Πέμπτη, 30 Νοεμβρίου 2017

Proximal total splenic artery embolization for refractory hepatic encephalopathy

Abstract

A Japanese woman with a history of Kasai operation for biliary atresia had living-donor liver transplantation at the age of 22. The first episode of refractory HE and late cellular rejection was treated by a high dose of methylprednisolone. The second episode of refractory HE was treated by balloon-occluded retrograde transvenous obliteration for a spleno-renal shunt. However, the third episode of refractory HE occurred 11 years after liver transplantation. The liver cirrhosis and hypersplenism were present with a Child–Pugh score of C-10. Although portal vein flow was hepatopetal, superior mesenteric vein flow regurgitated. We performed proximal total splenic artery embolization (TSAE). Superior mesenteric vein flow changed to a hepatopetal direction and she became clear. At a year after proximal TSAE, her spleen volume had decreased to 589 mL (20% decrease) on computed tomography. She is well and has a Child–Pugh score of 8 without overt HE. We report the first case of refractory HE treated by proximal TSAE that is a possible less invasive treatment option for a selected patient.



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Liver Function Tests “Gone Viral”: Acute Hepatitis of Uncertain Cause



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Editorial Board



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Efficacy of Rebamipide in Organic and Functional Dyspepsia: A Systematic Review and Meta-Analysis

Abstract

Objective

The role of gastritis in dyspepsia remains controversial. We aimed to examine the efficacy of rebamipide, a gastric mucosal protective agent, in both organic and functional dyspepsia.

Design

A systematic review and meta-analysis was performed. The following databases were searched using the keywords ("rebamipide" OR "gastroprotective agent*" OR "mucosta") AND ("dyspepsia" OR "indigestion" OR "gastrointestinal symptoms"): PubMed, Wed of Science, Embase, CINAHL, Cochrane Clinical Trials Register. The primary outcome was dyspepsia or upper GI symptom score improvement. Pooled analysis of the main outcome data were presented as risk ratio (RR) for dichotomous data and standardized mean difference (SMD) for continuous data.

Results

From an initial 248 records, 17 randomised controlled trial (RCT) publications involving 2170 subjects (1224 rebamipide, 946 placebo/control) were included in the final analysis. Twelve RCTs were conducted in subjects with organic dyspepsia (peptic ulcer disease, reflux esophagitis or NSAID-induced gastropathy) and five RCTs were conducted in patients with functional dyspepsia (FD). Overall, dyspepsia symptom improvement was significantly better with rebamipide compared to placebo/control drug (RR 0.77, 95% CI = 0.64–0.93; SMD −0.46, 95% CI = −0.83 to −0.09). Significant symptom improvement was observed both in pooled RR and SMD in subjects with organic dyspepsia (RR 0.72, 95% CI = 0.61–0.86; SMD −0.23, 95% CI = −0.4 to −0.07), while symptom improvement in FD was observed in pooled SMD but not RR (SMD −0.62, 95% CI = −1.16 to −0.08; RR 1.01, 95% CI = 0.71–1.45).

Conclusion

Rebamipide is effective in organic dyspepsia and may improve symptoms in functional dyspepsia.



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Impact of Bacterial Translocation on Hepatopulmonary Syndrome: A Prospective Observational Study

Abstract

Background/Aims

Hepatopulmonary syndrome (HPS) is characterized by a defect in oxygenation induced by pulmonary vascular dilatation in cirrhosis. While severe HPS is responsible for a high rate of mortality, the prevalence and pathophysiology of HPS are not fully elucidated. We evaluated the prevalence and pathophysiology of HPS in patients with cirrhosis.

Methods

A total of 142 patients with cirrhosis who underwent saline-agitated contrast echocardiography were enrolled in this prospective observational study. HPS was defined by positive findings on contrast echocardiography, cirrhosis, and the presence of an oxygenation defect (alveolar–arterial oxygen gradient > 15 mmHg). HPS grades from 0 to 3 were assigned based on the density and spatial distribution of microbubbles in the left ventricle. The primary endpoint was the prevalence of HPS. The secondary endpoints included clinical characteristics and levels of lipopolysaccharide (LPS), LPS-binding protein (LBP), nitric oxide, and endothelin-1 in HPS.

Results

Fifty-nine patients (41.5%) were diagnosed with HPS (grade 1: 24, grade 2: 23, and grade 3: 12 patients). The mean levels of LPS (0.36 ± 0.02, 1.02 ± 0.18, 2.86 ± 0.77, and 6.56 ± 1.46 EU/mL, p < 0.001) and LBP (7026 ± 3336, 11,445 ± 1247, 11,947 ± 1164, and 13,791 ± 2032 ng/mL, p = 0.045) were found to be increased according to HPS grade (negative, grade 1–3). Endothelin-1 levels were significantly elevated according to HPS grade (1.83 ± 0.17, 2.62 ± 0.22, 3.69 ± 0.28, and 4.29 ± 0.34 pg/mL, p < 0.001), demonstrating a significant difference between each grade (p < 0.05).

Conclusions

HPS is a common complication with a prevalence of 41.5% in patients with cirrhosis. Bacterial translocation and portal pulmonary vascular dilatation are key mechanism involved in the progression of HPS.



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Issue Information



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Using proprioception to get a better grasp on embodiment

Abstract

We interact with, interpret, and understand the world around us through our senses. We see our environment, touch the things in it, feel the ground beneath our feet, and know how we move within our surroundings.

This article is protected by copyright. All rights reserved



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Prior automatic posture and activity identification improves physical activity energy expenditure prediction from hip-worn triaxial accelerometry.

Accelerometry is increasingly used to quantify physical activity (PA) and related energy expenditure (EE). Linear regression models designed to derive PAEE from accelerometry-counts have shown their limits, mostly due to the lack of consideration of the nature of activities performed. Here we tested whether a model coupling an automatic activity/posture recognition (AAR) algorithm with an activity-specific count-based model, developed in 61 subjects in laboratory conditions, improved PAEE and total EE (TEE) predictions from hip-worn triaxial-accelerometer (ActigraphGT3X+TM) in free-living conditions. Data from two independent subject groups of varying body mass index and age were considered: 20 subjects engaged in a 3h urban-circuit, with activity-by-activity reference PAEE from combined heart-rate and accelerometry monitoring (ActiheartTM); and 56 subjects involved in a 14-day trial, with PAEE and TEE measured using the doubly-labeled-water method. PAEE was estimated from accelerometry using the activity-specific model coupled to the AAR algorithm (AAR-model), a simple linear model (SLM), and equations provided by the activity-devices companion -software of used activity-devices (Freedson and ActiheartTM models). AAR-model predictions were in closer agreement with selected references than those from other count-based models both for PAEE during the urban-circuit (RMSE=6.19 vs 7.90 for SLM and 9.62 kJ.min-1 for Freedson) and for EE over the 14-day trial, reaching ActiheartTM performances in the latter (PAEE: RMSE=0.93 vs 1.53 for SLM, 1.43 for Freedson, 0.91 MJ.day-1 for Actiheart; TEE: RMSE=1.05 vs 1.57 for SLM, 1.70 for Freedson, 0.95 MJ.day-1 for ActiheartTM). Overall, the AAR-model resulted in a 43% increase of daily PAEE variance explained by accelerometry predictions.



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Case Studies in Physiology: Exercise-induced diaphragm fatigue in a paralympic champion rower with spinal cord injury

Introduction. The aim of this case report was to determine whether maximal upper-body exercise was sufficient to induce diaphragm fatigue in a Paralympic champion adaptive rower with low-lesion spinal cord injury (SCI). Case Presentation. An elite arms-only oarsman (age 28 y, stature 1.89 m, mass 90.4 kg) with motor-complete SCI (T12) performed a 1000 m time-trial on an adapted rowing ergometer. Exercise measurements comprised pulmonary ventilation and gas exchange, diaphragm EMG-derived indices of neural respiratory drive and intrathoracic pressure-derived indices of respiratory mechanics. Diaphragm fatigue was assessed by measuring pre- to post-exercise changes in the twitch transdiaphragmatic pressure (Pdi,tw) response to anterolateral magnetic stimulation of the phrenic nerves. The time-trial (248 ± 25 W, 3.9 min) elicited a peak O2 uptake of 3.46 L·min–1 and a peak pulmonary ventilation of 150 L·min–1 (57% MVV). Breath-to-stroke ratio was 1:1 during the initial 400 m and 2:1 thereafter. The ratio of inspiratory transdiaphragmatic pressure to diaphragm EMG (neuromuscular efficiency) fell from rest to 600 m (16.0 vs. 3.0). Potentiated Pdi,tw was substantially reduced (–33%) at 15-20 min post-exercise, with only partial recovery (–12%) at 30-35 min. Conclusions. This is the first report of exercise-induced diaphragm fatigue in SCI. The decrease in diaphragm neuromuscular efficiency during exercise suggests that the fatigue was partly due to factors independent of ventilation (e.g., posture and locomotion).



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Alteration in upper airway dilator muscle co-activation during sleep: comparison of patients with OSA and healthy subjects.

In patients with OSA, substantial increases in genioglossus (GG) activity during hypopneas/apneas usually fail to restore normal airflow. We have previously suggested that sleep-induced alteration in tongue muscle coordination may explain this finding, as retractor muscles co-activation was reduced during sleep, as compared to wakefulness. The present study was undertaken to evaluate if these alterations in dilator muscle activation during sleep play a role in the pathogenesis of OSA, and whether co-activation of additional peri-pharyngeal muscles (non-GG muscles: styloglossus, geniohyoid, sternohyoid and sterno-cleido-mastoid) is also impaired during sleep. We compared GG and non-GG muscles EMG activity in 8 OSA patients and 12 healthy subjects during wakefulness, while breathing through inspiratory resistors, to the activity observed during sleep, towards the end of flow limitation, before arousal, at equivalent esophageal pressures. During wakefulness, resistive breathing triggered increases in both GG and non-GG muscles activity. During sleep, flow limitation was associated with increases in GG EMG that reached, on the average, more than two-fold the level observed while awake. In contrast, EMGs of the non-GG muscles, recorded simultaneously, reached on the average only about 2/3 the wakefulness level. We conclude that during sleep GG activity may increase to levels that exceed substantially those sufficient to prevent pharyngeal collapse during wakefulness, whereas other peri-pharyngeal muscles do not co-activate during sleep in both OSA and healthy subjects. We speculate that upper airway muscle dyssynchrony during sleep may explain why GG EMG activation fails to alleviate flow limitation and stabilize airway patency during sleep.



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Impact of training state on fasting-induced regulation of adipose tissue metabolism in humans

Recruitment of fatty acids from adipose tissue is essential during fasting. However, the molecular mechanisms behind fasting-induced metabolic regulation in human adipose tissue and the potential impact of training state in this are unknown. Therefore, the aim of the present study was to investigate 1) fasting-induced regulation of lipolysis and glyceroneogenesis in human adipose tissue as well as 2) the impact of training state on basal oxidative capacity and fasting-induced metabolic regulation in human adipose tissue. Untrained (VO2max < 45ml·min-1·kg-1) and trained subjects (VO2max > 55ml·min-1·kg-1) fasted for 36h and abdominal subcutaneous adipose tissue biopsies were obtained 2, 12, 24 and 36h after a standardized meal. Adipose tissue OXPHOS, phosphoenolpyruvate carboxykinase and pyruvate dehydrogenase (PDH) E1α protein as well as PDH kinase (PDK) 2, PDK4 and PDH phosphatase 2 mRNA content were higher in trained subjects than untrained subjects. In addition, trained subjects had higher adipose tissue hormone sensitive lipase Ser660 phosphorylation and adipose triglyceride lipase protein content as well as higher plasma free fatty acids concentration than untrained subjects during fasting. Moreover, adipose tissue PDH phosphorylation increased with fasting only in trained subjects. Taken together, trained subjects seem to possess higher basal adipose tissue oxidative capacity as well as higher capacity for regulation of lipolysis, glyceroneogenesis and substrate availability in adipose tissue than untrained subjects.



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Effects of Transcranial Direct Current Stimulation of Rat Cerebral Structures on Pentylenetetrazole-Induced Seizures

In rats with a kindling syndrome induced by injections of pentylenetetrazole (PTZ, 30.0 mg/kg, i.p., for three weeks), the latent periods of seizures induced by test doses of PTZ (30 mg/kg) were, on average, 37.5% greater than those in the control (P < 0.05) after transcranial direct current stimulation (TDCS, 600 μA, 15.0 min, cathode on the skull surface) oriented to the cerebellar cortex. Such stimulation prevented initiation of generalized clonico-tonic seizures; the duration of ictal discharges in brain structures decreased, on average, by 42.1% (P < 0.02). The latent periods of acute PTZ-induced (60.0 mg/kg) seizures in rats that were not subjected to the kindling procedure were 33.5% longer after TDCS (P < 0.05) than those in the control. Similar stimulation focused on the frontal cerebral cortex prevented the development of generalized seizures in 50% of the kindling rats (P < 0.05).



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Control of the Functionality of the Brachial Plexus during Robot-Assisted Transaxillary Thyroid Surgery

At present, such a type of endoscopic surgery as robot-assisted transaxillary thyroidectomy has become available. In this case, traumatization of the brachial plexus is a rare but possible complication. For the control of the function of the brachial plexus during the above operation we used monitoring of somatosensory evoked potentials (SSEPs) induced by stimulation of the median nerve. Fifteen patients (14 women and one man) were included in this study. All interventions were robot-assisted transaxillary thyroidectomies using the daVinci SI Surgical System. We found that such surgery induced mild but significant increases in the latency of the cortical N20 potential, especially when the tissue was tensioned by the autostatic retractor. The latency prolongation was a valuable signal given to the surgeon, followed by repositioning or loosening of the retractor. In the examined group, no significant decreases in the amplitude of the N20 potential were observed. We conclude that SSEP monitoring during robotic thyroid surgery is an available and safe method providing valuable information on the functional integrity of somatosensory pathways during surgical maneuvers.



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Effects of a Propolis Extract on the Viability of and Levels of Cytoskeletal and Regulatory Proteins in Rat Brain Astrocytes: an In Vitro Study

A potential for the use of propolis in preventive and therapeutic purposes has been acknowledged, but little attention has been paid to estimation of possible propolis cytotoxicity with respect to astrocytes. We tried to estimate how a propolis ethanol extract (PEE) affects rat brain astrocytes in vitro and also to uncover crucial molecular targets of the PEE action. Primary astrocytes were exposed to PEE in doses of 10, 25, or 100 μg/ml for 24 h, and then the cell viability was monitored by MTT assay. Levels of glial fibrillary acidic protein (GFAP), transcriptional nuclear factor-κB (NF-κB), poly(ADPribose) polymerase (PARP), and angiostatins were measured using Western blot to identify the molecular mechanisms underlying cell responses to PEE. The PEE treatment exerted a dose-dependent cytotoxic effect on cultured astrocytes. The PEE modulated astrocyte signaling pathways through inducing the expression of NF-κB and PARP. At the same time, the PEE stimulated GFAP synthesis and fibrillogenesis, which was indicative for activation of astrocytes preceding their suppression. The PEE significantly increased the production of angiostatin isoforms by astrocytes, thus contributing to an antiangiogenic potential of these cells. In summary, our results indicated that exposure to the PEE exerts certain cytotoxic effects on astrocytes in a dose-dependent manner; these effects are realized through modulation of cytoskeleton rearrangements and pro-apoptotic signaling pathways. A widely available, safe, and inexpensive substance, propolis, and its components and derivatives may be used in the prevention and treatment of neuronal impairments, including malignant tumors and neurodegenerative disorders associated with excessive astrocytic activation.



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Effects of Beta-Adrenergic Blockade on Diabetes-Induced Neurobehavioral Alterations in Mice

Neurobehavioral activities were estimated in three groups of male albino mice using the open field, elevated plus maze, light/dark board, and hole-board tests. The control group included intact animals, while alloxan-induced diabetes was evoked in the other two groups (single i.p. injection of 120 mg/kg alloxan). In the third group, a nonspecific beta-adrenoreceptor antagonist, propranolol, was i.p. injected (40 mg/kg) before the induction of diabetes. In diabetic mice, all neurobehavioral indices tested in the four above-mentioned tests were significantly (P< 0.05) smaller than those in the control group. The frequencies of rearings and grooming episodes in the open field, number of entries into the open arms and time spent in these arms in the elevated plus maze test, and number of head dips in the hole-board test demonstrated the most intense drops (more than twofold). Pretreatment with propranolol provided significant (P < 0.05) normalization of all neurobehavioral indices in diabetic mice; such normalization with respect to the locomotion intensity, frequency of grooming, time spent in the open arms, and both indices in the light/dark board was nearly complete. Thus, diabetes in the animal model used is accompanied by the development of the state of abnormally high anxiety. The activity of the betaadrenergic system is noticeably involved in the formation of this state; pharmacological blocking of beta-adrenoreceptors provides significant anxiolytic effects.



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Expression of Vascular Endothelial Growth Factor after Transfection of Human Neural Stem Cells with the Lentiviral Vector Encoding the VEGF165 Gene

We examined the expression of vascular endothelial growth factor (VEGF) and characteristics of human neural stem cells after transfection with the lentiviral vector encoding the VEGF 165 gene. The latter gene was amplified from the human breast cancer cell line MCF-7 using RT-PCR; then the target gene was cloned into the pCDH–CMV–MCS-EF1-copGFP, a lentiviral expressing plasmid. After transformation, enzyme digestion led to a correct length of the VEGF165 gene, and DNA sequencing analysis confirmed that the VEGF165 gene sequence was exactly the same as that reported by the GeneBank. Then, the recombinant lentivirus produced by 293T cells and packaging plasmids were transfected into fourthpassage human neural stem cells (NSCs). One week after transfection with pCDH-VEGF165, NSCs expressed VEGF stably, and their proliferation ability significantly increased. Furthermore, human NSCs kept their characteristics and multiple differentiation activity after transfection. Our results indicate that human NSCs can express vascular endothelial growth factor highly and stably via transfection with the lentiviral vector encoding the VEGF165 gene, which may be useful for future research on function recovery after stroke.



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Expression of Calcium-Binding Proteins, Calbindin D28k and Calretinin, in the Frog Taste Receptor Structures

Considering that information on the expression of calcium-binding proteins (CaBPs) in different cells of the taste receptors is rather limited, we investigated the distribution of such proteins, calbindin D28k (CB) and calretinin (CR), in the taste disc (TD) of the frog Lithobates catesbeianus. Western blot analysis revealed that CB and CR are expressed in cells of the fungiform papillae. CB-immunoreactive (ir) and CR-ir cell somata were located in the middle layer of the TD. Most CB-ir and CR-ir cells possessed one rod-shaped apical process and one basal process; in some cells there were several extended basal processes. Apical processes of CR-ir cells were thinner than those of CB-ir units, and CR-ir nerve fibers were ramified in the lamina propria directly below the TD. Most CR-ir fiber branches surrounded the TD; however, some penetrated this region, with both types of branches approaching the surface. CB and CR immunoreactivities did not co-occur in TD cells. In the TDs examined, the number of CB-ir cells was significantly greater than that of CR-ir units. Our observations suggest that CB-ir and CR-ir cells in the frog TD correspond to type-II and type-III cells, respectively.



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Hysteresis in EMG Activity of Muscles of the Human Upper Limb at Rotations of the Isometric Effort Vector

We studied the dynamics of EMG activity of muscles of the human upper limb under conditions of changes in the direction of a vector of the isometric effort created by the hand. Averaged EMG activities of seven muscles of the shoulder and shoulder girdle at nine combinations of the angles in the shoulder and elbow joints were evaluated. Mean values of the angles of the maximum activity (AMAs) of the muscle during slow counterclockwise and clockwise rotations of the above-mentioned vector were compared. A significant dependence of the AMA in a considerable part of the muscles on the direction of the target trajectory hodograph by the vector of the generated effort was observed; this dependence is, most likely, due to the manifestations of hysteresis in the activity of the examined muscles. In the case where the analysis was performed in more detail, AMA values for the mm. triceps brachii, pectoralis major, and deltoideus pars scapularis were significantly greater at clockwise rotation, while those for the m. brachioradialis demonstrated an opposite trend.



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The presynaptic scaffolding protein Piccolo organizes the readily releasable pool at the calyx of Held

Abstract

Synaptic vesicles (SVs) are released at the active zone (AZ), a specialized region of the presynaptic plasma membrane organized by a highly interconnected network of multi-domain proteins called cytomatrix of the active zone (CAZ). Two core components of the CAZ are the large, highly homologous scaffolding proteins Bassoon and Piccolo, whose function is not well understood. In order to investigate their role in synaptic transmission, we established the shRNA-mediated in vivo knock-down (KD) of Bassoon and Piccolo at the rat calyx of Held synapse. KD of Bassoon and Piccolo, separately or simultaneously, did not affect basic SV release. However, short-term depression (STD) was prominently increased by the KD of Bassoon while KD of Piccolo only had a minor effect. The observed alterations in STD were readily explained by reduced SV replenishment in synapses deficient in either of the proteins. Thus, the regulation of SV refilling during ongoing synaptic activity is a shared function of Bassoon and Piccolo, although Bassoon appears to be more efficient. Moreover, we observed the recruitment of slowly releasing SVs of the readily-releasable pool (RRP), which are normally not available for AP-induced release, during high frequency stimulation in Piccolo-deficient calyces. Therefore, our results suggest a novel and specific role for Piccolo in the organization of the subpools of the RRP.

This article is protected by copyright. All rights reserved



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Fasciculation: Does distance really matter?

The needle electromyography (EMG) discloses fasciculation potentials (FPs) so frequently in amyotrophic lateral sclerosis (ALS) that neurophysiologists are reluctant to accept this diagnosis unless FPs are shown (de Carvalho et al., 2008). Their presence has also been associated with hyperexcitability in ALS (de Carvalho et al., 2017), recently confirmed by the finding that the most excitable lower motor neuron is the one more susceptible to fasciculate (de Carvalho and Swash, 2017). Moreover, the presence of FPs is a very early finding in muscles of ALS patients (Lambert, 1969) and antedates morphological changes of the motor units (MUs) or signs of end-plate dysfunction (de Carvalho and Swash, 2013).

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Cortical inhibitory function in cervical dystonia

Dystonia is a hyperkinetic movement disorder characterized by the presence of involuntary muscle contractions producing abnormal twisting movements and/or postures(Albanese et al., 2013). In primary or isolated dystonia(Albanese et al., 2013), widespread and generalized loss of inhibition within the central nervous system is a key pathological finding(Berardelli et al., 1998; Hallett, 2011). The inhibitory deficit, however, may not reflect a single underlying neurophysiological mechanism. Rather, inhibition may refer to a range of neural mechanisms.

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Improve inventory and asset management with new technology

EMS panelists at AAA say embracing new technology to manage supplies helped them reduce waste, cut costs and improve patient care

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A nationwide population-based study of common bile duct stone recurrence after endoscopic stone removal in Korea

Abstract

Background

There is no consensus whether patients who underwent endoscopic common bile duct (CBD) stone removal should be followed up periodically and whether patients with gallbladder (GB) stones should undergo cholecystectomy. Thus, this study aimed to investigate the recurrence rate of CBD stones and the difference in recurrence rate according to cholecystectomy.

Methods

We conducted a population-based study using the National Health Insurance database. Patients diagnosed with CBD stones and with procedure registry of endoscopic stone removal were included. The primary outcome was the recurrence rate of CBD stones. The secondary outcome was the difference in recurrence rate of CBD stones according to cholecystectomy.

Results

A total of 46,181 patients were identified. The mean follow-up was 4.2 years. The first CBD stone recurrence occurred in 5228 (11.3%) patients. The cumulative first recurrence rate was low. However, the second and third recurrence rates were 23.4 and 33.4%, respectively. The cumulative second and third recurrence rates were high and gradually increased with time. The recurrence rate in the non-cholecystectomy group was higher than that in the cholecystectomy group (p < 0.0001). The relative risk for CBD stone recurrence in the non-cholecystectomy group was higher in younger patients, with 3.198 in patients < 50 years, 2.371 in 50–59 years, 1.618 in 60–69 years, and 1.262 in ≥ 70 years (p < 0.0001).

Conclusions

Regular follow-up is not routinely recommended for patients with first-time endoscopic stone removal, but is recommended for patients with recurrent stones. Cholecystectomy is recommended for patients with GB stones who are younger than 70 years.



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Pharmacogenetics of thiopurines for inflammatory bowel disease in East Asia: prospects for clinical application of NUDT15 genotyping

Abstract

The thiopurine drugs 6-mercaptopurine (6-MP) and azathiopurine (AZA) are widely used to treat inflammatory bowel disease. However, the incidence of adverse reactions is high, particularly in Asia, and the mechanisms of toxicity in Asian populations remain unclear. Thiopurine S-methyltransferase (TPMT) is a well-known enzyme that inactivates AZA or 6-MP through methylation and is one of the few pharmacogenetic predictors used in clinical settings in Western countries. Individuals carrying TPMT-deficient genetic variants require reduced drug doses, but this treatment modification is are not applicable to East Asian populations. Several genes code thiopurine-metabolizing enzymes, including TPMT, multidrug-resistance protein 4, and inosine triphosphatase. These genes have been studied as candidate pharmacogenetic markers; however, it remains unclear why Asian populations seem to be more intolerant than other ethnic groups to a full dose of thiopurines. A genome-wide association approach to identify Asian-specific pharmacogenetic markers in Korean patients with Crohn's disease revealed that a non-synonymous single nucelotide polymorphism in nucleoside diphosphate-linked moiety X-type motif 15 (NUDT15) which causes p.Arg139Cys was strongly associated with thiopurine-induced early leukopenia. Six common haplotypes of NUDT15 were reported, and five variants showed medium-to-low enzyme activities, compared with the wild haplotype. NUDT15 hydrolyzes the thiopurine active metabolites 6-thio-GTP and 6-thio-dGTP; variants of NUDT15 had lower enzyme activities, causing higher levels of thiopurine active metabolites, resulting in thiopurine-induced leukopenia. In clinical application, NUDT15 genotyping is a good candidate for predicting thiopurine toxicity in East Asian populations. However, the association of NUDT15 diplotypes with thiopurine toxicity remains unclear. Further analyses with large cohorts to confirm the clinical effects of each haplotype are planned.



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ZICO streamlines electrical accessory organization and transport

YARDLEY, Pa. — Ziamatic Corp. (ZICO) has developed a better, faster, easier way to keep your electrical accessories organized and transportable—the new Cord & Adapter Holder, Model QM-CADH. The innovative Cord & Adapter Holder secures electrical cord, adapters, and pigtails inside the compartment and keeps them organized and easy to locate on the scene. A sturdy, hook & loop ...

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Moped crash victim who lost her leg thanks responders who saved her

By EMS1 Staff OAK BLUFFS, Mass. — A college student who lost her leg in a moped crash thanked the first responders who saved her. Vineyard Gazette reported that Noelle Lambert rented a moped with her friend in July 2016 to take a tour of Martha's Vineyard. Lambert lost control and crashed into a dump truck, resulting in the loss of her left leg. The University of Massachusetts lacrosse player ...

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Inside EMS Podcast: How to develop into an EMS role model and mentor

Download this podcast on iTunes, SoundCloud or via RSS feed In this Inside EMS Podcast episode, co-hosts Chris Cebollero and Kelly Grayson reflect on the life of Lou Jordan. Chris and Kelly ask: who will be the next EMS role model and mentor of the future" They give some practical advice in getting to the next level and securing the influence needed to inspire a career field. Learn more about the EMS1 ...

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The role of ocelli in cockroach optomotor performance

Abstract

Insect ocelli are relatively simple eyes that have been assigned various functions not related to pictorial vision. In some species they function as sensors of ambient light intensity, from which information is relayed to various parts of the nervous system, e.g., for the control of circadian rhythms. In this work we have investigated the possibility that the ocellar light stimulation changes the properties of the optomotor performance of the cockroach Periplaneta americana. We used a virtual reality environment where a panoramic moving image is presented to the cockroach while its movements are recorded with a trackball. Previously we have shown that the optomotor reaction of the cockroach persists down to the intensity of moonless night sky, equivalent to less than 0.1 photons/s being absorbed by each compound eye photoreceptor. By occluding the compound eyes, the ocelli, or both, we show that the ocellar stimulation can change the intensity dependence of the optomotor reaction, indicating involvement of the ocellar visual system in the information processing of movement. We also measured the cuticular transmission, which, although relatively large, is unlikely to contribute profoundly to ocellar function, but may be significant in determining the mean activity level of completely blinded cockroaches.



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Ford F-150 vs. ambulance: Who wins?

Braun Ambulances put the two vehicles head to head to see how safe an ambulance actually is

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Pharmacogenetics: A strategy for personalized medicine for autoimmune diseases

Abstract

For many years, a considerable number of patients with autoimmune diseases (ADs) have suffered from a lack of drug response and drug-related toxicity. Despite the emergence of new therapeutic options such as biological agents, patients continue to struggle with these problems. Unfortunately, new challenges, including the paradoxical effects of biological drugs, have complicated the situation. In recent decades, efforts have been made to predict drug response as well as drug-related side-effects. Thanks to the many advances in genetics, evaluation of markers to predict drug response/toxicity before the initiation of treatment may be an avenue toward personalizing treatments. Implementing pharmacogenetics and pharmacogenomics in the clinic could improve clinical care; however, obstacles remain to effective personalized medicine for ADs. The present study attempted to clarify the concept of pharmacogenetics/pharmacogenomics for ADs. After an overview on the pathogenesis of the most common types of treatments, this paper focuses on pharmacogenetic studies related to the selected ADs. Bridging the gap between pharmacogenetics and personalized medicine is also discussed. Moreover, the advantages, disadvantages and recommendations related to making personalized medicine practical for ADs have been addressed.

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Targeted Gene Sequencing and Whole-Exome Sequencing in Autopsied Fetuses with Prenatally Diagnosed Kidney Anomalies

Abstract

Identification of fetal kidney anomalies invites questions about underlying causes and recurrence risk in future pregnancies. We therefore investigated the diagnostic yield of next-generation sequencing in fetuses with bilateral kidney anomalies and the correlation between disrupted genes and fetal phenotypes.

Fetuses with bilateral kidney anomalies were screened using an in-house-designed kidney-gene panel. In families where candidate variants were not identified, whole-exome sequencing was performed. Genes uncovered by this analysis were added to our kidney-panel.

We identified likely deleterious variants in 11 of 56 (20%) families. The kidney-gene analysis revealed likely deleterious variants in known kidney developmental genes in six fetuses and TMEM67 variants in two unrelated fetuses. Kidney histology was similar in the latter two fetuses – presenting a distinct prenatal form of nephronophthisis. Exome sequencing identified ROBO1 variants in one family and a GREB1L variant in another family. GREB1L and ROBO1 were added to our kidney-gene panel and additional variants were identified.

Next-generation sequencing substantially contributes to identifying causes of fetal kidney anomalies. Genetic causes may be supported by histological examination of the kidneys. This is the first time that SLIT-ROBO signaling is implicated in human bilateral kidney agenesis.

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Introduction to the Special Issue on Healthy Start



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Preserving the Shoulder Function of an Elite Para Triathlete: A Case Report

Abstract Shoulder pain in wheelchair users that participate in competitive adaptive sports can be a troublesome condition. Shoulder pain not only affects athletic performance, but also functional activities such as wheelchair propulsion and weight bearing during transfers. Managing pain in these athletes thus presents a unique challenge due to the difficulty in achieving relative rest and the need to modify athletic shoulder-focused rehabilitation strategies. In all athletes, it is vital to establish an early, accurate diagnosis and optimize conservative treatment prior to considering surgical interventions in order to avoid excessive shoulder-related morbidity, loss of function, and worse – loss of independence. Author Disclosures All authors disclose no competing interests, funding/grants, or financial benefits to the authors for this paper. All authors disclose no prior manuscript publication of this current paper. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Case report describing a new swallowing method to improve pharyngeal passage of a bolus by creating negative pressure in the esophagus—vacuum swallowing

No abstract available

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Shifting Practice in the Diagnosis of Paediatric Coeliac Disease in English District General Hospitals

No abstract available

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Hepatic Parenchymal Injury in Crigler-Najjar Type I

ABSTRACT Background: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate UGT1A1 activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Our aim was to investigate the association between hepatic histology and disease phenotype in CNI. Methods: We extracted data from the medical record at the time of liver transplant from 22 patients with CNI at the Children's Hospital of Pittsburgh, and reviewed explant histology. Continuous data were normally distributed, are presented as mean (±1SD), and analyzed using two-tailed Student's t-test. Categorical data were analyzed using the chi-square test. Results: Both alanine transaminase (ALT; mean 87.4 IU/L) and aspartate transaminase (AST; mean 54.6 IU/L) were elevated. Nine (41%) of 22 explants had significant fibrosis. Pericentral (n = 5), periportal (n = 2), and mixed (n = 2) patterns of fibrosis occurred. A significant difference in mean age of subjects with fibrotic versus non-fibrotic livers (16.1 yrs vs 10.5 yrs; p = 0.02) was seen. There were no indices of synthetic liver dysfunction or portal hypertension. Neither a history of gallstone disease nor excess weight appeared to contribute to the development of fibrosis. Conclusion: For the first time, we report a 41% prevalence of clinically silent, yet histologically significant fibrosis among subjects with Crigler-Najjar type 1. Risk for fibrosis appears to accrue with time, indicating that earlier intervention may be prudent when considering alternative treatments such as hepatocyte transplant, auxiliary liver transplant, or viral gene therapy. Address correspondence and reprint requests to James E. Squires, MD, MS, Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh, One Children's Hospital Drive, 6th Floor FP, 4401 Penn Avenue, Pittsburgh PA 15224 (e-mail: James.Squires2@chp.edu). Received 21 July, 2017 Accepted 4 November, 2017 Financial Support: The authors report no external sources of funding were utilized for the purposes of the research presented. Conflicts of Interest: Dr. McKiernan declares an advisory board position for Audentes Therapeutics. Author Contributions: Ellen Mitchell - background literature review, data collection, data analysis, drafting article, critical revision, approval of article; Sarangarajan Ranganathan – data collection, drafting article, critical revision, approval of article; Patrick McKiernan - drafting article, critical revision, approval of article; Robert H Squires - critical revision, approval of article; Kevin Strauss - critical revision, approval of article; Kyle Soltys - critical revision, approval of article; George Mazariegos - critical revision, approval of article; James E Squires - background literature review, data collection, data analysis, drafting article, critical revision, approval of article. © 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Pneumatosis Cystoides Intestinalis

No abstract available

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Safety and Efficacy of Reslizumab for Children and Adolescents With Eosinophilic Esophagitis Treated Over Nine Years

ABSTRACT Background: Eosinophilic esophagitis (EoE) is a chronic disease characterized by infiltration of eosinophils in the esophageal epithelium. There are limited treatment options for EoE. Rationale: To evaluate the long term safety and efficacy of reslizumab (RSZ) in pediatric patients who received RSZ in a randomized controlled trial (RCT) and expanded access program. Methods: Records of patients who received RSZ in our center were reviewed. Patients received RSZ 2 mg/kg (or placebo) every 4 weeks as part of the RCT, open label extension (OLE), and compassionate use (CU). Data were analyzed as of their most recent evaluation in August 2017. Labwork, history, and examinations were conducted every 12 weeks. Biopsy results were compared from baseline (prior to RCT) and at the most recent evaluation. Adverse events (AE) were recorded. Results: 12 patients entered the RCT at our center. 6 patients completed the OLE. 4 received RSZ through CU. Between the RCT, OLE, and CU periods, patients received 549 doses of RSZ (median 37, range 2–116). No serious AE were attributed to RSZ. Symptoms improved on treatment: dysphagia (42% vs 0%); abdominal pain (58% vs 0%); heartburn (18% vs. 0%); vomiting (67% vs. 17%); reflux (58% vs. 0%). Median esophageal eosinophil count improved (35 eos/hpf vs 3, p value 

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Noninvasive Methods of Predicting Large Esophageal Varices in Children With Intrahepatic Portal Hypertension

ABSTRACT Objective: Esophageal variceal bleeding is a severe complication of portal hypertension. The standard diagnostic screening test and therapeutic procedure for esophageal varices is endoscopy, which is invasive in pediatric patients. This study aimed to evaluate the role of noninvasive parameters as predictors of large varices in children with intrahepatic portal hypertension. Methods: Participants included in this cross-sectional study underwent a screening endoscopy. Variceal size, red marks, and portal gastropathy were assessed and rated. Patients were classified into two groups: G1, with small or no varices; and G2, with large varices. The population consisted of 98 children with no history of gastrointestinal bleeding, with a mean age of 8.9 ± 4.7 years. The main outcome evaluated was the presence of large varices. Results: The first endoscopy session revealed the presence of large varices in 32 children. The best noninvasive predictors for large varices were platelets (AUROC 0.67; 95%CI, 0.57–0.78), the Clinical Prediction Rule (AUROC 0.65; 95%CI, 0.54–0.76), and risk score (AUROC 0.66; 95%CI: 0.56–0.76). The logistic regression model showed that children with a Clinical Prediction Rule value under 114 were 8.59 times more likely to have large varices. Risk scores higher than −1.2 also increased the likelihood of large varices (OR6.09; P = 0.014), as did a platelet count/spleen size z-score lower than 25 (OR 3.99; P = 0.043). The combination of these three tests showed a high negative predictive value. Conclusion: The Clinical Prediction Rule, the risk score, and the platelet count/spleen size z-score could be helpful in identifying cirrhotic children who may be eligible for endoscopy. Address correspondence and reprint requests to Marina R. Adami, MD, Rua Jari 671/812; Zip Code: 91350-170, Porto Alegre, Rio Grande do Sul, Brazil (e-mail: marinaadami2008@gmail.com). Received 29 May, 2015 Accepted 13 September, 2017 This article has been developed as a Journal CME Activity by NASPGHAN. Visit http://ift.tt/2ioSD8w to view instructions, documentation, and the complete necessary steps to receive CME credit for reading this article. The other authors report no conflicts of interest. Source of Support: Fundação de Incentivo a Pesquisa e Eventos do Hospital de Clínicas de Porto Alegre: FIPE-HCPA. Non-Financial Disclosure. © 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Vitamin B1 Deficiency Related to Excessive Soft Drink Consumption in Japan

ABSTRACT The aim of this study was to clarify the picture of vitamin B1 deficiency (VB1D) caused by excessive soft drink consumption in Japan. A nationwide survey of children with soft drink-induced VB1D was conducted using a structured research form. Patient information was obtained from two sources: training hospitals for board-certified pediatricians and those identified by a database search. We obtained data from 33 children. Twenty-one children had a non-nurturing home environment. The median duration of excessive soft drink intake was 3.5 months and the daily intake was 1,000 mL or more in 25 children. Infection was the most common reason for excessive soft drink consumption. Only four children had the classical triad of Wernicke encephalopathy. One child died, and twelve had neurological sequelae. A majority of children with soft drink-induced VB1D did not have a nurturing home environment. It is imperative to establish measures to prevent future cases of VB1D. Address correspondence and reprint requests to Akihisa Okumura, MD, PhD, Department of Pediatrics, Aichi Medical University, 1-1 Yazako Karimata, Nagakute, Aichi, 480-1195 Japan (e-mail: okumura.akihisa.479@mail.aichi-med-u.ac.jp). Received 8 August, 2017 Accepted 15 November, 2017 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). Conflicts of Interest and sourses of funding: MM has received lecture fees from MSD K.K and Accvie LLC, and received consulting fees from Daiichi Sankyo Co., Ltd. And Taisho Pharmaceutical Co., Ltd. The other authors have no competing interests relevant to this article to disclose. This study was supported by the Japanese Pediatric Society. The other authors report no conflicts of interest. © 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Pancreatic Enzyme Replacement Therapy use in infants with Cystic Fibrosis Diagnosed by Newborn Screening

ABSTRACT Objectives: To describe pancreatic enzyme practices during the first year of life in infants with cystic fibrosis (CF) and evaluate associations between dosing and outcomes, including growth and gastrointestinal symptoms. Methods: We analyzed data from a subset of infants who were in a prospective cohort study conducted at 28 US CF centers. Anthropometric measurements and medications were recorded at each visit. Diaries with infant diet, pancreatic enzyme replacement therapy (PERT) dosing, stool frequency and consistency and pain were completed by a parent/guardian for three days prior to each visit. Results: 231 infants were enrolled in the main study; 205 of these met criteria for pancreatic insufficiency. PERT dose between birth and 6 months was on average 1882 LU/kg/meal (range: 492–3727) and was similar between 6 months and 12 months (mean: 1842 LU/kg/mean, range: 313–3612). PERT dose had a weak, negative association with weight z-score at 3 and 6 months (r = −0.16, 95% CI = −0.29, −0.02 and r = −0.18, 95% CI = −0.31, −0.04, respectively) but not at 12 months. There was not a clear relationship between PERT dosing and number of stools/day, stool consistency or pain. 144 infants (70%) were placed on acid suppression medication. Weight z-score mean was 0.37 higher in infants using proton pump inhibitors exclusively vs those using histamine-2 blockers exclusively (95% CI = −0.02, 0.76, p = 0.06). Conclusions: We did not observe that centers with a higher PERT dosing strategy yielded greater clinical benefit than dosing at the lower end of the recommended range. Address correspondence and reprint requests to Sonya L. Heltshe, Seattle Children's Research Institute, M/S CW805B, PO BOX 5371, Seattle WA 98145-5005 (e-mail: Sonya.Heltshe@seattlechildrens.org; http://ift.tt/1e5kjHX). Received 18 July, 2017 Accepted 9 October, 2017 See Appendix, Supplemental Digital Content 1, http://ift.tt/2ArRHqU for BONUS Study Investigators 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). Funding/support: BONUS and its lead investigators were supported by CFFT BONUS11KO, NIH R01DK095738, NIH P30DK089507, NIH UL1TR000423, CFF LEUNG14GE0 and NIH P30 DK072482. Conflict of interest disclosures and Sources of Funding: Dr Gelfond has served as a consultant for Vertex, Abbvie and Chiesi Pharmaceuticals. Dr. Leung has served as a consultant for Vertex and receives research/grant support from Bristol Meyers Squibb, Gilead, Abbvie, and Roche pharmaceuticals outside of the submitted work. Dr. Ramsey discloses that over the past 3 years she has received grant support from the Cystic Fibrosis Foundation and the National Institutes of Health. She has been the principal investigator on contracts between Seattle Children's Hospital and the following companies: Aridis Pharmaceuticals, LLC, Celtaxsys, Kalobios, Flatley Discovery Labs, LLV, Vertex Pharmaceuticals Inc, Laurent Therapeutics, Inc, Nilvalis Therapeutics, Inc, and Synedgen, Inc. Dr Heubi has received personal fees from Alynlam Pharma and has financial interest in Asklepion Pharma LLC. No other disclosures are reported. Author Contributions: Design and Conduct of the Study: Gelfond, Heltshe, Borowitz, Leung, Heubi, Ramsey; Collection, management, analysis and interpretation of data: All authors; Preparation, review or approval of the manuscript: All authors; Statistical Analysis: Heltshe, Skalland, Kloster © 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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The Omes of Inflammatory Bowel Disease–A Primer for Clinicians

ABSTRACT Recent advances in high-throughput laboratory technologies and bioinformatics tools are redefining how we view inflammatory bowel disease (IBD). Instead of two diseases we now see a diverse set of molecular subtypes. Large scale investigation of the genome, exome, transcriptome, proteome, metabolome, microbiome, and epigenome are providing transformative insights into the pathophysiology of IBD, with the promise of accurately predicting prognosis and targeting therapy. Understanding these tools and their application is crucial to navigating the molecular era of IBD. This review aims to help the IBD clinician understand, appreciate and eventually incorporate this coming paradigm shift in order to improve the care of children with IBD. Address correspondence and reprint requests to Neal S. LeLeiko, MD PhD, Department of Pediatrics, Hasbro Children's Hospital/Rhode Island Hospital, MPH Rm 134, 593 Eddy Street, Providence RI 02903 (e-mail: Neal_LeLeiko@brown.edu;nleleiko@gmail.com). Received 31 July, 2017 Accepted 13 November, 2017 Disclosure of funding: There was no funding for the preparation of this manuscript. © 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Adaptation of New Colombian Food-based Complementary Feeding Recommendations Using Linear Programming

imageABSTRACT Objective: The aim of the study was to use linear programming (LP) analyses to adapt New Complementary Feeding Guidelines (NCFg) designed for infants aged 6 to 12 months living in poor socioeconomic circumstances in Bogota to ensure dietary adequacy for young children aged 12 to 23 months. Design: A secondary data analysis was performed using dietary and anthropometric data collected from 12-month-old infants (n = 72) participating in a randomized controlled trial. LP analyses were performed to identify nutrients whose requirements were difficult to achieve using local foods as consumed; and to test and compare the NCFg and alternative food-based recommendations (FBRs) on the basis of dietary adequacy, for 11 micronutrients, at the population level. Results: Thiamine recommended nutrient intakes for these young children could not be achieved given local foods as consumed. NCFg focusing only on meat, fruits, vegetables, and breast milk ensured dietary adequacy at the population level for only 4 micronutrients, increasing to 8 of 11 modelled micronutrients when the FBRs promoted legumes, dairy, vitamin A–rich vegetables, and chicken giblets. None of the FBRs tested ensured population-level dietary adequacy for thiamine, niacin, and iron unless a fortified infant food was recommended. Conclusions: The present study demonstrated the value of using LP to adapt NCFg for a different age group than the one for which they were designed. Our analyses suggest that to ensure dietary adequacy for 12- to 23-month olds these adaptations should include legumes, dairy products, vitamin A–rich vegetables, organ meat, and a fortified food.

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New Celiac Icebergs Are Spotted, Other Are Slowly Emerging

No abstract available

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Mass Screening for Celiac Disease Among School-aged Children: Toward Exploring Celiac Iceberg in Saudi Arabia

imageABSTRACT Objectives: We conducted this mass screening study to determine the prevalence of celiac disease (CD) and characterize the celiac iceberg among Saudi pediatric population in Riyadh, the capital city of Saudi Arabia. Methods: During the study period (January 2014–June 2016), we have conducted a cross-sectional, mass screening, immunoglobulin A-tissue transglutaminase (TTG-IgA)-based study on 7930 Saudi students from primary and intermediate schools in Riyadh. Students with positive TTG-IgA (>20 U/L) were called in the hospital to undergo a repeat of TTG-IgA; in those with borderline positive TTG-IgA (20–60 U/L), IgA-endomyseal antibody (EMA-IgA) test was performed. Children with TTG-IgA >60 U/L and children with borderline positive TTG-IgA and positive EMA-IgA were advised to undergo upper endoscopy and intestinal biopsies. Results: We identified 221 students with positive TTG-IgA (2.8%). CD was diagnosed in 119 cases (1.5%, 1:67 Saudi children) (mean age 11.5 ± 2.62 years; girls 81 [68%]). Another 51 children had persistently borderline positive TTG-IgA but negative EMA (0.64%) and the remaining 51 had transiently positive TTG-IgA. We have identified 3 clinical patterns in the screening-identified cases with CD: a silent form (37%), a mild symptomatic form characterized by gastrointestinal symptoms in presence of normal growth or overweight/obesity (48%), and gastrointestinal symptoms associated with impaired growth in 15%. Conclusions: Our study provided evidence of a high prevalence of CD among Saudi children (1.5%), a rate that is at least twice the average prevalence rate in Europe and North America.

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Coagulopathy in Children With Liver Disease

ABSTRACT It was thought that a high international normalized ratio predicted bleeding in patients with chronic liver disease (CLD) and patients were "autoanticoagulated." Contrary to this belief, while patients with CLD experienced bleeding, they also developed thromboses. In the last decade, the prevailing literature challenged the idea that an elevated international normalized ratio increased bleeding risk. The global assays of coagulation such as thromboelastography (TEG)/rotational thromboelastometry and thrombin generation assays provide additional insight into coagulation processes. It has become apparent that a parallel reduction of procoagulant and anticoagulant factors leave patients in a new "balanced" state, albeit a fragile one, where the balance can be easily disrupted. The inherent differences in coagulation between children and adults such as differences in levels of procoagulant and anticoagulant factors, underlying liver disease, and the paucity of studies in children make extrapolation of these findings to the pediatric population problematic. Ultimately, this is an area that requires further investigation to avoid inappropriate use of blood products and medication.

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Cholic Acid to Treat HSD3B7 and AKR1D1 Deficiencies

No abstract available

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Lack of Association Between Interleukin 28B Polymorphism and Vertical Transmission of Hepatitis C

imageABSTRACT Objectives: Single genetic nucleotide polymorphism (rs12979860) near the gene for interleukin 28B (IL28B) is known to be of importance for frequency of spontaneous clearance and treatment outcome in interferon-based therapies in patients with hepatitis C virus (HCV) infection. The aim of the present study was to investigate whether IL28B polymorphism in children and/or their mothers plays a role in vertical transmission of HCV (HCV-VT). Methods: Plasma samples from 59 infected women, 76 uninfected children born to infected mothers, and 47 children with known vertically transmitted HCV infection, were analysed for IL28B polymorphism and classified by the IL28B genotype (C/C, C/T, and T/T) and by viral genotype. Results: The proportion of children with genotype C/C was the same in the vertically infected (36%, 17/47) and the exposed uninfected children (38%, 29/76). No difference was seen when stratifying for viral genotype. There was no association between mothers' IL28B genotype and the risk of vertical transmission. Conclusions: Regardless of viral genotype we found no association between IL28B genotype and the risk of HCV-VT. The IL28B genotype CC, which has been shown to be favourable in other settings, was not protective of HCV-VT. Thus, other factors possibly associated with the risk of HCV-VT need to be explored.

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Stability of Cortisol and Cortisone in Human Breast Milk During Holder Pasteurization

imageABSTRACT Human donor milk is the feeding of choice for preterm infants, when own mother's milk is not available. Holder pasteurization is necessary to secure the safety of donor milk, although it can affect milk quality by reduction of nutritional and bioactive components. Recently, research has focused on the potential role of breast milk glucocorticoids for infant development. At this moment, it is unknown whether pasteurization affects milk glucocorticoid levels. Therefore, we assessed whether Holder pasteurization, the most frequently used method nowadays, reduces breast milk cortisol and cortisone levels, using breast milk samples from 30 women who delivered at term. We found tight correlations between pre- and postpasteurization levels of cortisol (R2 = 0.99) and cortisone (R2 = 0.98), and good agreement in Passing and Bablok regression analysis. In conclusion, cortisol and cortisone in human term breast milk are not significantly affected by Holder pasteurization.

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Bile Acid Synthesis Disorders in Arabs: A 10-year Screening Study

imageABSTRACT Objectives: Early diagnosis of bile acid synthesis disorders (BASDs) is important because, untreated, these conditions can be fatal. Our objectives were to screen children with cholestasis or unexplained liver disease for BASD and in those with confirmed BASD to evaluate the effectiveness of cholic acid therapy. Methods: A routine serum total bile acid measurement was performed on children with cholestasis, liver cirrhosis, and liver failure. Patients were screened for BASD by fast atom bombardment ionization-mass spectrometry (FAB-MS) analysis of urine, and molecular analysis confirmed diagnosis. Treatment response to oral cholic acid (10–15 mg/kg bw/day) was assessed from liver function tests and fat-soluble vitamin levels. FAB-MS analysis of urine was used to monitor compliance and biochemical response. Results: Between 2007 and 2016, 626 patients were evaluated; 450 with infantile cholestasis. Fifteen cases of BASD were diagnosed: 12 presented with infantile cholestasis (2.7%, 7 boys), an 8-year-old boy presented with cirrhosis, and two 18-month-old boys presented with hepatomegaly and rickets. Eleven were caused by 3β-hydroxy-Δ5-C27-steroid oxidoreductase dehydrogenase deficiency, 3 from Δ4-3-oxosteroid 5β-reductase deficiency, and 1 had Zellweger spectrum disorder. In all but 1, serum total bile acids were normal or low. With cholic acid therapy, 10 are alive and healthy with their native liver. Liver failure developed in 3 infants despite therapy; 2 died and 1 underwent liver transplantation. Conclusions: BASDs are rare but treatable causes of metabolic liver disease in Saudi Arabia. BASD should be considered in infants with cholestasis and low or normal serum total bile acid concentrations.

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Sugar in Infants, Children and Adolescents: A Position Paper of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition Committee on Nutrition

imageABSTRACT The consumption of sugars, particularly sugar-sweetened beverages (SSBs; beverages or drinks that contain added caloric sweeteners (ie, sucrose, high-fructose corn syrup, fruit juice concentrates), in European children and adolescents exceeds current recommendations. This is of concern because there is no nutritional requirement for free sugars, and infants have an innate preference for sweet taste, which may be modified and reinforced by pre- and postnatal exposures. Sugar-containing beverages/free sugars increase the risk for overweight/obesity and dental caries, can result in poor nutrient supply and reduced dietary diversity, and may be associated with increased risk of type 2 diabetes mellitus, cardiovascular risk, and other health effects. The term "free sugars," includes all monosaccharides/disaccharides added to foods/beverages by the manufacturer/cook/consumer, plus sugars naturally present in honey/syrups/unsweetened fruit juices and fruit juice concentrates. Sugar naturally present in intact fruits and lactose in amounts naturally present in human milk or infant formula, cow/goat milk, and unsweetened milk products is not free sugar. Intake of free sugars should be reduced and minimised with a desirable goal of

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Body Composition Predicts Growth in Infants and Toddlers With Chronic Liver Disease

imageABSTRACT This cross-sectional study was conducted on 15 infants and toddlers with chronic liver disease to validate arm anthropometry as an accurate measure of body composition (BC) compared to dual-energy x-ray absorptiometry and to predict growth from BC. The z score means of the anthropometric indicators were

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Journal of Pediatric Gastroenterology and Nutrition 2017 Reviewer Acknowledgement

No abstract available

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Serum Markers of Necrotizing Enterocolitis: A Systematic Review

imageABSTRACT Objective: The aim of the study was to systematically review the diagnostic utility of serum biomarkers for the diagnosis of necrotizing enterocolitis (NEC). Methods: We conducted an electronic and manual search of the available evidence. We included studies reporting data on the diagnostic accuracy of "serum" biomarkers for the diagnosis of NEC, available until January 2016. Results: We selected 22 studies from the 1296 articles retrieved. Only S100 A8/A9 protein and apolipoprotein-CII showed high sensitivity (100% and 96.4%, respectively) and specificity (90% and 95%, respectively) in the studies using Bell stage II NEC as target condition. High sensitivity and specificity were reported for interleukin-10 (100% and 90%), interleukin1-receptor antagonist (100% and 91.7%), intestinal fatty acid–binding protein (100% and 91%) and ischemia-modified albumin (94.7% and 92%), when tested to predict the evolution from definite to advanced NEC. Given the amount of uncertainty, the limited availability of data and heterogeneity among the populations in the different studies, we were unable to perform a meta-analysis. Major concerns about the applicability stemmed from the spectrum of patients enrolled and the inclusion of diseases different from Bell stage ≥2 NEC as target conditions. Conclusions: We identified only few markers with good diagnostic accuracy and found an overall low quality of the studies on serum NEC biomarkers. In conclusion, data supporting their use are insufficient.

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The Development of the Healthy Pregnancy Stress Scale, and Validation in a Sample of Low-Income African American Women

Abstract

Objectives The association of stress with pregnancy health is well-known. However, few studies take a mixed methods approach to understand the stressors contributing to a woman's pregnancy-related stress. Among African American women, exposure to stressors during pregnancy likely contributes to disparities in pregnancy health outcomes. This work aimed to understand the types and magnitude of stressors African American women are exposed to during pregnancy. Methods Using a mixed methods research design, we developed and administered the Healthy Pregnancy Stress Scale to measure stressors within the stress environment of African American women living in poverty. Results Exploratory factor analysis with one random split-half sample (N = 85) identified a two-factor model. Factor 1, defined as general pregnancy stressors, had significant loadings for ten items that ranged in magnitude from 0.319 to 0.724. Factor 2, defined as relationship strain, had significant loadings for three items ranging in magnitude from 0.613 to 0.856. Confirmatory factor analysis in the second random split-half sample (N = 88) showed a strong fit for the two factor model with factor loadings similar in magnitude. Standard fit statistics and those that adjust for item non-normality suggested an adequate fit to the data (RMSEA = 0.057, CFI = 0.947, TLI = 0.932; Satorra-Bentler RMSEA = 0.037, CFI = 0.972, TLI = 0.965). Conclusions for Practice Our measurement tool may provide a way to determine differences in pregnancy stress experiences across diverse populations of women. Future research should include a test for construct validity by correlating the scale with other measures that should have a specific directional relationship in diverse populations.



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Heading in the right direction: the importance of direction selectivity for cerebellar motor learning

Abstract

In its most rudimentary form, information processing in the nervous system is typically bi-directional. Sensory afferent neurons relay sensory information to the central nervous system while efferent pathways carry processed nerve impulses to peripheral targets.

This article is protected by copyright. All rights reserved



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Norwegian Army train Afghan medics how to save lives. Sigh...

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"Norwegian Air Ambulance" -Gow. Style /-Afgan Style: https://youtube/pYvTnBuh8wI NOTE: The NGO Norwegian Air Ambulance Hawe NOTHING to do with this operation ..! ExEMTNor

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Thriller challenge for kids with eosinophilic diseases

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Tarpon Springs, we have accepted your challenge! Here is our Thriller Challenge Video to raise awareness for Eosinophilic Diseases! Eosinophilic Gastrointestinal Disorder (EGID) is a complicated digestive system disorder in which eosinophils, a type of white blood cell, are found in above-normal levels in the digestive system or blood. When the body senses an invader, eosinophils respond by moving into the area and releasing toxins. When the body creates too many eosinophils, they can cause chronic inflammation, resulting in tissue damage. Visit http://ift.tt/2zRseI4 or http://ift.tt/2iqran2 to find out more or to donate! We challenge Eastchester EMS, Pleasantville EMS, Ossining EMS, Capital Health of NJ EMS, and McCabe Ambulance Service to make your own video to raise awareness for Eosinophilic Diseases!

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Norwegian Army train Afghan medics how to save lives. Sigh...

hqdefault.jpg

"Norwegian Air Ambulance" -Gow. Style /-Afgan Style: https://youtube/pYvTnBuh8wI NOTE: The NGO Norwegian Air Ambulance Hawe NOTHING to do with this operation ..! ExEMTNor

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Thriller challenge for kids with eosinophilic diseases

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Tarpon Springs, we have accepted your challenge! Here is our Thriller Challenge Video to raise awareness for Eosinophilic Diseases! Eosinophilic Gastrointestinal Disorder (EGID) is a complicated digestive system disorder in which eosinophils, a type of white blood cell, are found in above-normal levels in the digestive system or blood. When the body senses an invader, eosinophils respond by moving into the area and releasing toxins. When the body creates too many eosinophils, they can cause chronic inflammation, resulting in tissue damage. Visit http://ift.tt/2zRseI4 or http://ift.tt/2iqran2 to find out more or to donate! We challenge Eastchester EMS, Pleasantville EMS, Ossining EMS, Capital Health of NJ EMS, and McCabe Ambulance Service to make your own video to raise awareness for Eosinophilic Diseases!

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Norwegian Army train Afghan medics how to save lives. Sigh...

hqdefault.jpg

"Norwegian Air Ambulance" -Gow. Style /-Afgan Style: https://youtube/pYvTnBuh8wI NOTE: The NGO Norwegian Air Ambulance Hawe NOTHING to do with this operation ..! ExEMTNor

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Thriller challenge for kids with eosinophilic diseases

mqdefault.jpg

Tarpon Springs, we have accepted your challenge! Here is our Thriller Challenge Video to raise awareness for Eosinophilic Diseases! Eosinophilic Gastrointestinal Disorder (EGID) is a complicated digestive system disorder in which eosinophils, a type of white blood cell, are found in above-normal levels in the digestive system or blood. When the body senses an invader, eosinophils respond by moving into the area and releasing toxins. When the body creates too many eosinophils, they can cause chronic inflammation, resulting in tissue damage. Visit http://ift.tt/2zRseI4 or http://ift.tt/2iqran2 to find out more or to donate! We challenge Eastchester EMS, Pleasantville EMS, Ossining EMS, Capital Health of NJ EMS, and McCabe Ambulance Service to make your own video to raise awareness for Eosinophilic Diseases!

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Father-daughter duo debut ‘AmbuPod’ microambulance

The solar-powered microambulance can be connected to any vehicle and its high-tech software allows patient information to be sent to doctors

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Masimo announces distribution of Rad-57® Pulse CO-Oximeters® by the Jeffrey Lee Williams Foundation to First Responders

Foundation awards devices to York County EMS departments to assist in monitoring for presence of carbon monoxide ROCK HILL, S.C. — Masimo (NASDAQ: MASI) announced today that The Jeffrey Lee Williams Foundation has distributed 20 Masimo Rad-57® Pulse CO-Oximeters® to EMS departments in York County, South Carolina, with the majority going to Piedmont Medical Center EMS. The Foundation purchases ...

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Spotlight: CATI Armor provides a lighter line of body armor

CATI Armor is protecting first responders with lighter, more secure body armor

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Wheezing, stridor ominous signs of impending airway loss in smoke inhalation

Treat aggressively with high-flow oxygen, rapid sequence intubation and Cyanokit in a patient with evidence of airway burns and CO2 poisoning

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Baltimore officials concerned about long wait times on 911 calls

Baltimore Fire Chief Niles Ford testified that the average wait time for the city's 911 is only six seconds

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How EMS is changing

In its second year, the EMS Trend Report describes revealing changes in clinical care, finance and the use of technology in EMS

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Norwegian Army train Afghan medics how to save lives. Sigh...

hqdefault.jpg

"Norwegian Air Ambulance" -Gow. Style /-Afgan Style: https://youtube/pYvTnBuh8wI NOTE: The NGO Norwegian Air Ambulance Hawe NOTHING to do with this operation ..! ExEMTNor

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Thriller challenge for kids with eosinophilic diseases

mqdefault.jpg

Tarpon Springs, we have accepted your challenge! Here is our Thriller Challenge Video to raise awareness for Eosinophilic Diseases! Eosinophilic Gastrointestinal Disorder (EGID) is a complicated digestive system disorder in which eosinophils, a type of white blood cell, are found in above-normal levels in the digestive system or blood. When the body senses an invader, eosinophils respond by moving into the area and releasing toxins. When the body creates too many eosinophils, they can cause chronic inflammation, resulting in tissue damage. Visit http://ift.tt/2zRseI4 or http://ift.tt/2iqran2 to find out more or to donate! We challenge Eastchester EMS, Pleasantville EMS, Ossining EMS, Capital Health of NJ EMS, and McCabe Ambulance Service to make your own video to raise awareness for Eosinophilic Diseases!

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EMS loses friend and mentor, Lou Jordan

He wore a T-shirt emblazoned with the caption, "EMS Artifact: Start conversation at your own risk," and walking an EMS conference exhibit hall with him was simultaneously a study in professional networking and an exercise in frustration. It didn't matter if you desperately wanted to see the new Tracheoblaster 5000 video laryngoscope or get pricing info on the latest generation of high-fidelity ...

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Cancer survivor becomes volunteer EMT while waiting for kidney donor

By EMS1 Staff EASTON, Conn. — A cancer survivor who is currently waiting for a kidney donor became an EMT after an EMS crew helped him while he fought for his life. WTNH reported that volunteer EMT Adam Goldstein, 37, was diagnosed with stage 4 testicular cancer when he was 21. "When I got to the hospital they did a CAT scan and they had told me that they had found cancer throughout my body," ...

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How EMS is changing

To an outsider, EMS probably looks pretty similar today to how it did decades ago. Call 911, and an ambulance arrives and takes you to the hospital – simple. Yet we know that while progress can sometimes feel slow, in other ways the profession might be going through its most transformative era. Whether it's a call to change "EMS" to "paramedicine," a push to reform reimbursement ...

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Is There More than One Variant of Noninvasive Follicular Neoplasm with Papillary-like Nuclear Features?

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 433-436.


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Does Antithyroid Drug Treatment Improve Outcomes in Wild-Type Offspring of Mothers with Thyroid Hormone Resistance?

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 426-429.


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Punctate Echogenic Foci on Thyroid Ultrasound Do Not Necessarily Represent Calcifications on Histopathology

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 415-418.


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Validation of American Thyroid Association Ultrasound Risk Assessment of Thyroid Nodules Selected for Ultrasound Fine-Needle Aspiration

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 411-414.


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Significant Variations of Thyroid Testing in the U.S. Argue for Improved Standardization of Practice Patterns

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 419-421.


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TPO Antibody Positivity in Pregnancy Is Associated with Metabolic Syndrome, Increased Waist Circumference, and Higher BMI in Offspring at Age 16

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 430-432.


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Are Wider TSH Cutoffs for Reflex Testing of Free T4 Feasible, Safe and Cost-Effective?

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 422-425.


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Multifocality Is Not an Independent Risk Factor for Recurrence of Papillary Thyroid Cancer

Clinical Thyroidology Nov 2017, Vol. 29, No. 11: 437-439.


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Antibiotic Resistance Gene Detection in the Microbiome Context

Microbial Drug Resistance , Vol. 0, No. 0.


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The Burden of Rifampicin- and Methicillin-Resistant Staphylococcus aureus in Italy

Microbial Drug Resistance , Vol. 0, No. 0.


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Molecular Epidemiology of Methicillin-Resistant Staphylococcus aureus in Cystic Fibrosis Patients from Argentina

Microbial Drug Resistance , Vol. 0, No. 0.


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Antimicrobial Resistance in Escherichia coli Isolated from Wild Animals in Poland

Microbial Drug Resistance , Vol. 0, No. 0.


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Sister Elizabeth Kenny and Polio in America: Doyenne or Demagogue in Her Role in Rehabilitation Medicine?



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Lumbar Medial Branch Block Volume Dependent Dispersion Patterns as a Predictor for Ablation Success: A Cadaveric Study

Lumbar facet arthropathy is a common cause of low back pain. Literature supports treatment with radiofrequency ablation (RFA) of associated nerves that innervate lumbar facets when alternative conservative therapies have failed. Diagnostic local anesthetic blocks precede therapeutic ablation, but have a false positive rate of 27-63%, and some have questioned their utility in predicting therapeutic response to RFA. The authors believe that injectate volume may be a contributing factor to false positivity.

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Body Satisfaction During Pregnancy: The Role of Health-Related Habit Strength

Abstract

Objectives Body satisfaction during pregnancy is an important determinant of maternal and fetal health outcomes. It is therefore critical to investigate factors related to changes in body satisfaction and to elucidate how body satisfaction changes over time in pregnant women. The purpose of this study was to examine the relation between two novel factors (i.e., healthy eating habit strength and physical activity habit strength) and body satisfaction during pregnancy. Methods Participants (n = 67 pregnant North American women) completed online questionnaires at the beginning of their second trimester (Time 1) and at the end of pregnancy. Maternal characteristics, relationship satisfaction, self-esteem, and psychological distress were assessed at Time 1 and habit strength, body satisfaction, and weight were assessed at both time points. Results Strength of healthy eating and physical activity habits remained stable over time and body satisfaction decreased over time. Healthy eating habit strength at Time 1 predicted increases in body satisfaction from the second trimester to the end of pregnancy, even when controlling for gestational weight gain. Conclusions This study suggests that health-related habit strength in women of reproductive age may offer protection against low levels of body satisfaction during pregnancy.



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Impact of a Usual Source of Care on Health Care Use, Spending, and Quality Among Adults With Mental Health Conditions

Abstract

Physical comorbidities associated with mental health conditions contribute to high health care costs. This study examined the impact of having a usual source of care (USC) for physical health on health care utilization, spending, and quality for adults with a mental health condition using Medicaid administrative data. Having a USC decreased the probability of inpatient admissions and readmissions. It decreased expenditures on emergency department visits for physical health, 30-day readmissions, and behavioral health inpatient admissions. It also had a positive effect on several quality measures. Results underscore the importance of a USC for physical health and integrated care for adults with mental health conditions.



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Behavioral and physiological polymorphism in males of the austral lizard Liolaemus sarmientoi

Abstract

Integrative behavioral studies show that the interplay between individual physiology and social behavior influences the ecology of the species, ultimately affecting individual fitness. Particularly in lizards, color polymorphism is associated with differential behaviors and reproductive strategies, which are evident in mature males during the mating season. Dominant males generally have greater endurance, higher body temperature, and larger bodies than submissive males, so they can acquire and defend larger territories and have greater access to females for mating. We studied whether the color morphs observed in males of one of the world's southernmost reptiles, Liolaemus sarmientoi, are related to behavioral variation during agonistic interactions, thermal physiology, morphology, and/or locomotor stamina. Liolaemus sarmientoi males exhibit three color morphs: red (RR), red–yellow (RY), and yellow (YY). These lizards exhibit subtle behavioral displays and we did not observe stamina differences among morphs. However, we found that RR males are more aggressive than YY males during agonistic encounters. In addition, greater body temperature change during trials, higher field body temperatures, and greater head sizes of RR males compared to RY or YY indicate that RR is a dominant morph, which may influence their ability to acquire and defend territory and tactics for achieving reproductive success.



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Riboflavin transporter deficiency mimicking mitochondrial myopathy caused by complex II deficiency

Biallelic likely pathogenic variants in SLC52A2 and SLC52A3 cause riboflavin transporter deficiency. It is characterized by muscle weakness, ataxia, progressive ponto-bulbar palsy, amyotrophy, and sensorineural hearing loss. Oral riboflavin halts disease progression and may reverse symptoms. We report two new patients whose clinical and biochemical features were mimicking mitochondrial myopathy. Patient 1 is an 8-year-old male with global developmental delay, axial and appendicular hypotonia, ataxia, and sensorineural hearing loss. His muscle biopsy showed complex II deficiency and ragged red fibers consistent with mitochondrial myopathy. Whole exome sequencing revealed a homozygous likely pathogenic variant in SLC52A2 (c.917G>A; p.Gly306Glu). Patient 2 is a 14-month-old boy with global developmental delay, respiratory insufficiency requiring ventilator support within the first year of life. His muscle biopsy revealed combined complex II + III deficiency and ragged red fibers consistent with mitochondrial myopathy. Whole exome sequencing identified a homozygous likely pathogenic variant in SCL52A3 (c.1223G>A; p.Gly408Asp). We report two new patients with riboflavin transporter deficiency, caused by mutations in two different riboflavin transporter genes. Both patients presented with complex II deficiency. This treatable neurometabolic disorder can mimic mitochondrial myopathy. In patients with complex II deficiency, riboflavin transporter deficiency should be included in the differential diagnosis to allow early treatment and improve neurodevelopmental outcome.



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