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Neonatal hemolytic

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https://www.readbyqxmd.com/read/28223188/next-generation-sequencing-unravels-homozygous-mutation-in-glucose-6-phosphate-isomerase-gpic-1040g-a-p-arg347his-causing-hemolysis-in-an-indian-infant
#1
Manu Jamwal, Anu Aggarwal, Anirban Das, Arindam Maitra, Prashant Sharma, Shekhar Krishnan, Neeraj Arora, Deepak Bansal, Reena Das
INTRODUCTION: Inherited anemias diagnostic workup requires a step-wise algorithm. Causal genes implicated in congenital hemolytic anemia are numerous, making a gene-by-gene approach by Sanger sequencing time consuming, expensive and labour intensive. Targeted resequencing can be of great use in explaining these cases. METHODOLOGY: Six months female presented with neonatal jaundice and negative family history. Clinical and laboratory evidences were suggestive of hemolytic anemia...
February 20, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28164304/blood-group-antigen-matching-influence-on-gestational-outcomes-amigo-study
#2
Meghan Delaney, Agneta Wikman, Leo van de Watering, Henk Schonewille, Jennie P Verdoes, Stephen P Emery, Michael F Murphy, Julie Staves, Susanne Flach, Donald M Arnold, Richard M Kaufman, Alyssa Ziman, Sarah K Harm, Mark Fung, Catherine S Eppes, Nancy M Dunbar, Andreas Buser, Erin Meyer, Helen Savoia, Padmakumari Abeysinghe, Nancy Heddle, Alan Tinmouth, Aicha N Traore, Mark H Yazer
BACKGROUND: Red blood cell (RBC) antigen matching policies to prevent alloimmunization in females of childbearing potential (FCP) vary between centers. To inform transfusion centers responsible for making decisions about matching policies for FCPs, the causal stimulus of the antibodies implicated in severe hemolytic disease of the fetus and newborn (HDFN) must be determined. STUDY DESIGN AND METHODS: We conducted a multinational retrospective study of women with offspring affected by severe HDFN requiring neonatal exchange transfusion and/or intrauterine transfusion...
February 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28134751/efficacy-of-urtoxazumab-tma-15-humanized-monoclonal-antibody-specific-for-shiga-toxin-2-against-post-diarrheal-neurological-sequelae-caused-by-escherichia-coli-o157-h7-infection-in-the-neonatal-gnotobiotic-piglet-model
#3
Rodney A Moxley, David H Francis, Mizuho Tamura, David B Marx, Kristina Santiago-Mateo, Mojun Zhao
Enterohemorrhagic Escherichia coli (EHEC) is the most common cause of hemorrhagic colitis and hemolytic uremic syndrome in human patients, with brain damage and dysfunction the main cause of acute death. We evaluated the efficacy of urtoxazumab (TMA-15, Teijin Pharma Limited), a humanized monoclonal antibody against Shiga toxin (Stx) 2 for the prevention of brain damage, dysfunction, and death in a piglet EHEC infection model. Forty-five neonatal gnotobiotic piglets were inoculated orally with 3 × 10⁸ colony-forming units of EHEC O157:H7 strain EDL933 (Stx1⁺, Stx2⁺) when 22-24 h old...
January 26, 2017: Toxins
https://www.readbyqxmd.com/read/28133914/molecular-basis-of-pyruvate-kinase-deficiency-among-tunisians-description-of-new-mutations-affecting-coding-and-noncoding-regions-in-the-pklr-gene
#4
M Jaouani, L Manco, M Kalai, L Chaouch, K Douzi, A Silva, S Macedo, I Darragi, I Boudriga, D Chaouachi, Z Fitouri, R Van Wijk, M L Ribeiro, S Abbes
INTRODUCTION: Pyruvate kinase (PK) deficiency is one of the most common hereditary nonspherocytic hemolytic anemias worldwide with clinical manifestations ranging from mild to severe hemolysis. However, investigation of this enzymopathy is lacking in Tunisia. We report here a pioneer investigation of PK deficiency among Tunisian cases referred to our laboratory for biological analysis of unknown cause of hemolytic anemia. METHODS: Two hundred and fifty-three patients with unknown cause of hemolytic anemia have been addressed to our laboratory in order to investigate for red blood cells genetic disorders...
January 30, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28099134/carboxyhemoglobin-the-forgotten-parameter-of-neonatal-hyperbilirubinemia
#5
Douggl G N Bailey, Hans Fuchs, Roland Hentschel
BACKGROUND: Neonatal hyperbilirubinemia is influenced by a wide variety of factors, one of which is hemolysis. Serious hyperbilirubinemia may lead to a kernicterus with detrimental neurologic sequelae. Patients suffering from hemolytic disease have a higher risk of developing kernicterus. Carbon monoxide (CO), a byproduct of hemolysis or heme degradation, was described by Sjöstrand in the 1960s. It is transported as carboxyhemoglobin (COHb) and exhaled through the lungs. We were interested in a potential correlation between COHb and total serum bilirubin (TSB) and the time course of both parameters...
January 18, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28090778/three-novel-spectrin-variants-in-jaundiced-neonates
#6
Robert D Christensen, Archana M Agarwal, Hassan M Yaish, N Scott Reading, Elizabeth A O'Brien, Josef T Prchal
Various mutations in the genes encoding alpha spectrin (SPTA1) or beta spectrin (SPTB) are known to cause erythrocyte membrane disorders, sometimes associated with severe neonatal jaundice and anemia. We used a next-generation sequencing panel to evaluate 3 unrelated neonates who had puzzling cases of nonimmune hemolytic jaundice. In each case, we identified novel mutations in either SPTA1 or SPTB. Correlating erythrocyte morphology, clinical course, and computational analysis, we submit that each of the 3 variants is a probable pathogenic cause of the hereditary hemolytic conditions in these patients...
January 1, 2017: Clinical Pediatrics
https://www.readbyqxmd.com/read/28076300/usefulness-of-maternal-red-cell-antibodies-to-predict-hemolytic-disease-of-the-fetus-and-newborn-and-significant-neonatal-hyperbilirubinemia-a-retrospective-study
#7
Bart Peeters, Inge Geerts, Anne-Mie Badts, Veroniek Saegeman, Jan Moerman
No abstract text is available yet for this article.
January 11, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28024505/-comparison-of-therapeutic-efficacy-for-neonatal-abo-hemolytic-disease-treated-with-intravenous-immunoglobin-g-by-different-modes-of-administration
#8
Yun-Feng Liu, Chao-Chun Zou, Hua-Qin Yang, Li-Jiang Lou
OBJECTIVE: To compare the therapeutic efficacy of patients with neonatal ABO hemolytic disease treated with introvenous immunoglobin G (IVIG) by different modes of administration. METHODS: Ninety-three in patients with neonatal ABO hemolytic disease treated in our hospital were divided into group A (31 cases), B(31 cases) and C (31 cases). Based on basic treatment, the patients in group A were treated by a single high dose of IVIG (1 g/kg), patients in group B were treated by multiple low-dose of IVIG (0...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27995496/neuroprotective-effect-of-erythropoietin-on-phenylhydrazine-induced-hemolytic-hyperbilirubinemia-in-neonatal-rats
#9
Asli Memisoglu, Meltem Kolgazi, Akan Yaman, Elif Bahadir, Serap Sirvanci, Berrak Ç Yeğen, Eren Ozek
Neonatal unconjugated hyperbilirubinemia might cause severe bilirubin neurotoxicity in especially hemolytic conditions. The study aimed to elucidate the potential neuroprotective effects of erythropoietin (EPO) in hemolysis-induced hyperbilirubinemia. In newborn rats, hyperbilirubinemia secondary to hemolysis was induced by injecting with phenylhydrazine hydrochloride (PHZ) and rats were injected with either vehicle or EPO. At 54th hour of the PHZ injection, rats were decapitated. Serum levels of TNF-α, IL-1β, IL-10, brain-derived neurotrophic factor (BDNF) and S100-B and brain malondialdehyde, glutathione levels and myeloperoxidase activities were measured...
December 19, 2016: Neurochemical Research
https://www.readbyqxmd.com/read/27994529/anti-d-antibodies-in-pregnant-d-variant-antigen-carriers-initially-typed-as-rhd
#10
Jelena Lukacevic Krstic, Slavica Dajak, Jasna Bingulac-Popovic, Vesna Dogic, Jela Mratinovic-Mikulandra
BACKGROUND: To evaluate the incidence, the consequences, and the prevention strategy of anti-D alloimmunizations of D variant carriers in the obstetric population of Split-Dalmatia County, Croatia. METHODS: RhD immunization events were evaluated retrospectively for the period between 1993 and 2012. Women were tested for RhD antigen and irregular antibodies. Those with anti-D antibody who were not serologically D- were genotyped for RHD. They were evaluated for their obstetric and transfusion history and their titer of anti-D...
November 2016: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/27980749/glucose-6-phosphate-dehydrogenase-deficiency-not-exclusively-in-males
#11
Leonie van den Broek, Evelien Heylen, Machiel van den Akker
Glucose-6-phosphate (G6PD) deficiency is the most common human enzyme defect, often presenting with neonatal jaundice and/or acute hemolytic anemia, triggered by oxidizing agents. G6PD deficiency is an X-linked, hereditary disease, mainly affecting men, but should also be considered in females with an oxidative hemolysis.
December 2016: Clinical Case Reports
https://www.readbyqxmd.com/read/27979672/-neonatal-expression-of-beta-thalassemia-trait-associated-with-hereditary-spherocytosis-in-two-monozygotic-twins
#12
H Ben Hamouda, B Mahjoub, H Soua, S Laradi, A Miled, M T Sfar
The beta-thalassemia trait is a heterozygous beta-thalassemia characterized by a partial deficiency of the synthesis of beta-globin chains of hemoglobin. It is usually asymptomatic and the diagnosis is often made on the occasion of the completion of a systematic blood count or a family survey. Clinical expression during the neonatal period is impossible and its association with hereditary spherocytosis is common. We report two monozygotic twins who, on the 3rd day of life, presented intense jaundice, unconjugated bilirubin associated with anemia, without hepatosplenomegaly...
February 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27978809/antibacterial-combination-of-oleoresin-from-copaifera-multijuga-hayne-and-biogenic-silver-nanoparticles-towards-streptococcus-agalactiae
#13
E S Otaguiri, Aeb Morguette, R P Biasi-Garbin, A T Morey, Cac Lancheros, D Kian, A G Oliveira, G Kerbauy, Mre Perugini, N Durán, C V Nakamura, V F Veiga Junior, G Nakazato, P Pinge-Filho, L M Yamauchi, S F Yamada-Ogatta
BACKGROUND: Streptococcus agalactiae (group B Streptococcus - GBS) remains a leading cause of neonatal infections and an important cause of invasive infections in adults with underlying conditions. METHODS: This study evaluated for the first time the effect of an oleoresin collected from Copaifera multijuga Hayne (copaiba oil) alone or in combination with silver nanoparticles produced by green synthesis using Fusarium oxysporum (AgNPbio) against planktonic and sessile cells of GBS isolated from colonized women...
December 13, 2016: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/27941691/glucose-6-phosphate-dehydrogenase-update-and-analysis-of-new-mutations-around-the-world
#14
REVIEW
Saúl Gómez-Manzo, Jaime Marcial-Quino, America Vanoye-Carlo, Hugo Serrano-Posada, Daniel Ortega-Cuellar, Abigail González-Valdez, Rosa Angélica Castillo-Rodríguez, Beatriz Hernández-Ochoa, Edgar Sierra-Palacios, Eduardo Rodríguez-Bustamante, Roberto Arreguin-Espinosa
Glucose-6-phosphate dehydrogenase (G6PD) is a key regulatory enzyme in the pentose phosphate pathway which produces nicotinamide adenine dinucleotide phosphate (NADPH) to maintain an adequate reducing environment in the cells and is especially important in red blood cells (RBC). Given its central role in the regulation of redox state, it is understandable that mutations in the gene encoding G6PD can cause deficiency of the protein activity leading to clinical manifestations such as neonatal jaundice and acute hemolytic anemia...
December 9, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27933591/charcoal-hemoperfusion-in-the-treatment-of-medically-refractory-pruritus-in-cholestatic-liver-disease
#15
Wonngarm Kittanamongkolchai, Ziad M El-Zoghby, J Eileen Hay, Russell H Wiesner, Patrick S Kamath, Nicholas F LaRusso, Kymberly D Watt, Carl H Cramer, Nelson Leung
BACKGROUND: Pruritus is a distressing symptom in a considerable proportion of cholestatic patients and a few of them do not respond to conventional treatment. Charcoal hemoperfusion (CH) is an extracorporeal technique that is effective in eliminating protein-bound substances which may have accumulated during cholestasis. Several case reports have shown significant reduction of bilirubin in mechanical jaundice and neonatal hemolytic jaundice. However, the published data of CH for the treatment of refractory pruritus in cholestatic patients are scarce...
December 8, 2016: Hepatology International
https://www.readbyqxmd.com/read/27861255/neonatal-nonimmune-hemolytic-anemia
#16
Hassan M Yaish, Robert D Christensen, Richard S Lemons
PURPOSE OF REVIEW: As in adults and older children, anemia in newborn infants can be the result of erythropoietic failure, hemorrhage, or hemolysis. When hemolysis is the prime consideration, it can be challenging for physicians caring for neonates to choose from the wide variety of available diagnostic tests. This review describes the authors' opinions regarding rational, consistent, and cost-effective means of making an exact diagnosis of a neonatal hemolytic condition. RECENT FINDINGS: Two recent advances in the diagnosis of neonatal nonimmune hemolytic disorders are highlighted in this review: introduction of flow cytometry-based Eosin-5-maleimide (EMA) uptake as a screening test to identify RBC membrane defects and next-generation sequencing (NGS)-based panels to uncover exact mutations causing hemolysis...
November 16, 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27819066/group-b-streptococcus-circumvents-neutrophils-and-neutrophil-extracellular-traps-during-amniotic-cavity-invasion-and-preterm-labor
#17
Erica Boldenow, Claire Gendrin, Lisa Ngo, Craig Bierle, Jay Vornhagen, Michelle Coleman, Sean Merillat, Blair Armistead, Christopher Whidbey, Varchita Alishetti, Veronica Santana-Ufret, Jason Ogle, Michael Gough, Sengkeo Srinouanprachanh, James W MacDonald, Theo K Bammler, Aasthaa Bansal, H Denny Liggitt, Lakshmi Rajagopal, Kristina M Adams Waldorf
Preterm birth is a leading cause of neonatal morbidity and mortality. Although microbial invasion of the amniotic cavity (MIAC) is associated with the majority of early preterm births, the temporal events that occur during MIAC and preterm labor are not known. Group B Streptococci (GBS) are β-hemolytic, gram-positive bacteria, which commonly colonize the vagina but have been recovered from the amniotic fluid in preterm birth cases. To understand temporal events that occur during MIAC, we utilized a unique chronically catheterized nonhuman primate model that closely emulates human pregnancy...
October 2016: Science Immunology
https://www.readbyqxmd.com/read/27807866/a-jordanian-family-with-three-sisters-apparently-homozygous-for-m-k-and-evidence-for-clinical-significance-of-antibodies-produced-by-m-k-m-k-individuals
#18
Nour Al-Huda Al-Jada
BACKGROUND: The rare M(k) M(k) phenotype is the result of a deletion of the coding regions of both GYPA and GYPB. Red blood cells (RBCs) of individuals homozygous for the rare M(k) gene lack all MNS blood group antigens and have no glycophorin A or glycophorin B. This phenotype is extremely rare and only four families have been reported. CASE REPORT: A 28-year-old woman was referred for assessment of recurrent early neonatal deaths. She was found to be apparently homozygous for M(k) ...
November 3, 2016: Transfusion
https://www.readbyqxmd.com/read/27746217/a-new-rat-model-of-neonatal-bilirubin-encephalopathy-kernicterus
#19
Naser Amini, Nasim Vousooghi, Mansoureh Soleimani, Ali Samadikuchaksaraei, Mehdi Akbari, Hosein Safakheil, Pezhman Atafimanesh, Ali Shahbazi, Peiman Brouki Milan, Sara Ramezani, Masoud Mozafari, Mohammad Taghi Joghataei
INTRODUCTION: Hemolytic kernicterus, an indirect bilirubin-induced brain dysfunction, is associated with hyper-bilirubinemia in mammalian neonates. In this study, a new model of kernicterus has been developed using intra-peritoneal injections of phenyl hydrazine and subcutaneous injections of sulfisoxazole. These drugs can potentially induce kernicterus in neonatal through changes in hemolysis and hypo-albumin. METHODS: For this purpose, 7-day-old male Wistar rats (n=72; mean weight 11±1g) were used...
October 13, 2016: Journal of Pharmacological and Toxicological Methods
https://www.readbyqxmd.com/read/27735024/the-effect-of-immunoglobulin-treatment-for-hemolysis-on-the-incidence-of-necrotizing-enterocolitis-a-meta-analysis
#20
Y Yang, J-J Pan, X-G Zhou, X-Y Zhou, R Cheng, Y-H Hu
OBJECTIVE: The application of intravenous immune globulin (IVIG) has been recommended for treating hemolysis in neonates for several years. But in clinical work, more than one study reported that IVIG treatment maybe increased the risk of NEC in hemolytic patients. In light of this situation, we performed this meta-analysis. MATERIALS AND METHODS: We searched in PubMed, Embase, Cochrane databases for English references, and in Wanfang, VIP, Cnki databases for Chinese references (all last launched on 2015/12/18)...
September 2016: European Review for Medical and Pharmacological Sciences
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