nihei, Mizuki Usami, Taro Taguchi, Seigo Amachi
Previously, we hypothesized that microbial laccase oxidizes iodide (I) in soils to molecular iodine (I2) or hypoiodous acid (HIO), both of which are easily incorporated into natural soil organic matter, and thus plays a role in iodine sorption on soils. In this study, soil iodide oxidase activity was determined by a colorimetric assay to evaluate if laccase is responsible for iodide oxidation in soils. Three types of Japanese soil showed significant iodide oxidase activities (0.751–2.87 mU g soil−1) at pH 4.0, which decreased with increasing pH, until it was no longer detected at pH 5.5. The activity was inhibited strongly by autoclaving or by the addition of common laccase inhibitors. Similar tendency of inhibition was observed in soil laccase activity, which was determined with 2,2′-azino-bis(3-ethylbenzothiazoline-6-sulfonic acid) (ABTS) as a substrate. Significant positive correlations (R2 values of 0.855–0.896) between iodide oxidase activity and laccase activity were observed in two of three soils. Commercially available fungal laccases showed only very low iodide oxidase activities (4.68–18.0 mU mg−1), but enhanced activities of 102–739 mU mg−1 were observed in the presence of redox mediators. Finally, we successfully isolated fungal strains with iodide-oxidizing phenotype in the presence of redox mediators. Polyacrylamide gel electrophoresis of the culture supernatant of Scytalidium sp. strain UMS and subsequent active stain revealed that the fungal laccase actually oxidized iodide in the presence of redox mediators. These results suggest that at least part of iodide in soils is oxidized by fungal laccase through the laccase-mediator system.



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Radiocesium interaction with clay minerals: Theory and simulation advances Post–Fukushima

Publication date: September 2018
Source:Journal of Environmental Radioactivity, Volume 189
Author(s): Masahiko Okumura, Sebastien Kerisit, Ian C. Bourg, Laura N. Lammers, Takashi Ikeda, Michel Sassi, Kevin M. Rosso, Masahiko Machida
Insights at the microscopic level of the process of radiocesium adsorption and interaction with clay mineral particles have improved substantially over the past several years, triggered by pressing social issues such as management of huge amounts of waste soil accumulated after the Fukushima Dai–ichi nuclear power plant accident. In particular, computer–based molecular modeling supported by advanced hardware and algorithms has proven to be a powerful approach. Its application can now generally encompass the full complexity of clay particle adsorption sites from basal surfaces to interlayers with inserted water molecules, to edges including fresh and weathered frayed ones. On the other hand, its methodological schemes are now varied from traditional force–field molecular dynamics on large–scale realizations composed of many thousands of atoms including water molecules to first–principles methods on smaller models in rather exacting fashion. In this article, we overview new understanding enabled by simulations across methodological variations, focusing on recent insights that connect with experimental observations, namely: 1) the energy scale for cesium adsorption on the basal surface, 2) progress in understanding the structure of clay edges, which is difficult to probe experimentally, 3) cesium adsorption properties at hydrated interlayer sites, 4) the importance of the size relationship between the ionic radius of cesium and the interlayer distance at frayed edge sites, 5) the migration of cesium into deep interlayer sites, and 6) the effects of nuclear decay of radiocesium. Key experimental observations that motivate these simulation advances are also summarized. Furthermore, some directions toward future solutions of waste soil management are discussed based on the obtained microscopic insights.



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The spatial and developmental expression of mouse Vwa8 (von Willebrand domain-containing protein 8)

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Publication date: Available online 13 April 2018
Source:Gene Expression Patterns
Author(s): Brian S. Grewe, Janet E. Richmond, David E. Featherstone
The Drosophila gene c12.2 was isolated in a screen examining mRNA binding proteins. Drosophila c12.2 is the mouse Vwa8 homolog. Various genome-wide associated studies have linked human Vwa8 to both neurological and oncological pathologies, which include autism, bipolar disorder, comorbid migraine, and acute myeloid leukemia, however, the function and role of the VWA8 protein remain poorly understood. To further analyze the Vwa8 gene in mouse, gene structure, protein homology modeling, and gene expression patterns were examined throughout mouse development. Our analyses indicate that the mouse Vwa8 gene produces two transcripts; the full-length Vwa8a is highly expressed relative to the truncated Vwa8b transcript across all developmental time points and tissues analyzed. Protein homology modeling indicates that VWA8a belongs to a novel protein superfamily containing both the midasin and cytoplasmic dynein 1 heavy chain 1 proteins. These data establish the development timeline and expression profile for both Vwa8a and Vwa8b, paving the way for future studies to determine the cellular role(s) of this highly conserved protein family.



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