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https://www.readbyqxmd.com/read/27914684/sickle-cell-disease-in-the-older-adult
#1
REVIEW
Mya S Thein, Norris E Igbineweka, Swee Lay Thein
Sickle cell disease (SCD) is an inherited haemoglobin disorder, associated with recurrent painful episodes, ongoing haemolytic anaemia and progressive multi-organ damage. Until the early 1990s, survival beyond the fourth decade for a patient with SCD was considered unusual and prompted case reports. Nowadays, in countries with developed health care systems, more than 90 percent of newborns with SCD survive into adulthood. Nevertheless, their life expectancy is still shortened by more than two decades compared to the general population...
November 30, 2016: Pathology
https://www.readbyqxmd.com/read/27905001/guidelines-for-diagnosis-and-management-of-the-cobalamin-related-remethylation-disorders-cblc-cbld-cble-cblf-cblg-cblj-and-mthfr-deficiency
#2
Martina Huemer, Daria Diodato, Bernd Schwahn, Manuel Schiff, Anabela Bandeira, Jean-Francois Benoist, Alberto Burlina, Roberto Cerone, Maria L Couce, Angeles Garcia-Cazorla, Giancarlo la Marca, Elisabetta Pasquini, Laura Vilarinho, James D Weisfeld-Adams, Viktor Kožich, Henk Blom, Matthias R Baumgartner, Carlo Dionisi-Vici
BACKGROUND: Remethylation defects are rare inherited disorders in which impaired remethylation of homocysteine to methionine leads to accumulation of homocysteine and perturbation of numerous methylation reactions. OBJECTIVE: To summarise clinical and biochemical characteristics of these severe disorders and to provide guidelines on diagnosis and management. DATA SOURCES: Review, evaluation and discussion of the medical literature (Medline, Cochrane databases) by a panel of experts on these rare diseases following the GRADE approach...
November 30, 2016: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27902286/macrococcus-canis-sp-nov-a-skin-bacterium-associated-with-infections-in-dogs
#3
Stefanie Gobeli Brawand, Kerstin Cotting, Elena Gómez-Sanz, Alexandra Collaud, Andreas Thomann, Isabelle Brodard, Sabrina Rodriguez Campos, Christian Strauss, Vincent Perreten
Gram-stain positive cocci were isolated from miscellaneous sites of the skin of healthy dogs as well as from infection sites in dogs. The closest relative by sequencing of the 16S rRNA was Macrococcus caseolyticus with 99.7% sequence identity, but compared to M. caseolyticus, the novel strains shared only 90.8% to 93.5% DNA identity to cpn60, dnaJ, rpoB, and sodA partial genes, respectively. The two species also exhibited other phenotypic characteristics and the majority displayed a visible haemolysis on sheep blood agar, while M...
November 16, 2016: International Journal of Systematic and Evolutionary Microbiology
https://www.readbyqxmd.com/read/27900629/dracorhodin-perochlorate-attenuates-staphylococcus-aureus-usa300-virulence-by-decreasing-%C3%AE-toxin-expression
#4
Yumin Liu, Dongxue Shi, Yan Guo, Meng Li, Yonghong Zha, Quankai Wang, Jianfeng Wang
α-Toxin, a pore-forming toxin secreted by most Staphylococcus aureus, plays critical role in the pathogenesis associated with various infectious diseases. The USA300 which is a major international epidemic methicilin-resisrant S. aureus has spread rapidly to multiple countries and become an emerging public health concern. In this study, the in vitro efficacy of Dracorhodin Perochlorate (DP) against USA300 virulence was evaluated. Using susceptibility testing, immunoblots, rabbit blood haemolytic assay and real-time RT-PCR, we observed that the α-toxin production was decreased when USA300 was co-cultured with different sub-inhibitory concentration of DP...
January 2017: World Journal of Microbiology & Biotechnology
https://www.readbyqxmd.com/read/27893356/single-stranded-dna-aptamer-targeting-and-neutralization-of-anti-d-alloantibody-a-potential-therapeutic-strategy-for-haemolytic-diseases-caused-by-rhesus-alloantibody
#5
Yinze Zhang, Fan Wu, Manni Wang, Naibao Zhuang, Huayou Zhou, Hua Xu
BACKGROUND: Rhesus (Rh) D antigen is the most important antigen in the Rh blood group system because of its strong immunogenicity. When RhD-negative individuals are exposed to RhD-positive blood, they may produce anti-D alloantibody, potentially resulting in delayed haemolytic transfusion reactions and Rh haemolytic disease of the foetus and newborn, which are difficult to treat. Inhibition of the binding of anti-D antibody with RhD antigens on the surface of red blood cells may effectively prevent immune haemolytic diseases...
November 11, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/27893348/cost-utility-and-budget-impact-of-methylene-blue-treated-plasma-compared-to-quarantine-plasma
#6
Joseph B Babigumira, Solomon J Lubinga, Emma Castro, Brian Custer
BACKGROUND: Methylene blue and visible light treatment and quarantine are two methods used to reduce adverse events, mostly infections, associated with the transfusion of fresh-frozen plasma. The objective of this study was to estimate and compare the budget impact and cost-utility of these two methods from a payer's perspective. MATERIALS AND METHODS: A budget impact and cost-utility model simulating the risks of hepatitis B virus, hepatitis C virus, cytomegalovirus, a West Nile virus-like infection, allergic reactions and febrile non-haemolytic transfusion reactions achieved using plasma treated with methylene blue and visible light (MBP) and quarantine plasma (QP) was constructed for Spain...
November 16, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/27891581/antigen-specificity-determines-anti-red-blood-cell-igg-fc-alloantibody-glycosylation-and-thereby-severity-of-haemolytic-disease-of-the-fetus-and-newborn
#7
Myrthe E Sonneveld, Joke Koelewijn, Masja de Haas, Jon Admiraal, Rosina Plomp, Carolien A M Koeleman, Agnes L Hipgrave Ederveen, Peter Ligthart, Manfred Wuhrer, C Ellen van der Schoot, Gestur Vidarsson
Haemolytic disease of the fetus and newborn (HDFN) is a severe disease in which fetal red blood cells (RBC) are destroyed by maternal anti-RBC IgG alloantibodies. HDFN is most often caused by anti-D but may also occur due to anti-K, -c- or -E. We recently found N-linked glycosylation of anti-D to be skewed towards low fucosylation, thereby increasing the affinity to IgG-Fc receptor IIIa and IIIb, which correlated with HDFN disease severity. Here, we analysed 230 pregnant women with anti-c, -E or -K alloantibodies from a prospective screening cohort and investigated the type of Fc-tail glycosylation of these antibodies in relation to the trigger of immunisation and pregnancy outcome...
November 28, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27891338/non-o157-h7-shiga-toxin-producing-diarrhoeagenic-escherichia-coli-stec-in-southern-india-a-tinderbox-for-starting-epidemic
#8
Shashank Purwar, Subrana Roy, Sharada Metgud
INTRODUCTION: Outbreaks due to non-O157:H7 Shiga toxin producing Escherichia coli (STEC) resulting in Haemolytic Uraemic Syndrome (HUS) have garnered much attention because of associated mortality transcending across continents and also because diarrhoea due to E.coli itself is rare in developed countries. The actual incidence of non-O157:H7 STEC in sporadic acute diarrhoea is not fully elucidated, both in developing as well as in developed countries. Due to larger extent of faecal-oral transmission in developing countries it is prudent to look for non-O157: H7 STEC in such epidemiological settings because of very high potential to spread across larger geographical regions and cause life threatening illness...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27889303/pgla-h-tandem-repeat-peptides-active-against-multidrug-resistant-clinical-bacterial-isolates
#9
Tomislav Rončević, Goran Gajski, Nada Ilić, Ivana Goić-Barišić, Marija Tonkić, Larisa Zoranić, Juraj Simunić, Monica Benincasa, Marijana Mijaković, Alessandro Tossi, Davor Juretić
Antimicrobial peptides (AMPs) are promising candidates for new antibiotic classes but often display an unacceptably high toxicity towards human cells. A naturally produced C-terminal fragment of PGLa, named PGLa-H, has been reported to have a very low haemolytic activity while maintaining a moderate antibacterial activity. A sequential tandem repeat of this fragment, diPGLa-H, was designed, as well as an analogue with a Val to Gly substitution at a key position. These peptides showed markedly improved in vitro bacteriostatic and bactericidal activity against both reference strains and multidrug resistant clinical isolates of Gram-negative and Gram-positive pathogens, with generally low toxicity for human cells as assessed by haemolysis, cell viability, and DNA damage assays...
November 23, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27878547/transcatheter-closure-of-paravalvular-leaks-state-of-the-art
#10
I Cruz-Gonzalez, J C Rama-Merchan, J Rodríguez-Collado, J Martín-Moreiras, A Diego-Nieto, M Barreiro-Pérez, P L Sánchez
Paravalvular leak (PVL) is a serious complication after surgical valve replacement or after transcatheter aortic valve replacement. Approximately 1-5% of PVLs can lead to serious clinical consequences, including congestive heart failure and/or haemolytic anaemia. For years, surgical re-intervention has been considered the treatment of choice for symptomatic patients with PVLs. However, surgical re-intervention is associated with a high risk of morbidity and mortality. Transcatheter PVL (TPVL) closure is a less invasive alternative to surgical re-intervention...
November 22, 2016: Netherlands Heart Journal
https://www.readbyqxmd.com/read/27877159/efficacy-of-antibacterial-peptides-against-peptide-resistant-mrsa-is-restored-by-permeabilization-of-bacteria-membranes
#11
Joshua Ravensdale, Zachary Wong, Frances O'Brien, Keith Gregg
Clinical application of antimicrobial peptides (AMPs), as with conventional antibiotics, may be compromised by the development of bacterial resistance. This study investigated AMP resistance in methicillin resistant Staphylococcus aureus, including aspects related to the resilience of the resistant bacteria toward the peptides, the stability of resistance when selection pressures are removed, and whether resistance can be overcome by using the peptides with other membrane-permeabilising agents. Genotypically variant strains of S...
2016: Frontiers in Microbiology
https://www.readbyqxmd.com/read/27871301/an-innovative-and-collaborative-partnership-between-patients-with-rare-disease-and-industry-supported-registries-the-global-ahus-registry
#12
REVIEW
Len Woodward, Sally Johnson, Johan Vande Walle, Joran Beck, Christoph Gasteyger, Christoph Licht, Gema Ariceta
BACKGROUND: Patients are becoming increasingly involved in research which can promote innovation through novel ideas, support patient-centred actions, and facilitate drug development. For rare diseases, registries that collect data from patients can increase knowledge of the disease's natural history, evaluate clinical therapies, monitor drug safety, and measure quality of care. The active participation of patients is expected to optimise rare-disease management and improve patient outcomes...
November 21, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27870210/risk-profiles-for-haemolytic-and-nonhaemolytic-neonatal-jaundice
#13
EDITORIAL
Vinod K Bhutani, Ronald J Wong
No abstract text is available yet for this article.
December 2016: Acta Paediatrica
https://www.readbyqxmd.com/read/27866816/nitric-oxide-donating-derivatives-of-hederacolchiside-a1-synthesis-and-biological-evaluation-in-vitro-and-in-vivo-as-potential-anticancer-agents
#14
Yuanying Fang, Rikang Wang, Mingzhen He, Hesong Huang, Qi Wang, Zunhua Yang, Yan Li, Shilin Yang, Yi Jin
A series of nitric oxide (NO) donating derivatives of hederacolchiside A1 bearing triterpenoid saponin motif were designed, synthesized and evaluated for their anticancer activity. All of the tested furoxan-based NO releasing compounds showed significant proliferation inhibitory activities. Especially compound 6a exhibited strong cytotoxicity (IC50=1.6-6.5μM) against four human tumor cell lines (SMMC-7721, NCI-H460, U251, HCT-116) in vitro and the highest level of NO releasing. Furthermore, compound 6a was revealed low acute toxicity to mice and weak haemolytic activity with potent tumor growth inhibition against mice H22 hepatocellular cells in vivo (51...
November 10, 2016: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/27863511/analysis-of-complement-biomarkers-in-systemic-sclerosis-indicates-a-distinct-pattern-in-scleroderma-renal-crisis
#15
Marcin Okrój, Martin Johansson, Tore Saxne, Anna M Blom, Roger Hesselstrand
BACKGROUND: The complement system has been implicated in pathogenesis of systemic sclerosis (SSc). The goal of the present study was to evaluate improved complement biomarkers in SSc. METHODS: The presence of C4d, reflecting activation of the classical/lectin pathways, C3bBbP corresponding to activation of the alternative pathway, and soluble terminal complement complexes (all complement pathways), was measured in plasma samples by enzyme-linked immunosorbent assay and correlated to clinical parameters...
November 18, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/27858138/engineered-biosynthesis-and-characterisation-of-disaccharide-modified-8-deoxyamphoteronolides
#16
Simon Walmsley, Eimear De Poire, Bernard Rawlings, Patrick Caffrey
Several polyene macrolides are potent antifungal agents that have severe side effects. Increased glycosylation of these compounds can improve water solubility and reduce toxicity. Three extending glycosyltransferases are known to add hexoses to the mycosaminyl sugar residues of polyenes. The Actinoplanes caeruleus PegA enzyme catalyses attachment of a D-mannosyl residue in a β-1,4 linkage to the mycosamine of the aromatic heptaene 67-121A to form 67-121C. NppY from Pseudonocardia autotrophica adds an N-acetyl-D-glucosamine to the mycosamine of 10-deoxynystatin...
November 17, 2016: Applied Microbiology and Biotechnology
https://www.readbyqxmd.com/read/27856325/effects-of-dietary-administration-of-fenugreek-seeds-alone-or-in-combination-with-probiotics-on-growth-performance-parameters-humoral-immune-response-and-gene-expression-of-gilthead-seabream-sparus-aurata-l
#17
A Bahi, F A Guardiola, C Messina, A Mahdhi, R Cerezuela, A Santulli, A Bakhrouf, M A Esteban
The use of immunostimulants is considered a promising preventive practice that may help to maintain animal welfare and a healthy environment, while increasing production and providing higher profits. The purpose of this study was to evaluate the effects on gilthead seabream (Sparus aurata L.) of the dietary administration of fenugreek (Trigonella foenum graecum) seeds, alone or combined with one of the following probiotic strains: Bacillus licheniformis (TSB27), Lactobacillus plantarum or Bacillus subtilis (B46)...
November 14, 2016: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/27855617/eryptosis-ally-or-enemy
#18
Marilena Briglia, Maria Antonia Rossi, Caterina Faggio
Prior to senescence, erythrocytes may, experience injury which compromises their integrity and thus triggers suicidal erythrocyte death or eryptosis. This mechanism is characterised by cell shrinkage, cell membrane blebbing, and cell membrane phospholipid scrambling after phosphatidylserine exposure on the cell surface that is identified by macrophages which engulf and degrade the eryptotic cells. The term eryptosis also includes typical mechanisms, which contribute to the triggering of this process. Among them: oxidative stress, Ca2+ entry with an increase in cytosolic Ca2+ activity ([Ca ]i) and the activation of p38 kinase, which is a kinase expressed in human erythrocytes and activated after hyperosmotic shock...
November 18, 2016: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/27837197/comparison-of-ethnicity-gender-age-of-onset-and-outcome-in-south-africans-with-systemic-lupus-erythematosus
#19
A Budhoo, G M Mody, T Dubula, N Patel, P G Mody
Ethnicity, gender and age of onset are reported to influence the expression and outcome of systemic lupus erythematosus. We studied a multi-ethnic cohort of 408 South Africans (91.2% females) comprising 237 (58.1%) Indians, 137 (33.6%) African Blacks, 17 (4.2%) Mixed ethnicity and 17 (4.2%) Whites. The most common manifestations were arthritis (80.6%), photosensitivity (67.2%), oral ulcers (50.0%), malar rash (49.0%) and renal (39.2%). The common laboratory findings were positive anti-nuclear factor (96.8%), haematological (74...
November 10, 2016: Lupus
https://www.readbyqxmd.com/read/27830112/mycoplasma-pneumonia-with-persistent-lymphadenopathy-and-severe-cold-agglutinin-haemolysis
#20
Mit Shah, Sajid Khan, Kenneth Chan
Mycoplasma pneumonia is an atypical pneumonia commonly affecting young patients with generally mild clinical course. We present a case of a 66-year-old female presenting with weight loss, night sweats and low-grade pyrexia. She acquired symptomatic haemolytic anaemia requiring blood transfusion, markedly raised erythrocyte sedimentation rate (ESR) to 114 mm/hr and extensive peri-hilar lymphadenopathy on computed tomography (CT) scan. After excluding malignancy and granulomatous diseases, she made good recovery although a 4 week follow-up CT scan showed persistent but resolving lymphadenopathy...
2016: Respiratory Medicine Case Reports
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