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https://www.readbyqxmd.com/read/28750490/secondary-adrenal-insufficiency-from-the-physiopathology-to-the-possible-role-of-modified-release-hydrocortisone-treatment
#1
Rosa M Paragliola, Salvatore M Corsello
Central adrenal insufficiency represents a life-threatening disorder that results from a reduced cortisol production due to an impairment production of adrenocorticotropic hormone. In particular, secondary AI results from pituitary disease that impedes the release of ACTH, while tertiary adrenal insufficiency is caused from an impaired synthesis of corticotropin-releasing hormone. Central adrenal insufficiency has an estimated prevalence of 150-280 per million, resulting more common than primary AI. Prompt diagnosis and management of this condition is crucial, but the diagnostic investigation can often be challenging, in particular in cases of recent onset of secondary adrenal insufficiency...
July 27, 2017: Minerva Endocrinologica
https://www.readbyqxmd.com/read/28750471/modulation-of-the-activated-protein-c-pathway-in-severe-haemophilia-a-patients-the-effects-of-thrombomodulin-and-a%C3%A2-factor-v-stabilizing-fab
#2
D F Brophy, E J Martin, B M Mohammed, J C Barrett, J G Kuhn, M E Nolte, B Wiinberg, H L Holmberg, J Lund, R Salbo, E K Waters
INTRODUCTION: The thrombomodulin (TM)/activated protein C (APC) system is a key regulator of haemostasis, limiting amplification and propagation of the formed blood clot to the injury site. Dampening APC's inhibition of factor V (FV) and factor VIII (FVIII) may be a future strategy in developing next-generation therapeutic targets for haemophilia treatment. AIMS: To determine ex vivo the respective concentration-dependent effects of TM and a FV-stabilizing Fab on the APC regulatory pathway in severe FVIII-deficient blood and plasma...
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28750470/clot-waveform-analysis-using-cs-2000i%C3%A2-distinguishes-between-very-low-and-absent-levels-of-factor-viii-activity-in-patients-with-severe-haemophilia-a
#3
T Matsumoto, K Nogami, Y Tabuchi, K Yada, K Ogiwara, H Kurono, N Arai, M Shima
INTRODUCTION: A recently developed method to assess comprehensive coagulation function, clot waveform analysis (CWA), accurately detect low levels (<1 IU/dL) of factor VIII activity (FVIII:C) in haemophilia A patients (HA-pts). Improvements are needed, however, to differentiate patients with very low from absent levels of FVIII:C. AIM: We attempted to optimize CWA using the coagulation analyser CS-2000i™ to distinguish between very low levels and absent FVIII:C in severe HA-pts...
July 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28750335/ironphone-mobile-device-coupled-point-of-care-diagnostics-for-assessment-of-iron-status-by-quantification-of-serum-ferritin
#4
Balaji Srinivasan, Dakota O'Dell, Julia L Finkelstein, Seoho Lee, David Erickson, Saurabh Mehta
Iron deficiency (ID) is an urgent public health problem that has devastating effects on maternal and child health. However, due to poor access and affordability, screening and diagnosis for ID is often limited to proxy hemoglobin measurements alone. Here, we report the development and validation of ironPhone, a mobile-device coupled portable diagnostics for quantification of serum ferritin concentrations, an iron status biomarker, within a few minutes, from a drop of fingerprick blood. The ironPhone diagnostic platform comprises of a smartphone accessory, an app, and a disposable lateral flow immunoassay test strip to quantify serum ferritin...
July 19, 2017: Biosensors & Bioelectronics
https://www.readbyqxmd.com/read/28750142/feline-leukocyte-adhesion-cd18-deficiency-caused-by-a-deletion-in-the-integrin-%C3%AE-2-itgb2-gene
#5
Thomas R Bauer, Suzanne M Pratt, Christina M Palena, Karthik Raj, Urs Giger
BACKGROUND: Leukocyte adhesion deficiency (LAD) or CD18 deficiency is an autosomal recessive immunodeficiency which has been described in people, cattle, dogs, and knockout mice. OBJECTIVES: The study goals were to characterize the clinicopathologic, immunologic, and molecular genetic features of feline LAD (FLAD) in a neutered male adult Domestic Longhair cat with severe leukocytosis and recurrent infections. METHODS: Flow cytometry evaluated surface expression of CD18 on neutrophils...
July 27, 2017: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/28750079/roles-of-the-low-density-lipoprotein-receptor-and-related-receptors-in-inhibition-of-lipoprotein-a-internalization-by-proprotein-convertase-subtilisin-kexin-type-9
#6
Rocco Romagnuolo, Corey A Scipione, Santica M Marcovina, Matthew Gemin, Nabil G Seidah, Michael B Boffa, Marlys L Koschinsky
Elevated plasma concentrations of lipoprotein(a) (Lp(a)) are a causal risk factor for cardiovascular disease. The mechanisms underlying Lp(a) clearance from plasma remain unclear, which is an obvious barrier to the development of therapies to specifically lower levels of this lipoprotein. Recently, it has been documented that monoclonal antibody inhibitors of proprotein convertase subtilisin/kexin type 9 (PCSK9) can lower plasma Lp(a) levels by 30%. Since PCSK9 acts primarily through the low density lipoprotein receptor (LDLR), this result is in conflict with the prevailing view that the LDLR does not participate in Lp(a) clearance...
2017: PloS One
https://www.readbyqxmd.com/read/28750066/cross-modulation-of-pathogen-specific-pathways-enhances-malnutrition-during-enteric-co-infection-with-giardia-lamblia-and-enteroaggregative-escherichia-coli
#7
Luther A Bartelt, David T Bolick, Jordi Mayneris-Perxachs, Glynis L Kolling, Gregory L Medlock, Edna I Zaenker, Jeffery Donowitz, Rose Viguna Thomas-Beckett, Allison Rogala, Ian M Carroll, Steven M Singer, Jason Papin, Jonathan R Swann, Richard L Guerrant
Diverse enteropathogen exposures associate with childhood malnutrition. To elucidate mechanistic pathways whereby enteric microbes interact during malnutrition, we used protein deficiency in mice to develop a new model of co-enteropathogen enteropathy. Focusing on common enteropathogens in malnourished children, Giardia lamblia and enteroaggregative Escherichia coli (EAEC), we provide new insights into intersecting pathogen-specific mechanisms that enhance malnutrition. We show for the first time that during protein malnutrition, the intestinal microbiota permits persistent Giardia colonization and simultaneously contributes to growth impairment...
July 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28750002/dna-pkcs-controls-calcineurin-mediated-il-2-production-in-t-lymphocytes
#8
Ara Kim Wiese, Marie Schluterman Burdine, Richard H Turnage, Alan J Tackett, Lyle J Burdine
Loss of DNA-dependent protein kinase catalytic subunit (DNA-PKcs) activity in mammals results in severe combined immuno-deficiency (SCID). This SCID phenotype has been postulated to be due solely to the function of DNA-PKcs in V(D)J recombination, a process critical for lymphocyte maturation. However; we show that DNA-PKcs is required for IL-2 production via regulation of the calcineurin signaling pathway. Reducing DNA-PKcs activity in activated T cells either by shRNA or an inhibitor significantly reduced IL-2 production by blocking calcineurin activity and the translocation of NFAT into the nucleus...
2017: PloS One
https://www.readbyqxmd.com/read/28749985/fatigue-in-out-patients-with-inflammatory-bowel-disease-prevalence-and-predictive-factors
#9
Albert Villoria, Víctor García, Angelina Dosal, Laura Moreno, Antònia Montserrat, Ariadna Figuerola, Diana Horta, Xavier Calvet, María José Ramírez-Lázaro
BACKGROUND AND AIM: Fatigue is a common and bothersome symptom in inflammatory bowel disease (IBD) patients. The study was aimed to determine the relationship of biological and psychological factors with IBD-related fatigue. METHODS: Consecutive clinically inactive IBD outpatients receiving immunosuppressants or biological drugs were enrolled between January and December 2013. Patients completed a Fatigue score (FACIT-F), various psychological, quality of life (IBDQ-9), and IBD activity scores...
2017: PloS One
https://www.readbyqxmd.com/read/28749951/vitamin-d-deficient-mice-have-more-invasive-urinary-tract-infection
#10
Olof Hertting, Petra Lüthje, Devin Sullivan, Pontus Aspenström, Annelie Brauner
Vitamin D deficiency is a common health problem with consequences not limited to bone and calcium hemostasis. Low levels have also been linked to tuberculosis and other respiratory infections as well as autoimmune diseases. We have previously shown that supplementation with vitamin D can induce the antimicrobial peptide cathelicidin during ex vivo infection of human urinary bladder. In rodents, however, cathelicidin expression is not linked to vitamin D and therefore this vitamin D-related effect fighting bacterial invasion is not relevant...
2017: PloS One
https://www.readbyqxmd.com/read/28749940/upregulation-of-an-inward-rectifying-k-channel-can-rescue-slow-ca2-oscillations-in-k-atp-channel-deficient-pancreatic-islets
#11
Vehpi Yildirim, Suryakiran Vadrevu, Benjamin Thompson, Leslie S Satin, Richard Bertram
Plasma insulin oscillations are known to have physiological importance in the regulation of blood glucose. In insulin-secreting β-cells of pancreatic islets, K(ATP) channels play a key role in regulating glucose-dependent insulin secretion. In addition, they convey oscillations in cellular metabolism to the membrane by sensing adenine nucleotides, and are thus instrumental in mediating pulsatile insulin secretion. Blocking K(ATP) channels pharmacologically depolarizes the β-cell plasma membrane and terminates islet oscillations...
July 27, 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/28749773/hemolytic-potential-of-tafenoquine-in-female-volunteers-heterozygous-for-glucose-6-phosphate-dehydrogenase-g6pd-deficiency-g6pd-mahidol-variant-versus-g6pd-normal-volunteers
#12
Ronnatrai Rueangweerayut, Germana Bancone, Emma J Harrell, Andrew P Beelen, Supornchai Kongpatanakul, Jörg J Möhrle, Vicki Rousell, Khadeeja Mohamed, Ammar Qureshi, Sushma Narayan, Nushara Yubon, Ann Miller, François H Nosten, Lucio Luzzatto, Stephan Duparc, Jörg-Peter Kleim, Justin A Green
Tafenoquine is an 8-aminoquinoline under investigation for the prevention of relapse in Plasmodium vivax malaria. This open-label, dose-escalation study assessed quantitatively the hemolytic risk with tafenoquine in female healthy volunteers heterozygous for the Mahidol(487A) glucose-6-phosphate dehydrogenase (G6PD)-deficient variant versus G6PD-normal females, and with reference to primaquine. Six G6PD-heterozygous subjects (G6PD enzyme activity 40-60% of normal) and six G6PD-normal subjects per treatment group received single-dose tafenoquine (100, 200, or 300 mg) or primaquine (15 mg × 14 days)...
July 24, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28749757/paracoccidioidomycosis-epidemiological-and-clinical-aspects-in-546-cases-studied-in-the-state-of-esp%C3%A3-rito-santo-brazil
#13
Paulo Mendes Peçanha, Mayara Elisa Batista Ferreira, Maria Angélica Massaroni Peçanha, Edilaine Brandão Schmidt, Mariceli Lamas de Araújo, Raphael Lubiana Zanotti, Felipe Fonseca Potratz, Nilo Eduardo Delboni Nunes, Carlos Urbano Gonçalves Ferreira, Délio Delmaestro, Aloísio Falqueto
Paracoccidioidomycosis (PCM) is an endemic disease in the southeast region of Brazil, which includes the state of Espírito Santo (ES). This historic case series analyses 546 patients treated in this state from 1978 to 2012. Patients aged from 7 to 83 years, 509 males and 37 females, yielding a ratio of 13.7:1. Most of the patients (81.4%) originated from rural areas in ES, 71.0% being farmers. A higher concentration of cases was observed in municipalities located along the western range of the state. Sixty patients (11...
July 24, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28749597/a-structural-model-of-nitro-porphyrin-dyes-based-on-spectroscopy-and-density-functional-theory
#14
Christopher Farley, Amit Aggarwal, Sunaina Singh, Aaron Dolor, Philip To, Alexander Falber, Maxwell Crossley, Charles Michael Drain
Nitro-porphyrins are an important class of commercial dyes with a range of potential applications. The nitro group is known to dramatically affect the photophysics of the porphyrin, but there are few systematic investigations of the contributing factors. To address this deficiency, we present spectroscopic studies of a series of nitro-porphyrins, accompanied by density functional theory calculations to elucidate their structures. In particular, we explore how the positions of the substituents affect the energy levels and nuclear geometry...
July 27, 2017: Journal of Computational Chemistry
https://www.readbyqxmd.com/read/28749522/palladium-catalyzed-decarboxylative-decarbonylative-and-dehydrogenative-c-sp-2-h-acylation-at-room-temperature
#15
Asik Hossian, Manash Kumar Manna, Kartic Manna, Ranjan Jana
Over the past few decades, an impressive array of C-H activation methodology has been developed for organic synthesis. However, due to the inherent inertness of the C-H bonds (e.g. ∼110 kcal mol(-1) for the cleavage of C(aryl)-H bonds) harsh reaction conditions have been realized to overcome high energetic transition states resulting in a limited substrate scope and functional group tolerance. Therefore, the development of mild C-H functionalization protocols is in high demand to exploit the full potential of the C-H activation strategy in the synthesis of a complex molecular framework...
July 27, 2017: Organic & Biomolecular Chemistry
https://www.readbyqxmd.com/read/28749486/milestones-and-controversies-in-maternal-and-child-health-examining-a-brief-history-of-micronutrient-fortification-in-the-us
#16
A Deavenport-Saman, A Britt, K Smith, R A Jacobs
Fortification of our food and drinking supply has decreased morbidity rates related to micronutrient deficiencies among mothers and their children, particularly during the perinatal and neonatal periods of development. The purpose of this historical review is to examine the impact of public policy changes related to micronutrient fortification. We provide a historical investigation of achievements and controversies related to iodine, vitamin D, fluoride and folic acid fortifications in our food and drinking supply...
July 27, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28749473/slc39a8-deficiency-biochemical-correction-and-major-clinical-improvement-by-manganese-therapy
#17
Julien H Park, Max Hogrebe, Manfred Fobker, Renate Brackmann, Barbara Fiedler, Janine Reunert, Stephan Rust, Konstantinos Tsiakas, René Santer, Marianne Grüneberg, Thorsten Marquardt
PurposeSLC39A8 deficiency is a severe inborn error of metabolism that is caused by impaired function of manganese metabolism in humans. Mutations in SLC39A8 lead to impaired function of the manganese transporter ZIP8 and thus manganese deficiency. Due to the important role of Mn(2+) as a cofactor for a variety of enzymes, the resulting phenotype is complex and severe. The manganese-dependence of β-1,4-galactosyltransferases leads to secondary hypoglycosylation, making SLC39A8 deficiency both a disorder of trace element metabolism and a congenital disorder of glycosylation...
July 27, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28749464/the-cohesin-complex-prevents-myc-induced-replication-stress
#18
Sara Rohban, Aurora Cerutti, Marco J Morelli, Fabrizio d'Adda di Fagagna, Stefano Campaner
The cohesin complex is mutated in cancer and in a number of rare syndromes collectively known as Cohesinopathies. In the latter case, cohesin deficiencies have been linked to transcriptional alterations affecting Myc and its target genes. Here, we set out to understand to what extent the role of cohesins in controlling cell cycle is dependent on Myc expression and activity. Inactivation of the cohesin complex by silencing the RAD21 subunit led to cell cycle arrest due to both transcriptional impairment of Myc target genes and alterations of replication forks, which were fewer and preferentially unidirectional...
July 27, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28749399/re-establishment-of-normal-radioactive-iodine-uptake-reference-range-in-the-era-of-universal-salt-iodization-in-the-indian-population
#19
Sanjana Ballal, Ramya Soundararajan, Chandrasekhar Bal
BACKGROUND & OBJECTIVES: The reference radioactive iodine uptake (RAIU) values established in the 1970s in the era of widespread iodine deficiency were relatively high. Now, after four decades of successful Universal Salt Iodization (USI) programme in India, there is a need to re-establish these reference ranges. The present study was aimed to quantify the two-hour and 24-h RAIU values in iodine sufficient euthyroid individuals and validate the results in Graves' and Hashimoto's thyroiditis patients...
March 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/28749359/systolic-time-interval-estimation-using-continuous-wave-radar-with-on-body-antennas
#20
Dilpreet Buxi, Evelien Hermeling, Marco Mercuri, Fabian Beutel, Roberto Garcia van der Westen, Tom Torfs, Jean-Michel Redoute, Mehmet Rasit Yuce
The estimation of systolic time interval (STI)s is done using continuous wave (CW) radar at 2.45GHz with an on-body antenna. MOTIVATION: In the state of the art, typically bioimpedance, heart sounds and / or ultrasound is used to measure STIs. All three methods suffer from insufficient accuracy of STI estimation due to various reasons. CW radar is investigated for its ability to overcome the deficiencies in the state of the art. METHODS: Ten healthy male subjects aged 25-45 were asked to lie down at a 30 degree incline...
July 25, 2017: IEEE Journal of Biomedical and Health Informatics
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