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Hemolytic disease

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https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#1
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28439485/treatment-of-atypical-hemolytic-uremic-syndrome-in-the-era-of-eculizumab
#2
Rawaa Ebrahem, Salam Kadhem, Quoc Truong
Hemolytic-uremic syndrome (HUS) is the triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and acute kidney injury (AKI); the main cause of multi-organ failure is related to thrombotic microangiopathy (TMA). Atypical HUS (aHUS) is a disease of uncontrolled complement activation associated with a high mortality rate and most cases progress to end-stage renal disease. About 50% of patients with this syndrome carry mutations in genes that encode complement proteins. Also, aHUS constitutes an over-activation of the complement pathway which is either inherited, acquired, or both...
March 23, 2017: Curēus
https://www.readbyqxmd.com/read/28439081/long-lasting-neutralization-of-c5-by-sky59-a-novel-recycling-antibody-is-a-potential-therapy-for-complement-mediated-diseases
#3
Taku Fukuzawa, Zenjiro Sampei, Kenta Haraya, Yoshinao Ruike, Meiri Shida-Kawazoe, Yuichiro Shimizu, Siok Wan Gan, Machiko Irie, Yoshinori Tsuboi, Hitoshi Tai, Tetsushi Sakiyama, Akihisa Sakamoto, Shinya Ishii, Atsuhiko Maeda, Yuki Iwayanagi, Norihito Shibahara, Mitsuko Shibuya, Genki Nakamura, Takeru Nambu, Akira Hayasaka, Futa Mimoto, Yuu Okura, Yuji Hori, Kiyoshi Habu, Manabu Wada, Takaaki Miura, Tatsuhiko Tachibana, Kiyofumi Honda, Hiroyuki Tsunoda, Takehisa Kitazawa, Yoshiki Kawabe, Tomoyuki Igawa, Kunihiro Hattori, Junichi Nezu
Dysregulation of the complement system is linked to the pathogenesis of a variety of hematological disorders. Eculizumab, an anti-complement C5 monoclonal antibody, is the current standard of care for paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS). However, because of high levels of C5 in plasma, eculizumab has to be administered biweekly by intravenous infusion. By applying recycling technology through pH-dependent binding to C5, we generated a novel humanized antibody against C5, SKY59, which has long-lasting neutralization of C5...
April 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28437723/clinical-course-and-disease-burden-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-by-hemolytic-status
#4
Mustafa N Yenerel, Petra Muus, Amanda Wilson, Jeff Szer
Disease characteristics of patients enrolled in the International PNH Registry were assessed during two follow-up periods based on hemolytic status while untreated with eculizumab: Non-hemolytic cohort: follow-up time defined as time from disease start until last reported untreated lactate dehydrogenase (LDH) value <1.5×upper limit normal (ULN); Hemolytic cohort: follow-up time defined as time from LDH ≥1.5×ULN at or post-disease start, to most recent untreated follow-up. A total of 1012 patients met criteria for the Non-hemolytic cohort and 1565 patients for the Hemolytic cohort; median (min, max) years of follow-up were 2...
March 27, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28434982/novel-monoclonal-antibodies-against-stx1d-and-1e-and-their-use-for-improving-immunoassays
#5
Xiaohua He, Stephanie Patfield, Reuven Rasooly, Daniela Mavrici
Shiga toxins (Stxs) are major causative agents for bloody diarrhea and hemolytic uremic syndrome, a life-threatening disease in humans. No effective treatment is available. Early detection of Stxs in clinical samples is critical for disease management. As bacteria evolve, new Stxs are produced; therefore, methods used to identify them need to be improved as well. In this study, new monoclonal antibodies (mAbs) against Stx1d and 1e were developed and used to improve a commercial Stx1 kit. Incorporation of the new mAbs into the Abraxis Stx1 kit not only increased the assay sensitivity to Stx1d, but the assay was conferred the ability to detect Stx1e, a newly identified subtype of Stx1 produced by an atypical Stx-producing bacterial strain, Enterobacter cloacae M12X01451, isolated from a clinical specimen...
April 20, 2017: Journal of Immunological Methods
https://www.readbyqxmd.com/read/28433715/aloe-vera-enhances-the-innate-immune-response-of-pacu-piaractus-mesopotamicus-after-transport-stress-and-combined-heat-killed-aeromonas-hydrophila-infection
#6
Fábio S Zanuzzo, Rafael E Sabioni, Luz Natalia F Montoya, Gisele Favero, Elisabeth C Urbinati
In this study, pacu (Piaractus mesopotamicus) were fed with diets containing Aloe vera for 10 days prior to transport stress and infection with heat killed Aeromonas hydrophila. A. vera is popular around the world due to its medicinal properties, including immunostimulatory effects which was observed in this study. The results show that transport causes immunosuppression, an effect that was prevented by A. vera. Specifically, A. vera prevented reductions of both leukocyte respiratory burst and hemolytic activity of complement system caused by transport...
April 19, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28425749/hemolytic-transfusion-reaction-attributable-to-anti-dia
#7
Arthur J Joyce, Kelli M Quantock, Ray Banh, Yew W Liew
In situations when a patient's antibody detection test is negative, many institutions have moved from an indirect antiglobulin test (IAT) crossmatch to an electronic crossmatch system. Here we report a case of an acute hemolytic transfusion reaction attributable to anti-Dia in a patient with a negative antibody detection test. A 22-year-old female patient with a diagnosis of β thalassemia and sickle cell anemia commenced a routine exchange transfusion of 5 units of red blood cells (RBCs) in the apheresis unit as part of her regular treatment...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28425257/role-of-hygienic-factors-in-the-etiology-of-allergic-disorders-in-children
#8
Şule Yeşil, Ahmet Kan, Olkar Abdulmajed, Arzu Bakirtaş, Nedim Sultan, Sadık M Demirsoy
BACKGROUND/AIM: We investigated the role of body flora and chronic inflammatory infections in the etiology of allergic disorders in Turkish children. MATERIALS AND METHODS: Forty pediatric asthma patients with positive skin prick tests and 40 age-matched healthy subjects with negative skin prick tests were enrolled in this cross-sectional study. Serum H. pylori IgG, viral hepatitis serology, IL-10, and TGF-beta levels were measured. Stool and throat cultures were taken and tested for occurrence of microorganisms...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28424685/analysis-of-linear-antibody-epitopes-on-factor-h-and-cfhr1-using-sera-of-patients-with-autoimmune-atypical-hemolytic-uremic-syndrome
#9
Eszter Trojnár, Mihály Józsi, Katalin Uray, Dorottya Csuka, Ágnes Szilágyi, Danko Milosevic, Vesna D Stojanović, Brankica Spasojević, Krisztina Rusai, Thomas Müller, Klaus Arbeiter, Kata Kelen, Attila J Szabó, György S Reusz, Satu Hyvärinen, T Sakari Jokiranta, Zoltán Prohászka
INTRODUCTION: In autoimmune atypical hemolytic uremic syndrome (aHUS), the complement regulator factor H (FH) is blocked by FH autoantibodies, while 90% of the patients carry a homozygous deletion of its homolog complement FH-related protein 1 (CFHR1). The functional consequence of FH-blockade is widely established; however, the molecular basis of autoantibody binding and the role of CFHR1 deficiency in disease pathogenesis are still unknown. We performed epitope mapping of FH to provide structural insight in the autoantibody recruitment on FH and potentially CFHR1...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28421309/genetic-makeup-of-shiga-toxin-producing-escherichia-coli-in-relation-to-clinical-symptoms-and-duration-of-shedding-a-microarray-analysis-of-isolates-from-swedish-children
#10
A Matussek, C Jernberg, I-M Einemo, S Monecke, R Ehricht, I Engelmann, S Löfgren, S Mernelius
Shiga toxin (Stx)-producing Escherichia coli (STECs) cause non-bloody diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome, and are the primary cause of acute renal failure in children worldwide. This study investigated the correlation of genetic makeup of STEC strains as revealed by DNA microarray to clinical symptoms and the duration of STEC shedding. All STEC isolated (n = 96) from patients <10 years of age in Jönköping County, Sweden from 2003 to 2015 were included. Isolates were characterized by DNA microarray, including almost 280 genes...
April 19, 2017: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/28420988/heme-as-a-target-for-therapeutic-interventions
#11
REVIEW
Stephan Immenschuh, Vijith Vijayan, Sabina Janciauskiene, Faikah Gueler
Heme is a complex of iron and the tetrapyrrole protoporphyrin IX with essential functions in aerobic organisms. Heme is the prosthetic group of hemoproteins such as hemoglobin and myoglobin, which are crucial for reversible oxygen binding and transport. By contrast, high levels of free heme, which may occur in various pathophysiological conditions, are toxic via pro-oxidant, pro-inflammatory and cytotoxic effects. The toxicity of heme plays a major role for the pathogenesis of prototypical hemolytic disorders including sickle cell disease and malaria...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28419995/hemolytic-uremic-syndrome-and-kidney-transplantation-a-case-series-and-review-of-the-literature
#12
Sabrina Milan Manani, Grazia Maria Virzì, Anna Giuliani, Anna Clementi, Alessandra Brocca, Daniela Dissegna, Francesca Martino, Emanuele Stefano Giovanni d'Amore, Claudio Ronco
BACKGROUND: Hemolytic uremic syndrome (HUS) can be triggered by Shiga toxin producing Escherichia coli (STEC) infection or it can be defined as atypical HUS (aHUS) if it is related to uncontrolled complement activation. aHUS is characterized by a high incidence of recurrence after kidney transplantation, and it can also occur de novo in transplant recipients. Eculizumab is used both to prevent and to treat aHUS following kidney transplantation. In this paper, we report our centre experience and we present 4 cases of HUS in patients who underwent kidney transplantation...
April 19, 2017: Nephron
https://www.readbyqxmd.com/read/28417547/a-long-term-follow-up-study-of-subtotal-splenectomy-in-children-with-hereditary-spherocytosis
#13
C W K Rosman, P M A Broens, M Trzpis, R Y J Tamminga
BACKGROUND: Hereditary spherocytosis (HS) is a heterogeneous hemolytic anemia treated with splenectomy in patients suffering from severe or moderate disease. Total splenectomy, however, renders patients vulnerable to overwhelming postsplenectomy infection despite preventive measures. Although subtotal splenectomy has been advocated as an alternative to total splenectomy, long-term follow-up data are scarce. We investigated how often hematologic recurrences requiring secondary total splenectomy occurred...
April 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28417064/adenocarcinoma-of-the-lung-presenting-as-thrombotic-thrombocytopenic-purpura
#14
Ahmed Dirweesh, Waqas Siddiqui, Muhammad Khan, Praneet Iyer, Marc Seelagy
Acquired thrombotic thrombocytopenic purpura (TTP) usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. It occurs in approximately three in one million adults and 1 in 10 million children annually. The incidence is increased in females and blacks. Diagnosing TTP and initiating therapy with plasma exchange is a must to avoid preventable complications. Acquired thromotic microangiopathy has been linked to collagen vascular diseases, use of certain medications, organ transplants, infections, pregnancy and cancer...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#15
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416508/hus-and-atypical-hus
#16
T Sakari Jokiranta
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by shiga-toxin producing E. coli (STEC) infection, atypical HUS (aHUS), usually caused by uncontrolled complement activation, or secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical (i.e. STEC-HUS) follows a gastrointestinal infection with STEC, while aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#17
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416183/inhibition-of-the-in-vitro-growth-of-babesia-bigemina-babesia-caballi-and-theileria-equi-parasites-by-trifluralin-analogues
#18
Marta G Silva, Donald P Knowles, Sandra Antunes, Ana Domingos, Maria A Esteves, Carlos E Suarez
Bovine and equine babesiosis caused by Babesia bovis, Babesia bigemina and Babesia caballi, along with equine theileriosis caused by Theileria equi are global tick-borne hemoprotozoan diseases characterized by fever, anemia, weight losses and abortions. A common feature of these diseases are transition from acute to chronic phases, in which parasites may persist in the hosts for life. Antiprotozoal drugs are important for managing infection and disease. Previous research demonstrated that trifluralin analogues, designated (TFLAs) 1-15, which specifically bind to regions of alpha-tubulin protein in plants and protozoan parasites, have the ability to inhibit the in vitro growth of B...
April 10, 2017: Ticks and Tick-borne Diseases
https://www.readbyqxmd.com/read/28414687/disseminated-cryptococcal-disease-with-diffuse-pulmonary-infiltrates-in-a-non-hiv-host
#19
M Hughes, K Trivedi, M Rudrappa
INTRODUCTION: We present a case of disseminated Cryptococcus in a non-HIV host, where the primary manifestation was pulmonary with diffuse pulmonary infiltrates. This patient was on high dose corticosteroids for autoimmune hemolytic anemia. CASE: A 79 year old Caucasian man with a history of autoimmune hemolytic anemia on 100 mg of prednisone daily, coronary artery disease s/p bypass surgery, ischemic cardiomyopathy, chronic obstructive pulmonary disease, sleep apnea, chronic kidney disease, and history of bilateral pulmonary emboli presented to Hematology/Oncology clinic with symptoms of productive cough, worsening shortness of breath, hemoptysis...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28409391/anti-inflammatory-activity-of-the-functional-groups-present-in-hippophae-rhamnoides-seabuckthorn-leaf-extract
#20
Himanshi Tanwar, Shweta, M Karthik Ananth, Divya Singh, S Narsimhan, Shashi Bala Singh, Lilly Ganju
ETHNO PHARMACOLOGICAL RELEVANCE: The study explores the anti-inflammatory activity of components present in fractions obtained from leaves of Hippophae rhamnoides in mouse peritoneal macrophages. AIM OF THE STUDY: Immunomodulators salvage the immune response by enhancing or reducing its capacity to the required level. Plant extracts are extensively used as immunomodulators because of their easy availability, simple methods of preparation and minimum side effects with maximum efficacy...
April 13, 2017: Inflammopharmacology
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