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Lehmberg K - - 2007
BACKGROUND: Splenic function may be reduced or absent in a range of medical conditions in childhood, most prominently in homozygous sickle cell disease, celiac disease, or after total or partial splenectomy. In neonates and patients with malignant disease, transient hyposplenia has been reported as well. A simple method with reliable ...
Davies E H - - 2007
The European Task Force for Neuronopathic Gaucher Disease (NGD) met in 2006 to review its 2001 guidelines. Fifty-five patients from five European countries were reviewed; 29 were male and 26 female. The majority of the patients were homozygous for the L444P mutation. All had been on enzyme replacement therapy (ERT). ...
Banerjee Antara - - 2010
Several homeopathic remedies, namely, Pulsatilla Nigricans (30th potency), Ceanothus Americanus (both mother tincture and 6th potency) and Ferrum Metallicum (30th potency) selected as per similia principles were administered to 38 thalassemic patients receiving Hydroxyurea (HU) therapy for a varying period of time. Levels of serum ferritin (SF), fetal hemoglobin (HbF), ...
Goodnough Lawrence Tim - - 2007
The decision to transfuse a patient is not always clear and straightforward, particularly because no single number, neither extraction ratio nor hemoglobin level, can serve as an absolute indicator of transfusion need. Clinical assessment of the patient in conjunction with physiologic values helps in determining the appropriateness of transfusion before ...
Bradai Mohamed - - 2007
BACKGROUND: Studies of evolution of transfusion requirements in thalassemic patients treated with hydroxyurea have produced somewhat conflicting results, especially in patients with thalassemia major. Our aims were to determine the proportion of good responders to hydroxyurea in a population of transfusion-dependent thalassemic patients and to identify the factors associated with ...
Olaniyi J A - - 2007
BACKGROUND: Hepatobiliary and splenic complications (amongst others) are common in sickle cell disease (SCD) as a consequence of the progressive injury resulting from repeated sickling of HbS red blood cells. OBJECTIVE: To determine the degree and the frequency of persistent hepatomegaly and splenomegaly in relation to determinants of clinical severity ...
Choy Y C - - 2007
The main goal of perioperative transfusion is to reduce the morbidity and mortality associated with inadequate delivery of oxygen to the tissues during surgery. In this audit, the primary trigger for transfusion was clinical anaemia assessed by examination of a patient's conjunctiva [40.7%] followed by estimation of blood loss of ...
Szczepanik A B - - 2007
Administration of vinca alkaloids (VA) to chronic corticosteroid refractory immune thrombocytopenia (ITP) patients results in a temporary increase of platelet count. The aim of the study was to evaluate the efficacy of vinca alkaloids in preparing adult corticosteroid refractory chronic ITP patients for splenectomy as well as to compare the ...
Sheikha Anwar K - - 2007
We aimed to evaluate the protective role of partial versus total splenectomy against sepsis in patients with thalassaemia when other preventive measures are not available. Overwhelming postsplenectomy infection is a serious complication of splenectomy in these patients, and most present with pneumococcal septicemia. Pneumococcal vaccine given before surgery is a ...
del Trujillo María Monte - - 2008
BACKGROUND: Total hip replacement (THR) is associated with a significant perioperative blood loss, and 30-50% of these patients receive allogeneic blood transfusion (ABT). We evaluated the clinical utility of washed shed blood (WSB) return to reduce ABT in THR patients. STUDY DESIGN AND METHODS: Data from 108 consecutive THR patients ...
Kim Justin - - 2007
BACKGROUND: Blood transfusion is associated with higher postoperative complication. With the availability of autologous blood and erythropoietin, it would be advantageous to identify patients who are at higher risk for requiring blood transfusion. Our aim is to identify possible predictive factors for perioperative blood transfusion in patients undergoing colorectal resection. ...
Akinyoola A L - - 2007
BACKGROUND: Avascular necrosis (AVN) especially of the femoral head, has long been recognised as a manifestation of sickle cell disease (SCD). Despite this knowledge the actual prevalence of this complication among sickle cell patients in Nigeria is not known. OBJECTIVE: To determine the prevalence and patterns of presentation of avascular ...
Anyanwu, R. A.
The role of alpha (α) and beta (β) haemolysins in blood transfusion has been well documented. However, the occurrence of haemolysins and its attending problems in sickle cell anaemia (SCA) patients has limited appearance in the literatures especially in black Africa. This study was therefore designed to investigate the occurrence ...
Worel Nina - - 2007
BACKGROUND: Delayed severe immune hemolysis due to donor-derived passenger lymphocytes is observed in minor and/or bidirectional ABO-mismatched transplants, especially after reduced-intensity conditioning (RIC). The incidence is reported in up to 30 percent of patients and can result in multiorgan failure (MOF) and death. STUDY DESIGN AND METHODS: A first group ...
Johansson Pär I - - 2007
BACKGROUND: Continued hemorrhage remains a major cause of mortality in massively transfused patients of whom many develop coagulopathy. Reviewing transfusion practice for these patients, we found that at our hospital more than 10 percent received a suboptimal transfusion therapy and that survivors had a higher platelet count than nonsurvivors. We ...
Boga C - - 2007
Red cell exchange transfusion is the recommended therapy for patients with sickle cell disease who have complicated vaso-occlusive episodes. However, the role of the therapeutic plasma exchange in the management of the potentially life-threatening complications in patients with sickle cell disease is not well known. To determine whether plasma exchange ...
Hall K - - 2007
While there is laboratory evidence that the activity of recombinant activated factor VII (rFVIIa) is reduced by the presence of acidosis and hypothermia, there is limited clinical data to support this observation. Recombinant FVIIa may be used as rescue therapy in surgical patients who have bleeding that is refractory to ...
Fasola Foluke - - 2007
Congenital hemoglobin mutations may alter the delicate balance of free-radical generation and antioxidant defense systems in the red cell. Oxidative stress may thus play a role in the pathophysiology of the clinical manifestations of the disease. We assessed the total antioxidant status in steady-state sickle cell anemia (SCA) patients and ...
Giagounidis Aristoteles A N - - 2007
Lenalidomide is a novel thalidomide analogue with enhanced immunomodulatory and antiangiogenic action lacking most of the typical thalidomide-associated adverse events. In myelodysplastic syndromes (MDS), it has been used primarily in the IPSS low- and intermediate-1 risk setting. Several trials have demonstrated its potential to lead to both erythroid and cytogenetic ...
Wiwanitkit Viroj - - 2007
Diabetes mellitus (DM) is a frequent disorder affecting individuals of all ages. Glycohemoglobin (GHb) has a key role in the assessment of glycemic control in diabetic patients. Generally, GHb is measured as hemoglobin (Hb) A1C and is the result of an irreversible non-enzymatic glycation of the beta chain of hemoglobin ...
Yousif, A; ;
Background: Abnormal haemoglobins lead to significant morbidity and mortality. Design: This is a prospective study, evaluating types of haemoglobin and haematological parameters in blood samples of 631 Sudanese patients. Materials and Methods: Participants were patients who attended different departments in Khartoum Teaching Hospital in the period from March 2005 through ...
Ozdogu Hakan - - 2007
BACKGROUND: The apoptosis of human polymorphonuclear leukocytes (PMNs) in patients with sickle cell disease (SCD) is not well understood. The goal of this study was to examine the apoptosis of PMNs in patients with SCD and in controls. METHODS: Flow cytometric quantitation of PMN apoptosis was performed in 17 patients ...
Masiello David - - 2007
An infant with Hb SE disease is reported. He was clinically well. Review of the literature shows that patients aged 18 and younger are usually well. On the other hand, more than half of those aged 20 and older developed sickling-related complications, including potentially life-threatening acute chest syndrome. These patients ...
Steiner Laurie A - - 2007
OBJECTIVE: Our goal was to identify trends in patient demographics and indications for and complications related to neonatal exchange transfusion over a 21-year period in a single institution using a uniform protocol for performing the procedure. METHODS: A retrospective chart review of 107 patients who underwent 141 single- or double-volume ...
Rivkin Anastasia - - 2007
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia. Patients with TTP often have an increased reticulocyte count, elevated serum lactate dehydrogenase level, and decreased serum haptoglobin level. Other symptoms include fever, neurologic changes, and renal abnormalities. Thrombotic thrombocytopenic purpura may occur in ...
Borgna-Pignatti Caterina - - 2007
The term thalassaemia intermedia includes a large spectrum of conditions of varying severity. Blood transfusion and chelation are necessary in some patients, especially during childhood, in order to promote growth and prevent bone deformities. Alloimunisation, however, is frequent and can be difficult to control. Splenectomy is usually needed at some ...
Shimoda Kazuya - - 2007
Between 1999 and 2005, 285 patients received new diagnoses of myelofibrosis with myeloid metaplasia (MMM) in Japan. Anemic symptoms were present in 162 patients, and hemoglobin (Hb) concentrations were <10 g/dL in 197 patients. Fifty-five MMM patients were treated with anabolic steroids, and their effect on anemia during MMM was ...
Aessopos Athanassios - - 2007
beta-Thalassemia is an inherited hemoglobin disorder characterized by reduced synthesis of beta-globin chain. The severity of clinical course distinguishes this heterogeneous disease in two main subtypes: thalassemia major (TM) and thalassemia intermedia (TI). TI has a later clinical onset with a milder anemia that does not require transfusions at least ...
Sadat-Ali M - - 2007
OBJECTIVE: The risk of osteoporosis and osteopenia i s patients with sickle cell anaemia is not well established. This prospective study was conducted to assess the prevalence of osteoporosis and osteopenia in sickle cell anaemia. METHODS: Consecutive adult sickle cell anaemia patients who were treated at the department of orthopaedic ...
Kotila Taiwo R - - 2007
BACKGROUND: The sickle cell trait is a benign asymptomatic condition that should not ordinarily be associated with clinical manifestations of a haemoglobinopathy. METHOD: This is a case control study of sickle cell trait patients who presented with symptomatology of a haemoglobinopathy. HbA2, HbF and HbS levels as well as the ...
Ogunbiyi A O - - 2007
BACKGROUND: Patients with Sickle cell disease present with a wide range of symptoms and signs which overlap with other chronic illnesses. This often leads to a delay in diagnoses of the associated disorder. OBJECTIVE: In view of the high prevalence of SCD in our environment, it is important to know ...
Schizas A M P - - 2007
Patients with anaemia are commonly referred for bidirectional endoscopy. The aim of this study was to determine if any haematological parameters could predict positive findings at endoscopy. A total of 209 patients had bidirectional endoscopies performed for anaemia between September 2002 and March 2004. The endoscopy reports, histology and full ...
Kotila Rachel - - 2007
BACKGROUND & OBJECTIVES: Sickle-cell trait confers protection against malaria while homozygote sickle-cell disease (SCD) patients are at greater risk of malaria infection, hence the use of malaria chemoprophylaxis in SCD patients. The use of malaria chemoprophylaxis and asymptomatic parasitaemia were studied in SCD and non-SCD patients. STUDY DESIGN: A semi-structured ...
Botta Luca - - 2008
beta-Thalassaemia is an inherited haemoglobin (Hb) disorder resulting in chronic haemolytic anaemia. The most anaemic patients require regular red blood cell (RBC) transfusions for survival but iron accumulation leads to multisystem dysfunction. Heart complications represent the leading cause of mortality in beta-thalassaemia patients. In this case report we present a ...
Muñoz M - - 2007
INTRODUCTION: Requirements for allogeneic blood transfusion (ABT) after total knee replacement (TKR) are still high (30-50%), and salvage of unwashed filtered postoperative shed blood (USB) may represent an alternative to ABT. We evaluated that patients are more likely to benefit of USB reinfusion after TKR. PATIENTS AND METHODS: Data from ...
Schnedl Wolfgang J - - 2007
BACKGROUND: Evaluation of conditions associated with glycated hemoglobin (HbA1c) values below the reference range in HbA1c determinations. METHODS: Over a time period of 5 years, HbA1c results were determined with the ion-exchange high-performance liquid chromatography (HPLC) method HA-8140 Menarini. RESULTS: Of approximately 20 000 HbA1c results analyzed, 9 were below ...
Abuzallouf S - - 2007
To determine the frequency of anemia in patients with advanced or metastatic non-small cell lung cancer (NSCLC) at the time of initial oncologic consultation. We reviewed 144 consecutive charts of patients with advanced or metastatic NSCLC, who were seen in consultation at the Hamilton Regional Cancer Center (Canada) between January ...
Noronha J F A - - 2007
BACKGROUND AND PURPOSE: Reticulated platelet (RP) count provides an estimate of thrombopoiesis. The objective was to evaluate RP in patients in different stages of sickle cell disease (SCD) and to determine the relationship between interleukin-6 (IL-6), interleukin-3 (IL-3) and thrombopoietin (TPO) and RP count and degree of activation. METHODS: Eighty-nine ...
Yesilipek M Akif - - 2007
beta-Thalassemia (thal) and sickle cell disease are the most common genetic diseases worldwide. Although supportive therapies such as regular transfusion and chelation for beta-thal and hydroxyurea (HU) for sickle cell disease have significantly improved clinical manifestations and the quality of life, they cannot eliminate disease and therapy-related complications. Today, hematopoietic ...
Birgegård Gunnar - - 2007
Approximately 60% of anaemic cancer patients respond to epoetin treatment. An early marker of response would be valuable in order to avoid ineffective treatment. We have previously shown that beta globin mRNA increases rapidly after epoetin beta treatment of healthy controls. In the present study we have evaluated whether a ...
Kratovil Tonya - - 2006
INTRODUCTION: Hydroxyurea (HU) improves hematologic parameters and decreases adverse events in patients with sickle cell disease (SCD). HU has been proposed as an alternative to chronic transfusions for secondary stroke prevention. Transcranial doppler (TCD) is an accepted method of stroke risk stratification in patients with SCD. We sought to determine ...
Sadis C - - 2007
BACKGROUND AND OBJECTIVES: Multiple blood transfusions are considered a common cause of acute respiratory distress syndrome (ARDS). We hypothesized that ARDS is more a consequence of ARDS risk factors (in particular circulatory shock) requiring transfusions than a result of the transfusions themselves. METHODS: This retrospective study included 103 patients admitted ...
Ahmed Sagir G - - 2006
BACKGROUND: Priapism was associated with certain hematological parameters in sickle cell anemia (SCA) patients in one report but not in another. We studied differences in haematological parameters between SCA patients with and without priapism. PATIENTS AND METHODS: Eighteen patients with SCA who presented with acute priapism during the years 2001-2004 ...
Thomas Merlin C - - 2006
BACKGROUND: Anemia is a common finding in patients with diabetes, for whom it constitutes an additional burden. The aim of this study is to clarify the natural history of anemia in patients with type 2 diabetes and describe factors that predict a decrease in hemoglobin (Hb) levels. METHODS: A 5-year ...
Priftis Kostas N - - 2006
Transfusional iron overload may occur in the lungs. We hypothesized that quantitating siderophages in the bronchoalveolar fluid (BALF) of heavily transfused patients may prove to be a useful tool in determining lung iron overload in transfusion-dependent patients. The study included six patients (7-20 years) with thalassemia major (TM) who had ...
Buseri Fiekumo I - - 2006
The plasma levels of some blood coagulation parameters; prothrombin time, (PT), partial thromboplastin time with kaolin (PTTK), thrombin clotting time, (TCT), fibrinogen and factor X assay were determined in 50 Nigerian homozygous (HbSS) patients and 50 HbAA healthy individuals for the purpose of assessing their baseline values and susceptibility of ...
Sanchorawala V - - 2006
AL amyloidosis, a systemic disorder characterized by widespread deposition of amyloid fibrils derived from monoclonal Ig light chains in organs and soft tissues, is typically caused by an underlying plasma cell dyscrasia. However, this disease can also be associated rarely with a B-cell lymphoproliferative disorder. In this report, we describe ...
Hassan Hilali - - 2006
BACKGROUND: Bronchial hyperresponsiveness and/or bronchospasm are recognized complications of sickle cell disease. OBJECTIVE: The aim of this study was to investigate the presence of bronchospasm during painful crises, using simple spirometry in patients with sickle cell disease. METHODS: A prospective, non-randomized study was undertaken in patients with homozygous sickle cell ...
Bachmeyer C - - 2007
Salmonella osteoarticular infections involve mainly long bones such as the femur, tibia, and humerus in patients with sickle cell disease (SCD). We report here two unusual cases of Salmonella osteoarthritis affecting sacroiliac and sternoclavicular joints in two patients with SCD, one patient also being followed for rheumatoid arthritis. Because of ...
Harrison M J - - 2006
The aim of this pilot study was to investigate anaesthetists' assessment of the ability of patients to increase cardiac output over a range of clinical scenarios and of their perceived 'likelihood of transfusion' in these scenarios. Specialist anaesthetists were given a questionnaire with clinical cues in the form of diagnoses ...
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