Search Results
Results 251 - 300 of 909
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Bernaudin Françoise - - 2005
Cerebral arteriopathy can be detected in children with sickle cell disease (SCD) by transcranial Doppler (TCD). Abnormally high velocities are predictive of high stroke risk, which can be reduced by transfusion therapy. We report the results of the screening of 291 SCD children followed in our center, including the clinical ...
Cappellini M D - - 2005
Venous thromboembolic events, such as pulmonary embolism, deep venous thrombosis, and portal vein thrombosis, have been observed in adult thalassemia patients, mainly in beta-thalassemia intermedia. The clinical findings are consistent with the observation of several alterations that indicate a state of activation of the hemostatic mechanisms in thalassemias. These alterations ...
Olteanu H - - 2005
A hemolytic transfusion reaction due to anti-Fy3 is reported in an African American patient with no history of sickle cell disease. This 82-year-old African American woman received two units of RBCs for anemia (Hab 7 g/dL) on admission for a left hip fracture. On hospital Day 7, the patient underwent ...
Aessopos Athanasios - - 2005
Cardiac involvement represents the leading cause of mortality in both forms of beta-thalassemia, namely, thalassemia major (TM) and thalassemia intermedia (TI), and pulmonary hypertension (PHT) is part of the cardiopulmonary complications of the disease. PHT was initially documented in a small group of TI patients with right heart failure. In ...
Kano Gen - - 2004
Hemoglobin (Hb) Bristol-Alesha is caused by a GTG --> ATG mutation at codon 67 in the Hb beta chain, resulting in abnormal beta globin chains with mutated molecules from normal beta67 valine (Val) to beta67 methionine (Met) or beta67 aspartate (Asp). We describe a Japanese child with this rare hemoglobinopathy ...
Deeg H Joachim - - 2004
Myelodysplastic syndrome (MDS) comprises a spectrum of heterogeneous diseases. Most patients present with ineffective hematopoiesis. The pathophysiology involves immune-mediated effects, cytokine dysregulation, and apoptosis, among others. We treated 14 transfusion-requiring patients with MDS, 10 with refractory anemia (RA) and four with RA with excess blasts (RAEB) with a 4-day course ...
Shalev Hanna - - 2004
Congenital dyserythropoietic anemia (CDA) type I is an inherited disorder characterized by macrocytic anemia with pathognomonic morphologic ultrastructural features of the erythroid precursors. The authors recently cloned the CDAN1 gene and identified one founder missense mutation in all of their Bedouin patients. In a previous study, the authors found that ...
Fielding David D Department of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, - - 2004
Practical aspects of Storz Dlight autofluorescence bronchoscopy are presented from a series of 73 patients. 80 biopsies were taken of which 23 had at least moderate dysplasia, including 8 CIS lesions. Causes of false positive fluorescence abnormalities were suction trauma, overlying mucus, depressions in the leading edge of bronchial spurs, ...
Cacciavillano Walter Daniel - - 2004
The study sought to evaluate the response to cyclophosphamide (CPM) in hepatoblastoma (HB). Patients with a refractory or relapsing HB after first-line therapy as per SIOPEL 2 and 3 protocols were eligible. All patients were to receive two courses of CPM 2 g/m(2) on days 1 and 2 at 3-week ...
Melissas J - - 2004
PURPOSE: In the ongoing effort to improve patient treatment, a deeper understanding of the symptomatology, physical signs and management options of rare splenic, non-traumatic, benign diseases is extremely important. PATIENTS AND METHODS: The records of eight consecutive patients with benign splenic conditions, other than injury and abscess, were reviewed retrospectively ...
Aderinto J - - 2004
We have reviewed prospective data on 1016 patients who underwent unilateral total hip replacement to establish the pre-operative risk factors associated with peri-operative blood transfusion. Most patients who required transfusion were older and were of lower weight, height, pre-operative haemoglobin level and body mass index than patients who were not ...
DemirbaƟ Kaya A - - 2004
AIM: To investigate radiographic manifestations of sickle cell anaemia (SCA) and whether or not a pulpal necrosis may develop without a pathological history. METHODOLOGY: Thirty-six patients with homozygous SCA were evaluated, and a further 36 individuals without SCA were included in the study as a control group. All 72 patients ...
Boschetti Carla - - 2004
We reviewed clinical and molecular data of 23 consecutive unrelated patients affected by paroxysmal nocturnal hemoglobinuria (PNH) (19 with hemolytic PNH, 3 with aplastic anemia/PNH, and 1 with myelodysplasia/PNH syndrome) with a mean follow-up of 11.8 years. Five patients had thrombotic episodes, and 10 needed regular blood transfusions; 2 died ...
Marawaha R K - - 2004
Wheat grass juice is the juice extracted from the pulp of wheat grass and has been used as a general-purpose health tonic for several years. Several of our patients in the thalassemia unit began consuming wheat grass juice after anecdotal accounts of beneficial effects on transfusion requirements. These encouraging experiences ...
Juwah A I - - 2004
BACKGROUND: Anaemic crises in paediatric patients with sickle cell anaemia are major causes of morbidity and mortality. Some children admitted to hospitals' emergency rooms or paediatric wards of the hospitals with severe anaemia die before blood transfusion. AIMS AND METHODS: A total of 108 episodes of anaemic crises were prospectively ...
Kattamis Antonis - - 2004
The authors studied the long-term clinical and hematological response to hydroxyurea (HU) therapy in young patients, with either S/beta-thalassemia (beta(thal)) (8 patients) or SS (6 patients). All patients with S/beta(thal) responded well to treatment. Longitudinal evaluation of Hb, HbF, and MCV showed a significant increase compared to baseline levels, but ...
Mamman A I - - 2004
Pure red cell Aplasia (PRCA) is a transfusion dependent condition characterised by anaemia, reticulocytopaenia and a paucity of erythroid precursors in the bone marrow. Records of patients with PRCA seen in Ahmadu Bello University Teaching Hospitals between 1991 and 2001 were reviewed and the outcome of their management was assessed. ...
Hardwick Mary E - - 2004
This study evaluated whether patients receiving a two-dose regime of epoetin alfa received less allogeneic blood than patients donating autologous blood before primary hip arthroplasty. Consenting patients with a hemoglobin level between 12 and 15 g/dL were randomly assigned to a two-dose administration of epoetin alfa (19 patients) or autologous ...
Dhar Meekoo - - 2004
Homocysteine has associations with both vitamin insufficiency and vascular complications, and its status is therefore of interest in sickle cell disease (SCD). However, information is limited, especially in adults. We studied plasma total homocysteine (tHcy) and three of its major modifiers, cobalamin, folate, and creatinine, in 90 adult patients with ...
Verhelst David - - 2004
BACKGROUND: Recombinant human erythropoietin is the standard treatment for anaemia related to chronic kidney disease, and its widespread use has been favoured by a very high therapeutic index. However, since 1998, more than 200 patients worldwide with chronic kidney disease treated in this way have developed neutralising antibodies to erythropoietin, ...
Shander Aryeh - - 2004
BACKGROUND: Hemoglobin-based oxygen carriers hold promise for the treatment of acute anemia. CASE: We report a patient with severe dysfunctional uterine bleeding. During her hospitalization, her lowest hemoglobin level was 3.1 g/dL, with a hematocrit of 9.3%. An investigational product, o-raffinose cross-linked human hemoglobin solution (hemoglobin raffimer), was infused along ...
Udezue Emmanuel - - 2004
BACKGROUND: Sickle cell disease (SCD) is highly prevalent in the Al-Hasa area of eastern Saudi Arabia. We analyzed our patient data to try and find an explanation for the unexpected observation that more males than females with SCD were transferred to the hospital after a stay in the stabilization unit. ...
Stuart Brian J - - 2004
Polycythemia vera is a chronic myeloproliferative disorder characterized by increased red blood cell mass. The resultant hyperviscosity of the blood predisposes such patients to thrombosis. Polycythemia vera should be suspected in patients with elevated hemoglobin or hematocrit levels, splenomegaly, or portal venous thrombosis. Secondary causes of increased red blood cell ...
Shander Aryeh - - 2004
The anemia of critical illness is a distinct clinical entity with characteristics similar to that of chronic disease anemia. Several solutions to the processes of anemia, such as blunted erythropoietin production and erythropoietin response and abnormalities in iron metabolism have been developed. The transfusion of RBCs provides immediate correction of ...
Lee K-H - - 2004
The outcomes of patients who experience the failure to reconstitute a trilineage of blood cells after initial neutrophil engraftment were evaluated in 178 patients with hematologic disorders, who underwent allogeneic HCT. Of 165 qualified patients (five with primary engraftment failure; eight deaths before day 60 of HCT), 43 (26%) satisfied ...
Silliman Christopher C - - 2004
Transfusion of the injured patient with packed red blood cells (PRBCs) is a dynamic process requiring vigilance during the acute resuscitative and recovery phases postinjury. Although adverse events have been reported in 2% to 10% of injured patients, the advent of new detection techniques for viral pathogens has markedly decreased ...
Ludwig Heinz - - 2004
Anemia is a common complication in patients with multiple myeloma (MM) and occurs in more than two thirds of all patients. The most frequent underlying pathophysiological mechanism is anemia of chronic disease (ACD), relative erythropoietin (EPO) deficiency (due partly to renal impairment) and myelosuppressive effects of chemotherapy, but many other ...
Ootaki Yoshio - - 2004
OBJECTIVES: Low-hematocrit bypass is one technique used to prevent allogeneic transfusion during cardiopulmonary bypass. The purpose of this study is to determine the efficacy of a criterion-driven transfusion protocol and the effect of low-hematocrit bypass with moderate hypothermia in pediatric cardiac surgery. METHODS: Seventy-five children who underwent cardiopulmonary bypass with ...
Camargo J L - - 2004
To identify the causes of very low glycohaemoglobin (GHb) values in a sample of patients with diabetes in southern Brazil using high performance liquid chromatography. Between August 1996 and December 2001 all samples from patients with diabetes at a university hospital with GHb values below the reference range (4.7-6.0% HbA(1c)) ...
Farmakis Dimitrios - - 2004
The development of a diffuse elastic tissue defect resembling pseudoxanthoma elasticum (PXE) is a recently established and frequently encountered clinical entity in beta-thalassemia. The clinical spectrum of this disorder is not yet completely understood as it is continuously being enriched with novel complications that are often serious. We present here ...
Barosi Giovanni - - 2004
Neoangiogenesis is an integral component of bone marrow myeloproliferation in patients with myelofibrosis with myeloid metaplasia (MMM). As extramedullary haematopoiesis is a constitutive feature of MMM, we studied spleen neoangiogenesis by a computerized image analysis in MMM patients. Compared with five normal subjects, spleen CD34-staining capillary vascular density (CVD) was ...
Abdulla Al-Ghamdi Abdulmohsin - - 2004
A 27-yr-old male patient, with homozygous sickle cell disease was scheduled for bilateral total knee replacement under tourniquet. The use of tourniquet in sickle cell patients is not without hazard. After preoperative exchange transfusion, total knee replacement was performed. The patient tolerated the procedure well. Patients with sickle cell disease ...
Das Nandita - - 2004
The oxidative stress status of the transfusion-dependent Ebeta- and beta-thalassemia patients were studied before and after treatment with vitamin E for a period of four weeks. The level of cellular vitamin antioxidants viz. ascorbic acid and vitamin E in the thalassemia patients were found to be considerably lower compared to ...
Chaidos Aristeidis - - 2004
The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is beta-thalassemia. Current therapeutic approaches for homozygous beta-thalassemia entail blood transfusions and iron chelation therapy with deferoxamine or deferiprone for preventing tissue hemosiderosis. Recently, much effort has focused on various inducers of ...
Boonsa Supap - - 2004
We defined the molecular basis and correlated the hematological phenotypes with the globin genotypes in 52 patients with Hb H disease and 29 patients with AEBart's disease of northeast Thailand. Among the former group, the most prevalent molecular defect was found to be the interaction of alpha-thalassemia 1 (SEA type) ...
Strupp C - - 2004
Except rare instances of allogeneic stem cell transplantation, treatment of idiopathic myelofibrosis (IMF) is only palliative and based on cytostatic treatment (hydroxyurea and anagrelide), androgen therapy, steroids and splenectomy. Thalidomide is an anti-angiogenic and immunomodulatory drug with a wide spectrum of activities, which are not clearly understood. Current data suggest ...
Walsh R M - - 2004
BACKGROUND: Elective laparoscopic splenectomy (LS) achieves excellent results for benign hematologic diseases. The role of LS for hematologic malignancies is harder to define owing to associated splenomegaly and patient disease that may alter outcome. METHODS: Retrospective review of single institution experience 1996 through 2002. To limit variability of disease processes, ...
McLellan S A - - 2003
Anaemia is a common finding in critically ill patients. There are often multiple causes. Obvious causes include surgical bleeding and gastrointestinal haemorrhage but many patients have no overt bleeding episodes. Phlebotomy can be a significant source of blood loss. In addition, critically ill patients have impaired erythropoiesis as a consequence ...
Waters Jonathan H - - 2003
In this case report, we report a patient with a placenta accreta and thalassemia intermedia undergoing cesarean delivery. There are no data regarding the use of cell salvage in patients with thalassemia. During the course of her surgery, she lost approximately 9000 mL of blood. Of this blood, 2250 mL ...
Rahimi Z - - 2003
Sickle cell anemia in Iran is accompanied by a high level of HbF and mild clinical presentation. Here we report haplotypes of the beta gene cluster found in 81 randomly selected sickle cell patients, including 47 sickle cell anemia (SS), 17 sickle cell trait (AS), and 17 sickle/thalassemia (S/thal) from ...
Jansen T - - 2003
A young man was accidentally intoxicated with isobutyl nitrite by a threefold lethal dose. Due to nitrites' ability to change hemoglobine into methemoglobine the patient showed signs of severe hypoxia, which could not be treated by conventional means. Therefore the patient was rapidly transferred to a hyperbaric chamber. The patient ...
Fujita Fumihiko - - 2003
Portal vein thrombosis (PVT) following splenectomy is a potentially life-threatening complication, and the true incidence of PVT in splenectomized patients is unknown. The objective of this study was to determine the incidence of symptomatic PVT after splenectomy. The hospital database was searched to identify cases of PVT associated with splenectomy ...
Shanafelt Tait D TD Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA. - - 2003
To evaluate the efficacy of rituximab for the treatment of adult patients with immune cytopenia, including idiopathic thrombocytopenic purpura (ITP), autoimmune hemolytic anemia, and Evans syndrome. We retrospectively reviewed the medical charts of all patients treated with rituximab for immune cytopenia at the Mayo Clinic in Rochester, Minn, through January ...
Gartaganis S P - - 2003
PURPOSE: Patients with beta-thalassemia (beta-tha) represent a group with lifelong transfusion-dependent anemias. This study aimed to describe the conjunctival changes and tear film parameters in these patients. METHODS: A total of 52 patients (104 eyes) with beta-tha major and 22 normal control subjects (44 eyes) were studied during 1999 through ...
Mendek-Czajkowska E - - 2003
Our study investigated two groups of adult patients with established diagnoses of primary myelofibrosis (21 patients) and myelodysplastic syndromes (MDS) (21 patients). The objective was to assess fetal hemoglobin (HbF) concentration and to investigate correlations with organomegaly and extramedullary hematopoiesis and with the level of anemia and blood transfusion requirement. ...
Lechapt Emmanuelle - - 2003
Previous reports have shown that in more than 40% of adults with acute chest syndrome (ACS), fat droplets suggestive of pulmonary fat embolism were present in alveolar macrophages. To determine whether induced sputum (IS) is a reliable test for detecting this embolism, we compared bronchoalveolar lavage and IS results in ...
Kizaki Masahiro - - 2003
To date, there are no curative therapeutic options for patients with myelodysplastic syndromes (MDS) other than allogeneic stem cell transplantation. We treated an MDS patient with 10 microg/kg pegylated recombinant human megakaryocyte growth and development factor (rHuMGDF) for more than 450 d. The patient's platelet counts increased from <10 x ...
Steensma David P - - 2003
The syndrome of chronic myelomonocytic leukemia (CMML) includes a heterogeneous group of patients who exhibit both myelodysplastic and myeloproliferative clinicopathological features. Troublesome splenomegaly is uncommon in myelodysplastic syndrome (MDS), but when organomegaly occurs, this complication is more likely to be associated with myelodysplastic-myeloproliferative overlap syndromes such as CMML rather than ...
Fogarty P F - - 2003
In recent years, a pathophysiological role for T cells in immune thrombocytopenia (ITP) has been established. We applied cDNA size distribution analysis of the T cell receptor (TCR) beta-variable (VB) complementarity-determining region 3 (CDR3) in order to investigate T cell repertoire diversity among immune thrombocytopenia patients who had either responded ...
Lombardo Turiddu - - 2003
Acute Chest Syndrome (ACS) describes a syndrome characterized by the presence of a new pulmonary infiltrate on a chest X-ray, fever, and respiratory symptoms and is the leading cause of death and hospitalization in sickle cell disease (SCD). We studied 21 patients affected by SCD (13 HbSbeta+, 4 HbS beta(o), ...
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