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https://www.readbyqxmd.com/read/28345437/clinical-profile-dosing-and-quality-of-life-outcomes-in-primary-immune-deficiency-patients-treated-at-home-with-immunoglobulin-g-data-from-the-ideal-patient-registry
#1
Sean Kearns, Loretta Kristofek, William Bolgar, Luqman Seidu, Samantha Kile
BACKGROUND: Patients with primary immune deficiency (PID) often require immunoglobulin G (IgG, commonly referred to as Ig) replacement therapy to prevent infections and associated comorbidities. Ig therapy can be given either through intravenous or subcutaneous routes, and both can be done in the home setting. There is limited information available on the real-world diagnosis, management, and outcomes of this patient population, given the variable disease presentation and treatment options...
April 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28342087/pelvic-inflammatory-disease-and-the-risk-of-ovarian-cancer-a-meta-analysis
#2
Zhiyi Zhou, Fangfang Zeng, Jianhui Yuan, Jinling Tang, Graham A Colditz, Shelley S Tworoger, Britton Trabert, Xuefen Su
PURPOSE: Previous studies on pelvic inflammatory disease (PID) and the risk of ovarian cancer have found inconsistent results. We performed an updated meta-analysis to summarize the evidence of this association. METHODS: PubMed, Embase, and ISI web of science databases were searched through October 2016 for studies that investigated the PID and ovarian cancer association. Summary risk estimates were calculated using random-effects meta-analysis. RESULT: Thirteen studies were eligible for analysis, which included six cohort studies and seven case-control studies...
March 24, 2017: Cancer Causes & Control: CCC
https://www.readbyqxmd.com/read/28333195/genome-wide-genetic-analyses-highlight-mitogen-activated-protein-kinase-mapk-signaling-in-the-pathogenesis-of-endometriosis
#3
Outi Uimari, Nilufer Rahmioglu, Dale R Nyholt, Katy Vincent, Stacey A Missmer, Christian Becker, Andrew P Morris, Grant W Montgomery, Krina T Zondervan
STUDY QUESTION: Do genome-wide association study (GWAS) data for endometriosis provide insight into novel biological pathways associated with its pathogenesis? SUMMARY ANSWER: GWAS analysis uncovered multiple pathways that are statistically enriched for genetic association signals, analysis of Stage A disease highlighted a novel variant in MAP3K4, while top pathways significantly associated with all endometriosis and Stage A disease included several mitogen-activated protein kinase (MAPK)-related pathways...
February 9, 2017: Human Reproduction
https://www.readbyqxmd.com/read/28325526/enhanced-imc-based-pid-controller-design-for-non-minimum-phase-nmp-integrating-processes-with-time-delays
#4
K Ghousiya Begum, A Seshagiri Rao, T K Radhakrishnan
Internal model control (IMC) with optimal H2 minimization framework is proposed in this paper for design of proportional-integral-derivative (PID) controllers. The controller design is addressed for integrating and double integrating time delay processes with right half plane (RHP) zeros. Blaschke product is used to derive the optimal controller. There is a single adjustable closed loop tuning parameter for controller design. Systematic guidelines are provided for selection of this tuning parameter based on maximum sensitivity...
March 18, 2017: ISA Transactions
https://www.readbyqxmd.com/read/28318548/a-uniform-lmi-formulation-for-tuning-pid-multi-term-fractional-order-pid-and-tilt-integral-derivative-tid-for-integer-and-fractional-order-processes
#5
Farshad Merrikh-Bayat
In this paper first the Multi-term Fractional-Order PID (MFOPID) whose transfer function is equal to [Formula: see text] , where kj and αj are unknown and known real parameters respectively, is introduced. Without any loss of generality, a special form of MFOPID with transfer function kp+ki/s+kd1s+kd2s(μ) where kp, ki, kd1, and kd2 are unknown real and μ is a known positive real parameter, is considered. Similar to PID and TID, MFOPID is also linear in its parameters which makes it possible to study all of them in a same framework...
March 15, 2017: ISA Transactions
https://www.readbyqxmd.com/read/28318545/fractional-order-pi-%C3%AE-d-%C3%AE-controller-design-for-satisfying-time-and-frequency-domain-specifications-simultaneously
#6
WeiJia Zheng, Ying Luo, XiaoHong Wang, YouGuo Pi, YangQuan Chen
In order to achieve a desired control performance characterized by satisfying specifications in both frequency-domain and time-domain simultaneously, an optimal fractional order proportional integral derivative (PI(λ)D(μ)) controller design strategy is proposed based on analytical calculation and Differential Evolution algorithm for a permanent magnet synchronous motor (PMSM) servo system in this paper. In this controller design, the frequency-domain specifications can guarantee the system stability with both gain margin and phase margin, and also the system robustness to loop gain variations...
March 16, 2017: ISA Transactions
https://www.readbyqxmd.com/read/28316003/evaluation-of-the-safety-tolerability-and-pharmacokinetics-of-gammaplex-%C3%A2-10-versus-gammaplex-%C3%A2-5-in-subjects-with-primary-immunodeficiency
#7
Richard L Wasserman, Isaac R Melamed, Mark R Stein, Stephen Jolles, Miranda Norton, James N Moy
PURPOSE: This phase 3, multicenter, open-label, randomized, two-period, crossover bioequivalence trial evaluated the safety, tolerability, and pharmacokinetics of intravenous immunoglobulins (IVIGs) Gammaplex 5% and Gammaplex 10% in 33 adults and 15 children with primary immunodeficiency diseases (PIDs). METHODS: Eligible adults received five Gammaplex 5% infusions followed by five Gammaplex 10% infusions, or vice versa, stratified by a 21- or 28-day dosing regimen...
March 18, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28314962/pd-l1-expression-in-pancreatic-ductal-adenocarcinoma-is-a-poor-prognostic-factor-in-patients-with-high-cd8-tumor-infiltrating-lymphocytes-highly-sensitive-detection-using-phosphor-integrated-dot-staining
#8
So Yamaki, Hiroaki Yanagimoto, Koji Tsuta, Hironori Ryota, Masanori Kon
BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) has an extremely poor prognosis. For the development of more effective immunotherapies, it is first necessary to elucidate the immunological escape mechanisms. In this study, we applied our recently developed highly sensitive immunostaining method employing fluorescent phosphor-integrated dot (PID) nanoparticles to evaluate the prevalence of programmed death ligand 1 (PD-L1) in patients with PDAC. METHODS: This study included 42 patients with PDAC who underwent pancreatectomy...
March 18, 2017: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28293793/ultrasensitive-and-selective-detection-of-3-repeat-tau-seeding-activity-in-pick-disease-brain-and-cerebrospinal-fluid
#9
Eri Saijo, Bernardino Ghetti, Gianluigi Zanusso, Adrian Oblak, Jennifer L Furman, Marc I Diamond, Allison Kraus, Byron Caughey
The diagnosis and treatment of diseases involving tau-based pathology such as Alzheimer disease and certain frontotemporal dementias is hampered by the inability to detect pathological forms of tau with sufficient sensitivity, specificity and practicality. In these neurodegenerative diseases, tau accumulates in self-seeding filaments. For example, Pick disease (PiD) is associated with frontotemporal degeneration and accumulation of 3-repeat (3R) tau isoforms in filaments constituting Pick bodies. Exploiting the self-seeding activity of tau deposits, and using a 3R tau fragment as a substrate, we have developed an assay (tau RT-QuIC) that can detect tau seeds in 2 µl aliquots of PiD brain dilutions down to 10(-7)-10(-9)...
March 14, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28290977/actinomyces-related-tubo-ovarian-abscess-in-a-poorly-controlled-type-ii-diabetic-with-a-copper-intrauterine-device
#10
Ashley L Sawtelle, Nicole P Chappell, Caela R Miller
BACKGROUND: The historic association of Actinomyces israelii infection with intrauterine devices (IUDs) has long been recognized. In recent years, the risk of developing pelvic inflammatory disease with a copper or levonorgestrel IUD has been less than 1% in women who are low risk for sexually transmitted infections. IUD-related pelvic infections secondary to actinomyces have largely vanished from contemporary practice. CASE: A 49-year-old using a copper IUD for contraception with poorly controlled type II diabetes mellitus was admitted for suspected tubo-ovarian abscess on the basis of abdominopelvic pain, leukocytosis, and computed tomography findings...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28288231/-primary-immunodeficiencies-in-seriously-ill-children-report-of-3-clinical-cases
#11
Leticia Yáñez, Pamela Lama, Carolina Rivacoba, Juanita Zamorano, María Angélica Marinovic
Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. OBJECTIVE: To present and discuss 3 infants diagnosed with PID. CLINICAL CASES: The cases are presented of three patients with PID diagnosed during their first admission to a Paediatric Intensive Critical Care Unit. The first patient, a 4-month-old infant affected by a severe pneumonia, and was diagnosed as a Severe Combined Immunodeficiency Disease...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28271158/physicians-perspectives-on-the-diagnosis-and-management-of-periodic-fever-aphthous-stomatitis-pharyngitis-and-cervical-adenitis-pfapa-syndrome
#12
Kalpana Manthiram, Suzanne C Li, Jonathan S Hausmann, Gil Amarilyo, Karyl Barron, Hanna Kim, Simona Nativ, Geraldina Lionetti, Andrew Zeft, Donald Goldsmith, David Kimberlin, Kathryn Edwards, Fatma Dedeoglu, Sivia Lapidus
To assess the practice patterns of pediatric rheumatology and infectious diseases subspecialists in the diagnosis and treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. An online survey assessing diagnostic and treatment approaches was sent to 424 members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) and 980 members of the Pediatric Infectious Disease Society (PIDS). 277 physicians (123 from CARRA and 154 from PIDS representing 21% of the total membership) completed the survey...
March 7, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28270364/new-frontiers-in-the-therapy-of-primary-immunodeficiency-from-gene-addition-to-gene-editing
#13
REVIEW
Donald B Kohn, Caroline Y Kuo
The most severe primary immune deficiency diseases (PIDs) have been successfully treated with allogeneic hematopoietic stem cell transplantation for more than 4 decades. However, such transplantations have the best outcomes when there is a well-matched donor available because immune complications, such as graft-versus-host disease, are greater without a matched sibling donor. Gene therapy has been developed as a method to perform autologous transplantations of a patient's own stem cells that are genetically corrected...
March 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28270363/targeted-strategies-directed-at-the-molecular-defect-toward-precision-medicine-for-select-primary-immunodeficiency-disorders
#14
REVIEW
Luigi D Notarangelo, Thomas A Fleisher
Primary immunodeficiency disorders (PIDs) represent a range of genetically determined diseases that typically have increased susceptibility to infections and in many cases also have evidence of immune dysregulation that often presents as autoimmunity. Most recently, the concept of gain-of-function mutations associated with PIDs has become well recognized and adds a new dimension to the understanding of this group of disorders, moving beyond the more commonly seen loss-of-function mutations. The rapidly expanding genetic defects that have been identified in patients with previously uncharacterized PIDs has opened up the potential for targeted therapy directed at the specific disease-causing abnormality...
March 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28268387/cascaded-pid-controller-for-anaesthesia-delivery
#15
Alena Simalatsar, Monia Guidi, Thierry Buclin
The technologies for continuous measurement of the anaesthetic agents circulating in body fluids are not mature yet, though some preliminary prototypes exist already. We present a control algorithm that based on the real measurement of propofol plasma concentration may adjust the delivery rate. This opens a possibility for a safer anesthesia when the technologies for online measurement of drug concentration will be mature enough to be combined with our model.
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28261277/gene-therapy-for-primary-immune-deficiencies-a-canadian-perspective
#16
REVIEW
Xiaobai Xu, Chetankumar S Tailor, Eyal Grunebaum
The use of gene therapy (GT) for the treatment of primary immune deficiencies (PID) including severe combined immune deficiency (SCID) has progressed significantly in the recent years. In particular, long-term studies have shown that adenosine deaminase (ADA) gene delivery into ADA-deficient hematopoietic stem cells that are then transplanted into the patients corrects the abnormal function of the ADA enzyme, which leads to immune reconstitution. In contrast, the outcome was disappointing for patients with X-linked SCID, Wiskott-Aldrich syndrome and chronic granulomatous disease who received GT followed by autologous gene corrected transplantations, as many developed hematological malignancies...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28240985/a-palb2-interacting-domain-in-rnf168-couples-homologous-recombination-to-dna-break-induced-chromatin-ubiquitylation
#17
Martijn S Luijsterburg, Dimitris Typas, Marie-Christine Caron, Wouter W Wiegant, Diana van den Heuvel, Rick A Boonen, Anthony M Couturier, Leon H Mullenders, Jean-Yves Masson, Haico van Attikum
DNA double-strand breaks (DSB) elicit a ubiquitylation cascade that controls DNA repair pathway choice. This cascade involves the ubiquitylation of histone H2A by the RNF168 ligase and the subsequent recruitment of RIF1, which suppresses homologous recombination (HR) in G1 cells. The RIF1-dependent suppression is relieved in S/G2 cells, allowing PALB2-driven HR to occur. With the inhibitory impact of RIF1 relieved, it remains unclear how RNF168-induced ubiquitylation influences HR. Here, we uncover that RNF168 links the HR machinery to H2A ubiquitylation in S/G2 cells...
February 27, 2017: ELife
https://www.readbyqxmd.com/read/28237128/primary-immunodeficiency-diseases-in-northern-iran
#18
I Mohammadzadeh, B Moazzami, J Ghaffari, A Aghamohammadi, N Rezaei
INTRODUCTION: Primary immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders, characterised by recurrent severe infections, autoimmunity and lymphoproliferation. Despite impressive progress in identification of novel PID, there is an unfortunate lack of awareness among physicians in identification of patients with PID, especially in non-capital cities of countries worldwide. RESULT: This study was performed in a single-centre paediatric hospital in Northern Iran during a 21-year period (1994-2015)...
February 22, 2017: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/28236292/the-role-of-genomics-in-common-variable-immunodeficiency-disorders
#19
REVIEW
Anne-Kathrin Kienzler, Chantal E Hargreaves, Smita Y Patel
The advent of next generation sequencing (NGS) and 'omic' technologies has revolutionised the field of genetics and its implementation in healthcare has the potential to realise precision medicine. Primary immunodeficiencies (PID) are a group of rare diseases which have benefitted from NGS, with a massive increase in causative genes identified in the past few years. Common Variable Immunodeficiency Disorders (CVID) are a heterogeneous form of PID and the most common form of antibody failure in children and adults...
February 25, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28228821/security-of-patient-data-when-decommissioning-ultrasound-systems
#20
James Moggridge
BACKGROUND: Although ultrasound systems generally archive to Picture Archiving and Communication Systems (PACS), their archiving workflow typically involves storage to an internal hard disk before data are transferred onwards. Deleting records from the local system will delete entries in the database and from the file allocation table or equivalent but, as with a PC, files can be recovered. Great care is taken with disposal of media from a healthcare organisation to prevent data breaches, but ultrasound systems are routinely returned to lease companies, sold on or donated to third parties without such controls...
February 2017: Ultrasound: Journal of the British Medical Ultrasound Society
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