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https://www.readbyqxmd.com/read/29738043/complement-activation-in-atypical-hemolytic-uremic-syndrome-and-scleroderma-renal-crisis-a-critical-analysis-of-pathophysiology
#1
Roman Zuckerman, Arif Asif, Eric J Costanzo, Tushar Vachharajani
Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the role of complement pathways in the development of atypical hemolytic uremic syndrome and scleroderma renal crisis. The abundant similarities in their presentation as well as the clinical course are raising the possibility of a common underlying pathogenesis. Recent reports are emphasizing that complement pathways appear to be the unifying link...
May 7, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29724898/how-i-safely-transfuse-patients-with-sickle-cell-disease-and-manage-delayed-hemolytic-transfusion-reactions
#2
France Pirenne, Karina Yazdanbakhsh
Transfusions can be a life-saving treatment for patients with sickle cell disease (SCD). However, availability of matched units can be limiting due to distinctive blood group polymorphisms in patients of African descent. Development of antibodies against the transfused red blood cells (RBCs), resulting in delayed hemolytic transfusion reactions (DHTRs), can be life-threatening and pose unique challenges for this population with regard to treatment strategies and transfusion management protocols. In cases where the transfused cells as well as patient's own RBCs are destroyed, diagnosis of DHTR can be difficult because symptoms may mimic vaso-occlusive crisis, and frequently antibodies are undetectable...
May 3, 2018: Blood
https://www.readbyqxmd.com/read/29590553/aps-sickle-cell-disease-conference-mini-review-targeting-pain-at-its-source-in-sickle-cell-disease
#3
Kanika Gupta, Om Jahagirdar, Kalpna Gupta
Sickle cell disease (SCD) is a genetic disorder associated with hemolytic anemia, end-organ damage, reduced survival and pain. One of the unique features of SCD is, recurrent and unpredictable episodes of acute pain due to vaso-occlusive crisis, requiring hospitalization. Additionally, SCD patients often develop chronic persistent pain. Currently, sickle pain is treated with opioids, an approach limited by adverse effects. Because pain can start at infancy and continue throughout life, preventing the genesis of pain may be relatively better than treating the pain once it has been evoked...
March 28, 2018: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
https://www.readbyqxmd.com/read/29551999/design-synthesis-and-evaluation-of-branched-rrwqwr-based-peptides-as-antibacterial-agents-against-clinically-relevant-gram-positive-and-gram-negative-pathogens
#4
Sandra C Vega, Diana A Martínez, María Del S Chalá, Hernán A Vargas, Jaiver E Rosas
Multidrug resistance of pathogenic bacteria has become a public health crisis that requires the urgent design of new antibacterial drugs such as antimicrobial peptides (AMPs). Seeking to obtain new, lactoferricin B (LfcinB)-based synthetic peptides as viable early-stage candidates for future development as AMPs against clinically relevant bacteria, we designed, synthesized and screened three new cationic peptides derived from bovine LfcinB. These peptides contain at least one RRWQWR motif and differ by the copy number (monomeric, dimeric or tetrameric) and structure (linear or branched) of this motif...
2018: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29540210/a-vegetable-induced-hemolytic-crisis-in-a-g6pd-deficient-person-a-case-report
#5
N D B Ehelepola, A N Abayagunawardana, T N Sudusinghe
BACKGROUND: Hemolysis can occur in people with G6PD deficiency under oxidative stress. Acalypha indica is a tropical plant used as a medicinal plant as well as a vegetable. There are a few reported cases of Acalypha indica ingestion induced hemolysis in G6PD deficient people. All except one of them are from Sri Lanka. The information available at present (2017) about G6PD deficiency prevalence and variants of the G6PD gene among Sri Lankans is very sparse. There are no past reports on hemolytic crisis in a G6PD deficient person presenting mimicking leptospirosis...
March 14, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29524230/transfusion-transmitted-malaria-masquerading-as-sickle-cell-crisis-with-multisystem-organ-failure
#6
Cheryl L Maier, Phillip J Gross, Christina L Dean, Satheesh Chonat, Andrew Ip, Morgan McLemore, Fuad El Rassi, Sean R Stowell, Cassandra D Josephson, Ross M Fasano
BACKGROUND: Fever accompanying vaso-occlusive crisis is a common presentation in patients with sickle cell disease (SCD) and carries a broad differential diagnosis. Here, we report a case of transfusion-transmitted malaria in a patient with SCD presenting with acute vaso-occlusive crisis and rapidly decompensating to multisystem organ failure (MSOF). CASE REPORT: An 18-year-old African American male with SCD was admitted after multiple days of fever and severe generalized body pain...
March 9, 2018: Transfusion
https://www.readbyqxmd.com/read/29473172/ceftriaxone-induced-drug-reaction-mimicking-acute-splenic-sequestration-crisis-in-a-child-with-hemoglobin-sc-disease
#7
Nancy L Van Buren, Jed B Gorlin, Robyn C Reed, Jerome L Gottschall, Stephen C Nelson
BACKGROUND: Acute splenic sequestration crisis is a complication of sickle cell disease (SCD) occurring when intrasplenic red blood cell (RBC) sickling prevents blood from leaving the spleen, causing acute splenic enlargement. Although typically seen in young children, it has been reported in older children with hemoglobin (Hb)SC disease, eventually resulting in functional asplenia. Ceftriaxone is a frequently used antibiotic of choice for children with SCD, because of its efficacy against invasive pneumococcal disease...
April 2018: Transfusion
https://www.readbyqxmd.com/read/29302103/-babesia-odocoilei-as-a-cause-of-mortality-in-captive-cervids-in-canada
#8
Amélie Mathieu, Adriana R Pastor, Charlene N Berkvens, Carolyn Gara-Boivin, Michel Hébert, Alexandre N Léveillé, John R Barta, Dale A Smith
Nine cases of fatal infection with Babesia odocoilei were confirmed in reindeer (Rangifer tarandus tarandus) and elk (Cervus canadensis) housed in zoological institutions located in southern Quebec, Ontario, and Manitoba, Canada between 2013 and 2016. All animals died of a hemolytic crisis. Frequent postmortem findings were extensive hemorrhage, pigmenturia, and intrahepatic cholestasis. The described ante- and postmortem signs are consistent with those of previously reported cases in the United States. Diagnosis was confirmed in all cases by polymerase chain reaction performed on DNA extracted from whole blood or frozen spleen...
January 2018: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/29248295/nota-analogue-a-first-dithiocarbamate-inhibitor-of-metallo-%C3%AE-lactamases
#9
En Zhang, Ming-Ming Wang, Shu-Chao Huang, Shuai-Min Xu, De-Yun Cui, Yuan-Li Bo, Peng-Yan Bai, Yong-Gang Hua, Chun-Ling Xiao, Shangshang Qin
The emergence of antibiotic drug (like carbapenem) resistance is being a global crisis. Among those resistance factors of the β-lactam antibiotics, the metallo-β-lactamases (MBLs) is one of the most important reasons. In this paper, a series of cyclic dithiocarbamate compounds were synthesized and their inhibition activities against MBLs were initially tested combined with meropenem (MEM) by in vitro antibacterial efficacy tests. Sodium 1,4,7-triazonane-1,4,7-tris(carboxylodithioate) (compound 5) was identified as the most active molecule to restore the activity of MEM...
January 15, 2018: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/29222291/evolving-treatment-paradigms-in-sickle-cell-disease
#10
REVIEW
Ramasamy Jagadeeswaran, Angela Rivers
Sickle cell disease (SCD) is an inheritable hemoglobinopathy characterized by polymerization of hemoglobin S in red blood cells resulting in chronic hemolytic anemia, vaso-occlusive painful crisis, and multiorgan damage. In SCD, an increased reactive oxygen species (ROS) generation occurs both inside the red blood cells and inside the vascular lumen, which augment hemolysis and cellular adhesion. This review discusses the evolving body of literature on the role of ROS in the pathophysiology of SCD as well as some emerging therapeutic approaches to SCD with a focus on the reduction of ROS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29174516/retracted-delayed-hemolytic-transfusion-reaction-and-hyperhemolysis-syndrome-without-detectable-alloantibodies-or-autoantibodies-in-a-patient-with-sickle-cell-disease-a-fatal-case-report-and-literature-review
#11
Basile Nsimba
L'éditeur a le regret de vous informer que cet article a déjà été publié dans: Journal of Blood Disorders & Transfusion, 2017, 8:4. DOI: 10.4172/2155-9864.1000388. Cette seconde publication faite par erreur a été retirée.
November 22, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28982343/study-of-glucose-6-phosphate-dehydrogenase-deficiency-5-years-retrospective-egyptian-study
#12
Adel A Hagag, Ibrahim M Badraia, Mohamed S Elfarargy, Mohamed M Abd Elmageed, Ehab A Abo-Ali
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide that causes a spectrum of diseases including neonatal hyperbilirubinemia, acute and chronic hemolysis after exposure to oxidative stress. AIM OF THE WORK: This five years retrospective study was carried out to study the demographic, clinical and laboratory data of 1000 patients with G6PD deficiency anemia registered in Hematology Unit, Pediatric Department, Tanta University Hospital...
February 13, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28924974/incidence-and-predictive-score-for-delayed-hemolytic-transfusion-reaction-in-adult-patients-with-sickle-cell-disease
#13
David Narbey, Anoosha Habibi, Philippe Chadebech, Armand Mekontso-Dessap, Mehdi Khellaf, Jean-Daniel Lelièvre, Bertrand Godeau, Marc Michel, Frédéric Galactéros, Rachid Djoudi, Pablo Bartolucci, France Pirenne
Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication of transfusion in sickle cell disease (SCD). The frequency of DHTR is underestimated because its symptoms mimic those of vaso-occlusive crisis and antibodies (Abs) are often not detectable. No predictive factors for identifying patients likely to develop DHTR have yet been defined. We conducted a prospective single-center observational study over 30 months in adult sickle cell patients. We included 694 transfusion episodes (TEs) in 311 patients, divided into occasional TEs (OTEs: 360) and chronic transfusion program (CTEs: 334)...
December 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28828525/maternal-consumption-of-quinine-containing-sodas-may-induce-g6pd-crises-in-breastfed-children
#14
S Bichali, D Brault, C Masserot, C Boscher, M L Couec, G Deslandes, S Pissard, G Leverger, C Vauzelle, E Elefant, J C Rozé, A Cortey, Alexis Chenouard
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect often presenting with neonatal jaundice and/or hemolytic anemia. G6PD hemolytic events are linked with exposure to a pro-oxidant agent. We here report three cases of initial G6PD crises in breastfed children secondary to maternal consumption of a tonic drink which contains quinine. Quinine was found in breast milk of one of the mothers after she consumed tonic water. CONCLUSION: The amount of quinine that is transmitted through breast milk appears to be sufficient to induce G6PD crises in breastfed children...
October 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28801086/slowly-progressive-leukodystrophy-in-an-adolescent-male-with-phosphoglycerate-kinase-deficiency
#15
Shimpei Baba, Ayumi Kobayashi, Haruna Yokoyama, Kengo Moriyama, Ayako Kashimada, Jun Oyama, Ayako Owada, Shoichi Oyama, Tomohiro Morio, Masatoshi Takagi
We report the case of an 18-year-old man with a phosphoglycerate kinase (PGK) deficiency who had slowly progressive leukodystrophy during adolescence. The patient had a history of severe neonatal jaundice, hemolytic crisis with rhabdomyolysis triggered by febrile viral infections, dysarthria, and intellectual disability during early childhood. Clumsiness in walking and writing became obvious at ∼10years of age. Evaluations performed by us on the 18-year-old patient confirmed the presence of pyramidal tract signs, increased muscle tone, and generalized dystonia...
February 2018: Brain & Development
https://www.readbyqxmd.com/read/28799315/glucose-6-phosphate-dehydrogenase-deficiency-unveiled-by-diabetic-ketoacidosis-a-dual-dilemma
#16
Pankti Mehta, Vishal Srivastav, Priya Bhate, Vishal Gupta, Milind Y Nadkar
An 18 year old male, known case of Type 1 Diabetes Mellitus was admitted in view of diabetic ketoacidosis. With normalization of blood sugars patient developed gross reddish discoloration of urine. Urine routine microscopy did not reveal RBCs or RBC casts. Peripheral blood smear revealed bite cells, Heinz bodies and spherocytes. Thus a diagnosis of hemolytic anemia with hemoglobinuria was made. Patient's glucose-6-phosphate dehydrogenase (G6PD) levels were below the normal range. G6PD, an enzyme of the HMP shunt, is the most common enzyme defect causing hemolytic anemia...
August 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28754279/-g6pd-deficiency-in-females-with-neonatal-revelation-report-of-four-cases
#17
A Renault, D Mitanchez, A Cortey
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human erythrocyte enzyme defect, estimated to affect approximately 4 million people worldwide. It is associated with severe neonatal hyperbilirubinemia, which may lead to bilirubin encephalopathy and kernicterus, and with hemolytic crisis. G6PD deficiency is an X-linked enzymopathy affecting hemizygous males, homozygous females, and also a subset of heterozygous females via chromosome X inactivation. We report four cases of female newborns with neonatal hyperbilirubinemia related to a G6PD deficiency and followed by the Centre national de référence en hémobiologie périnatale (CNRHP) from November 2013 to July 2014...
July 25, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28669521/management-of-delayed-hemolytic-transfusion-reaction-in-sickle-cell-disease-prevention-diagnosis-treatment
#18
F Pirenne, P Bartolucci, A Habibi
Transfusion remains a key treatment of sickle cell disease complications. However, delayed hemolytic transfusion reaction, the most serious complication of transfusion, may be life-threatening if hyperhemolysis develops. This syndrome is generally underdiagnosed because its biological and clinical features resemble those of vaso-occlusive crisis, and red blood cell antibodies are frequently absent. Further transfusions may aggravate the symptoms, leading to severe multiple organ failure and death. It is therefore essential to prevent, diagnose and treat this syndrome efficiently...
September 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28657766/a-suspected-delayed-hemolytic-transfusion-reaction-mediated-by-anti-joa
#19
Ryan P Jajosky, Wendy C Lumm, Scott C Wise, Roni J Bollag, James F Shikle
A 32-year-old African-American woman with a history of sickle cell disease presented for surgical evaluation of left total hip arthroplasty due to avascular necrosis of the femoral head. In anticipation of a complex orthopedic procedure, pre-surgical blood work was ordered. The patient's Fenwal blood sample typed as group O, D+. Although the patient had a history of anti-Fya, the antibody identification was inconclusive, so the workup was sent to a reference laboratory. The patient was last transfused with red blood cells (RBCs) 2 years earlier, but had no history of transfusion reactions...
June 2017: Immunohematology
https://www.readbyqxmd.com/read/28401102/human-parvovirus-b19-in-patients-with-beta-thalassemia-major-from-tehran-iran
#20
Seyed Ali Mohammad Arabzadeh, Farideh Alizadeh, Ahmad Tavakoli, Hamidreza Mollaei, Farah Bokharaei-Salim, Gharib Karimi, Mohammad Farahmand, Helya Sadat Mortazavi, Seyed Hamidreza Monavari
BACKGROUND: Due to the tropism of human parvovirus B19 to erythroid progenitor cells, infection in patients with an underlying hemolytic disorder such as beta-thalassemia major leads to suppression of erythrocyte formation, referred to as transient aplasia crisis (TAC), which may be life-threatening. We investigated the prevalence of parvovirus B19 among patients with beta thalassemia major attending the Zafar Adult Thalassemia Clinic in Tehran, Iran. METHODS: This cross-sectional study was performed to determine the presence of parvovirus B19 DNA in blood samples and parvovirus B19 genotypes in plasma samples of patients with thalassemia major...
March 2017: Blood Research
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