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https://www.readbyqxmd.com/read/28449343/cost-effectiveness-of-the-highly-effective-direct-acting-antivirals-in-the-treatment-of-chronic-hepatitis-c-in-hong-kong
#1
Angeline Oi-Shan Lo, Henry Lik-Yuen Chan, Vincent Wai-Sun Wong, Grace Lai-Hung Wong
BACKGROUND AND AIM: In Asia-Pacific where cost is a major concern, peginterferon plus ribavirin (PR) often remain as the standard of care in chronic hepatitis C (CHC) treatment, while the direct-acting antivirals (DAAs) are commonly recommended as retreatment. Newer DAAs can achieve a sustained virological response (SVR) of nearly 100% with pan-genotypic coverage, that is "Highly Effective DAAs." We aimed to investigate the most desirable cost range for the Highly Effective DAAs using Hong Kong as an example...
May 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28448325/combined-spinal-epidural-anesthesia-with-dexmedetomidine-based-sedation-for-multiple-corrective-osteotomies-in-a-child-with-osteogenesis-imperfecta-type-iii-a-case-report
#2
Anju Gupta, Geeta Kamal, Nishkarsh Gupta, Anil Aggarwal
Osteogenesis imperfecta (OI) is a rare disabling genetic connective tissue disorder. General anesthesia in these patients is associated with increased risks. Regional anesthesia is favored wherever feasible, but there are limited reports of use of a sole regional technique in OI in pediatric patients. Moreover, combined spinal-epidural anesthesia has never been described previously. We are reporting the use of combined spinal-epidural anesthesia for a prolonged surgery (multiple osteotomies) of lower limbs in a 10-year-old wheelchair-bound child with OI type III...
April 26, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28444369/three-dimensional-intracellular-structure-of-a-whole-rice-mesophyll-cell-observed-with-fib-sem
#3
Takao Oi, Sakiko Enomoto, Tomoyo Nakao, Shigeo Arai, Koji Yamane, Mitsutaka Taniguchi
Background and Aims: Ultrathin sections of rice leaf blades observed two-dimensionally using a transmission electron microscope (TEM) show that the chlorenchyma is composed of lobed mesophyll cells, with intricate cell boundaries, and lined with chloroplasts. The lobed cell shape and chloroplast positioning are believed to enhance the area available for the gas exchange surface for photosynthesis in rice leaves. However, a cell image revealing the three-dimensional (3-D) ultrastructure of rice mesophyll cells has not been visualized...
April 21, 2017: Annals of Botany
https://www.readbyqxmd.com/read/28442844/a-rare-case-of-bilateral-morgagni-s-hernia-with-intestinal-obstruction-and-malrotation-of-the-gut-in-an-adult-patient-with-severe-osteogenesis-imperfecta-presenting-as-severe-respiratory-distress
#4
S Y Kulgod, Amit Shivashankar Ammanagi, Pradeep Vagarali, Ravi Patil
Osteogenesis imperfecta (OI) is associated with congenital inguinal hernia that can occur due to genetic mutation. Morgagni's hernia is a rare congenital diaphragmatic herniation and is usually diagnosed in childhood. We present a case of an 18-year-old male presenting with bilateral Morgagni's hernia with intestinal obstruction with osteogenesis imperfecta with malrotation of the gut.
April 2017: Indian Journal of Surgery
https://www.readbyqxmd.com/read/28438452/muscle-damage-after-total-hip-arthroplasty-through-the-direct-anterior-approach-for-developmental-dysplasia-of-the-hip
#5
Masashi Kawasaki, Yukiharu Hasegawa, Toshiaki Okura, Satoshi Ochiai, Takayoshi Fujibayashi
BACKGROUND: Total hip arthroplasty (THA) through the direct anterior approach (DAA) is known to cause less muscle damage than other surgical approaches. However, more complex primary cases, such as developmental dysplasia of the hip (DDH), might often cause muscle damage. The objective of the present study was to clarify the muscle damage observed 1 year after THA through the DAA for DDH using magnetic resonance imaging. METHODS: We prospectively compared the muscle cross-sectional area (M-CSA) and fatty atrophy (FA) in muscles by magnetic resonance imaging and the Harris hip score before and at 1-year follow-up after THA through the DAA in 3 groups: 37 patients with Crowe group 1 DDH (D1), 13 patients with Crowe group 2 and 3 DDH (D2 + 3), and 12 patients with osteonecrosis as a control...
March 27, 2017: Journal of Arthroplasty
https://www.readbyqxmd.com/read/28436160/compound-heterozygous-mutations-in-col1a1-associated-with-an-atypical-form-of-type-i-osteogenesis-imperfecta
#6
Amanda M Ackermann, Michael A Levine
Heterozygous mutations in the genes encoding the proα1(I) or proα2(I) chains of type I procollagen (COL1A1 and COL1A2, respectively) account for most cases of osteogenesis imperfecta (OI), a disorder characterized by reduced bone strength and increased fracture risk. COL1A1 mutations can also cause rare cases of Ehlers-Danlos syndrome (EDS), a disorder that primarily affects connective tissue and often includes reduced bone mass. Here we present a kindred of three young siblings ages 1-4 years old whose mother has a history of mild type I OI...
April 24, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28435282/managing-the-patient-with-osteogenesis-imperfecta-a-multidisciplinary-approach
#7
REVIEW
Caroline Marr, Alison Seasman, Nick Bishop
Osteogenesis imperfecta (OI) is a heterogeneous heritable connective tissue disorder characterized by low bone density. The type and severity of OI are variable. The primary manifestations are fractures, bone deformity, and bone pain, resulting in reduced mobility and function to complete everyday tasks. OI affects not only the physical but also the social and emotional well-being of children, young people, and their families. As such, medical, surgical, and allied health professionals' assessments all play a role in the management of these children...
2017: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/28433581/therapeutic-burst-suppression-coma-in-pediatric-febrile-refractory-status-epilepticus
#8
Jainn-Jim Lin, Cheng-Che Chou, Shih-Yun Lan, Hsiang-Ju Hsiao, Yu Wang, Oi-Wa Chan, Shao-Hsuan Hsia, Huei-Shyong Wang, Kuang-Lin Lin
BACKGROUND: Evidence for the beneficial effect of therapeutic burst-suppression coma in pediatric patients with febrile refractory status epilepticus is limited, and the clinical outcomes of this treatment strategy are largely unknown. Therefore, the aim of this study was to explore the outcomes of therapeutic burst-suppression coma in a series of children with febrile refractory status epilepticus. METHODS: We retrospectively reviewed consecutive pediatric patients with febrile refractory status epilepticus admitted to our pediatric intensive care unit between January 2000 and December 2013...
April 19, 2017: Brain & Development
https://www.readbyqxmd.com/read/28433416/static-postural-control-in-youth-with-osteogenesis-imperfecta-type-i
#9
Annie Pouliot-Laforte, Martin Lemay, Frank Rauch, Louis-Nicolas Veilleux
OBJECTIVE: To assess static postural control in eyes-open and eyes-closed conditions in individuals with osteogenesis imperfecta (OI) type I as compared to typically developing (TD) individuals. The second aim was to explore the relation between postural control and lower-limbs muscle function. DESIGN: This is a cross-sectional study. SETTINGS: The study was carried out in the outpatient department of a pediatric orthopedic hospital. PARTICIPANTS: 22 individuals with OI type I (mean age [range]: 13...
April 19, 2017: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/28431466/next-generation-sequencing-reveals-one-novel-missense-mutation-in-col1a2-gene-in-an-iranian-family-with-osteogenesis-imperfecta
#10
Farah Talebi, Farideh Ghanbari Mardasi, Mohammadi Asl Javad, Bavarsad Amir Hooshang, Salehi Kambo Masoumeh
BAckground: Osteogenesis imperfecta (OI) is a clinically and genetically heterogeneous disorder characterized by bone loss and bone fragility. The aim of this study was to investigate the variants of three genes involved in the pathogenesis of OI. Methods: Molecular genetic analyses were performed for COL1A1, COL1A2, and CRTAP genes in an Iranian family with OI. The DNA samples were analyzed by next-generation sequencing (NGS) gene panel and Sanger sequencing. Results: Five different variants were identified in COL1A1 and COL1A2, including two variants in COL1A1 and three variants in COL1A2...
April 22, 2017: Iranian Biomedical Journal
https://www.readbyqxmd.com/read/28421169/high-prevalence-of-virulence-genes-in-specific-genotypes-of-atypical-enteropathogenic-escherichia-coli
#11
Yanmei Xu, Xiangning Bai, Yujuan Jin, Bin Hu, Hong Wang, Hui Sun, Ruyue Fan, Shanshan Fu, Yanwen Xiong
Atypical enteropathogenic Escherichia coli (aEPEC) strains are emerging enteropathogens that have been detected worldwide. A collection of 228 aEPEC strains (121 from diarrheal patients, 27 from healthy carriers, 47 from animals and 33 from raw meats) were investigated for serotypes, virulence gene profiles and phylogenetic relationships. Sixty-six O serogroups were identified. Serogroup O51 was the most prevalent, followed by O119, O26 and O76. For the 20 virulence genes detected, statistically significant differences were observed in the overall prevalence of efa1 (lifA), nleB, nleE, set/ent, paa, and ehxA genes among strains from diarrheal patients, healthy carriers, animals and raw meats, respectively...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28413750/an-optically-isotropic-antiferroelectric-liquid-crystal-oi-aflc-display-mode-operating-over-a-wide-temperature-range-using-ternary-bent-core-liquid-crystal-mixtures
#12
Leah Bergquist, Cuiyu Zhang, Roberta R Ribeiro de Almeida, Brittany Pellegrene, Miroslaw Salamonczyk, Matthew Kim, Jung-Im Hwang, Kyeong-Jin Kim, Joun-Ho Lee, Antal Jákli, Torsten Hegmann
We report on the synthesis and characterization of bent-core liquid crystal (LC) compounds and the preparation of mixtures that provide an optically isotropic antiferroelectric (OI-AFLC) liquid crystal display mode over a very wide temperature interval and well below room temperature. From the collection of compounds synthesized during this study, we recognized that several ternary mixtures displayed a modulated SmCaPA phase down to below -40 °C and up to about 100 °C on both heating and cooling, as well as optical tilt angles in the transformed state of approximately 45° (optically isotropic state)...
April 2017: ChemistryOpen
https://www.readbyqxmd.com/read/28413186/the-characterization-of-a-full-thickness-excision-open-foot-wound-model-in-n5-streptozotocin-stz-induced-type-2-diabetic-rats-that-mimics-diabetic-foot-ulcer-in-terms-of-reduced-blood-circulation-higher-c-reactive-protein-elevated-inflammation-and-reduced-cell
#13
Caroline Oi-Ling Yu, Kwok-Sui Leung, Kwok-Pui Fung, Francis Fu-Yuen Lam, Ethel Sau-Kuen Ng, Kit-Man Lau, Simon Kwoon-Ho Chow, Wing-Hoi Cheung
Delayed foot wound healing is a major complication attributed to hyperglycemia in type 2 diabetes mellitus (DM) patients, and these wounds may develop into foot ulcers. There are at least two types of DM wound models used in rodents to study delayed wound healing. However, clinically relevant animal models are not common. Most models use type 1 DM rodents or wounds created on the back rather than on the foot. An open full-thickness excision wound on the footpad of type 2 DM rats is more clinically relevant, but such a model has not yet been characterized systematically...
April 11, 2017: Experimental Animals
https://www.readbyqxmd.com/read/28401152/hemodynamic-analysis-of-pediatric-septic-shock-and-cardiogenic-shock-using-transpulmonary-thermodilution
#14
En-Pei Lee, Shao-Hsuan Hsia, Jainn-Jim Lin, Oi-Wa Chan, Jung Lee, Chia-Ying Lin, Han-Ping Wu
Septic shock and cardiogenic shock are the two most common types of shock in children admitted to pediatric intensive care units (PICUs). The aim of the study was to investigate which hemodynamic variables were associated with mortality in children with shock. We retrospectively analyzed 50 children with shock (37 septic shock cases and 13 cardiogenic shock cases) in the PICU and monitored their hemodynamics using transpulmonary thermodilution from 2003 to 2016. Clinical factors were analyzed between the patients with septic and cardiogenic shock...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28400738/grammar-is-differentially-impaired-in-subgroups-of-autism-spectrum-disorders-evidence-from-an-investigation-of-tense-marking-and-morphosyntax
#15
Nadezhda Modyanova, Alexandra Perovic, Ken Wexler
Deficits in the production of verbal inflection (tense marking, or finiteness) are part of the Optional Infinitive (OI) stage of typical grammatical development. They are also a hallmark of language impairment: they have been used as biomarkers in guiding genetic studies of Specific Language Impairment (SLI), and have also been observed in autism spectrum disorders (ASD). To determine the detailed nature of finiteness abilities in subgroups of ASD [autism with impaired language (ALI) vs. autism with normal language (ALN)], we compared tense marking abilities in 46 children with ALI and 37 children with ALN with that of two groups of nonverbal mental age (MA) and verbal MA-matched typically developing (TD) controls, the first such study described in the literature...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28398160/should-young-children-with-tbi-be-compared-to-community-or-orthopedic-control-participants
#16
Miriam Helen Beauchamp, Catherine Landry-Roy, Jocelyn Gravel, Cindy Beaudoin, Annie Bernier
Pediatric traumatic brain injury (TBI) research depends on comparisons of profiles and outcome between brain injured individuals and groups consisting either of injured controls (e.g., orthopedic injuries, OI) or uninjured, typically developing children recruited from the community (community controls, CC). Children with OI are thought to provide optimal comparisons for individuals with TBI because they share injury-related experiences and pre-morbid characteristics; however, a study by Mathias and colleagues (2013) in adults has called into question the added value of injury control groups in TBI research...
April 11, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28396763/neonatal-mucolipidosis-type-ii-alpha-beta-due-to-compound-heterozygosity-for-a-known-and-novel-gnptab-mutation-and-a-concomitant-heterozygous-change-in-serpinf1-inherited-from-the-mother
#17
Kirsten A Wood, Regina M Zambrano, Bradley J Cheek, Christopher Arcement, Marie Haymon, Jessica Steinkampf, Srirangan Sampath, James C Hyland, Yves Lacassie
We report on a newborn with IUGR, rhizomelic dwarfism, and suspected chondrodysplasia punctata. At birth, OI was suspected; however, a skeletal survey suggested ML II alpha/beta. Sequencing revealed compound heterozygosity for a reported pathogenic and novel but expected pathogenic GNPTAB variant. Molecular testing for autosomal recessive OI identified a SERPINF1 variant.
April 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28396251/gene-expression-profiling-of-bone-marrow-mesenchymal-stem-cells-from-osteogenesis-imperfecta-patients-during-osteoblast-differentiation
#18
Carla Martins Kaneto, Patrícia S Pereira Lima, Karen Lima Prata, Jane Lima Dos Santos, João Monteiro de Pina Neto, Rodrigo Alexandre Panepucci, Houtan Noushmehr, Dimas Tadeu Covas, Francisco José Alburquerque de Paula, Wilson Araújo Silva
Mesenchymal stem cells (MSCs) are precursors present in adult bone marrow that are able to differentiate into osteoblasts, adipocytes and chondroblasts that have gained great importance as a source for cell therapy. Recently, a number of studies involving the analysis of gene expression of undifferentiated MSCs and of MSCs in the differentiation into multiple lineage processes were observed but there is no information concerning the gene expression of MSCs from Osteogenesis Imperfecta (OI) patients. Osteogenesis Imperfecta is characterized as a genetic disorder in which a generalized osteopenia leads to excessive bone fragility and severe bone deformities...
April 7, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28396189/external-counterpulsation-increases-beat-to-beat-heart-rate-variability-in-patients-with-ischemic-stroke
#19
Li Xiong, Ge Tian, Li Wang, Wenhua Lin, Xiangyan Chen, Thomas Wai Hong Leung, Yannie Oi Yan Soo, Lawrence Ka Sing Wong
BACKGROUND AND PURPOSE: External counterpulsation (ECP) is a noninvasive method used to augment cerebral perfusion in ischemic stroke. However, the response of beat-to-beat heart rate variability (HRV) in patients with ischemic stroke during ECP remains unknown. METHODS: Forty-eight patients with unilateral ischemic stroke at the subacute stage and 14 healthy controls were recruited. Beat-to-beat heart rate before, during, and after ECP was monitored. The frequency components of HRV were calculated using power spectral analysis...
April 7, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28394261/folate-cycle-enzyme-mthfd1l-confers-metabolic-advantages-in-hepatocellular-carcinoma
#20
Derek Lee, Iris Ming-Jing Xu, David Kung-Chun Chiu, Robin Kit-Ho Lai, Aki Pui-Wah Tse, Lynna Lan Li, Cheuk-Ting Law, Felice Ho-Ching Tsang, Larry Lai Wei, Cerise Yuen-Ki Chan, Chun-Ming Wong, Irene Oi-Lin Ng, Carmen Chak-Lui Wong
Cancer cells preferentially utilize glucose and glutamine, which provide macromolecules and antioxidants that sustain rapid cell division. Metabolic reprogramming in cancer drives an increased glycolytic rate that supports maximal production of these nutrients. The folate cycle, through transfer of a carbon unit between tetrahydrofolate and its derivatives in the cytoplasmic and mitochondrial compartments, produces other metabolites that are essential for cell growth, including nucleotides, methionine, and the antioxidant NADPH...
April 10, 2017: Journal of Clinical Investigation
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