Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation and anti-TNF Treatment.

Background: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). Methods: Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. Results: 55 patients (16.7%) suffered from 1-4 EIM (39 Crohn's disease, 12 ulcerative colitis and 4 IBD-Unclassified (IBD-U) patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs. 5.0%, p = 0.014). EIM were more frequent in CD when compared to UC/IBD-U (22.5 vs. 10.3%, p = 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5-149.0). 31 of the 55 patients (56.4%) were treated with one or more anti-TNF agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs. 35.0%, p = 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). Conclusions: In a cohort of pediatric IBD patients, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings might translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti- TNF for the treatment of certain EIM is effective although a substantial proportion of new EIM might present despite ongoing anti-TNF therapy. (C) 2016 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Survey on Clinical Practice of Primary Prophylaxis in Portal Hypertension in Children.

Primary prophylaxis in portal hypertension in children is controversial, as there are few studies documenting its efficacy on the risk of bleeding. 28 centres out of the 38 we contacted returned a completed questionnaire about their clinical practices. More than 75% of the centres use endoscopy to screen patients diagnosed with portal cavernoma, biliary atresia, cystic fibrosis and other fibrotic chronic liver diseases with suspected portal hypertension. In cases of grade 2 varices with red marks and grade 3 varices more than 90% of centres perform sclerotherapy or endoscopic variceal ligation (EVL). Non-cardio selective beta blockers where used by approximately 20% of centres. In conclusion, despite the absence of scientific recommendations there is a tacit consensus concerning the need to screen children with clinical signs of portal hypertension, and to provide primary prophylaxis in cases of endoscopic patterns of high risk varices. (C) 2016 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Effect of Differential Enteral Protein on Growth and Neurodevelopment in Infants

Objective: To determine if higher enteral protein intake leads to improved head growth at 40 weeks postmenstrual age (PMA) in preterm infants less than 32 weeks or 1500 g. Method: Randomized controlled trial in which 120 infants were assigned to either group A with higher enteral protein intake achieved by fortification with higher protein containing fortifier [1 g/100 ml expressed breast milk (EBM)] or to group B with lower enteral protein intake where fortification was done with standard available protein fortifier (0.4 g /100 ml EBM). Results: The mean (SD) protein intake was higher in group A as compared to group B; 4.2 (0.47) vs 3.6 (0.37) g/kg/day, P

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Correlation of Transient Elastography With Severity of Cystic Fibrosis Related Liver Disease.

Objective: To evaluate whether liver stiffness measurement (LSM), determined by transient elastography (TE), correlates with presence and severity of liver disease (LD) in children and young adults with cystic fibrosis (CF). Methods: Subjects underwent LSM at routine CF visits. Presence and severity of CFLD was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN. LSM was compared to AST/platelet ratio index (APRI) as a correlate to severity of CFLD. Results: 249 subjects [53%male; mean age 14 +/- 7y; 7(3%)

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The destination defines the journey: an examination of the kinematics of hand-to-mouth movements

Long-train electrical stimulation of the motor and premotor cortices of nonhuman primates can produce either hand-to-mouth or grasp-to-inspect movements, depending on the precise location of stimulation. Furthermore, single-neuron recording studies identify discrete neuronal populations in the inferior parietal and ventral premotor cortices that respond uniquely to either grasp-to-eat or grasp-to-place movements, despite their identical mechanistic requirements. These studies demonstrate that the macaque motor cortex is organized around producing functional, goal-oriented movements, rather than simply fulfilling muscular prerequisites of action. In humans, right-handed hand-to-mouth movements have a unique kinematic signature; smaller maximum grip apertures are produced when grasping to eat than when grasping to place identical targets. This is evidence that the motor cortex in humans is also organized around producing functional movements. However, in both macaques and humans, grasp-to-eat/hand-to-mouth movements have always been elicited using edible targets and have (necessarily) been paired with mouth movement. It is therefore unknown whether the kinematic distinction is a natural result of grasping food and/or is simply attributable to concurrent opening of the mouth while grasping. In experiment 1, we used goal-differentiated grasping tasks, directed toward edible and inedible targets, to show that the unique kinematic signature is present even with inedible targets. In experiment 2, we used the same goal-differentiated grasping tasks, either coupled with or divorced from an open-mouth movement, to show that the signature is not attributable merely to a planned opening of the mouth during the grasp. These results are discussed in relation to the role of hand-to-mouth movements in human development, independently of grasp-to-eat behavior.

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White matter and cognition: making the connection

Whereas the cerebral cortex has long been regarded by neuroscientists as the major locus of cognitive function, the white matter of the brain is increasingly recognized as equally critical for cognition. White matter comprises half of the brain, has expanded more than gray matter in evolution, and forms an indispensable component of distributed neural networks that subserve neurobehavioral operations. White matter tracts mediate the essential connectivity by which human behavior is organized, working in concert with gray matter to enable the extraordinary repertoire of human cognitive capacities. In this review, we present evidence from behavioral neurology that white matter lesions regularly disturb cognition, consider the role of white matter in the physiology of distributed neural networks, develop the hypothesis that white matter dysfunction is relevant to neurodegenerative disorders, including Alzheimer's disease and the newly described entity chronic traumatic encephalopathy, and discuss emerging concepts regarding the prevention and treatment of cognitive dysfunction associated with white matter disorders. Investigation of the role of white matter in cognition has yielded many valuable insights and promises to expand understanding of normal brain structure and function, improve the treatment of many neurobehavioral disorders, and disclose new opportunities for research on many challenging problems facing medicine and society.

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Cerebellar tDCS dissociates the timing of perceptual decisions from perceptual change in speech

Neuroimaging studies suggest that the cerebellum might play a role in both speech perception and speech perceptual learning. However, it remains unclear what this role is: does the cerebellum help shape the perceptual decision, or does it contribute to the timing of perceptual decisions? To test this, we used transcranial direct current stimulation (tDCS) in combination with a speech perception task. Participants experienced a series of speech perceptual tests designed to measure and then manipulate (via training) their perception of a phonetic contrast. One group received cerebellar tDCS during speech perceptual learning, and a different group received sham tDCS during the same task. Both groups showed similar learning-related changes in speech perception that transferred to a different phonetic contrast. For both trained and untrained speech perceptual decisions, cerebellar tDCS significantly increased the time it took participants to indicate their decisions with a keyboard press. By analyzing perceptual responses made by both hands, we present evidence that cerebellar tDCS disrupted the timing of perceptual decisions, while leaving the eventual decision unaltered. In support of this conclusion, we use the drift diffusion model to decompose the data into processes that determine the outcome of perceptual decision-making and those that do not. The modeling suggests that cerebellar tDCS disrupted processes unrelated to decision-making. Taken together, the empirical data and modeling demonstrate that right cerebellar tDCS dissociates the timing of perceptual decisions from perceptual change. The results provide initial evidence in healthy humans that the cerebellum critically contributes to speech timing in the perceptual domain.

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