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https://www.readbyqxmd.com/read/29054114/characterization-of-melanosomes-and-melanin-in-japanese-patients-with-hermansky-pudlak-syndrome-types-1-4-6-and-9
#1
Ken Okamura, Yuko Abe, Yuta Araki, Kazumasa Wakamatsu, Mariko Seishima, Takafumi Umetsu, Atsushi Kato, Masakazu Kawaguchi, Masahiro Hayashi, Yutaka Hozumi, Tamio Suzuki
Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by oculocutaneous albinism (OCA), a bleeding tendency and ceroid deposition. Most of the causative genes for HPS encode subunits of the biogenesis of lysosome-related organelles complex (BLOC). In this study, we identified one patient each with HPS4, HPS6 and HPS9 by whole-exome sequencing. Next, we analyzed hair samples from the three patients and representative patients with HPS1 and controls using electron microscopy and chemical methods...
October 20, 2017: Pigment Cell & Melanoma Research
https://www.readbyqxmd.com/read/29050864/outpatient-parenteral-antibiotic-therapy-evaluation-of-practices-and-limits-of-use-in-rural-areas-in-france
#2
C Triffault-Fillit, T Ferry, T Perpoint, L Adélaïde, S Le Ngoc Tho, F Ader, C Chidiac, F Valour
OBJECTIVES: To evaluate outpatient parenteral antibiotic therapy (OPAT) practices in a French rural area. MATERIAL AND METHODS: Descriptive study assessing knowledge, practices, and limitations of OPAT use among hospital practitioners (HP), family physicians (FP), and private nurses (PN). RESULTS: OPAT (mainly ceftriaxone and penicillins) was used by 69.6%, 73.3%, and 97.7% of the 23 HPs, 45 FPs, and 46 PNs mostly for respiratory or urinary tract infections, bacteremia, and/or multidrug-resistant bacterial infections...
October 16, 2017: Médecine et Maladies Infectieuses
https://www.readbyqxmd.com/read/29050676/effects-of-gripping-volume-in-the-mechanical-strengths-of-orthodontic-mini-implant
#3
Yu-Chuan Tseng, Ju-Hui Wu, Hong-Sen Chen, Chun-Ming Chen, Chun-Chan Ting
The objective of study was to investigate the correlation between the mechanical strengths [insertion torque (IT); resonance frequency (RF); and horizontal pullout strength (HPS)] and gripping volume (GV) of mini-implants. Thirty mini-implants of three types (Type A: 2 mm × 10 mm, cylindrical, titanium alloy; Type B: 2 mm × 10 mm, tapered, stainless steel; and Type C: 2 mm × 11 mm, cylindrical, titanium alloy) were inserted 7 mm into artificial bones. One-way analysis of variance and Spearman's test were applied to assess intergroup comparisons and intragroup correlations...
November 2017: Kaohsiung Journal of Medical Sciences
https://www.readbyqxmd.com/read/29050114/-effect-of-interferon-%C3%AE-on-airway-inflammation-following-respiratory-syncytial-virus-reinfection-in-mice
#4
X R Long, J Xie, W Li, K T Zhao, X H Xie, L J Wang, L Ren, E M Liu, Y Deng
Objective: To identify the role of interferon (IFN)-γ during respiratory syncytial virus (RSV) re-infection in mice. Method: Female wild type C57BL/6 mice and IFN-γ knockout mice (IFN-γ(-/-) mice) at the age of 6 to 8 weeks were randomly divided into two groups: control group and RSV group, according to random number table.Each group was further divided into primary infection group and re-infection group.There were 8 groups.Mice were sacrificed on days 5, 7, 14 to collect samples.There were 5-8 mice in each group at each time point...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29049128/changing-pathological-diagnosis-from-hyperplastic-polyp-to-sessile-serrated-adenoma-systematic-review-and-meta-analysis
#5
Yaron Niv
The WHO published a new classification of colonic polyps in 2010, including the group of serrated polyps, which can be divided into hyperplastic polyps (HP), traditional serrated adenomas, and sessile serrated adenomas (SSA) or polyps. To assess the rate of re-diagnosis of HP to SSA and to look for possible predictors for changing the diagnosis. English Medical literature searches were performed for 'reassessment' OR 'reclassification' AND 'hyperplastic polyp' OR 'sessile serrated adenoma' till 31 January 2017...
October 18, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29044644/hermansky-pudlak-syndrome-report-of-two-patients-with-updated-genetic-classification-and-management-recommendations
#6
REVIEW
Manuela Loredana Asztalos, Kristian T Schafernak, Jayla Gray, Adam Berry, Amy S Paller, Anthony J Mancini
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder caused by mutations in one of nine genes involved in the packaging and formation of specialized lysosomes, including melanosomes and platelet-dense granules. The cardinal features are pigmentary dilution, bleeding diathesis, and accumulation of ceroid-like material in reticuloendothelial cells. Pulmonary fibrosis induced by tissue damage is seen in the most severe forms, and one subtype is characterized by immunodeficiency. We describe two patients with HPS type 1 and review the updated gene-based classification, clinical features, and recommendations for evaluation and follow-up...
October 16, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29044469/co-differentiation-of-human-pluripotent-stem-cells-derived-cardiomyocytes-and-endothelial-cells-from-cardiac-mesoderm-provides-a-three-dimensional-model-of-cardiac-microtissue
#7
Elisa Giacomelli, Milena Bellin, Valeria V Orlova, Christine L Mummery
The formation of cardiac mesodermal subtypes is highly regulated in time and space during heart development. In vitro models based on human pluripotent stem cells (hPS cells) provide opportunities to study mechanisms underlying fate choices governing lineage specification from common cardiovascular progenitors in human embryos. The generation of cardiac endothelial cells in particular allows the creation of complex models of cardiovascular disorders in which either cardiomyocytes or endothelial cells are affected...
October 18, 2017: Current Protocols in Human Genetics
https://www.readbyqxmd.com/read/29036666/do-israeli-health-promoting-schools-contribute-to-students-healthy-eating-and-physical-activity-habits
#8
Samah Hayek, Riki Tessler, Shiran Bord, Ronit Endevelt, Carmit Satran, Irit Livne, Mohammed Khatib, Yosi Harel-Fisch, Orna Baron-Epel
The Israeli Health Promoting School Network (HPSN) is actively committed to enhancing a healthy lifestyle for the entire school population. This study aimed to explore the contribution of school participation in the HPSN and students' individual characteristics to healthy eating and physical activity habits among Israeli school children aged 10-12 years. A cross-sectional survey was conducted among 4166 students in grades 4-6 from 28 schools. The schools were selected from a sample of HPSN affiliated and non-HPSN schools...
October 4, 2017: Health Promotion International
https://www.readbyqxmd.com/read/29030700/pyloric-stenosis-an-enigma-more-than-a-century-after-the-first-successful-treatment
#9
REVIEW
Yousef El-Gohary, Abdelhafeez Abdelhafeez, Elizabeth Paton, Ankush Gosain, Andrew J Murphy
Despite hypertrophic pyloric stenosis (HPS) being one of the most frequently treated pediatric surgical conditions, its etiology remains incompletely understood. We review the diagnosis and treatment of this condition with an emphasis on the evolution of surgical techniques that led to laparoscopic pyloromyotomy, the most frequently performed technique for HPS today. In addition, we review key developments in the understanding of HPS etiology and treatment, including the postulated etiology of work-induced hypertrophy of the pylorus, its association with prokinetic macrolide antibiotics, and the emerging role of atropine sulfate as a medical treatment for HPS or a rescue treatment for incomplete myotomy...
October 13, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29023811/placental-growth-factor-inhibition-targets-pulmonary-angiogenesis-and-represents-a-novel-therapy-for-hepatopulmonary-syndrome-in-mice
#10
Sarah Raevens, Anja Geerts, Annelies Paridaens, Sander Lefere, Xavier Verhelst, Anne Hoorens, Jo Van Dorpe, Tania Maes, Ken R Bracke, Christophe Casteleyn, Bart Jonckx, Thomas Horvatits, Valentin Fuhrmann, Hans Van Vlierberghe, Christophe Van Steenkiste, Lindsey Devisscher, Isabelle Colle
Hepatopulmonary syndrome (HPS) is a severe complication of cirrhosis with increased risk of mortality. Pulmonary microvascular alterations are key features of HPS, but underlying mechanisms are incompletely understood, and studies on HPS are limited to rats. Placental growth factor (PlGF), a pro-angiogenic molecule that is selectively involved in pathological angiogenesis, may play an important role in HPS development, however, its role has never been investigated. In this study, we validated an HPS model by common bile duct ligation (CBDL) in mice, investigated the kinetic changes in pulmonary angiogenesis and inflammation during HPS development, and provide evidence for a novel therapeutic strategy by targeting pathological angiogenesis...
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28987478/clinical-impact-of-intrapulmonary-vascular-dilatation-in-liver-transplant-candidates
#11
Hilary M DuBrock, Michael J Krowka, Kimberly A Forde, Karen Krok, Mamta Patel, Tiffany Sharkoski, Michael Sprys, Grace Lin, Jae K Oh, Carl D Mottram, Paul D Scanlon, Michael B Fallon, Steven M Kawut
BACKGROUND: Intrapulmonary vascular dilatations (IPVD) are frequently detected in patients with liver disease by the delayed appearance of microbubbles on contrast-enhanced echocardiography. IPVD with an elevated alveolar-arterial (A-a) gradient define hepatopulmonary syndrome, however the importance of IPVD in the absence of abnormal gas exchange is unknown. We aimed to determine the clinical impact of IPVD in patients with liver disease. METHODS: We performed a cross-sectional study within the Pulmonary Vascular Complications of Liver Disease (PVCLD2) Study, a multicenter prospective cohort study of patients being evaluated for liver transplantation...
October 5, 2017: Chest
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#12
Frederick M Karrer, Bradley J Wallace, Arturo E Estrada
Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28975529/pulse-contour-analysis-of-arterial-waveform-in-a-high-fidelity-human-patient-simulator
#13
Paolo Persona, Elisabetta Saraceni, Francesca Facchin, Enrico Petranzan, Matteo Parotto, Fabio Baratto, Carlo Ori, Sandra Rossi
The measurement of cardiac output (CO) may be useful to improve the assessment of hemodynamics during simulated scenarios. The purpose of this study was to evaluate the feasibility of introducing an uncalibrated pulse contour device (MostCare, Vytech, Vygon, Padova, Italy) into the simulation environment. MostCare device was plugged to a clinical monitor and connected to the METI human patient simulator (HPS) to obtain a continuous arterial waveform analysis and CO calculation. In six different simulated clinical scenarios (baseline, ventricular failure, vasoplegic shock, hypertensive crisis, hypovolemic shock and aortic stenosis), the HPS-CO and the MostCare-CO were simultaneously recorded...
October 3, 2017: Journal of Clinical Monitoring and Computing
https://www.readbyqxmd.com/read/28973644/is-an-iranian-health-promoting-school-status-associated-with-improving-school-food-environment-and-snacking-behaviors-in-adolescents
#14
Vahid Yazdi-Feyzabadi, Nasrin Omidvar, Nastaran Keshavarz Mohammadi, Saharnaz Nedjat, Akram Karimi-Shahanjarini, Arash Rashidian
The Iranian Health Promoting Schools (IHPS) program was first piloted and then formally established in Iran in 2011 as a framework to promote healthy environment and behaviors such as proper dietary practice among adolescents. This study examined the role of IHPS in improving the school food environment and snacking behaviors among adolescents. In this cross-sectional study, 1320 eighth grade students from 40 middle schools with IHPS and non-IHPS program were selected using a proportional stratified random sampling method...
August 29, 2017: Health Promotion International
https://www.readbyqxmd.com/read/28972388/hepatopulmonary-syndrome-which-blood-gas-analysis-criteria-and-position-should-we-use-for-diagnosis
#15
Israel Grilo, Juan Manuel Pascasio, Francisco-Jesús López-Pardo, Francisco Ortega-Ruiz, Juan Luis Tirado, José Manuel Sousa, María José Rodríguez-Puras, María Teresa Ferrer, Miguel Ángel Gómez-Bravo, Antonio Grilo
INTRODUCTION: Different blood gas criteria have been used in the diagnosis of hepatopulmonary syndrome (HPS). PATIENTS AND METHODS: Arterial blood gases were prospectively evaluated in 194 cirrhotic candidates for liver transplantation (LT) in the supine and seated position. Three blood gas criteria were analyzed: classic (partial pressure of oxygen [PaO2] < 70 mmHg and/or alveolar-arterial gradient of oxygen [A-a PO2] ≥ 20 mmHg), modern (A-a PO2 ≥ 15 mmHg or ≥ 20 mmHg in patients over 64) and the A-a PO2 ≥ threshold value adjusted for age...
October 3, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28969989/the-effects-of-pioglitazone-in-cirrhotic-rats-with-hepatopulmonary-syndrome
#16
Tsung-Yi Cheng, Wen-Shin Lee, Hui-Chun Huang, Fa-Yauh Lee, Ching-Chih Chang, Han-Chieh Lin, Shou-Dong Lee
BACKGROUND: Hepatopulmonary syndrome (HPS) is characterized by oxygen desaturation and increased intrapulmonary shunting formation in cirrhosis. Due to an unclarified mechanism, there is still no effective therapy except liver transplantation. Recent studies revealed that pulmonary angiogenesis may participate in pathogenesis, in which nitric oxide (NO) and vascular endothelial growth factor (VEGF) play roles. Pioglitazone, a peroxisome proliferator-activated receptor gamma agonist, exerts anti-angiogenesis effect...
September 29, 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28964108/stochastic-spontaneous-calcium-release-events-and-sodium-channelopathies-promote-ventricular-arrhythmias
#17
Fernando O Campos, Yohannes Shiferaw, Edward J Vigmond, Gernot Plank
Premature ventricular complexes (PVCs), the first initiating beats of a variety of cardiac arrhythmias, have been associated with spontaneous calcium release (SCR) events at the cell level. However, the mechanisms underlying the degeneration of such PVCs into arrhythmias are not fully understood. The objective of this study was to investigate the conditions under which SCR-mediated PVCs can lead to ventricular arrhythmias. In particular, we sought to determine whether sodium (Na(+)) current loss-of-function in the structurally normal ventricles provides a substrate for unidirectional conduction block and reentry initiated by SCR-mediated PVCs...
September 2017: Chaos
https://www.readbyqxmd.com/read/28961571/screening-for-hepatopulmonary-syndrome-in-cirrhotic-patients-using-technetium-99m-macroaggregated-albumin-perfusion-lung-scan-tc-maa-diagnostic-approach-and-clinical-correlations
#18
Maria Fragaki, Dimitra Sifaki-Pistolla, Dimitrios N Samonakis, Mairi Koulentaki, Sofia Koukouraki, Maria Stathaki, Elias Kouroumalis
BACKGROUND AND AIMS: The aims of this study were to prospectively screen cirrhotic patients with arterial blood gas test and albumin perfusion scan, identify those fulfilling the classic hepatopulmonary syndrome (HPS) criteria, correlate with clinical parameters, and evaluate the survival of patients with HPS compared with those without HPS in a genetically homogenous Cretan cirrhotic population. MATERIALS AND METHODS: Data on consecutive 102 patients within 1 year were collected and analyzed...
September 28, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28959477/staged-carotid-artery-angioplasty-and-stenting-for-patients-with-high-grade-carotid-stenosis-with-high-risk-of-developing-hyperperfusion-injury-a-retrospective-analysis-of-44-cases
#19
Dapeng Mo, Gang Luo, Bo Wang, Ning Ma, Feng Gao, Xuan Sun, Xiaotong Xu, Zhongrong Miao
BACKGROUND: Hyperperfusion syndrome (HPS) is a rare but potentially a life-threatening complication after carotid artery angioplasty and stenting (CAS). Staged CAS has been an alternative to prevent HPS. MATERIALS AND METHODS: 44 of 908 patients with high-grade internal carotid artery stenosis or near occlusion were at risk of HPS because of poor collateral flow and impaired cerebral blood flow (CBF). They were treated with first (stage 1), followed by a full CAS (stage 2) 1 month later...
December 2016: Stroke and Vascular Neurology
https://www.readbyqxmd.com/read/28951116/assessment-of-learning-curves-for-180-w-greenlight-xps-photoselective-vaporisation-of-the-prostate-a-multicentre-study
#20
Claire Bastard, Kevin Zorn, Benoit Peyronnet, Pierre Alain Hueber, Benjamin Pradère, Morgan Rouprêt, Vincent Misrai
BACKGROUND: The learning curve for photoselective vaporisation of the prostate (PVP) has never been assessed accurately. OBJECTIVE: To compare 180-W GreenLight XPS PVP learning curves for three surgeons with different levels of surgical experience and different institutional backgrounds. DESIGN, SETTING, AND PARTICIPANTS: A multicentre retrospective study of the first patients treated with PVP by three operators in three different centres (n=152 in group 1, n=112 in group 2, n=101 in group 3) was conducted...
September 23, 2017: European Urology Focus
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