Τρίτη 23 Μαΐου 2017

A mutation in GABRB3 associated with Dravet syndrome

Dravet syndrome is a rare and severe type of epilepsy in infants. Approximately, 70–80% of patients with Dravet syndrome have mutations in SCN1A, the gene encoding the alpha-1 subunit of the sodium channel, while some simplex patients have variants in one of several other genes, including but not limited to GABRA1, SCN2A, STXBP1, GABRG2, and SCN1B. In this study, we performed exome sequencing in six patients with SCN1A-negative Dravet syndrome to identify other genes related to this disorder. In one affected individual, we detected a novel de novo heterozygous missense variant, c.695G>A, p.(Arg232Gln), in GABRB3, the gene encoding the β3-subunit of the gamma-aminobutyric acid type A (GABAA) receptor, which mediates inhibitory signaling within the central nervous system. In summary, the data in this study identify GABRB3 as a candidate gene for Dravet syndrome.



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37th Annual David W. Smith Workshop on Malformations and Morphogenesis: Abstracts of the 2016 Annual Meeting

The 37th Annual David W. Smith Workshop on Malformations and Morphogenesis occurred on September 9th–14th, 2016 at the University of California—Los Angeles Conference Center in Lake Arrowhead, CA. The Workshop, which honors the legacy of David W. Smith, brought together clinicians and researchers interested in congenital malformations and their underlying mechanisms of morphogenesis. The Workshop highlighted five themes besides mechanisms of morphogenesis and New Syndromes: Neural Crestopathies, Mosaicism, Disorders of Skin Pigmentation, Therapies, and Ear Malformations and Hearing Loss. This Conference Report includes the abstracts presented at the 2016 Workshop.



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Discordant phenotypes in monozygotic twins with 16p11.2 microdeletions including the SH2B1 gene

A 200∼240 kb SH2B1-containing deletion region on 16p11.2 is associated with early-onset obesity and developmental delay. Here, we describe monozygotic twin brothers with discordant clinical presentations. Intrauterine fetal growth restriction was present in both twins. Additionally, twin A exhibited coarctation of aorta, left ventricular noncompaction, atrial septal defect, pericardial effusion, left hydronephrosis, and moderate developmental delay, whereas twin B exhibited single umbilical artery. Chromosome microarray analysis was performed on both twins and their parents. An identical 244 kb microdeletion on 16p11.2 including 9 Refseq genes, including SH2B1, was identified in the twins. The novel findings in monozygotic twins may expand the phenotypic spectrum of 16p11.2 microdeletion. Further studies are needed to strengthen the correlation between genotypes and abnormal clinical features.



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Obstructive sleep apnea in Down syndrome: Benefits of surgery and noninvasive respiratory support

Children with Down syndrome are at increased risk of obstructive sleep apnea (OSA). The aim of the study was to describe the management of OSA in a large cohort of children with Down syndrome. A retrospective analysis of sleep studies and consequent management was performed for all consecutive Down syndrome patients evaluated between September 2013 and April 2016. The data of 57 patients were analyzed: 51/53 had an interpretable overnight polygraphy and 4 the recording of nocturnal gas exchange. Mean age at baseline sleep study was 6.2 ± 5.9 years. Eighteen patients (32%) had prior upper airway surgery. Mean apnea-hypopnea index (AHI) was 14 ± 16 events/hr with 41 of the 51 (80%) patients having OSA with an AHI >1 event/hr and 20 patients (39%) having an AHI ≥10 events/hr. Consequently, eight patients (14%) had upper airway surgery. OSA improved in all patients except two who needed noninvasive respiratory support. Nineteen (33%) patients required noninvasive respiratory support. Mean age at noninvasive respiratory support initiation was 7 ± 7 years. On 11 patients with objective adherence data available, mean compliance at 2 ± 1 years of treatment was excellent with an average use per night of 8 hr46 ± 3 hr59 and 9 patients using the noninvasive respiratory support >4 hr/night. Noninvasive respiratory support was associated with an improvement of nocturnal gas exchange. The prevalence of OSA is high in Down syndrome. Upper airway surgery is not always able to correct OSA. Noninvasive respiratory support represents then an effective treatment for OSA and good compliance may be achieved in a majority of patients.



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Haploinsufficiency of NR4A2 is associated with a neurodevelopmental phenotype with prominent language impairment

Non-recurrent deletions in 2q24.1, minimally overlapping two genes, NR4A2 and GPD2, were recently described in individuals with language impairment and behavioral and cognitive symptoms. We herewith report on a female patient with a similar phenotype of severe language and mild cognitive impairment, in whom we identified a de novo deletion covering only NR4A2. NR4A2 encodes a transcription factor highly expressed in brain regions critical for speech and language and implicated in dopaminergic neuronal development. Our findings of a de novo deletion of NR4A2 in an individual with mild intellectual disability and prominent speech and language impairment provides further evidence for NR4A2 haploinsufficiency being causative for neurodevelopmental and particularly language phenotypes.



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Preoperative platelet function predicts perioperative bleeding complications in ticagrelor-treated cardiac surgery patients: a prospective observational study

British Journal of Anaesthesia, 2016; 117(3): 309–15, DOI 10.1093/bja/aew189

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Chronic postsurgical pain in the Evaluation of Nitrous Oxide in the Gas Mixture for Anaesthesia (ENIGMA)-II trial

British Journal of Anaesthesia, 2016; 117(6): 801–11, DOI 10.1093/bja/aew338

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Modern hydroxyethyl starch and acute kidney injury after cardiac surgery: a prospective multicentre cohort

British Journal of Anaesthesia, 2016; 117(4): 458–63, DOI 10.1093/bja/aew258

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Surgical orthodontics.

Purpose of review: The article reviews some commonly used orthodontic treatments as well as new strategies to assist in the correction of malocclusion. Many techniques are used in conjunction with surgical intervention and are a necessary compliment to orthognathic surgery. Basic knowledge of these practices will aid in the surgeon's ability to adequately treat the patient. Recent findings: Many orthodontists and surgeons are eliminating presurgical orthodontics to adopt a strategy of 'surgery first' orthodontics in orthognathic surgery. This has the benefit of immediate improvement in facial aesthetics and shorter treatment times. The advent of virtual surgical planning has helped facilitate the development of this new paradigm by making surgical planning faster and easier. Furthermore, using intraoperative surgical navigation is improving overall precision and outcomes. Summary: A variety of surgical and nonsurgical treatments may be employed in the treatment of malocclusion. It is important to be familiar with all options available and tailor the patient's treatment plan accordingly. Surgery-first orthodontics, intraoperative surgical navigation, virtual surgical planning, and 3D printing are evolving new techniques that are producing shorter treatment times and subsequently improving patient satisfaction without sacrificing long-term stability. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Airway management during induction of anaesthesia, spontaneous ventilation (SV) and controlled mechanical ventilation (CMV), using an endotracheal tube (ETT), laryngeal mask (LM), rabbit-specific supraglottic airway device (v-gel) or facemask (FM).






Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182
6948891480

Stabilization of Visual Function After Optic Nerve Sheath Fenestration for Optic Nerve Meningocele.

A 10-year-old boy with bilateral colobomatous cavitary disc anomalies presented with a 3-month history of vision loss in his right eye. MRI of the head and orbit revealed bilateral tubular cystic enlargement of the optic nerve/optic sheath complex with thickening of the optic nerves without inflammation or neoplasm, suggestive of bilateral optic nerve meningocele. An optic nerve sheath fenestration was performed OD, and he experienced an improvement and stabilization of vision in his right eye during a 1-year follow-up period. The authors recommend that surgical decompression, particularly optic nerve sheath fenestration, should be considered in cases with progressive vision loss due to optic nerve meningocele. (C) 2017 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.

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Scleral Contact Lenses in an Academic Oculoplastics Clinic: Epidemiology and Emerging Considerations.

Purpose: To describe the role and efficacy of scleral contact lenses (SCLs) in the treatment of progressive keratopathy in patients who have undergone periocular surgical procedures, to investigate the financial impact of these surgical interventions, and to demonstrate the role of oculoplastic surgery in improving scleral contact lens fit. Methods: A retrospective medical record review was performed to identify patients who both received SCLs and were examined by the oculoplastics service at the University of Iowa between January 1990 and December 2015. Inclusion criteria also required a minimum of 12 months of patient follow up after being fit with a SCL. The indication for SCL use, as well as clinical outcomes and cumulative relative value units (RVUs) of prior oculoplastic treatments and SCL therapy were recorded for each patient. Results: Six hundred and fifty-nine patients were fitted with SCLs at the authors' institution during the 25-year study period, 43 of whom were examined by the oculoplastics service for reasons related to their SCL. Patients who were fitted for SCLs before (27 patients) or after (16 patients) evaluation in the oculoplastics clinic presented with a variety of corneal and periocular pathology. Corneal indications for SCLs in patients seen secondarily in the oculoplastics clinic included decreased corneal sensation (from CN V palsy or neurotrophic keratopathy), decreased corneal healing from limbal stem cell deficiency, exposure keratopathy, and keratoconjunctivitis sicca secondary to Sjogren's syndrome or orbital radiation. Indications for oculoplastic clinic evaluation in current scleral lens patients included lagophthalmos, trichiasis, epiphora, cicatricial changes in the eyelids or fornices, and eyelid or eyebrow malposition affecting SCL centration. In all 27 patients, surgical intervention resulted in improved SCL centration. Sixteen patients (5 with CN VII palsy, 4 with CN V and CN VII palsy, 4 with neurotrophic keratitis, and 3 with cicatricial entropion) had progressive corneal decompensation despite primary oculoplastics procedures to protect the cornea and ultimately benefitted from SCL treatment secondarily. Fourteen of these 16 patients demonstrated an improved visual acuity of 1 line or more using SCLs. Procedures performed before referral for SCLs included tarsorrhaphies, gold weights, ectropion repair with lateral tarsal strip, retraction repair with spacer grafts, full-thickness skin grafts, cheek lifts, and punctal occlusion. The total RVUs of these procedures performed per patient were tabulated. The RVU range was 10.47-33.96 with an average of 19.5 RVUs (standard deviation = 9.4 RVUs) per patient. Conclusions: Scleral contact lenses may offer a useful alternative to stabilize the ocular surface, improve vision, and minimize the financial impact and morbidity of multiple periocular procedures in select patients with progressive keratopathy of varying etiologies. The therapeutic indications and utilization of SCLs are likely to increase in oculoplastic practices with optometric support, particularly in academic settings. Surgical correction of eyelid and eyebrow malposition, as well as epiphora, may also be useful adjunctive procedures to optimize SCL fit. (C) 2017 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.

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Diagnosis, Treatment, and Surgical Repair in a Case of Isolated Conjunctival Lichen Planus Causing Cicatricial Entropion.

A 50-year-old male presented with bilateral cicatricial entropion with subepithelial fibrosis and fornix foreshortening. Conjunctival biopsy showed lymphocytic infiltrate along the interface of the epithelium and lamina propria with linear, shaggy fibrinogen deposition along the epithelial basement membrane zone, supporting a diagnosis of lichen planus. The patient was treated with commercially available topical cyclosporine for 5 months after which bilateral lower eyelid splitting procedure with recession of the anterior lamella and complete excision of the lower eyelid lashes was performed. (C) 2017 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.

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Curcumin attenuates lipopolysaccharide/d-galactosamine-induced acute liver injury

Curcumin, a polyphenol in curry spice isolated from the rhizome of turmeric, has been reported to possess versatile biological properties including anti-inflammatory, anti-oxidant, antifibrotic, and anticancer activities. In this study, the hepatoprotective effect of curcumin was investigated in lipopolysaccharide (LPS)/d-galactosamine (d-GalN)-induced acute liver injury (ALI) in rats. Experimental ALI was induced with an intraperitoneal (ip) injection of sterile 0.9% sodium chloride (NaCl) solution containing 8μg LPS and 800mg/kg d-GalN. Curcumin was administered once daily starting three days prior to LPS/d-GalN treatment. Results indicated that curcumin could attenuate hepatic pathological damage, decrease serum ALT and AST levels, and reduce malondialdehyde (MDA) content in experimental ALI rats. Moreover, higher dosages of curcumin pretreatment inhibited NF-κB activation and reduced serum TNF-α and liver TNF-α levels induced by LPS/d-GalN ip injection. Furthermore, we found that curcumin up-regulated the expression of nuclear Nrf2 and Nrf2-dependent antioxidant defense genes including heme oxygenase-1 (HO-1), glutamate-cysteine ligase (GCLC), NAD(P)H dehydrogenase, and quinone (NQO-1) in a dose-dependent manner. Our results showed that curcumin protected experimental animals against LPS/d-GalN-induced ALI through activation of Nrf2 nuclear translocation and inhibition of NF-κB activation.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182
6948891480

Body Composition Trajectories From Infancy to Preschool in Children Born Premature Versus Full-term

imageObjectives: The aim of the study was to longitudinally characterize infancy to preschool body composition trajectories and the association of early fat and fat-free mass gains with preschool age body composition in children born premature versus full-term. Methods: A cohort of appropriate-for-gestational age preterm (n = 20) and term (n = 51) infants were followed at 3 visits: "neonatal" visit 1 at 2 weeks of age for term and near term corrected age for preterm; "infancy" visit 2 at 3 to 4 months (preterm corrected age); "preschool" visit 3 at 4 years. Body composition via air displacement plethysmography and anthropometrics were measured at all visits. Tracking of infancy weight and body composition with preschool measurements was tested using Pearson partial correlation coefficients. Associations between serial body composition measurements were assessed using multiple linear regression. Results: Early differences in body composition between premature (mean gestational age 31.9 weeks, mean birth weight 1843 g) and full-term (mean gestational age 39.8 weeks) infants were not present at preschool age. Visit 1 body composition was not correlated with preschool measurements in the preterm infants. Visit 2 measurements were correlated with preschool measures. Fat-free mass accretion from visit 1 to visit 2 was positively associated with preschool lean mass (β = 0.038, P = 0.049) in preterm children, whereas fat accretion was not associated with preschool body composition. Conclusions: Children born prematurely and full-term have similar body composition at preschool age. For preterms infancy fat-free mass gains, and not adiposity gains, are positively associated with preschool fat-free mass; this may be associated with lower risk of later obesity and adverse metabolic outcomes.

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Comparison of Cytokine and Efflux Transporter Expression in Pediatric Versus Adult-onset Ulcerative Colitis

imageObjectives: Ulcerative colitis (UC), a chronic inflammation of the colon, is often more severe in children than adults. Identification of altered expression of efflux transporters, cytokines, and suppressor of cytokine signaling (SOCS) molecules in pediatric versus adult patients could provide insight into the differential molecular patterns related to the age and disease pathology. Methods: Mucosal samples from terminal ileum and colon in pediatric (9 UC-New, 4 UC-Remission) and adult (9 UC-New, 8 UC-Remission) patients were compared with healthy subjects (15 children and 10 adults) for mRNA expressions of several efflux transporters, cytokines, and SOCS molecules. Results: The inflamed colon interleukin (IL)-6, IL-17A, and interferon-γ levels were elevated in UC-New subgroups but close to control values in UC-Remission. IL-1β expression was increased only in UC-New children. Interestingly, uninflamed ileum also showed increased IL-6 and IL-1β levels in UC-New subgroups. SOCS1/SOCS3 expression pattern followed a trend observed for inflammatory cytokines only in children. Both children and adults had decreased multidrug resistance protein 1 expression in colon, which inversely correlated with disease score, IL-6 and interferon-γ levels in UC-New children. IL-2 expression was upregulated in UC-Remission, compared with controls. Conclusions: Efflux transporter expression varies between UC children and adults except for decreased multidrug resistance protein 1. UC is characterized by a dysregulated TH1 and TH17 cytokine response irrespective of age at disease onset, with higher cytokine levels detected in children. Increased IL-2 levels in remission imply a protective role for regulatory T cells (Tregs).

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Seeing Without Looking: Predicting Mucosal Healing Without Endoscopic Evaluation in Pediatric Ulcerative Colitis

No abstract available

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Functional Pelvic Floor Disorders: Concurrent Bowel and Bladder Symptoms

No abstract available

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Two Inborn Errors of Bile Acid Biosynthesis: The Need for Recognition and Treatment by Primary Bile Acid Replacement

No abstract available

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Medically Graded Honey Supplementation Formula to Preterm Infants as a Prebiotic: A Randomized Controlled Trial

imageObjectives: The aim of the study was to assess the effect of medically graded enteral honey supplementation on the intestinal microbiota, immune response, and somatic growth of preterm infants. Methods: A prospective randomized controlled trial was conducted on preterm infants with gestational age ≤34 weeks and postnatal age >3 days. After reaching 1/2 goal enteral feeds, medically graded bee honey was added to milk at a dose of 5, 10, 15, and 0 g/day for 2 weeks in groups A, B, C, and D, respectively. Anthropometric measurements, CD4 and CD8 cytokines, stool cultures, and stool polymerase chain reaction assays for molecular detection of microbiomes were performed at 0, 7, and 14 days of intervention. Analysis of variance test was used to detect differences among the 4 groups. Results: A total of 40 subjects were enrolled; 10 in each arm of the study. Compared with group D, all 3 intervention groups demonstrated significant increase in weight (P 

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The Challenge of Treating Children With Hepatitis C Virus Infection

imageABSTRACT: The development of oral hepatitis C virus (HCV) direct-acting antivirals (DAAs) has revolutionized the therapeutic field. Nowadays, multiple safe and highly effective antiviral regimens are commercially available to treat adults with hepatitis C infection. These new regimens for the first time genuinely raise the prospects of eradicating HCV. Many challenges, however, remain from identifying infected individuals to optimizing treatment and ensuring global access to antiviral therapy to all population groups, including children. Recently, in April 2017, the association of sofosbuvir with ribavirin and the fixed-dose combination sofosbuvir/ledipasvir have been approved by the Food and Drug Administration for treatment of children with chronic HCV infection 12 years of age and older. The only drugs currently approved for children younger than 12 years are pegylated interferon and ribavirin. There are 6 registered ongoing pediatric trials assessing safety and efficacy of DAAs, but their current completion timelines are years away. Herein, we summarize the state of the art of DAAs' development for adult and children and highlight the crucial importance of overcoming barriers to treating children with HCV.

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Isolated Appendiceal Pinworms on Colonoscopy

No abstract available

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Extracorporeal Liver Support Systems in Paediatric Liver Failure

imageABSTRACT: Extracorporeal liver support systems (ELSS), encompassing artificial and bioartificial devices, have been used for decades, with the aim of supporting patients with acute liver failure and acute-on chronic liver failure, as a bridge to recovery (acute liver failure only) or liver transplantation, in an era of organ donation shortage. Although biochemical efficacy has been consistently demonstrated by these devices, translation into clinical and survival benefits has been unclear, due to study limitations and lack of reliable prognostic scoring in liver failure. Consequently, extracorporeal devices are not widely accepted as routine therapy in adult liver failure. Recent large multicentre trials using artificial liver systems have not revealed beneficial outcomes associated with albumin dialysis but plasma exchange practices have shown some potential. In paediatric liver failure, data on extracorporeal systems is scarce, comprising few reports on albumin dialysis (namely, Molecular Adsorbent Recirculating System; MARS) and plasma exchange. When extrapolating data from adult studies differences in disease presentation, aetiology, prognosis and the suitability, and safety of such devices in children must be considered. The aim of this review is to critically appraise current practices of extracorporeal liver support systems to help determine efficacy in paediatric liver failure.

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Combination Steroid and Test-based Food Elimination for Eosinophilic Esophagitis: A Retrospective Analysis

imageObjectives: Eosinophilic esophagitis (EoE) is a clinicopathologic disorder characterized by infiltration of eosinophils into the esophagus. Primary treatment approaches include topical corticosteroids and/or food elimination. The aim of the present study was to compare the effectiveness of combination therapy (topical corticosteroid plus test-based food elimination [FS]) with single therapy (topical corticosteroid [S] or test-based food elimination [F]). Methods: Chart review of patients with EoE at Texas Children's Hospital (age

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Attempt to Determine the Prevalence of Two Inborn Errors of Primary Bile Acid Synthesis: Results of a European Survey

imageObjective: Inborn errors of primary bile acid (BA) synthesis are genetic cholestatic disorders leading to accumulation of atypical BA with deficiency of normal BA. Unless treated with primary BA, chronic liver disease usually progresses to cirrhosis and liver failure before adulthood. We sought to determine the prevalence of 2 common disorders, 3β-hydroxy-Δ5-C27-steroid dehydrogenase (3β-HSD) and Δ4–3-oxosteroid-5β-reductase (Δ4–3-oxoR) deficiencies and to describe current diagnostic and treatment strategies among different European paediatric hepatology centres. Methods: A total of 52 clinical paediatric centres were approached and 39 centres in 21 countries agreed to participate in the Web-based survey. The survey comprised questions regarding general information, number of cases, diagnostic, and therapeutic management. Results: Seventeen centres located in 11 countries reported patients with inborn errors in primary BA synthesis, 22 centres never had cases diagnosed. In total, we could identify 63 patients; 55 with 3β-HSD and 8 with Δ4–3-oxoR deficiency in 21 countries. The minimum estimated combined prevalence of these diseases was 1.13 cases per 10 million (0.99 and 0.14 for 3β-HSD and Δ4–3-oxoR deficiencies, respectively). The surveyed colleagues indicated their main challenges to be the rarity of diseases and the lack of convenient laboratory facilities nearby. Conclusion: We have identified the largest cohort of patients with 3β-HSD or Δ4–3-oxoR deficiency described so far. These diseases are likely underdiagnosed mainly due to unawareness of their existence and the lack of laboratory facilities.

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Prostaglandin E-major Urinary Metabolite as a Biomarker for Pediatric Ulcerative Colitis Activity

imageObjectives: Prostaglandin E-major urinary metabolite (PGE-MUM) is a useful biomarker for adult ulcerative colitis (UC) activity. In the present study, we evaluated whether PGE-MUM can also be a biomarker of pediatric UC activity and compared its efficacy in predicting UC activity with that of C-reactive protein and erythrocyte sedimentation rate. Methods: Twenty-nine pediatric patients with UC (8–18 years) and 29 healthy age- and sex-matched subjects were enrolled. UC activity was evaluated using the Pediatric Ulcerative Colitis Activity Index, highest Mayo endoscopic scoring (Mayo), and Matts grading (Matts) for histologic scoring, and the sum of Mayo (total of 6 segments) and Matts in all patients with UC. PGE-MUM levels were measured using a radioimmunoassay. Results: PGE-MUM levels were elevated in endoscopically and histologically active UC patients, but not in patients with endoscopic and histologic remission or controls. PGE-MUM levels positively and significantly correlated with UC activity. PGE-MUM levels were positively correlated with Pediatric Ulcerative Colitis Activity Index (r = 0.594), highest Mayo (r = 0.462), the sum of Mayo (r = 0.694), and the sum of Matts (r = 0.613), but not with highest Matt (r = 0.352). The sum of Mayo and the sum of Matts, which reflect total colon inflammation, showed highest correlation with PGE-MUM. C-reactive protein levels did not correlate with any UC activity scores. Erythrocyte sedimentation rate exhibited correlation (r = 0.490) with the sum of Mayo only. Conclusions: PGE-MUM is a reliable biomarker that reflects both the endoscopic and histologic activity of the entire colon in pediatric UC.

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Study of Carnitine/Acylcarnitine and Amino Acid Profile in Children and Adults With Acute Liver Failure

imageObjectives: Fatty acid oxidation defects (FAODs) may underlie or modify the course of acute liver failure (ALF). Overall significance of carnitine/acylcarnitine and amino acid profile in ALF is similarly undetermined. Thus, this study was undertaken to study the abnormalities in carnitine/acylcarnitine and amino acid profile in ALF. Methods: A prospective study was performed including all patients with ALF, and detailed evaluation including metabolic testing was done. Results: A total of 55 patients (33 pediatric and 22 adult patients) were included in the study. Three patients (a 1-year 6-month-old child, a 13-year-old adolescent, and a 21-year-old adult, ie, 5.5% of all) were identified for the study with underlying metabolic etiology, that is, carnitine palmitoyl transferase-1 deficiency, based on the abnormal carnitine/acylcarnitine profile. Almost three-fourths of patients (78%) had evidence of serum hyperaminoacidemia. Thirty-one patients (56%) had evidence of abnormal carnitine/acylcarnitine profile with predominant abnormality being low free carnitine (C0). Higher levels of serum tyrosine (P = 0.002) and lower levels of serum C0 (P = 0.032) in children and higher levels of serum phenyalanine (P = 0.047) in adults predicted poor outcome (death/liver transplant) on univariate analysis. Conclusions: FAODs are not uncommon in ALF with a suggested prevalence of approximately 5.5%. FAODs can cause ALF or modify the natural course of ALF caused by other etiologies. Serum hyperaminoacidemia and low serum free carnitine may predict poor outcome in patients with acute liver failure.

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Nutrient-enriched Infant Formula Is Associated With Higher Weight Gain for Low Birth Weight Infants

imageObjective: Nutrient-enriched formulas were developed to provide extra nutrients and facilitate optimal growth for low birth weight infants. This study examined the association between use of nutrient-enriched formulas and weight gain among low birth weight infants (birth weight 1500–2500 g). Methods: This retrospective cohort study used data from infants enrolled in the Illinois Special Supplemental Nutrition Program for Women, Infants, and Children. Analyses were limited to infants born in 2010 with low birth weight (1500–2500 g, n = 3130). We examined weight gain by use of nutrient-enriched versus standard term formula, which was assigned to the infant by the Women, Infants, and Children program during the first month of life. The analyses used a multilevel mixed-effects linear regression model with random intercepts to determine the association between use of enriched versus standard term formula and weight gain while simultaneously adjusting for confounders including gestational age, birth weight, and history of breast-feeding. Results: Among 670 infants in the 1500 to 1999 g birth weight group, those fed enriched formula gained 46.4 g (95% confidence interval 7.4–85.3, P 6 to 12 months of age compared with infants using standard term infant formula after adjustment for covariates. Similar findings were noted among the 2460 infants in the 2000 to 2500 g birth weight group. Conclusions: Use of nutrient-enriched formulas is associated with higher weight gain in low birth weight infants.

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Autophagy in Hepatocytes in Infants With Alpha-1 ATD and Different Liver Disease Outcomes: A Retrospective Analysis

imageObjectives: It is unclear whether a distinct activity of pathways removing the antitrypsin (AT) protein in Alpha-1-Antitrypsin Deficiency (α1ATD) are associated with an unfavorable predisposition to liver disease in the future. The aim of this study was to determine whether liverspecific activity of AT protein disposal occurs at infancy in α1ATD with PiZZ phenotype (ATZ). Methods: Liver samples of 17 infants with unfavorable ATZ outcome (Group I, n = 8, median age  = 0.35 year) and good outcome (Group II, n = 9, 0.17 year), and 9 with biliary atresia (BA, median age = 0.17 year) as control, were enrolled. For each subject were investigated autophagy activity by mRNA, protein expression (Calnexin, Beclin-1, p62, and Parkin), and hepatocyte ultrastructure with morphometric analyses. Results: No significant differences in gene expression in the liver of infants were found between the 2 ATZ groups. Although a correlation between patients' age and protein expression was observed, the ATZ groups differed Parkin immunohistochemical expression. Moreover, the hepatocytes in ATZ infants with unfavorable outcome were characterized by low Parkin expression and the presence of isolated mitophagosoms and numerous enlarged mitochondria. The mentioned findings differed in patients with BA. Conclusions: Thus, mentioned specific features occurring at infancy may suggest association with poor liver outcome. Parkin low expression could have a potential for disease prognosis and treatment; however, further studies in a greater number of patients are needed.

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Joint ESPGHAN/NASPGHAN Guidelines for the Management of Helicobacter pylori in Children and Adolescents (Update 2016)

imageBackground: Because of the changing epidemiology of Helicobacter pylori infection and low efficacy of currently recommended therapies, an update of the European Society for Paediatric Gastroenterology Hepatology and Nutrition/North American Society for Pediatric Gastroenterology, Hepatology and Nutrition recommendations for the diagnosis and management of H pylori infection in children and adolescents is required. Methods: A systematic review of the literature (time period: 2009–2014) was performed. Representatives of both societies evaluated the quality of evidence using GRADE (Grading of Recommendation Assessment, Development, and Evaluation) to formulate recommendations, which were voted upon and finalized using a Delphi process and face-to-face meeting. Results: The consensus group recommended that invasive diagnostic testing for H pylori be performed only when treatment will be offered if tests are positive. To reach the aim of a 90% eradication rate with initial therapy, antibiotics should be tailored according to susceptibility testing. Therapy should be administered for 14 days, emphasizing strict adherence. Clarithromycin-containing regimens should be restricted to children infected with susceptible strains. When antibiotic susceptibility profiles are not known, high-dose triple therapy with proton pump inhibitor, amoxicillin, and metronidazole for 14 days or bismuth-based quadruple therapy is recommended. Success of therapy should be monitored after 4 to 8 weeks by reliable noninvasive tests. Conclusions: The primary goal of clinical investigation is to identify the cause of upper gastrointestinal symptoms rather than H pylori infection. Therefore, we recommend against a test and treat strategy. Decreasing eradication rates with previously recommended treatments call for changes to first-line therapies and broader availability of culture or molecular-based testing to tailor treatment to the individual child.

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Quality of Life and Parental Worrying in a National Cohort of Biliary Atresia Children Living With Their Native Livers

imageObjectives: The aim of the study was to evaluate health-related quality of life (HRQoL) and parental distress in a national cohort of children with biliary atresia (BA) with their native livers in relation to BA complications and HRQoL of normal population controls. Methods: We invited all Finnish children with BA surviving with their native livers at age 2 to 18 years to participate in 2009 and in 2014. Parents filled the Pediatric Quality of Life Inventory (PedsQL) proxy questionnaire, a survey of their child's health and evaluated parental distress on a visual-analog scale from 0 to 7. Overall participation rates were 80% (12/15) for the longitudinal and 83% (20/24) for the cross-sectional assessment. A control population of 324 children matched for age and sex was randomly picked, and 108 (33%) participated. Results: Overall, patients and controls had comparable HRQoL. Patients reported significantly lower scores for school functioning (P = 0.004) as depicted by missing school or day care due to hospital visits. Eighty-five percent of parents reported extreme worry (7.0) when hearing their child's BA diagnosis. At 6 years after diagnosis, parents reported significantly less worry: median score 3.8 (interquartile range 3.0–5.4, P 

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Apoptotic Enteropathy in a Neonate With Allergic Enterocolitis

imageNo abstract available

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Abdominal Arterial Anomalies in Children With Alagille Syndrome: Surgical Aspects and Outcomes of Liver Transplantation

imageObjectives: Angiogenic defects secondary to gene mutations of JAG1 and NOTCH2, causing arterial anomalies in Alagille syndrome (AGS), are well described in the literature. The study analyzes the frequency of abdominal arterial anomalies in children with AGS with an emphasis on outcomes following liver transplantation (LT). Methods: Between 1988 and 2013, 242 children with AGS were treated at our institution. We performed a retrospective analysis of 55 who underwent LT during the study period. Preoperative abdominal arterial findings, operative reports, arterial reconstruction technique, and early as well as late complications following LT were reviewed specifically focusing on arterial thrombosis. Results: Twenty-five patients had preoperative imaging available for analysis. Twelve of these patients showed celiac trunk stenosis (48.0%), 2, a superior mesenteric artery stenosis (8.0%) and one a stenosis of both renal arteries. Twenty patients (36.3%) underwent standard hepatic reconstruction using the native recipient hepatic artery. Thirty-five patients (63.7%) underwent aortic conduit reconstruction (ACR) from the infrarenal aorta using donor arterial conduits. Hepatic artery thrombosis occurred in 9 patients (16.3%). This number was higher in the standard arterial anastomosis group 7/20 (35.0%) than in those with ACR 2/35 (5.7%, P = 0.0079). Conclusions: In this series, children with AGS pretransplant have a high prevalence of abdominal arterial anomalies. Preoperative abdominal vascular imaging makes it possible to anticipate whether or not a classical arterial revascularization can be performed or whether an ACR is required.

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Rumination Syndrome and Dental Erosions in Children

imageABSTRACT: Rumination syndrome is the effortless regurgitation of recently ingested food with subsequent reswallowing or spitting out. Dental erosion (DE) affects 2% to 5% of the population. DE is defined as loss of tooth structure by a chemical process that does not involve bacteria. Our objective was to compare the frequency of DE among children with rumination syndrome with healthy controls. We enrolled 30 patients 4 to 21 years of age diagnosed with rumination syndrome, and 30 age- and sex-matched healthy control subjects. Patients were evaluated by pediatric dentists for presence of DE with Taji et al a validated grading system. Patients with rumination were more likely to have DE (P 

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Clinical, Biochemical, and Genetic Aspects of Sjögren-Larsson Syndrome

Abstract

Sjögren-Larsson syndrome (SLS) is caused by an autosomal recessive mutation in ALDH3A2, which encodes the fatty aldehyde dehydrogenase responsible for the metabolism of long-chain aliphatic aldehydes and alcohols. The pathophysiologic accumulation of aldehydes in various organs, including the skin, brain, and eyes, leads to characteristic features of ichthyosis, intellectual disability, spastic di-/quadriplegia, and low visual acuity with photophobia. The severity of the clinical manifestations thereof can vary greatly, although most patients are bound to a wheelchair due to contractures. To date, correlations between genotype and phenotype have proven difficult to document due to low disease incidence and high heterogenetic variability in mutations. This review summarizes the clinical characteristics of SLS that have been found to contribute to the prognosis thereof, as well as recent updates from genetic and brain imaging studies. Additionally, the differential diagnoses of SLS are briefly illustrated, covering cerebral palsy and other genetic or neurocutaneous syndromes mimicking the syndrome.

Thumbnail image of graphical abstract

Graphical abstract

Pathogenic variations of ALDH3A2 encoding fatty aldehyde dehydrogenase (FALDH) cause Sjögren-Larsson syndrome



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On the complexity of clinical and molecular bases of neurodegeneration with brain iron accumulation

ABSTRACT

Neurodegeneration with brain iron accumulation (NBIA) are a group of inherited heterogeneous neurodegenerative rare disorders. These patients present with dystonia, spasticity, parkinsonism and neuropsychiatric disturbances, along with brain MRI evidence of iron accumulation. In sum, they are devastating disorders and to date, there is no specific treatment. Ten NBIA genes are accepted: PANK2, PLA2G6, C19orf12, COASY, FA2H, ATP13A2, WDR45, FTL, CP, and DCAF17, and. Nonetheless, a relevant percentage of patients remain without genetic diagnosis, suggesting that other novel NBIA genes remain to be discovered. Overlapping complex clinical pictures render an accurate differential diagnosis difficult. Little is known about the pathophysiology of NBIAs. The reported NBIA genes take part in a variety of pathways: CoA synthesis, lipid and iron metabolism, autophagy, and membrane remodeling. The next generation sequencing revolution has achieved relevant advances in genetics of Mendelian diseases and will provide new genes for NBIAs, which will be investigated according to two main strategies: genes involved in disorders with similar phenotype and genes that play a role in a pathway of interest. To achieve an effective therapy for NBIA patients, a better understanding of the biological process underlying disease is crucial, moving towards a new age of precision medicine.

Thumbnail image of graphical abstract

Graphical abstract



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Disruption of YWHAE gene at 17p13.3 causes learning disabilities and brain abnormalities.

Abstract

There is a broad phenotypic spectrum of patients with 17p13.3 deletions. One of the most prominent features is lissencephaly caused by haploinsufficiency of the gene PAFAH1B1. The deletion of this gene and those distal to it, results in Miller-Dieker syndrome, however there have been many reports of patients with haploinsufficiency of the distal genes alone. The deletions of these genes including YWHAE, CRK and TUSC5 have been studied extensively and YWHAE has been postulated to be the cause of neurological abnormalities. The patients with deletions of the Miller-Dieker syndrome distal region present with variable clinical features including brain abnormalities, growth retardation, developmental delay, facial dysmorphisms and seizures. While there have been many patients reported to have deletions involving the YWHAE gene along with other genes, here we present the first detailed clinical description of a patient with deletion of YWHAE alone, allowing a more accurate characterization of the pathogenicity of YWHAE haploinsufficiency. The patient reported here demonstrated brain abnormalities, learning disabilities, and seizures supporting the role of YWHAE in these features. We review the literature and use this case report to better characterize and further confirm the genotype phenotype relationship of the genes within the critical region of Miller-Dieker Syndrome.

Thumbnail image of graphical abstract

Graphical abstract



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Gait initiation and termination strategies in patients with Prader-Willi syndrome

Gait Initiation (GI) is a functional task representing one of the first voluntary destabilizing behaviours observed in the development of a locomotor pattern as the whole body centre of mass transitions from a...

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Anal cancer: French Intergroup Clinical Practice Guidelines for diagnosis, treatment and follow-up

This document is a summary of the French Intergroup guidelines regarding the management of anal carcinomas, published in November 2016.

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Ductal Stones Recurrence After Extracorporeal Shock Wave Lithotripsy For Difficult Common Bile Duct Stones: Predictive Factors

Extra-corporeal shock wave lithotripsy (ESWL) can be considered in difficult common bile duct stones (DCBDS), with a success rate greater than 90% but data on stone recurrence after ESWL are limited. We performed a retrospective analysis to evaluate long-term outcomes in patients who underwent ESWL for DCBDS.

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Anal cancer: French Intergroup Clinical Practice Guidelines for diagnosis, treatment and follow-up

This document is a summary of the French Intergroup guidelines regarding the management of anal carcinomas, published in November 2016.

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Ductal Stones Recurrence After Extracorporeal Shock Wave Lithotripsy For Difficult Common Bile Duct Stones: Predictive Factors

Extra-corporeal shock wave lithotripsy (ESWL) can be considered in difficult common bile duct stones (DCBDS), with a success rate greater than 90% but data on stone recurrence after ESWL are limited. We performed a retrospective analysis to evaluate long-term outcomes in patients who underwent ESWL for DCBDS.

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Does corrective spine surgery improve the standing balance in patients with adult spinal deformity?

The effect of corrective spine surgery on standing stability in adult spinal deformity (ASD) has not been fully documented.

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Band-specific atypical functional connectivity pattern in childhood autism spectrum disorder

Autism spectrum disorder (ASD), a polygenetic neurodevelopmental disorder that is strongly influenced by genetic factors (Rutter, 2000; Geschwind, 2011), is characterized by impairment of social communication skills and by repetitive and stereotypic behaviors. Increasing evidence has supported the notion that altered neural connectivity is a key neural underpinning of the brain of ASD individuals (Belmonte et al., 2004; Courchesne et al., 2005; Geschwind, 2011; Wass, 2011; Vissers et al., 2012).

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Reduced Electrode Arrays for Acute Electroencephalography: Can Less be More?

Sixty years ago, when Dr. Herbert Jasper and colleagues published the 10/20 method for electroencephalographic (EEG) electrode placement (10/20) (Jasper, 1958), they accomplished two goals. One was the standardization of EEG electrode placement across EEG laboratories. The other was consistent localization of each scalp electrode over a distinct portion of cerebral cortex (proven by drilling holes in cadaver skulls and marking the underlying cortex with India-ink).

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Immune-mediated neuropathies: role of single-fiber conduction velocity.

Immune-mediated neuropathies are acquired diseases that considerably vary in their pattern of nerve involvement. Peripheral nerves may have segmental or diffuse involvement, creating great variability in the exact clinical presentation (Magy et al., 2011; Padua et al., 2013). The diagnosis is based upon clinical examination, which may demonstrate weakness and/or sensory impairment, depending on affected nerves, degree of suffering and type of involvement (Van den Bergh et al., 2010). Neurophysiology supports the diagnostic process, providing information about nerve function.

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A Cluster RCT to Reduce Workers' Sitting Time: Impact on Cardiometabolic Biomarkers.

Purpose: To evaluate the initial and long-term impacts on cardio-metabolic health indicators of the Stand Up Victoria intervention - a 12-month, multicomponent workplace-delivered intervention that successfully reduced overall sitting time, primarily by increasing standing time. Methods: Office worksites (>=1km apart) from a single organization were cluster randomized to intervention (n=7) or control (n=7). Participants were 136 intervention and 95 control desk-based workers (5-39 per worksite; 68% women; mean+/-SD age = 45.6+/-9.4 years). Outcomes, assessed at baseline (0 months), three months, and 12 months, were 14 individual biomarkers of body composition, blood pressure, glucose metabolism, lipid metabolism and a composite overall cardio-metabolic risk score. Intervention effects were assessed by linear mixed models, accounting for repeated measures and clustering, baseline values and potential confounders. Missing data were multiply imputed. Significance was set at p

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Children with Burn Injury Have Impaired Cardiac Output during Submaximal Exercise.

Introduction: Burn trauma damages resting cardiac function; however, it is currently unknown if the cardiovascular response to exercise is likewise impaired. We tested the hypothesis that, in children, burn injury lowers cardiac output (Q) and stroke volume (SV) during submaximal exercise. Methods: Five children with 49+/-4% total body surface area (BSA) burned (2 female, 11.7+/-1 y, 40.4+/-18 kg, 141.1+/-9 cm) and eight similar non-burned controls (5 female, 12.5+/-2 y, 58.0+/-17 kg, 147.3+/-12 cm) with comparable exercise capacity (peak oxygen consumption [peak VO2]: 31.9+/-11 vs. 36.8+/-8 ml O2[middle dot]kg[middle dot]min-1, P=0.39) participated. The exercise protocol entailed a pre-exercise (pre-EX) rest period followed by 3-minute exercise stages at 20 W and 50 W. VO2, heart rate (HR), Q (via non-rebreathing), SV (Q/HR), and arteriovenous O2 difference ([a-v] O2 dif, Q/VO2) were the primary outcome variables. Results: Using a 2-way factorial ANOVA (group [G] x exercise [EX]), we found that Q was ~27% lower in the burned than the non-burned group at 20 W of exercise (burned 5.7+/-1.0 vs. nonburned: 7.9+/-1.8 L[middle dot]min-1) and 50 W of exercise (burned 6.9+/-1.6 vs. nonburned 9.2 +/-3.2 L[middle dot]min-1) (G x EX interaction, P=0.012). SV did not change from rest to exercise in burned children but increased by ~24% in the non-burned group (main effect for EX, P=0.046). Neither [a-v] O2 dif nor VO2 differed between groups at rest or exercise, but HR response to exercise was reduced in the burn group (G x EX interaction, P=0.004). When normalized to BSA, SV (index) was similar between groups; however, Q (index) remained attenuated in the burned group (G x EX interaction, P

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Ectopic protein interactions within BRD4-chromatin complexes drive oncogenic megadomain formation in NUT midline carcinoma [Genetics]

To investigate the mechanism that drives dramatic mistargeting of active chromatin in NUT midline carcinoma (NMC), we have identified protein interactions unique to the BRD4–NUT fusion oncoprotein compared with wild-type BRD4. Using cross-linking, affinity purification, and mass spectrometry, we identified the EP300 acetyltransferase as uniquely associated with BRD4 through the...

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Hsp104 disaggregase at normal levels cures many [PSI+] prion variants in a process promoted by Sti1p, Hsp90, and Sis1p [Genetics]

Overproduction or deficiency of many chaperones and other cellular components cure the yeast prions [PSI+] (formed by Sup35p) or [URE3] (based on Ure2p). However, at normal expression levels, Btn2p and Cur1p eliminate most newly arising [URE3] variants but do not cure [PSI+], even after overexpression. Deficiency or overproduction of Hsp104...

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Selective targeting of point-mutated KRAS through artificial microRNAs [Genetics]

Mutated protein-coding genes drive the molecular pathogenesis of many diseases, including cancer. Specifically, mutated KRAS is a documented driver for malignant transformation, occurring early during the pathogenesis of cancers such as lung and pancreatic adenocarcinomas. Therapeutically, the indiscriminate targeting of wild-type and point-mutated transcripts represents an important limitation. Here, we...

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Shelterin components mediate genome reorganization in response to replication stress [Genetics]

The dynamic nature of genome organization impacts critical nuclear functions including the regulation of gene expression, replication, and DNA damage repair. Despite significant progress, the mechanisms responsible for reorganization of the genome in response to cellular stress, such as aberrant DNA replication, are poorly understood. Here, we show that fission...

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CertaDose™, the revolutionary children’s syringe can prevent accidental overdoses, launches on Indiegogo

CertaDose™ provides a fast, accurate and safe way to measure children's medication. Independent clinical studies have proven that CertaDose™ can reduce critical medicine dosing errors to zero.

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[Webcast] It’s About Time: Every Second Counts with STEMI Patient Care

Time is critical in the ability to care efficiently and effectively for patients, especially for STEMI cases. To remain viable in today's cost- and quality-focused healthcare environment, hospitals must strive to achieve best practice patient care (or exceed such measures) while demonstrating strong clinical competencies for top results at the lowest operational cost. Meanwhile, technology and treatment advances are also changing the way patient care is provided and streamlined, including care from prehospital entry through discharge.During this special 2017 Heart Month education session, Corazon is partnering with Pulsara to discuss:- Patient safety, continuing education and satisfaction within the care continuum;- Technology as a means to provide real-time data to accelerate time-to-treatment for critical care patients;- Developing standardized, evidence-based algorithms to enforce best-practice patient care, compliance, and efficient workflows;- Using measurable data outcomes to drive continuous quality improvement (CQI) and increasing cost savings;And more!Join the Corazon and Pulsara experts this Heart Month to learn how these key elements can enhance your organization's quality of care, treatment efficiencies, and technology resources. Participants will gain an understanding of the best ways to evaluate and then use the information gained to optimize STEMI care delivery within the context of the ever-changing healthcare industry.

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[Webcast] JEMS Webcast with James Woodson & Stein Bronsky

According to the Johns Hopkins School of Medicine, medical error in the U.S. accounts for 250,000 deaths each year. Additionally, the Joint Commission found that 80% of those involve miscommunication — a poor excuse for loss of life.BUT, there are care teams out there who are tackling the crisis. Communication is the most important tool we have in healthcare. With all the advancements we have made in clinical care, why haven't we improved this important tool? Why are we still using a mix of outdated technologies? This is especially true in time sensitive emergencies, where additional factors compound not only the complexity of treatment but the size of the teams we communicate with.Join Dr. E. Stein Bronsky, medical director for the Colorado Springs Fire Department and AMR in El Paso County, and Dr. James Woodson, emergency physician and founder of Pulsara, and recent JEMS Innovator in EMS Award winner, as they explore the steps Colorado Springs took to improve communication in their community.

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[Webcast] It’s About Time: Every Second Counts with STEMI Patient Care

Time is critical in the ability to care efficiently and effectively for patients, especially for STEMI cases. To remain viable in today's cost- and quality-focused healthcare environment, hospitals must strive to achieve best practice patient care (or exceed such measures) while demonstrating strong clinical competencies for top results at the lowest operational cost. Meanwhile, technology and treatment advances are also changing the way patient care is provided and streamlined, including care from prehospital entry through discharge.During this special 2017 Heart Month education session, Corazon is partnering with Pulsara to discuss:- Patient safety, continuing education and satisfaction within the care continuum;- Technology as a means to provide real-time data to accelerate time-to-treatment for critical care patients;- Developing standardized, evidence-based algorithms to enforce best-practice patient care, compliance, and efficient workflows;- Using measurable data outcomes to drive continuous quality improvement (CQI) and increasing cost savings;And more!Join the Corazon and Pulsara experts this Heart Month to learn how these key elements can enhance your organization's quality of care, treatment efficiencies, and technology resources. Participants will gain an understanding of the best ways to evaluate and then use the information gained to optimize STEMI care delivery within the context of the ever-changing healthcare industry.

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[Webcast] JEMS Webcast with James Woodson & Stein Bronsky

According to the Johns Hopkins School of Medicine, medical error in the U.S. accounts for 250,000 deaths each year. Additionally, the Joint Commission found that 80% of those involve miscommunication — a poor excuse for loss of life.BUT, there are care teams out there who are tackling the crisis. Communication is the most important tool we have in healthcare. With all the advancements we have made in clinical care, why haven't we improved this important tool? Why are we still using a mix of outdated technologies? This is especially true in time sensitive emergencies, where additional factors compound not only the complexity of treatment but the size of the teams we communicate with.Join Dr. E. Stein Bronsky, medical director for the Colorado Springs Fire Department and AMR in El Paso County, and Dr. James Woodson, emergency physician and founder of Pulsara, and recent JEMS Innovator in EMS Award winner, as they explore the steps Colorado Springs took to improve communication in their community.

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Pulsara Intro - Mary’s Having A Stroke [STOP STROKE]

Learn how having the Pulsara Platform works as an entire emergency network in the palm of your hand.The easy-to-adopt platform links your entire Emergency Response Team with a tap — eliminating unnecessary pages, calls, operators, faxes and emails. Breathtakingly simple, fast and information rich.

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MagneGrip completes Exhaust Removal System installation in St. Louis

CINCINNATI — MagneGrip Group recently completed a large-scale urban program in St. Louis, Missouri for the St. Louis Fire Department. Comprised of 30 engine houses -- each with unique specifications -- the project encompassed installation of Exhaust Removal Systems within a contract stipulated prompt and efficient timeframe. The new MagneGrip Vehicle Exhaust Removal System replaced a high maintenance ...

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New ambulance crash test methods aim to keep EMS providers safe

NIOSH's video series highlights demos and improvements to patient compartment design

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[Webcast] It’s About Time: Every Second Counts with STEMI Patient Care

Time is critical in the ability to care efficiently and effectively for patients, especially for STEMI cases. To remain viable in today's cost- and quality-focused healthcare environment, hospitals must strive to achieve best practice patient care (or exceed such measures) while demonstrating strong clinical competencies for top results at the lowest operational cost. Meanwhile, technology and treatment advances are also changing the way patient care is provided and streamlined, including care from prehospital entry through discharge.During this special 2017 Heart Month education session, Corazon is partnering with Pulsara to discuss:- Patient safety, continuing education and satisfaction within the care continuum;- Technology as a means to provide real-time data to accelerate time-to-treatment for critical care patients;- Developing standardized, evidence-based algorithms to enforce best-practice patient care, compliance, and efficient workflows;- Using measurable data outcomes to drive continuous quality improvement (CQI) and increasing cost savings;And more!Join the Corazon and Pulsara experts this Heart Month to learn how these key elements can enhance your organization's quality of care, treatment efficiencies, and technology resources. Participants will gain an understanding of the best ways to evaluate and then use the information gained to optimize STEMI care delivery within the context of the ever-changing healthcare industry.

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[Webcast] JEMS Webcast with James Woodson & Stein Bronsky

According to the Johns Hopkins School of Medicine, medical error in the U.S. accounts for 250,000 deaths each year. Additionally, the Joint Commission found that 80% of those involve miscommunication — a poor excuse for loss of life.BUT, there are care teams out there who are tackling the crisis. Communication is the most important tool we have in healthcare. With all the advancements we have made in clinical care, why haven't we improved this important tool? Why are we still using a mix of outdated technologies? This is especially true in time sensitive emergencies, where additional factors compound not only the complexity of treatment but the size of the teams we communicate with.Join Dr. E. Stein Bronsky, medical director for the Colorado Springs Fire Department and AMR in El Paso County, and Dr. James Woodson, emergency physician and founder of Pulsara, and recent JEMS Innovator in EMS Award winner, as they explore the steps Colorado Springs took to improve communication in their community.

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Pulsara Intro - Mary’s Having A Stroke [STOP STROKE]

Learn how having the Pulsara Platform works as an entire emergency network in the palm of your hand.The easy-to-adopt platform links your entire Emergency Response Team with a tap — eliminating unnecessary pages, calls, operators, faxes and emails. Breathtakingly simple, fast and information rich.

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Pulsara Stop STEMI Case Demonstration

Do you have one protocol for incoming STEMI patients from EMS? Another one for in-hospital? What about a patient who's being transferred? Instead, use one standardized communication protocol regardless of patient location. STOP STEMI provides online and wireless delivery of acute care coordination information to teams of healthcare providers.Why do we have protocols in the first place? To reduce variability. Using the same telecommunications platform for all acute care conditions will improve STEMI communications.

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[Webcast] It’s About Time: Every Second Counts with STEMI Patient Care

Time is critical in the ability to care efficiently and effectively for patients, especially for STEMI cases. To remain viable in today's cost- and quality-focused healthcare environment, hospitals must strive to achieve best practice patient care (or exceed such measures) while demonstrating strong clinical competencies for top results at the lowest operational cost. Meanwhile, technology and treatment advances are also changing the way patient care is provided and streamlined, including care from prehospital entry through discharge.During this special 2017 Heart Month education session, Corazon is partnering with Pulsara to discuss:- Patient safety, continuing education and satisfaction within the care continuum;- Technology as a means to provide real-time data to accelerate time-to-treatment for critical care patients;- Developing standardized, evidence-based algorithms to enforce best-practice patient care, compliance, and efficient workflows;- Using measurable data outcomes to drive continuous quality improvement (CQI) and increasing cost savings;And more!Join the Corazon and Pulsara experts this Heart Month to learn how these key elements can enhance your organization's quality of care, treatment efficiencies, and technology resources. Participants will gain an understanding of the best ways to evaluate and then use the information gained to optimize STEMI care delivery within the context of the ever-changing healthcare industry.

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[Webcast] JEMS Webcast with James Woodson & Stein Bronsky

According to the Johns Hopkins School of Medicine, medical error in the U.S. accounts for 250,000 deaths each year. Additionally, the Joint Commission found that 80% of those involve miscommunication — a poor excuse for loss of life.BUT, there are care teams out there who are tackling the crisis. Communication is the most important tool we have in healthcare. With all the advancements we have made in clinical care, why haven't we improved this important tool? Why are we still using a mix of outdated technologies? This is especially true in time sensitive emergencies, where additional factors compound not only the complexity of treatment but the size of the teams we communicate with.Join Dr. E. Stein Bronsky, medical director for the Colorado Springs Fire Department and AMR in El Paso County, and Dr. James Woodson, emergency physician and founder of Pulsara, and recent JEMS Innovator in EMS Award winner, as they explore the steps Colorado Springs took to improve communication in their community.

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Pulsara Intro - Mary’s Having A Stroke [STOP STROKE]

Learn how having the Pulsara Platform works as an entire emergency network in the palm of your hand.The easy-to-adopt platform links your entire Emergency Response Team with a tap — eliminating unnecessary pages, calls, operators, faxes and emails. Breathtakingly simple, fast and information rich.

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Pulsara Stop STEMI Case Demonstration

Do you have one protocol for incoming STEMI patients from EMS? Another one for in-hospital? What about a patient who's being transferred? Instead, use one standardized communication protocol regardless of patient location. STOP STEMI provides online and wireless delivery of acute care coordination information to teams of healthcare providers.Why do we have protocols in the first place? To reduce variability. Using the same telecommunications platform for all acute care conditions will improve STEMI communications.

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Pulsara Intro - Mary’s Having A Stroke [STOP STROKE]

Learn how having the Pulsara Platform works as an entire emergency network in the palm of your hand.The easy-to-adopt platform links your entire Emergency Response Team with a tap — eliminating unnecessary pages, calls, operators, faxes and emails. Breathtakingly simple, fast and information rich.

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Pulsara Stop STEMI Case Demonstration

Do you have one protocol for incoming STEMI patients from EMS? Another one for in-hospital? What about a patient who's being transferred? Instead, use one standardized communication protocol regardless of patient location. STOP STEMI provides online and wireless delivery of acute care coordination information to teams of healthcare providers.Why do we have protocols in the first place? To reduce variability. Using the same telecommunications platform for all acute care conditions will improve STEMI communications.

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Pulsara Stop STEMI Case Demonstration

Do you have one protocol for incoming STEMI patients from EMS? Another one for in-hospital? What about a patient who's being transferred? Instead, use one standardized communication protocol regardless of patient location. STOP STEMI provides online and wireless delivery of acute care coordination information to teams of healthcare providers.Why do we have protocols in the first place? To reduce variability. Using the same telecommunications platform for all acute care conditions will improve STEMI communications.

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Predictors of Clinical Response and Remission at 1 Year Among a Multicenter Cohort of Patients with Inflammatory Bowel Disease Treated with Vedolizumab

Abstract

Background

Vedolizumab (VDZ) has demonstrated long-term efficacy in Crohn's disease (CD) and ulcerative colitis (UC) in phase III trials.

Aims

Our aim was to evaluate the efficacy of VDZ at week 54 in inflammatory bowel disease (IBD) in a multicenter cohort of patients.

Methods

Adult patients completing induction therapy with VDZ were eligible for this study. Clinical response and remission was assessed using the Harvey–Bradshaw Index (HBI) for CD, the Simple Clinical Colitis Activity Index for UC and physician assessment.

Results

Among 136 total patients (96 CD and 40 UC), 76 (56%) demonstrated clinical response or remission at week 54. In univariate analysis, for patients with CD concomitant initiation of immunomodulator therapy (2.71, 95% CI 1.11–6.57), the addition of an immunomodulator (OR 11.49, 3.16–41.75) and CRP < 3 (4.92, 95% CI 1.99–12.15) was associated with increased odds of clinical response or remission at week 54. For UC patients, hospitalization after VDZ induction was associated with decreased odds of response or remission at week 54 (OR 0.22, 95% CI 0.05–0.88). On multivariate analysis in CD, addition of an immunomodulator (OR 8.33, 95% CI 2.15–32.26) remained significant predictors of clinical response or remission at week 54.

Conclusions

Among a multicenter cohort of patients with IBD demonstrating primary response to VDZ, the addition of combination therapy with an immunomodulator is a significant predictor of clinical response or remission at week 54 in patients with CD.



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Choledochoscopic Identification of a Hepatic/Cystic Artery Pseudoaneurysm in a Patient with Hematemesis After Laparoscopic Cholecystectomy



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Immunological Mechanisms of Adsorptive Cytapheresis in Inflammatory Bowel Disease

Abstract

Ulcerative colitis and Crohn's disease are the two main forms of inflammatory bowel disease (IBD). The study of immunological pathways involved in the onset of IBD is of fundamental importance to identify potential biological markers of disease activity and specific targets for therapy. Removing excess and activated circulating leukocytes with adsorptive cytapheresis has been shown to be a potentially effective treatment for patients with an inflamed bowel. Adsorptive cytapheresis is a non-pharmacological approach for active IBD, in which known sources of inflammatory cytokines such as activated myeloid lineage leucocytes are selectively depleted from the circulatory system. The decrease in inflammatory load caused by removing these cells is thought to enhance drug therapy and thereby promote disease remission. The benefit of cytapheresis appears to rest upon its ability to reduce levels of certain immune cell populations; however, whether this depletion results in further changes in lymphocyte populations and cytokine production needs further clarification. In this review, we aim to summarize existing evidence on the role of cytapheresis in patients with IBD, its effect on cytokine levels and cellular populations, and to discuss its potential impact on disease activity.



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Initial Report of Outpatient Management of Acute Cholangitis via ERCP



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Assessment of Anti-vinculin and Anti-cytolethal Distending Toxin B Antibodies in Subtypes of Irritable Bowel Syndrome

Abstract

Background

Antibodies to cytolethal distending toxin B (CdtB) and vinculin are novel biomarkers that rule-in and differentiate irritable bowel syndrome with diarrhea (IBS-D) from other causes of diarrhea and healthy controls.

Aim

To determine whether these antibodies can also diagnose and differentiate other IBS subtypes.

Methods

Subjects with IBS-D based on Rome III criteria (n = 2375) were recruited from a large-scale multicenter clinical trial (TARGET 3). Healthy subjects without gastrointestinal (GI) diseases or symptoms (n = 43) and subjects with mixed IBS (IBS-M) (n = 25) or IBS with constipation (IBS-C) (n = 30) were recruited from two major medical centers. Plasma levels of anti-CdtB and anti-vinculin antibodies in all subjects were determined by enzyme-linked immunosorbent assay. Optical densities of ≥1.68 and ≥2.80 were considered positive for anti-vinculin and anti-CdtB, respectively. Plasma levels of anti-CdtB and anti-vinculin antibodies were highest in IBS-D and lowest in IBS-C and healthy controls (P < 0.001). Levels in IBS-C subjects were not statistically different from controls (P > 0.1). Positivity for anti-CdtB or anti-vinculin resulted in a statistically significant negative gradient from IBS-D (58.1%) to IBS-M (44.0%), IBS-C (26.7%), and controls (16.3%) (P < 0.001).

Conclusions

Anti-CdtB and anti-vinculin titers and positivity rates differ in IBS subtypes, with higher antibody levels and positivity rates in IBS-D and IBS-M, and lower levels in IBS-C subjects that are similar to those in healthy controls. These antibodies appear useful in the diagnosis of IBS-M and IBS-D, but not IBS-C. Furthermore, these findings suggest that IBS-C is pathophysiologically distinct from subtypes with diarrheal components (i.e., IBS-M and IBS-D).



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Low-Dose Cannabidiol Is Safe but Not Effective in the Treatment for Crohn’s Disease, a Randomized Controlled Trial

Abstract

Background

Cannabidiol (CBD) is an anti-inflammatory cannabinoid shown to be beneficial in a mouse model of IBD. Lacking any central effect, cannabidiol is an attractive option for treating inflammatory diseases.

Aim

To assess the effects of cannabidiol on Crohn's disease in a randomized placebo-controlled trial.

Patients and Methods

Twenty patients aged 18–75 years with a Crohn's disease activity index (CDAI) >200 were randomized to receive oral (10 mg) CBD or placebo twice daily. Patients did not respond to standard treatment with steroids (11 patients), thiopurines (14), or TNF antagonists (11). Disease activity and laboratory parameters were assessed during 8 weeks of treatment and 2 weeks thereafter. Other medical treatment remained unchanged.

Results

Of 20 patients recruited 19 completed the study. Their mean age was 39 ± 15, and 11 were males. The average CDAI before cannabidiol consumption was 337 ± 108 and 308 ± 96 (p = NS) in the CBD and placebo groups, respectively. After 8 weeks of treatment, the index was 220 ± 122 and 216 ± 121 in the CBD and placebo groups, respectively (p = NS). Hemoglobin, albumin, and kidney and liver function tests remained unchanged. No side effects were observed.

Conclusion

In this study of moderately active Crohn's disease, CBD was safe but had no beneficial effects. This could be due to lack of effect of CBD on Crohn's disease, but could also be due to the small dose of CBD, the small number of patients in the study, or the lack of the necessary synergism with other cannabinoids. Further investigation is warranted.

ClinicalTrials.gov

NCT01037322.



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Incidence of and Risk Factors for Free Bowel Perforation in Patients with Crohn’s Disease

Abstract

Background

Incidence of and risk factors for intestinal free perforation (FP) in patients with Crohn's disease (CD) are not established.

Aim

To establish rate of and risk factors for FP in a large cohort of CD patients.

Methods

Medical records of CD patients who visited Asan Medical Center from June 1989 to December 2012 were reviewed. After matching the FP patients to controls (1:4) by gender, year, and age at CD diagnosis, and disease location, their clinical characteristics were compared using conditional logistic regression analysis.

Results

Among 2043 patients who were included in our study cohort, 44 patients (2.15%) developed FP over a median follow-up period of 79.8 months (interquartile range 37.3–124.6), with an incidence of 3.18 per 1000 person-years [95% confidence interval (CI) 2.37–4.28]. All 44 patients underwent emergency surgery, and eight patients underwent reoperation within 12 months (8/44, 18.2%). Multivariable-adjusted analysis revealed that anti-TNF therapy [odds ratio (OR), 3.73; 95% CI 1.19–11.69; p = 0.024] was associated with an increased risk of FP.

Conclusions

The incidence of FP in a large cohort of Korean CD patients was 2.15%, which was similar to that in Western reports. Anti-TNF therapy could be risk factors for FP.



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Helicobacter pylori -Associated Upper Gastrointestinal Symptoms: FD or HpD?



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Safer VL intubation: Recapitate the device after blade insertion

Another tip for safer intubations.

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Remember 2 Things: BVM use

Do you know how to properly use your BVM? Steve Whitehead gives two pieces of advice when it comes to BVM use.

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Safer VL intubation: Recapitate the device after blade insertion

Another tip for safer intubations.

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Remember 2 Things: BVM use

Do you know how to properly use your BVM? Steve Whitehead gives two pieces of advice when it comes to BVM use.

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Why I came to accept being called an 'ambulance driver'

It is time for all of us to embrace our inner ambulance driver. As much as we fight the label, we are still ambulance drivers. It's possible to be ambulance drivers, as well as highly-trained and experienced medical professionals. I can only hope that others realize our training and experience as well. Nevertheless, without ambulance drivers there would be no reason for any of us, so we might as ...

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EMS Week 2017: Top tweets from chiefs, departments and field personnel

By EMS1 Staff During EMS Week 2017, paramedic chiefs, agency public information officers, hospitals and field personnel have taken to Twitter to share their appreciation for EMS. Here is a collection of our favorite tweets. Share this article with the hashtag #EMSWeek or #EMSWeek2017 and tweet with us @EMS1. Here's another terrific thank you note going out to a lucky customer. Watch your deliveries! ...

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A novel variant in MED12 gene: Further delineation of phenotype

MED12 is a multiprotein mediator complex, which has a role in cell growth and differentiation and has been implicated in three distinct X-linked intellectual disability syndromes with distinctive clinical features. These include Opitz–Kaveggia syndrome (FG syndrome), Lujan syndrome, and X-linked Ohdo syndrome. Recently MED12 variants have been implicated in isolated X-linked intellectual disability. We describe a 5-year-old male patient with intellectual disability and facial dysmorphism and a novel variant in MED12 gene identified by Whole Exome Sequencing. His dysmorphic facial features are distinct from the previously described phenotypes. With a strong genotype–phenotype correlation that is already known for MED12, this could be a new phenotype linked to MED12, thus expanding the phenotypic spectrum of MED12-related disorders.



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Renal anomalies and lymphedema distichiasis syndrome. A rare association?

Lymphedema distichiasis syndrome (LDS) is a rare, autosomal dominant genetic condition, characterized by lower limb lymphedema and distichiasis. Other associated features that have been reported include varicose veins, cleft palate, congenital heart defects, and ptosis. We update a previously reported family with a pathogenic variant in FOXC2 (c.412-413insT) where five affected individuals from the youngest generation had congenital renal anomalies detected on prenatal ultrasound scan. These included four fetuses with hydronephrosis and one with bilateral renal agenesis. A further child with LDS had prominence of the left renal pelvis on postnatal renal ultrasound. We also describe a second family in whom the proband and his affected son had congenital renal anomalies; left ectopic kidney, right duplex kidney, and bilateral duplex collecting systems with partial duplex kidney with mild degree of malrotation, respectively. Foxc2 is expressed in the developing kidney and therefore congenital renal anomalies may well be associated, potentially as a low penetrance feature. We propose that all individuals diagnosed with LDS should have a baseline renal ultrasound scan at diagnosis. It would also be important to consider the possibility of renal anomalies during prenatal ultrasound of at risk pregnancies, and that the presence of hydronephrosis may be an indication that the baby is affected with LDS.



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Safer VL intubation: Recapitate the device after blade insertion

Another tip for safer intubations.

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Remember 2 Things: BVM use

Do you know how to properly use your BVM? Steve Whitehead gives two pieces of advice when it comes to BVM use.

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Safer VL intubation: Recapitate the device after blade insertion

Another tip for safer intubations.

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Remember 2 Things: BVM use

Do you know how to properly use your BVM? Steve Whitehead gives two pieces of advice when it comes to BVM use.

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Bottom of an iceberg: undiagnosed aortic aneurysm masquerading as vocal cord palsy

http://otorhinolaryngology-crete.blogspot.com/2017/05/undiagnosed-aortic-aneurysm.html
 από Rao, V. U. S., Chatterjee, S., Swamy, S. S. στο BMJ Case Reports Last 6 Issues Μετάφραση άρθρου Description A previously healthy woman aged 60 years was referred to a tertiary referral cancer centre with change of voice for 1 week suspecting neoplastic aetiology on account of her tobacco chewing habit of more than 20 years. No history of voice abuse, fever or cough was there preceding the onset of the change of voice. She did not have any previous history of hospitalisation or diagnosed comorbidities. On clinical examination, her pulse rate was 82 bpm; blood pressure was 130/90 mm Hg and respiratory rate was 12/min.  Video laryngoscopy examination revealed left vocal cord palsy with no obvious lesion. A whole-body F18 FDG PET–CT scan revealed the presence of 6.6x4.8x6.7 cm lobulated sacullar aneurysm arising from the aortic arch between the origins of the left common carotid and subclavian arteries (figures 1 and 2). The likely mycotic aneurysm caused significant surrounding metabolically active inflammatory changes (figure 3).

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182
6948891480

Tap, tap, who's there? It's localized muscle activity elicited by the human stretch reflex

Abstract

Skeletal muscles are the motors that make us move. Discharge of α-motoneurones causes muscle fibres they innervate to contract and generate force.

This article is protected by copyright. All rights reserved



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Long-range transport of Xe-133 emissions under convective and non-convective conditions

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Publication date: September 2017
Source:Journal of Environmental Radioactivity, Volumes 175–176
Author(s): J. Kuśmierczyk-Michulec, M. Krysta, M. Kalinowski, E. Hoffmann, J. Baré
To investigate the transport of xenon emissions, the Provisional Technical Secretariat (PTS) operates an Atmospheric Transport Modelling (ATM) system based on the Lagrangian Particle Dispersion Model FLEXPART. The air mass trajectory ideally provides a "link" between a radionuclide release and a detection confirmed by radionuclide measurements. This paper investigates the long-range transport of Xe-133 emissions under convective and non-convective conditions, with special emphasis on evaluating the changes in the simulated activity concentration values due to the inclusion of the convective transport in the ATM simulations. For that purpose a series of 14 day forward simulations, with and without convective transport, released daily in the period from 1 January 2011 to 30 June 2013, were analysed. The release point was at the ANSTO facility in Australia. The simulated activity concentrations for the period January 2011 to February 2012 were calculated using the daily emission values provided by the ANSTO facility; outside the aforementioned period, the median daily emission value was used. In the simulations the analysed meteorological input data provided by the European Centre for Medium-Range Weather Forecasts (ECMWF) were used with the spatial resolution of 0.5°. It was found that the long-range transport of Xe-133 emissions under convective conditions, where convection was included in the ATM simulation, led to a small decrease in the activity concentration, as compared to transport without convection. In special cases related to deep convection, the opposite effect was observed. Availability of both daily emission values and measured Xe-133 activity concentration values was an opportunity to validate the simulations. Based on the paired t-test, a 95% confidence interval for the true mean difference between simulations without convective transport and measurements was constructed. It was estimated that the overall uncertainty lies between 0.08 and 0.25 mBq/m3. The uncertainty for the simulations with the convective transport included is slighted shifted to the lower values and is in the range between 0.06 and 0.20 mBq/m3.



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Environmental radiation dosimetry at Argentine Antarctic Marambio Base (64° 13′ S, 56° 43′ W): preliminary results

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Publication date: September 2017
Source:Journal of Environmental Radioactivity, Volumes 175–176
Author(s): Alba Zanini, Vicente Ciancio, Monica Laurenza, Marisa Storini, Adolfo Esposito, Juan Carlos Terrazas, Paolo Morfino, Alessandro Liberatore, Gustavo Di Giovan
The preliminary results obtained in the first environmental radiation dosimetry campaign performed in the Antarctic region are presented. This experiment is carried out in the framework of CORA (COsmic Rays in Antarctica) Project, a collaboration between Argentine and Italian institutions. After a feasibility study performed in the Antarctic summer 2013, a new campaign has been carried out, started in March 2015, to measure various components of cosmic ray induced secondary atmospheric radiation at the Argentine Marambio Base (Antarctica; 196 m a.s.l., 64°13′ S, 56°43′ W). Due to a very few dosimetric data available in literature at high southern latitudes, accurate measurements are performed by using a set of different active and passive detectors. Special attention is dedicated to measure the neutron ambient dose equivalent in different energy ranges, by using an active detector, the Atomtex Rem Counter, for neutron energy between 0.025 eV–14 MeV and a set of passive bubble dosimeters, sensitive to thermal neutrons and neutrons in the energy range 100 keV–20 MeV. The results obtained in the first six months of measurements for X and γ radiation and for low and intermediate energy neutrons (En ≤ 20 MeV) are presented in this paper and show that at high latitude, also at sea level and at distance from the South Magnetic Pole, the ambient dose equivalent is significant, in particular for the high contribution of neutron component. This involves that at higher altitude (i.e. Antarctic Plateau, over 3000 m a.s.l.) the yearly ambient dose equivalent could be higher than the limit of 1 mSv recommended for general public by the International Commission on Radiological Protection (ICRP).



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Longitudinal Physical Activity, Body Composition, and Physical Fitness in Preschoolers.

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Purpose: To investigate longitudinal associations of objectively-measured physical activity (PA) and sedentary behavior (SB) with body composition and physical fitness at a 12-month follow-up in healthy Swedish 4-year-old children. Methods: The data from the population-based MINISTOP trial were collected between 2014-2016, and this study included the 138 children who were in the control group. PA and SB were assessed using the wrist-worn ActiGraph (wGT3x-BT) accelerometer during seven 24-hour periods, and subsequently, defined as SB, light-intensity PA (LPA), moderate-intensity PA (MPA), vigorous-intensity PA (VPA), and moderate-to-vigorous PA (MVPA). Body composition was measured using air-displacement plethysmography, and physical fitness (cardiorespiratory fitness, lower and upper muscular strength as well as motor fitness) by the PREFIT fitness battery. Linear regression and isotemporal substitution models were applied. Results: Greater VPA and MVPA at the age of 4.5 were associated with higher fat-free mass index (FFMI) at 5.5 years of age (p

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Physical Activity and Sleep Quality in Breast Cancer Survivors: A Randomized Trial.

Purpose: Data from large randomized controlled trials confirming sleep quality improvements with aerobic physical activity have heretofore been lacking for post-primary treatment breast cancer survivors. Our primary purpose for this report was to determine the effects of a physical activity behavior change intervention, previously reported to significantly increase physical activity behavior, on sleep quality in post-primary treatment breast cancer survivors. Methods: Post-primary treatment breast cancer survivors (n=222) were randomized to a 3-month physical activity behavior change intervention (BEAT Cancer) or usual care. Self-report (Pittsburgh Sleep Quality Index [PSQI]) and actigraphy (latency and efficiency) sleep outcomes were measured at baseline, 3 months (M3), and 6 months (M6). Results: After adjusting for covariates, BEAT Cancer significantly improved PSQI global sleep quality when compared with usual care at M3 (mean between group difference [M] = -1.4; 95% CI = -2.1 to -0.7; p

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