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Neonatal hemolytic

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https://www.readbyqxmd.com/read/28633864/perinatal-group-b-streptococcal-infections-virulence-factors-immunity-and-prevention-strategies
#1
REVIEW
Jay Vornhagen, Kristina M Adams Waldorf, Lakshmi Rajagopal
Group B streptococcus (GBS) or Streptococcus agalactiae is a β-hemolytic, Gram-positive bacterium that is a leading cause of neonatal infections. GBS commonly colonizes the lower gastrointestinal and genital tracts and, during pregnancy, neonates are at risk of infection. Although intrapartum antibiotic prophylaxis during labor and delivery has decreased the incidence of early-onset neonatal infection, these measures do not prevent ascending infection that can occur earlier in pregnancy leading to preterm births, stillbirths, or late-onset neonatal infections...
June 17, 2017: Trends in Microbiology
https://www.readbyqxmd.com/read/28583873/a-trade-off-between-catalytic-activity-and-protein-stability-determines-the-clinical-manifestations-of-glucose-6-phosphate-dehydrogenase-g6pd-deficiency
#2
Usa Boonyuen, Kamonwan Chamchoy, Thitiluck Swangsri, Thanyaphorn Junkree, Nicholas P J Day, Nicholas J White, Mallika Imwong
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common polymorphism and enzymopathy in humans, affecting approximately 400 million people worldwide. It is responsible for various clinical manifestations, including favism, hemolytic anemia, chronic non-spherocytic hemolytic anemia, spontaneous abortion, and neonatal hyperbilirubinemia. Understanding the molecular mechanisms underlying the severity of G6PD deficiency is of great importance but that of many G6PD variants are still unknown. In this study, we report the construction, expression, purification, and biochemical characterization in terms of kinetic properties and stability of five clinical G6PD variants-G6PD Bangkok, G6PD Bangkok noi, G6PD Songklanagarind, G6PD Canton+Bangkok noi, and G6PD Union+Viangchan...
June 3, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28580721/persistent-hemolytic-disease-of-the-fetus-and-newborn-hdfn-associated-with-passive-acquisition-of-anti-d-in-maternal-breast-milk
#3
Marissa Li, John C Blaustein
BACKGROUND: Anti-D is a well-documented, significant cause of hemolytic disease of the fetus and newborn (HDFN), but its presence in breast milk is not routinely described. Theoretically, breast milk containing anti-D could have the potential to exacerbate HDFN if ingested by the affected infant. STUDY DESIGN AND METHODS: This is a case report of a 28-week premature male neonate with hydrops fetalis born to a 32-year-old woman (gravidity 3/parity 3) with anti-D and anti-G...
June 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28571779/clinical-and-molecular-characterization-of-6-children-with-glutamate-cysteine-ligase-deficiency-causing-hemolytic-anemia
#4
Fatima Almusafri, Hiba E Elamin, Tamam E Khalaf, Alaa Ali, Tawfeg Ben-Omran, Ayman W El-Hattab
Glutathione (gamma-glutamylcysteinylglycine) has diverse functions including free radicals scavenging and modulating many critical cellular processes. Glutathione is synthesized by the consecutive action of the enzymes glutamate-cysteine ligase (GCL) and glutathione synthetase. GCL is composed of a catalytic subunit encoded by the GCLC gene and a regulatory subunit encoded by the GCLM gene. GCL deficiency due to homozygous mutations in GCLC has been reported in 6 individuals from 4 independent families. All presented with hemolytic anemia and 4 had additional neurological manifestations including cognitive impairment, neuropathy, ataxia, and myopathy...
June 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28503958/neonatal-management-and-outcome-in-alloimmune-hemolytic-disease
#5
Isabelle M C Ree, Vivianne E H J Smits-Wintjens, Johanna G van der Bom, Jeanine M M van Klink, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management...
June 5, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#6
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28412685/tcm-comprehensive-nursing-intervention-to-pathological-jaundice-on-neonate
#7
Jie Li, Weihua Chen, Guihua Xu
Pathological Jaundice on neonate differs from that of physiologic jaundice. They perform differently on clinical cases, which is an effective way to distinguish them. There are many causes for pathological jaundice, common ones include hemolytic jaundice, infectious jaundice, obstructive jaundice, breast-feeding jaundice, etc. Clinically proven that pathological jaundices has significant differences in symptoms. This brings problems to its treatment and nursing. In recent years, TCM comprehensive nursing shows great results in treating process...
November 2016: Pakistan Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28357148/anti-k1-kell-antibody-expressed-in-maternal-breastmilk-a-case-report-of-a-neonate-with-multiple-intrauterine-transfusions-and-postnatal-exposure-to-kell-antibody-in-maternal-breastmilk
#8
Patrick DeMoss, Mohamed Asfour, Kelly Hersey
Hemolytic disease of the fetus and newborn is a common consideration in newborn medicine, especially among the jaundiced. Maternal breastmilk provides numerous benefits to the infant, including nutrition and immunologic factors. Here, we present an infant who received three intrauterine transfusions for anemia secondary to anti-K1 (Kell), anti-C, and anti-e antibodies and whose maternal breastmilk tested positive for anti-Kell antibodies. The infant required another transfusion at 4 weeks of life for anemia...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28349448/neonatal-onset-hereditary-coproporphyria-a-new-variant-of-hereditary-coproporphyria
#9
Kosei Hasegawa, Hiroyuki Tanaka, Miho Yamashita, Yousuke Higuchi, Takayuki Miyai, Junko Yoshimoto, Ayumi Okada, Norihiro Suzuki, Keiji Iwatsuki, Hirokazu Tsukahara
Genetic mutation of the coproporphyrinogen oxidase (CPOX) gene causes either hereditary coproporphyria (HCP) or harderoporphyria. HCP, a rare hepatic porphyria, causes acute attacks after puberty and rarely accompanies cutaneous symptoms. In contrast, harderoporphyria is an erythropoietic porphyria that represents photosensitivity and hemolytic anemia from the neonatal period. In patients with harderoporphyria, the p.Lys404Glu mutation is found in the homozygous or compound heterozygous state with another mutation, and a marked increase in harderoporphyrin is observed...
March 28, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28283555/developmental-genetic-dietary-and-xenobiotic-influences-on-neonatal-hyperbilirubinemia
#10
REVIEW
Mei-Fei Yueh, Shujuan Chen, Nghia Nguyen, Robert H Tukey
Hyperbilirubinemia, caused by the accumulation of unconjugated bilirubin, is one of the most common clinical diagnoses in both premature and term newborns. Owing to the fact that bilirubin is metabolized solely through glucuronidation by UDP-glucuronosyltransferase (UGT) 1A1, it is now known that immaturity of UGT1A1, in combination with the overproduction of bilirubin during the developmental stage, acts as a bottleneck to bilirubin elimination and predisposes the infant to high total serum bilirubin levels...
May 2017: Molecular Pharmacology
https://www.readbyqxmd.com/read/28275334/clinical-significance-of-an-alloantibody-against-the-kell-blood-group-glycoprotein
#11
Stella Maris Mattaloni, Carine Arnoni, Rosario Céspedes, Claudia Nonaka, Carolina Trucco Boggione, Melina Eliana Luján Brajovich, Andrea Trejo, Néstor Zani, Claudia Silvia Biondi, Lilian Castilho, Carlos Miquel Cotorruelo
BACKGROUND: Kell null (K0) individuals can produce anti-Ku, an antibody against many epitopes in the Kell glycoprotein, after transfusion and/or pregnancy. Since sensitized K0 patients are rare, little is known about anti-Ku clinical relevance and in particular about its association to hemolytic disease of the fetus and newborn. CASE REPORT: This work describes a case of neonatal hyperbilirubinemia due to immune-mediated erythrocyte destruction by an alloantibody directed against the Kell glycoprotein...
January 2017: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/28267090/a-rare-cause-of-neonatal-hemolytic-anemia-glutathione-synthetase-deficiency
#12
Pembe Soylu Ustkoyuncu, Fatma Türkan Mutlu, Aslihan Kiraz, Zuhal Tag Balkis, Sibel Yel
BACKGROUND: Isolated hemolysis or hemolytic anemia and 5-oxoprolinuria are 2 distinct medical conditions in the clinical spectrum associated with glutathione synthetase deficiency. CLINICAL OBSERVATION: A 1-day-old female baby presented with anemia and respiratory distress. Her hemoglobin level was 9.5 g/dL and the total serum bilirubin level was 5.6 mg/dL. Metabolic acidosis was detected in her blood gas analysis. Metabolic acidosis recurred despite treatment and further investigation was required...
March 6, 2017: Journal of Pediatric Hematology/oncology
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
#13
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
#14
MULTICENTER STUDY
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...
March 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
#15
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
#16
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...
April 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28099134/carboxyhemoglobin-the-forgotten-parameter-of-neonatal-hyperbilirubinemia
#17
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
#18
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
#19
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
#20
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
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