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https://www.readbyqxmd.com/read/28534860/hemophilia-care-in-the-pediatric-age
#1
REVIEW
Marta Bertamino, Francesca Riccardi, Laura Banov, Johanna Svahn, Angelo Claudio Molinari
Hemophilia is the most common of the severe bleeding disorders and if not properly managed since early infancy can lead to chronic disease and lifelong disabilities. However, it enjoys the most efficacious and safe treatment among the most prevalent monogenic disorders. Hemophilia should be considered in the neonatal period in the case of unusual bleeding or in the case of positive family history. Later, hemophilia should be suspected mainly in males because of abnormal bruising/bleeding or unusual bleeding following invasive procedures-for example, tonsillectomy or circumcision...
May 19, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28533975/an-exhaustive-survey-of-regular-peptide-conformations-using-a-new-metric-for-backbone-handedness-h
#2
Ranjan V Mannige
The Ramachandran plot is important to structural biology as it describes a peptide backbone in the context of its dominant degrees of freedom-the backbone dihedral angles φ and ψ (Ramachandran, Ramakrishnan & Sasisekharan, 1963). Since its introduction, the Ramachandran plot has been a crucial tool to characterize protein backbone features. However, the conformation or twist of a backbone as a function of φ and ψ has not been completely described for both cis and trans backbones. Additionally, little intuitive understanding is available about a peptide's conformation simply from knowing the φ and ψ values of a peptide (e...
2017: PeerJ
https://www.readbyqxmd.com/read/28533757/multiscale-modeling-of-bone-healing-toward-a-systems-biology-approach
#3
REVIEW
Edoardo Borgiani, Georg N Duda, Sara Checa
Bone is a living part of the body that can, in most situations, heal itself after fracture. However, in some situations, healing may fail. Compromised conditions, such as large bone defects, aging, immuno-deficiency, or genetic disorders, might lead to delayed or non-unions. Treatment strategies for those conditions remain a clinical challenge, emphasizing the need to better understand the mechanisms behind endogenous bone regeneration. Bone healing is a complex process that involves the coordination of multiple events at different length and time scales...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28533023/the-rna-modification-n-6-methyladenosine-and-its-implications-in-human-disease
#4
REVIEW
Pedro J Batista
Impaired gene regulation lies at the heart of many disorders, including developmental diseases and cancer. Furthermore, the molecular pathways that control gene expression are often the target of cellular parasites, such as viruses. Gene expression is controlled through multiple mechanisms that are coordinated to ensure the proper and timely expression of each gene. Many of these mechanisms target the life cycle of the RNA molecule, from transcription to translation. Recently, another layer of regulation at the RNA level involving RNA modifications has gained renewed interest of the scientific community...
May 19, 2017: Genomics, Proteomics & Bioinformatics
https://www.readbyqxmd.com/read/28532688/progress-in-the-contemporary-management-of-hemophilia-the-new-issue-of-patient-aging
#5
REVIEW
Pier Mannuccio Mannucci, Massimo Iacobelli
The management of inherited coagulation disorders such as hemophilia A and B has witnessed dramatic progresses since the last few decades of the last century. Accordingly, persons with hemophilia (PWH) now enjoy a life expectancy at birth not different from that of males in the general population, at least in high income countries. Nowadays, a substantial proportion of PWH are aging, like their peers in the general population. This outstanding progress is accompanied by problems that are in part similar to those of any old person (multiple concomitant diseases and the resulting intake of multiple drugs other than those specific for hemophilia treatment)...
May 20, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28529779/crystal-structures-of-di-bromido-n-pyridin-2-yl-%C3%AE%C2%BAn-methyl-idene-picolinohydrazide-%C3%AE%C2%BA-2-n-o-cadmium-methanol-monosolvate-and-di-iodido-n-pyridin-2-yl-%C3%AE%C2%BAn-methyl-idene-picolinohydrazide-%C3%AE%C2%BA-2-n-o-cadmium
#6
Ali Akbar Khandar, Farhad Akbari Afkhami, Harald Krautscheid, Kenneth Aase Kristoffersen, Zeliha Atioğlu, Mehmet Akkurt, Carl Henrik Görbitz
The title compounds, [CdBr2(C12H10N4O)]·CH3OH, (I), and [CdI2(C12H10N4O)], (II), are cadmium bromide and cadmium iodide complexes of the ligand (E)-N'-(pyridin-2-yl-methyl-ene)picolinohydrazide. Complex (I) crystallizes as the methanol monosolvate. In both compounds, the Cd(2+) cation is ligated by one O atom and two N atoms of the tridentate ligand, and by two bromide anions forming a Br2N2O penta-coordination sphere for (I), and by two iodide anions forming an I2N2O penta-coordination sphere for (II), both with a distorted square-pyramidal geometry...
May 1, 2017: Acta Crystallographica. Section E, Crystallographic Communications
https://www.readbyqxmd.com/read/28527059/personality-disorders-in-cluster-headache-a-study-using-the-millon-clinical-multiaxial-inventory-iii
#7
Sylvie Hélène Marie Jeanne Piacentini, Lara Draghi, Alberto Proietti Cecchini, Massimo Leone
A great deal of studies suggests that cluster headache (CH) patients are usually comorbid to anxiety-mood spectrum disorders and psychopathological symptoms; however, the personality profiles reported in the literature strictly depend on type of assessment used. Psychiatric comorbidities have been extensively studied in migraine and they are recognized to represent a major risk factor associated with poorer outcome, playing a role in the headache chronification process at once as cause and consequence of it...
May 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28524225/-how-must-we-manage-epileptic-encephalopathies-in-infants-conclusions
#8
V Soto-Insuga
Epileptic encephalopathies are defined as epileptic syndromes in which the epileptic activity per se (in the form of frequent seizures or the presence of interictal epileptiform activity) contributes to a cognitive and behavioural disorder that is more important than could be expected from the causation of the disorder. Their aetiological diagnosis is fundamental to allow an early treatment to be established. We propose a diagnostic algorithm for patients with epileptic encephalopathy with onset during the first year of life, which includes management coordinated with electroencephalographic studies, neuroimaging, and screening for metabolic and genetic disorders...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28524033/-procedural-analysis-of-acid-base-balance-disorder-case-serials-in-4-patents
#9
Chunyuan Ma, Guijie Wang
OBJECTIVE: To establish the standardization process of acid-base balance analysis, analyze cases of acid-base balance disorder with the aid of acid-base balance coordinate graph. METHODS: The acid-base balance theory were reviewed systematically on recent research progress, and the important concepts, definitions, formulas, parameters, regularity and inference in the analysis of acid-base balance were studied. The analysis of acid-base balance disordered processes and steps were figured...
May 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28516196/coupling-dynamics-in-speech-gestures-amplitude-and-rate-influences
#10
Pascal H H M van Lieshout
Speech is a complex oral motor function that involves multiple articulators that need to be coordinated in space and time at relatively high movement speeds. How this is accomplished remains an important and largely unresolved empirical question. From a coordination dynamics perspective, coordination involves the assembly of coordinative units that are characterized by inherently stable coupling patterns that act as attractor states for task-specific actions. In the motor control literature, one particular model formulated by Haken et al...
May 17, 2017: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/28515711/the-impact-of-glucagon-like-peptide-1-on-bone-metabolism-and-its-possible-mechanisms
#11
REVIEW
Chenhe Zhao, Jing Liang, Yinqiu Yang, Mingxiang Yu, Xinhua Qu
The impact of antidiabetic drugs on bone metabolism is drawing increasing attention due to the discovery of a correlation between type 2 diabetes mellitus (T2DM) and osteoporosis. Glucagon-like peptide-1 (GLP-1) receptor agonists are a novel and promising class of drugs for T2DM, which may also have clinical applications in bone tissue disorders. This review examines the impact of GLP-1 on bone metabolism, including enhancement of bone mineral density and improvement of bone quality. However, the precise effect of GLP-1 on fracture risk has not been unambiguously defined...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28512766/mental-health-outcomes-of-developmental-coordination-disorder-in-late-adolescence
#12
Ian Harrowell, Linda Hollén, Raghu Lingam, Alan Emond
AIM: To assess the relationship between developmental coordination disorder (DCD) and mental health outcomes in late adolescence. METHOD: Data were analyzed from the Avon Longitudinal Study of Parents and Children. Moderate-to-severe DCD was defined at 7 to 8 years according to the DSM-IV-TR criteria. Mental health was assessed at 16 to 18 years using self-reported questionnaires: Strengths and Difficulties Questionnaire, Short Moods and Feelings Questionnaire, and the Warwick-Edinburgh Mental Well-being Scale...
May 16, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28508655/-prevention-of-eating-disorders
#13
Hana Papežová
The quality of the prevention of eating disorders represents in several last decades frequently discussed issue in the context of rapidly changing socio-economic conditions, a significant increase of influence of the media, new technologies and knowledge of risk factors. Primary prevention aims to reduce the risk of developing eating disorders, but secondary and tertiary prevention play the important role as well. Effective and coordinated prevention is still missing. Our experience of international cooperation of the last 20 years led to the development and evaluation of prevention programs...
2017: Casopís Lékar̆ů C̆eských
https://www.readbyqxmd.com/read/28507985/control-of-neuronal-migration-and-aggregation-by-reelin-signaling-in-the-developing-cerebral-cortex
#14
REVIEW
Yuki Hirota, Kazunori Nakajima
The mammalian cerebral neocortex has a well-organized laminar structure, achieved by the highly coordinated control of neuronal migration. During cortical development, excitatory neurons born near the lateral ventricle migrate radially to reach their final positions to form the cortical plate. During this process, dynamic changes are observed in the morphologies and migration modes, including multipolar migration, locomotion, and terminal translocation, of the newborn neurons. Disruption of these migration processes can result in neuronal disorders such as lissencephaly and periventricular heterotopia...
2017: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/28498676/intermolecular-interactions-in-highly-disordered-confined-dense-n2
#15
Mario Santoro, Federico A Gorelli, Roberto Bini, Julien Haines
Molecular nitrogen is a benchmark system for condensed matter and, in particular, for looking at universal properties of strongly confined dense systems. We conducted Raman and X-ray diffraction measurements on a dense and disordered form of molecular nitrogen subnanoconfined in a noncatalytic pure SiO2 zeolite under pressure, up to 50 GPa. In this form, N2-N2 interactions and, consequently, distances are found to be very close to those of bulk N2 and intramolecular interactions progressively weaken upon increasing pressure...
May 15, 2017: Journal of Physical Chemistry Letters
https://www.readbyqxmd.com/read/28498279/effectiveness-of-a-stepped-collaborative-and-coordinated-health-care-network-for-somatoform-disorders-sofu-net-a-controlled-cluster-cohort-study
#16
Bernd Löwe, Katharina Piontek, Anne Daubmann, Martin Härter, Karl Wegscheider, Hans-Helmut König, Meike Shedden-Mora
OBJECTIVE: Stepped, collaborative, and coordinated approaches have been proposed as the optimal treatment strategy for somatic symptom and related disorders (SSRD), but evidence supporting this strategy is lacking. The aim of this study was to assess the effectiveness of a guideline-based health care network for patients who are at high risk of somatoform disorder (Sofu-Net). METHODS: In a controlled, prospective, observer-blinded cluster cohort study, patients who were at high risk of somatoform disorder were recruited at 18 primary care practices in the Sofu-Net and at 15 primary care practices that provided care as usual (CAU)...
May 12, 2017: Psychosomatic Medicine
https://www.readbyqxmd.com/read/28497909/the-therapeutic-alliance-in-different-mental-disorders-a-comparison-of-patients-with-depression-somatoform-and-eating-disorders
#17
Johannes Mander, Andreas B Neubauer, Angelika Schlarb, Martin Teufel, Hinrich Bents, Martin Hautzinger, Stephan Zipfel, Andreas Wittorf, Isa Sammet
OBJECTIVE: The therapeutic alliance is intensively investigated in psychotherapy research. However, there is scarce research on the role of the specific diagnosis of the patient in the formation of the therapeutic alliance. Hence, the aim of this study was to address this research gap by comparing the alliance in different mental disorders. DESIGN: Our sample comprised 348 patients (mean age = 40 years; 68% female; 133 patients with depression, 122 patients with somatoform disorders, and 93 patients with eating disorders)...
May 12, 2017: Psychology and Psychotherapy
https://www.readbyqxmd.com/read/28493231/-drug-therapy-of-fibromyalgia-syndrome-updated-guidelines-2017-and-overview-of-systematic-review-articles
#18
C Sommer, R Alten, K-J Bär, M Bernateck, W Brückle, E Friedel, P Henningsen, F Petzke, T Tölle, N Üçeyler, A Winkelmann, W Häuser
BACKGROUND: The regular update of the guidelines on fibromyalgia syndrome, AWMF number 145/004, was scheduled for April 2017. METHODS: The guidelines were developed by 13 scientific societies and 2 patient self-help organizations coordinated by the German Pain Society. Working groups (n =8) with a total of 42 members were formed balanced with respect to gender, medical expertise, position in the medical or scientific hierarchy and potential conflicts of interest...
May 10, 2017: Der Schmerz
https://www.readbyqxmd.com/read/28493226/-etiology-and-pathophysiology-of-fibromyalgia-syndrome-updated-guidelines-2017-overview-of-systematic-review-articles-and-overview-of-studies-on-small-fiber-neuropathy-in-fms-subgroups
#19
N Üçeyler, M Burgmer, E Friedel, W Greiner, F Petzke, M Sarholz, M Schiltenwolf, A Winkelmann, C Sommer, W Häuser
BACKGROUND: The regular update of the guidelines on fibromyalgia syndrome, AWMF number 145/004, was planned for April 2017. METHODS: The guidelines were developed by 13 scientific societies and 2 patient self-help organizations coordinated by the German Pain Society. Working groups (n =8) with a total of 42 members were formed balanced with respect to gender, medical expertise, position in the medical or scientific hierarchy and potential conflicts of interest...
May 10, 2017: Der Schmerz
https://www.readbyqxmd.com/read/28492620/novel-red-phosphors-kbaeu-xo4-3-x-mo-w-show-high-color-purity-and-high-thermostability-from-a-disordered-chained-structure
#20
G Q Wang, X H Gong, Y J Chen, J H Huang, Y F Lin, Z D Luo, Y D Huang
Two novel red phosphors KBaEu(XO4)3 (X = Mo, W) have been synthesized by high-temperature solid-state reactions and the crystal structures were determined for the first time. Single-crystal X-ray diffraction data reveal that their space groups are C2/c. The crystalline structure is constituted of K/BaO8 distorted square antiprisms and distorted EuO8 polyhedra which form chains lying along the c-axis and two kinds of distorted XO4 tetrahedra. This high disorder of K/Ba which might lower the crystal field symmetry around Eu(3+) results in the high purity of red emission around 615 nm originating from (5)D0 → (7)F2 transition under near-ultraviolet (NUV) excitation...
May 11, 2017: Dalton Transactions: An International Journal of Inorganic Chemistry
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