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Liu Judy Fong - - 2010
We describe an 80-year-old woman with atrial fibrillation, anti-coagulated with warfarin, who on two separate occasions developed black tarry stools and an elevated international normalized ratio (INR) after eating a pound of Black Licorice. During her most recent episode, her hematocrit was 14 (baseline 34) and her INR was 5.5 ...
Prakobkaew Nattaphol - - 2010
Secondary erythrocytosis may arise from several causes, but an association with oxygen transport is rare. We describe for the first time a form of secondary erythrocytosis caused by compound heterozygosity for hemoglobin (Hb) Tak and (δβ)(0)-thalassemia found in an adult Thai individual. The patient had marked erythrocytosis and microcytosis with ...
Kalantri Ashwini - - 2010
BACKGROUND: Anaemia is a common disorder. Most health providers in resource poor settings rely on physical signs to diagnose anaemia. We aimed to determine the diagnostic accuracy of pallor for anaemia by using haemoglobin as the reference standard. METHODOLOGY/PRINCIPAL FINDINGS: In May 2007, we enrolled consecutive patients over 12 years ...
Melchiori Luca - - 2010
beta-thalassemia encompasses a group of monogenic diseases that have in common defective synthesis of beta-globin. The defects involved are extremely heterogeneous and give rise to a large phenotypic spectrum, with patients that are almost asymptomatic to cases in which regular blood transfusions are required to sustain life. As a result ...
Salah Hosam - - 2010
Osteopenia and osteoporosis are considered major health problems in patients suffering from thalassemia due to increased life expectancy of those patients. Osteoprotegerin (OPG) and receptor activator of NF-kappa-B ligand (RANKL) have been recently implicated in the pathogenesis of various types of osteoporosis. The aim of this study is to evaluate ...
Jetsrisuparb Arunee - - 2010
The authors performed a prospective, controlled, 3-year, follow-up study on infections and illnesses in Hb E beta-thalassemic pediatric patients. Fifty severe and 24 non-severe patients and 24 controls were included. Siblings with an age difference of no more than 4 years served as controls. All patients and controls were asked ...
Vayá Amparo - - 2010
There is little information regarding the behaviour of red blood cell deformability in macrocytosis. We have determined in 114 patients with macrocytosis (MCV > 97 fL) and in 115 age and sex-matched subjects with normocytosis (VCM < 97 fL) erythrocyte deformability by means of ektacytometric techniques (Rheodyn SSD) measuring the ...
Fawibe A E - - 2010
BACKGROUND: Sickle cell chronic lung disease (SCLD) is often underappreciated by health care providers because its exact prevalence and methods of diagnosis have not been well studied. OBJECTIVE: To describe the pattern of SCLD among young adult Nigerians with sickle cell anaemia (SCA).its exact prevalence and methods of diagnosis have ...
Cakmak Alpay - - 2010
AIM: Prolidase is a specific imidodipeptidase involved in collagen degradation. The increase in the enzyme activity is believed to be correlated with the increased intensity of collagen degradation. The study aimed to evaluate the relationship between prolidase activity and oxidative status in patients with thalassemia major. METHODS: Comparison was made ...
Yin Xiao-Llin - - 2010
The clinical characteristics of 357 patients with hemoglobin H (HbH) disease from the Guangxi province of Southern China were studied. One hundred and ninety-one (53.3%) patients were diagnosed with HbH-Constant Spring, 19 were diagnosed with HbH Westmead. Ten patients were shown to have coinherited HbH-Constant Spring/QS with a β-thalassemia mutation. ...
Fendel Rolf - - 2010
Falciparum Malaria, an infectious disease caused by the apicomplexan parasite Plasmodium falciparum, is among the leading causes of death and morbidity attributable to infectious diseases worldwide. In Gabon, Central Africa, one out of four inpatients have severe malarial anemia (SMA), a life-threatening complication if left untreated. Emerging drug resistant parasites ...
Fitzhugh Courtney D CD Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute and National Institute of Diabetes, Digestive, and Kidney Diseases/NIH, 9000 Rockville Pike, Bethesda, MD 20892, USA. - - 2010
Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and ...
Brittain Julia E - - 2010
Placenta growth factor (PlGF) is released by immature erythrocytes and is elevated in sickle cell disease (SCD). Previous data generated in vitro suggest that PlGF may play a role in the pathophysiology of SCD-associated pulmonary hypertension (PHT) by inducing the release of the vasoconstrictor, endothelin-1. In this cross-sectional study of ...
Ozoene J O - - 2009
Liberal fluid intake is one of the key management strategies in sickle cell anaemia [SCA] patients in steady state, but less work has been done on the desire of patients to drink water. Using the Visual Analogue Scale we studied thirst perception [TP] in 20 euhydrated SCA patients and 28 ...
Thom Giddel G A - - 2009
S-glutathionyl hemoglobin is a proposed biomarker of oxidative stress but has not been measured in sickle cell disease patients. Unlike the S-glutathionyl adduct of normal adult hemoglobin, S-glutathionyl sickle hemoglobin (HbSSG) cannot be directly measured by capillary isoelectric focusing, because it coelutes with fetal hemoglobin (HbF). This suggests that HbF, ...
Jung Chae Lim CL Department of Laboratory Medicine, School of Medicine, Ewha Womans University, Seoul, - - 2009
Hemoglobin (Hb) Yamagata is a rare Hb variant, which has been reported only twice-one case each in Japan and Korea. This variant arises from a Lys --> Asn substitution due to a mutation of AAA to AAC or AAT at codon 133 of the beta-globin gene. This study reports the ...
Wuchter Patrick - - 2010
As more efficient agents for stem cell mobilization are being developed, there is an urgent need to define which patient population might benefit from these novel drugs. For a precise and prospective definition of "poor mobilization" (PM), we have analyzed the efficiency of mobilization in patients intended to receive autologous ...
Macdougall Iain C - - 2009
BACKGROUND: We investigated whether a novel, synthetic, peptide-based erythropoietin-receptor agonist (Hematide, Affymax) can stimulate erythropoiesis in patients with anemia that is caused by antierythropoietin antibodies. METHODS: In this open-label, single-group trial, we enrolled patients with chronic kidney disease who had pure red-cell aplasia or hypoplasia due to antierythropoietin antibodies and ...
O'Donnell A - - 2009
In many Asian populations, the commonest form of severe thalassemia results from the coinheritance of HbE and beta thalassemia. The management of this disease is particularly difficult because of its extreme clinical diversity; although some genetic and adaptive factors have been identified as phenotypic modifiers, the reasons remain unclear. Because ...
Abu Zeid Haitham A - - 2009
Sickle cell disease patients with relatively high hemoglobin (> or = 12 g x dl) and those who have elevated alloimmunizations antibodies with rare phenotype subgroups, are problems challenging anesthesiologists. Acute Normovolemic Hemodilution (ANH) is rarely used in the perioperative management of homozygous sickle cell disease (SCD) in patients undergoing ...
Das Sudipta Sekhar - - 2009
Various adsorption techniques are available to remove serum autoantibodies and subsequently detect the underlying alloantibody in previously transfused patients with autoimmune haemolytic anaemia. We planned to establish a suitable adsorption technique in our transfusion service which can remove all autoantibodies and detect underlying alloantibodies rapidly, cheaply and effectively. We evaluated ...
Olgar Seref - - 2010
BACKGROUND: Thalassemia major (TM) is an important cause of severe anemia that necessitates regular blood transfusion to prevent the profound weakness and cardiac decompensation caused by the anemia. However, iron overloading is an inevitable consequence of prolonged transfusion therapy. In addition, extramedullary hematopoiesis and hemosiderosis cause spleen, liver and marrow ...
Harada Hironori - - 2009
Lenalidomide is an immunomodulatory agent recently reported to be effective in the treatment of transfusion-dependent anemia due to low- or intermediate-1 risk myelodysplastic syndromes (MDS) associated with a deletion 5q (del 5q) cytogenetic abnormality. We conducted a multicenter, single-arm clinical trial to evaluate the safety and efficacy of lenalidomide in ...
Nur Erfan - - 2009
Sickle cell disease (SCD) is associated with a high incidence of ischemic stroke. SCD is characterized by hemolytic anemia, resulting in reduced nitric oxide-bioavailability, and by impaired cerebrovascular hemodynamics. Cerebrovascular CO2 responsiveness is nitric oxide dependent and has been related to an increased stroke risk in microvascular diseases. We questioned ...
Ambruso Daniel R - - 2009
BACKGROUND: Pathogen inactivation technologies provide a potential solution to donor screening and blood testing strategies reducing the risk of transfusion-transmitted infectious diseases. The Mirasol pathogen reduction technology (PRT) system (CaridianBCT) uses riboflavin and UV light to introduce modifications in nucleic acids, reducing the infectious pathogen load in blood components. This ...
Spencer John David - - 2009
Patients with sickle cell disease commonly experience painless hematuria. Hematuria may be found in patients with sickle cell trait, sickle cell anemia, and sickle cell hemoglobin C disease, but it is believed to be uncommon in patients with other hemoglobinopathies, such as hemoglobin C disease and hemoglobin C trait. We ...
Nijland Roel - - 2009
STUDY OBJECTIVE: To study whether a deficiency in galactose-1-phosphate uridyl transferase (GALT) activity of mothers was an explanation for the occurrence of Müllerian aplasia of their daughters. DESIGN: A case control study. SETTING: The patients were selected from the outpatient clinic of the University Medical Center Nijmegen, and compared with ...
Nakazawa Harumasa - - 2009
BACKGROUND: To reduce the risk of transfusion-related acute lung injury (TRALI), plasma products are mainly made from male donors in some countries because of the lower possibility of alloimmunization; other countries are considering this policy. The advantage of male-only fresh-frozen plasma (FFP) should be examined in a prospective case-control study. ...
Palmerini C A - - 2009
Erectile dysfunctions are not uncommon, especially in patients suffering from metabolic syndrome and from a number of circulatory and psychiatric problems. cGMP diesterase inhibitors, such as sildenafil, have proven to be beneficial in the treatment of many such conditions. Our patients, all of them complaining of erectile dysfunction, were treated ...
Otterman Marie-Louise - - 2009
BACKGROUND: Increased production of red blood cells (RBCs) should be reflected by increased reticulocyte counts (RC). With the introduction of modern fully automated measurements of RC, the recovery of hemoglobin (Hb) after blood loss might be assessed earlier. We investigated the temporal relation of Hb and RC in trauma patients. ...
Olayemi Edeghonghon E - - 2009
The cause of chronic leg ulcer (CLU) in patients with sickle cell anemia (SCA) is unknown; it has been attributed to hypercoagulability associated with the disease. Recently, it has been suggested that a subset of SCA patients may be prone to developing CLU and that hemolysis may be an underlying ...
Italia Khushnooma Y - - 2009
BACKGROUND: The clinical and hematological response to hydroxyurea was evaluated in beta thalassemia patients in western India with variable clinical severity and correlated with genetic factors. MATERIALS AND METHODS: Seventy-nine patients-[38-beta thalassemia intermedia-(group I), 41-beta thalassemia major-(group II)] on hydroxyurea therapy were followed-up for 20-24months. RESULTS: Among the frequently transfused ...
Ilan Yaron - - 2009
Gaucher disease (GD) is caused by the reduced activity of a lysosomal enzyme, glucocerebrosidase, leading to the accumulation of glucocerebroside (GC). The relatively high prevalence of this disease within an ethnic group is believed to reflect a selective advantage. Treatment with enzyme replacement therapy (ERT) is safe and effective in ...
Almeida Roberto Paulo - - 2009
OBJECTIVE: to describe clinical, laboratory and ultrasonographic abnormalities in the hepatobiliary system of pediatric patients with sickle cell disease in the city of Salvador, Brazil. MATERIAL AND METHODS: pediatric patients with sickle cell disease were clinically evaluated, their charts were reviewed and findings of supplementary tests were examined to identify ...
Kar Rakhee - - 2009
Hereditary spherocytosis (HS) is an inherited membranopathy characterized by phenotypic and genotypic heterogeneity. This study describes the clinico-hematological profile of 70 HS patients diagnosed at a tertiary care center in North India over a period of five years. Patients commonly presented with intermittent jaundice (82.9%), pallor (80%) and dark colored ...
Claster Susan S Division of Hematology Oncology, Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California 90027, USA. - - 2009
One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies have been reported in TM and nontransfused SCD patients, a comprehensive evaluation of vitamin and ...
Heilmann Claudia - - 2009
INTRODUCTION: Implantation of a ventricular assist device (VAD) is a seminal therapeutic option for patients with terminal cardiac failure. However, haemolysis remains a clinically relevant adverse effect of several VAD types. Lysed erythrocytes release lactate dehydrogenase (LDH) and haemoglobin. Free haemoglobin in plasma is bound by haptoglobin with high affinity. ...
Boudville Neil C - - 2009
Anemia and hemoglobin (Hb) variability are associated with mortality in hemodialysis patients who are on erythropoiesis-stimulating agents (ESA). Our aim was to describe the degree of Hb variability present in nondialysis patients with chronic kidney disease (CKD), including those who were not receiving ESA, and to investigate the association between ...
Issaivanan Magimairajan - - 2009
Childhood lead poisoning is common, affects multiple organs and is preventable. Co-existence of plumbism and sickle cell disease (SCD) is uncommon, with major overlap in signs and symptoms. We describe a patient with SCD and plumbism. Dysfunctional eating patterns may cause ingestion and increased absorption of lead in these patients. ...
Chadebech Philippe - - 2009
BACKGROUND: Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication in sickle cell disease (SCD) characterized by recurrence of disease complications, recipient red blood cell (RBC) destruction, and frequently no detectable antibody. Phosphatidylserine (PS) exposure signs suicidal RBC death or eryptosis and is involved in vasoocclusive crisis (VOC). STUDY DESIGN ...
DeFrancisco A L M - - 2009
To help identify factors contributing to intra-patient Hb variability, pooled records were analyzed from 5,592 patients undergoing hemodialysis (HD) in European, multicenter, open-label, single-arm Phase 3b trials. Patients previously treated with recombinant human erythropoietin (rHuEPO) were switched to darbepoietin-alpha administered once a week (QW) or once every 2 weeks (Q2W), ...
Spinella Philip C PC United States Army Institute of Surgical Research, Fort Sam Houston, Texas, USA. - - 2009
Increased understanding of the pathophysiology of the acute coagulopathy of trauma has lead many to question the current transfusion approach to hemorrhagic shock. We hypothesized that warm fresh whole blood (WFWB) transfusion would be associated with improved survival in patients with trauma compared with those transfused only stored component therapy ...
Pornprasert Sakorn - - 2009
OBJECTIVES: To assess the effects of highly active antiretroviral therapy (HAART) on hematological parameters in HIV-1-infected patients with and without thalassemia carriages. METHODS: Prospective study was conducted in HIV-1-infected Thai patients receiving HAART. Their hematological parameters were measured at baseline and during follow-up of 1 year. beta-thalassemia and hemoglobin-E trait ...
Renzulli Pietro - - 2009
BACKGROUND: Splenic involvement in amyloidosis is rather frequent (5-10%). An atraumatic rupture of the affected spleen is however an extremely rare event. We report on a patient with undiagnosed amyloidosis who underwent emergency splenectomy for atraumatic splenic rupture. METHODS: Review of the literature and identification of 31 patients, including our ...
Al-Nood Hafiz - - 2009
Blood samples were collected from 699 patients attending out patient clinics in Sana'a City, Yemen, to obtain some idea of the prevalence of the thalassemias in our country. Complete blood count, hemoglobin (Hb) electrophoresis, quantitation of Hb A(2) and Hb F, and serum ferritin were determined. Microcytic, hypochromic red cells ...
Karimi Mehran - - 2009
Hydroxyurea (HU) is an antineoplastic agent that enhances fetal hemoglobin. The clinical significance induced by this compound is well known in sickle cell disease. This clinical significance could also be expected in beta-thalassemia patients. Although studies on beta-thalassemia major patients showed significant results, but these clinical responses are expected to ...
Lee Byeong-Taek - - 2009
Hwa-Byung (HB) is a Korean culture-bound psychiatric syndrome caused by the suppression of anger. HB patients have various psychological and somatic symptoms, such as chest discomfort, a sensation of heat, and the sensation of having an epigastric mass. In this study, we measured brain activity in HB patients and healthy ...
El-Beshlawy Amal - - 2009
Thalassemia patients with persistently high levels of fetal globin typically have less severe anemia, have milder clinical syndromes, and are often transfusion independent. Therefore, the search for molecules exhibiting the property of inducing gamma-globin gene expression and fetal hemoglobin (HbF) production is of great interest. Different pharmacological agents have been ...
Landburg P P - - 2009
Inflammation and angiogenesis are of importance in the pathophysiology of sickle cell disease (SCD). Recently, the chemokine stromal-derived factor-1 (SDF-1) has been shown to be a key mediator of angiogenesis and inflammation. In this study we determined serum SDF-1 levels in consecutive adult sickle cell patients during the clinically asymptomatic ...
Tantawy Azza A G - - 2009
The problem of spinal cord compression (SCC) related to extramedullary hematopoiesis (EMH) in beta-thalassemia (beta-thal) patients, both clinically and radiologically and its correlation with laboratory parameters of anemia and hemosiderosis was assessed. Sixty beta-thal patients were included and divided into group I: 40 beta-thal major patients (beta-TM), aged 7-30 years ...
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