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Claire Gendrin, Sean Merillat, Jay Vornhagen, Michelle Coleman, Blair Armistead, Lisa Ngo, Anjali Aggarwal, Phoenicia Quach, Jacob Berrigan, Lakshmi Rajagopal
Group B streptococci (GBS) are encapsulated, β-hemolytic bacteria that are a common cause of infections in human newborns and certain adults. Two factors important for GBS virulence are the sialic acid capsular polysaccharide that promotes immune evasion and the hemolytic pigment that induces host cell cytotoxcity. These virulence factors are often oppositely regulated by the CovR/CovS two-component system. Clinical GBS strains exhibiting hyperhemolysis and low capsule due to pathoadaptive covR/S mutations have been isolated from patients...
March 13, 2018: Journal of Infectious Diseases
Maria I Harrell, Kellie Burnside, Christopher Whidbey, Jay Vornhagen, Kristina M Adams Waldorf, Lakshmi Rajagopal
Infection of the amniotic cavity remains a major cause of preterm birth, stillbirth, fetal injury and early onset, fulminant infections in newborns. Currently, there are no effective therapies to prevent in utero infection and consequent co-morbidities. This is in part due to the lack of feasible and appropriate animal models to understand mechanisms that lead to in utero infections. Use of mouse and rat models do not fully recapitulate human pregnancy, while pregnant nonhuman primate models are limited by ethical considerations, technical constraints, and cost...
September 2017: Journal of Infectious Diseases and Medicine
Marie Dubert, Jacques Elion, Aissata Tolo, Dapa Aly Diallo, Saliou Diop, Ibrahima Diagne, Ibrahima Sanogo, Suzanne Belinga, Odette Guifo, Guillaume Wamba, Françoise Ngo Sack, Kouakou Boidy, Ismael Kamara, Youssouf Traore, Cheick Oumar Diakite, Valérie Gbonon, Blaise Felix Faye, Moussa Seck, Indou Deme Ly, David Chelo, Roland N'Guetta, Ibrahima Bara Diop, Bamba Gaye, Xavier Jouven, Brigitte Ranque
The hyperhemolysis paradigm that describes overlapping "hyperhemolytic-endothelial dysfunction" and "high hemoglobin-hyperviscous" subphenotypes of sickle cell disease (SCD) patients is based on North American studies. We performed a transversal study nested in the CADRE cohort to analyze the association between steady-state hemolysis and vascular complications of SCD among sub-Saharan African patients. In Mali, Cameroon, and Ivory Coast, 2407 SCD patients (1751 SS or sickle β-zero-thalassemia [Sβ0 ], 495 SC, and 161 sickle β+ -thalassemia [Sβ+ ]), aged 3 years old and over, were included at steady state...
November 16, 2017: Blood
Zoe Bezirgiannidou, Anna Christoforidou, Eftychia Kontekaki, Athanasios G Anastasiadis, Spyros I Papamichos, Helen Menexidou, Dimitrios Margaritis, Georges Martinis, Elpis Mantadakis
BACKGROUND: Hyperhemolytic Syndrome or Hyperhemolytic Transfusion Reaction (HHTR), a life-threatening subset of Delayed Hemolytic Transfusion Reaction (DHTR) is characterized by destruction of both transfused and autologous erythrocytes evidenced by a fall in post transfusion hemoglobin below the pre-transfusion level. CASE REPORT: We describe a case of DHTR due to anti-P1 alloimmunization manifesting with hyperhemolysis in a 30-year-old Greek Pomak woman with thalassemia intermedia (HbO-Arab/β-thalassemia), during the11(th) week of her first gestation...
2016: Mediterranean Journal of Hematology and Infectious Diseases
Christopher Whidbey, Kellie Burnside, Raquel M Martinez, Claire Gendrin, Jay Vornhagen, Andrew Frando, Maria Isabel Harrell, Ryan McAdams, Lakshmi Rajagopal
Group B Streptococci (GBS) are ß-hemolytic, gram-positive bacteria that are typically associated with infections in human newborns or immunocompromised adults. However, mutation in the two-component regulator CovR/S relieves repression of hemolysin, potentially increasing virulence of GBS. We report the isolation of hyperhemolytic/hyperpigmented GBS strain from an adolescent patient who presented to the University of Washington clinic with symptoms of sore throat. While the patient also tested positive for mononucleosis, a GBS strain with increased hemolysis was isolated from the throat swab obtained from the patient...
2015: Clinical Research in Infectious Diseases
Claire Gendrin, Jay Vornhagen, Lisa Ngo, Christopher Whidbey, Erica Boldenow, Veronica Santana-Ufret, Morgan Clauson, Kellie Burnside, Dionne P Galloway, Kristina Adams Waldorf, Adrian M Piliponsky, Lakshmi Rajagopal
Ascending infection of microbes from the lower genital tract into the amniotic cavity increases the risk of preterm birth, stillbirth, and newborn infections. Host defenses that are critical for preventing ascending microbial infection are not completely understood. Group B Streptococcus (GBS) are Gram-positive bacteria that frequently colonize the lower genital tract of healthy women but cause severe infections during pregnancy, leading to preterm birth, stillbirth, or early-onset newborn infections. We recently described that the GBS pigment is hemolytic, and increased pigment expression promotes GBS penetration of human placenta...
July 17, 2015: Science Advances
Mark Boonyasampant, Ilene C Weitz, Brian Kay, Chaiyaporn Boonchalermvichian, Howard A Liebman, Ira A Shulman
BACKGROUND: Hyperhemolysis in sickle cell disease is a rare and potentially life-threatening complication of transfusion. STUDY DESIGN AND METHODS: In this article we report a case of delayed hemolytic transfusion reaction with resultant hyperhemolysis triggered by an anti-IH autoantibody with alloantibody behavior. RESULTS: The anti-IH was reactive at room temperature as well as 37 °C, but only weakly reactive with autologous red blood cells...
October 2015: Transfusion
Pablo Bartolucci, François Lionnet
Chronic complications of sickle cell disease take an increasing role in the management of patients due to their morbidity and mortality impact. The prevalence of chronic organ damages increases as the age of patients followed in France. Few organs seem unaffected by the disease. The natural history of chronic complication is highly variable from one patient to another, and the distribution of those manifestations throughout life, is different depending on their nature and pathophysiology. Thus we can, for example, distinguish SS patients presenting a "hyperhemolytic" phenotype associated with dense red blood cells that have a high risk of vascular complications including kidney disease, pulmonary hypertension, leg ulcers and priapism, from SC patients with high hemoglobin levels, who have a higher risk of retinopathy, osteonecrosis and sensory syndrome, probably related to hyperviscosity...
October 2014: La Revue du Praticien
Agnese Lupo, Corinne Ruppen, Andrew Hemphill, Barbara Spellerberg, Parham Sendi
Group B Streptococcus (GBS) causes invasive infections in neonates, older adults and patients with comorbidities. β-hemolysin/cytolysin is an important GBS virulence factor. It is encoded by the cyl operon and confers GBS hemolytic activity. Isolates displaying hyperpigmentation are typically hyperhemolytic. Comparison of clonally identical isolates displaying different levels of pigmentation has shown transcriptional dysregulation due to mutations in components of the control of the virulence S/R (CovS/R) regulatory system...
July 2014: International Journal of Medical Microbiology: IJMM
Christopher Whidbey, Maria Isabel Harrell, Kellie Burnside, Lisa Ngo, Alexis K Becraft, Lakshminarayan M Iyer, L Aravind, Jane Hitti, Kristina M Adams Waldorf, Lakshmi Rajagopal
Microbial infection of the amniotic fluid is a significant cause of fetal injury, preterm birth, and newborn infections. Group B Streptococcus (GBS) is an important human bacterial pathogen associated with preterm birth, fetal injury, and neonatal mortality. Although GBS has been isolated from amniotic fluid of women in preterm labor, mechanisms of in utero infection remain unknown. Previous studies indicated that GBS are unable to invade human amniotic epithelial cells (hAECs), which represent the last barrier to the amniotic cavity and fetus...
June 3, 2013: Journal of Experimental Medicine
Elena Aragona, Michael J Kelly
An 18-year-old female with sickle cell disease presented with thigh pain, dark urine, and hematuria within 72 hours of receiving a blood transfusion. Her clinical picture was consistent with hemolysis. Subsequent laboratory workup, however, demonstrated reticulocytopenia without evidence of an antibody-mediated transfusion reaction. As her hemoglobin continued to decrease, she was treated with IVIG and steroids for presumed hyperhemolysis. Clinicians should have a high index of suspicion for hyperhemolysis in sickle cell patients with evidence of hemolysis after a recent transfusion...
January 2014: Journal of Pediatric Hematology/oncology
Ingrid Prkacin, Jasna Mesarić, Gordana Cavrić, Silvana Jurenec, Zeljka Hundrić-Haspl, Ika Kardum-Skelin
Hyperhemolysis syndrome usually occurs in patients with sickle cell disease and possibly thalassemia who receive multiple transfusions. There are only few clinical reports on patients without hemoglobinopathies as in this report. Our patient was diagnosed with hyperhemolytic reaction and was infused with IVIG and methylprednisolone for several days. Signs of tissue hypoxia developed along with increased cardiac enzymes, hepatocellular and cerebrovascular injury, and finally death. On autopsy, there was no evidence for hemolytic uremic syndrome or thrombotic thrombocytopenic purpura...
September 2011: Acta Medica Croatica: C̆asopis Hravatske Akademije Medicinskih Znanosti
Masanori Hayashi, Agustin Calatroni, Brittany Herzberg, Allison K Ross, Henry E Rice, Courtney Thornburg
Hydroxyurea has enhanced the treatment for children with sickle cell anemia. The objectives of this study were to compare perioperative transfusions and outcomes for children taking hydroxyurea versus those not taking hydroxyurea. We retrospectively reviewed perioperative management and outcomes for 51 children with sickle cell anemia (HbSS genotype) who underwent surgery in our center between January 2003 and April 2008. Of the 51 patients, 30 (59%) were taking hydroxyurea and 21 (41%) were not taking hydroxyurea...
October 2011: Journal of Pediatric Hematology/oncology
Robert P Hebbel
The "hyperhemolytic paradigm" (HHP) posits that hemolysis in sickle disease sequentially and causally establishes increased cell-free plasma Hb, consumption of NO, a state of NO biodeficiency, endothelial dysfunction, and a high prevalence of pulmonary hypertension. The basic science underpinning this concept has added an important facet to the complexity of vascular pathobiology in sickle disease, and clinical research has identified worrisome clinical issues. However, this critique identifies and explains a number of significant concerns about the various HHP component tenets...
February 2011: American Journal of Hematology
Amr El-Husseini, Alaa Sabry
No abstract text is available yet for this article.
November 2010: American Journal of Emergency Medicine
Nay Win, Smita Sinha, Edmond Lee, Wendy Mills
Hyperhemolytic transfusion reaction (HHTR) is a serious and potentially life-threatening complication of red blood cell (RBC) transfusion and has been well described in patients with sickle cell disease (SCD) and non-SCD patients. Awareness of this condition is important because subsequent transfusion may exacerbate hemolysis and may lead to a chronic protracted course or even death. If hemolysis is rapid and severe, subsequent transfusion may be necessary. Additional transfusion has been given together with intravenous immunoglobulin (IVIG) and steroids...
January 2010: Transfusion Medicine Reviews
M A Reyes, O C Illoh
A case of hyperhemolytic transfusion reaction attributable to anti-Fy3 in a 30-year-oldAfricanAmerican woman with a history of sickle cell disease is reported. The patient was admitted for vaso-occlusive sickle cell crisis and received 4 units of packed RBCs secondary to worsening symptomatic anemia (Hb 5.0 g/dL). On admission, the patient's antibody screen and identification showed anti-V and anti-E, and her antibody history included anti-E,-C,-Jk(b),-N, -V,-S,-Sl(a), and a cold agglutinin with possible anti-I specificity...
2008: Immunohematology
Daniel Glikman, Phuc Nguyen-Dinh, Jacquelin M Roberts, Christopher P Montgomery, Robert S Daum, John F Marcinak
Malaria is a disease of global importance and accounts for up to 500 million cases per year. Nearly all malaria cases in the United States occur among persons who have traveled to areas with ongoing malaria transmission. Among the cases of malaria reported in the United States in 2000-2005, 695 were in US residents under the age of 18 years. The association of malaria with the sickle cell hemoglobin is well described in Africa but is a rare occurrence in the United States. Here we report 5 cases of Plasmodium falciparum malaria in siblings of a family who had traveled to Africa without taking chemoprophylaxis...
September 2007: Pediatrics
Jeremy M Yarwood, Kara M Paquette, Ilya B Tikh, Esther M Volper, E Peter Greenberg
Several serious diseases are caused by biofilm-associated Staphylococcus aureus. Colonial variants occur in biofilms of other bacterial species, and S. aureus variants are frequently isolated from biofilm-associated infections. Thus, we studied the generation of variants with altered expression of virulence factors in S. aureus biofilms. We observed that the number of variants found in biofilms, as measured by hemolytic activity, varied for different strains. Further study of hemolytic activity and signaling by the accessory gene regulator (Agr) quorum-sensing system in one S...
November 2007: Journal of Bacteriology
Mohammad Al-Haggar, Hala Al-Marsafawy, Nabeel Abdel-Razek, Rizk Al-Baz, Abdel-Hamid Mostafa
The objective of this study was to predict which infants with sickle cell disease (SCD) are prone to develop severe painful crises. In a mixed hospital - and community-based population (76 cases), demographic data, SCD diagnostic parameters, and basal blood counts were correlated with 2 indices of SCD severity: pain rate (average number of days of painful episodes per year of follow-up) and serious life-threatening complications, such as hyperhemolytic crises. Data were analyzed blind to these indices. The Student t test, analysis of variance, and Pearson correlation were used to determine association with pain rate...
April 2006: International Journal of Hematology
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