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Results 401 - 450 of 894
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Holmberg L A - - 1999
High-dose therapy with autologous peripheral blood stem cell (PBSC) rescue is widely used for the treatment of malignant disease. CD34 selection of PBSC has been applied as a means of reducing contamination of the graft. Although CD34 selection results in a 2 to 3 log reduction in contaminating tumor cells ...
Piaggio G - - 1999
Cytogenetic abnormalities and paroxysmal nocturnal haemoglobinuria (PNH) phenotype are frequent findings in aplastic anaemia patients treated with immunosuppressive therapy (IST). In this study we investigated whether the appearance of clonal haemopoiesis influences patient outcome and survival. 97 patients entered this study and were followed from the onset of the disease ...
Manfrè L - - 1999
OBJECTIVE: The purpose of this study was to evaluate the spectrum of MR findings of the brain in asymptomatic patients affected with thalassemia intermedia or sickle cell-thalassemia disease to prevent brain damage by identifying patients at risk for stroke so that transfusional or pharmacologic treatment could be implemented. SUBJECTS AND ...
Hoppe C - - 1999
Hydroxyurea (HU) and sodium phenylbutyrate (SPB) have been shown to increase fetal hemoglobin (Hb F) levels in patients with thalassemia intermedia. The reported effects of these agents in increasing total Hb, however, have been inconsistent and there have been no studies on the combination of these medications. We describe the ...
Willig T N - - 1999
Diamond-Blackfan anemia (DBA) is a constitutional disease characterized by a specific maturation defect in cells of erythroid lineage. We have assembled a registry of 229 DBA patients, which includes 151 patients from France, 70 from Germany, and eight from other countries. Presence of malformations was significantly and independently associated with ...
Reed W F - - 1999
The practice of transfusion for patients with sickle cell disease is changing. Diminished childhood mortality is resulting in greater patient longevity and a higher prevalence of chronic systemic complications. Many of these chronic complications, as well as several acute ones, are treatable with erythrocyte transfusion. Proactive or preventive uses for ...
Diop S - - 1999
Despite the unicity of its genetic mutation, Sickle cell homozygosity presents different clinical features. Our objectives were to evaluate disease severity in Senegalese patients. Sixty (60) homozygous sickle cell patients were followed up monthly during one year and disease severity was assessed using the "severity index" (SI) which is resulting ...
Mantadakis E - - 1999
A questionnaire survey was conducted of patients with homozygous sickle cell anemia (Hb SS) and sickle cell beta(0)-thalassemia (Hb S-beta(0)) between 5 and 20 years of age to determine the prevalence and characteristics (number of episodes, timing, duration, cause, or precipitating event) of priapism. Ninety-eight male patients or their parents ...
Booz M M - - 1999
We have studied 50 patients with sickle cell disease who presented with musculoskeletal pain over a 2-year period to assess the use of ultrasound in differentiating infection from infarction. All the patients were evaluated by ultrasound. Five had soft tissue oedema and no fluid collection adjacent to the bone. Forty-five ...
Al-Salem A H - - 1999
To analyze the authors' experience with splenectomy for sickling disorders and evaluate the indications, complications, and outcome. Over a period of 10 years (1987-1997), 113 patients with sickling disorders (100 with sickle cell disease and 13 with sickle-beta-thalassemia) had splenectomy at the authors' hospital as part of their management. The ...
Hume H A - - 1999
Modern transfusion support of pediatric patients requires attention to the necessity to provide specialized or modified blood components to these patients who are often immunocompromised and/or affected by very complex medical and surgical illnesses. In this review we will address three potential complications of transfusion that may require specialized components ...
Legler T J - - 1999
BACKGROUND: In chronically transfused patients, conventional blood group typing may be impossible because of mixed-field agglutination. STUDY DESIGN AND METHODS: In 27 patients with congenital anemia and lifelong transfusion history, genotyping for D, RHD, and RHCE was performed with polymerase chain reactions. These results were compared with the blood group ...
Bordin J O - - 1999
BACKGROUND: The immunomodulatory effects of allogeneic blood transfusions have been attributed to the white cells (WBCs) present in the cellular blood components transfused to patients. STUDY DESIGN AND METHODS: The effect of the transfusion of allogeneic red cells (RBCs) or allogeneic prestorage WBC-reduced RBCs (WBC-reduced RBCs) on host immune responsiveness ...
Al-Salem A H AH Division of Pediatric Surgery, Department of Surgery, and Department of Internal Medicine, Qatif Central Hospital, Qatif, Saudi - - 1999
In the Eastern Province of Saudi Arabia, an area known for various hemoglobinopathies, splenectomy is performed rather frequently. This study is an analysis of our experience with splenectomy performed for various hematological disorders between 1988 and 1997, outlining the indications, complications and outcome. This is a retrospective analysis of all ...
Alessandrino P - - 1999
Bone marrow (BM) and/or peripheral blood progenitor cells (PBPC) given after high-dose chemo-radiotherapy are commonly cryopreserved. Re-infusion of the thawed product can cause cardiovascular and other complications. We compared two groups of adult patients receiving autologous BM or PBPC transplant to assess the incidence of adverse events occurring during infusion. ...
Tancabelic J - - 1999
In the management of patients requiring chronic transfusion, various parameters may be used to evaluate the degree of erythroid marrow suppression. The aim of our study was to assess which of these parameters provide the most useful assessment of erythropoiesis. We studied 27 chronically transfused patients, 19 with sickle cell ...
Vermi W - - 1999
The Wiskott-Aldrich syndrome (WAS) is a X-linked hematologic disorder characterized by thrombocytopenia, eczema, and immunodeficiency of variable severity. Reported here are the results of a morphologic, morphometric, and immunophenotypic analysis of splenic lymphoid tissue in 12 WAS patients with documented molecular defect and with different disease severity. Spleens from 29 ...
Faris P M - - 1999
Preoperative hemoglobin concentration may be an important predictor of transfusion risk in surgical procedures with significant expected blood loss. Contemporary studies investigating transfusion risk with regard to the relationship between perioperative administration of Epoetin alfa and baseline hemoglobin provide data to test this hypothesis. The predictive power of seven preoperative ...
Sidhu P S - - 1999
Pain due to bone marrow infarction is common in homozygous sickle cell disease (SS disease). Musculoskeletal pathology may be responsible for persistent atypical symptoms. We have assessed the frequency and ultrasound appearances of soft tissue and joint abnormalities in SS disease. SS disease patients with atypical musculoskeletal symptoms were examined ...
Mouélé R - - 1999
Clinical, hematological and molecular features of 116 unrelated sickle-cell anemia patients from Brazzaville were investigated. The mean age of the patients was 9.4 +/- 5.3 years. 232 beta(s)-chromosomes were haplotyped and almost all sickle cell anemia patients (91%) were homozygous for the Bantu haplotype. All hematological indices were similar for ...
Singer S T - - 1999
Cerebrovascular accident (CVA) is a major complication of sickle cell disease during childhood. Long-term transfusion reduces the hemoglobin S level and generally prevents recurrent stroke, but it also results in progressive iron overload that requires regular chelation therapy. Erythrocytapheresis offers an alternative approach aimed at reducing the iron accumulation. We ...
Miller A M - - 1999
Twenty-two patients with myelodysplastic syndrome were treated with combined recombinant human erythropoietin and recombinant human interleukin 3 (rHuIL-3). All 22 patients were evaluable for toxicity and 21/22 for response. Thirteen patients (62%) required rHuIL-3 dose reduction because of toxicity. Nineteen experienced a 50% or greater rise in neutrophil count. Of ...
Sokol R. J. - - 1999
52 patients (30 male, 22 female) with paroxysmal cold haemoglobinuria (PCH) were identified by critically examining the records of all cases with Donath-Landsteiner antibodies seen over a 37 year period. Although ages ranged from 1-82 years, PCH was much commoner in young children; the median age at presentation was 5 ...
Hutagalung R - - 1999
To determine if hemoglobin E trait influences the course of acute malaria, adults hospitalized for the treatment of symptomatic infection with Plasmodium falciparum were studied retrospectively. Forty-two patients with hemoglobin E trait were compared with 175 reference subjects who did not have hemoglobin E, beta-thalassemia, glucose-6-phosphate dehydrogenase deficiency, or alpha-thalassemia. ...
Biesma D H - - 1999
The correction of anemia in patients with chronic renal failure (CRF) has become the most important application of recombinant human erythropoietin (rHuEpo). The merits of rHuEpo therapy in patients with CRF are overt. Firstly, patients with CRF have an absolute deficiency in endogenous erythropoietin production and a relatively low maintenance ...
Haider M Z - - 1998
Chronic hemolysis, with consequent hyperbilirubinemia, predisposes SS patients to pigment gallstones. The other factors which influence the development of stones in these patients have not been identified. We have carried out a combined prospective and retrospective study of SS patients in Kuwait and specifically investigated the influence of coexistent alpha-thal ...
Economou-Petersen E - - 1998
In homozygous beta-thalassemia, the organ damage is mainly attributed to excessive iron deposition through the formation of oxygen free radicals. Despite appropriate transfusion and chelation therapy and low ferritin levels, patients still develop organ failure, heart failure being the main cause of death. This study was designed to determine whether ...
Schönhofer B - - 1998
OBJECTIVE: To study in anemic patients with chronic obstructive pulmonary disease (COPD) whether blood transfusion reduces minute ventilation and work of breathing (WOB). DESIGN: We prospectively evaluated the minute ventilation and WOB in 20 anemic adults (hemoglobin of <11 g/dL). Ten patients had severe COPD and ten patients were without ...
Childs R - - 1998
Nine of 56 (20% actuarial) patients receiving a T cell-depleted, HLA-identical sibling BMT for hematological malignancy developed hemorrhagic cystitis (HC) 15-368 days post BMT. Hematuria was severe and prolonged (median duration 18 days). In eight patients (89%), a viral etiology was confirmed (four adenovirus, four polyomavirus). HC was associated with ...
Bagasra O - - 1998
The spleen and lymph nodes are major sites of human immunodeficiency virus type 1 (HIV-1) replication, mutation, and genetic variation in vivo. If a major portion of the lymphatic tissue, such as the spleen, is removed or otherwise is unavailable for invasion by the HIV-1 virus, will the course of ...
Sesso R - - 1998
Patients with sickle cell anemia (Hb SS) or sickle cell trait (Hb AS) may present several types of renal dysfunction; however, comparison of the prevalence of these abnormalities between these two groups and correlation with the duration of disease in a large number of patients have not been thoroughly investigated. ...
Oshima Y - - 1998
A rare hemoglobin variant, Hb JLome, was identified by chance in a male patient with diabetes mellitus (DM). The patient had no evidence of anemia or hemolysis. However, when his glycated hemoglobin (Hb A1c) was examined by high-performance liquid chromatography (HPLC) to assess the state of his DM, an abnormal ...
Bando K - - 1998
BACKGROUND: Modified ultrafiltration (MUF) after cardiopulmonary bypass (CPB) in children decreases body water, removes inflammatory mediators, improves hemodynamics, and decreases transfusion requirements. The optimal target population for MUF needs to be defined. This prospective, randomized study attempted to identify the best candidates for MUF during operations for congenital heart disease. ...
Kjaergard H K - - 1998
BACKGROUND: The Vivostat System is a medical device for the preparation of an autologous fibrin sealant from 120 mL of the patient's blood in the operating room. The system is fully automated and microprocessor controlled and is made up of three components: an automated processor unit, an automated applicator unit, ...
Vitale M G - - 1998
Concerns about the transmission of the human immunodeficiency virus (HIV) have driven the evolution of surgical transfusion practices including the use of preoperative erythropoietin (rhEPO). Although there is significant experience documenting the efficacy of preoperative rhEPO in reducing transfusion requirements for adult patients, there is little experience in the pediatric ...
Rodríguez J N - - 1998
BACKGROUND AND OBJECTIVE: Experience with recombinant human erythropoietin (rHuEPO) in the treatment of the anemia secondary to myelofibrosis with myeloid metaplasia (MMM) is slight up to now. We present our results of the treatment of 6 patients and a review of the literature in search of possible parameters predicting response ...
Sadat-Ali M - - 1998
We prospectively evaluated the results of ultrasonography in 53 patients of sickle cell disease suspected to have vasoocclusive crisis/acute hematogenous osteomyelitis. The average age was 8.4 +/- 3.40 years (range, 1-14). Twenty-six children were boys and 27 were girls. Seventeen (32%) patients had ultrasonographic changes that suggested acute osteomyelitis. The ...
Dover G J - - 1998
Homozygous beta thalassemia affects thousands of people around the world. Current management of this condition includes regular transfusion of red cells, which leads to transfusional iron overload requiring chelation therapy: increasing hemoglobin levels while decreasing or eliminating iron overload is therefore a major therapeutic goal in the treatment of thalassemia. ...
Omonge E - - 1998
Cholelithiasis is a common clinical condition in patients with sickle cell disease and there are conflicting reports on laboratory indices useful in predicting those patients who are likely to have gallstones. There is however lack of similar studies from Kenya. We therefore studied the role of clinical (Body Mass Index), ...
Zavadil D P - - 1998
The physical process of extracorporeal membrane oxygenation (ECMO) results in derangement of the hemostatic mechanism, which may lead to increased morbidity, secondary to the disease process. The purpose of this study was to evaluate the hematological status of neonates undergoing ECMO therapy, and to evaluate coagulation tests in predicting hemorrhagic ...
Roeckel I E - - 1998
The AIDS crisis and the fear of blood product contamination stimulated the development of a designated plasma collection and transfusion for patients with common variable immune deficiency. Four patients have been maintained over 1 year; 5 years and 10 months; 8 years and 7 months; and 12 years. A new ...
Yung G L - - 1998
Patients with sickle cell disease have been reported to have an increased risk of thromboembolism and pulmonary hypertension. Some of these patients may benefit from pulmonary thromboendarterectomy (PTE), a procedure that requires profound hypothermia, cardiopulmonary bypass, and periods of circulatory arrest, factors that may potentially increase the risk of sickling. ...
Al-Salem A H AH Departments of Surgery, Internal Medicine, Pediatrics and Radiology, Qatif Central Hospital, Qatif, Saudi - - 1998
In patients with SCD, the spleen commonly enlarges during the first two decades of life but then undergoes autosplenectomy due to repeated attacks of vaso-occlusion and infarction. This, however, is not the case in Saudi patients with SCD, where splenomegaly sometimes persists into adult life. Ultrasonography was used to evaluate ...
Weiss R B - - 1998
PURPOSE: A report of the clinical features, treatment, and outcome of patients who developed hemolytic anemia (HA) temporally associated with fludarabine (Fludara; Berlex Laboratories, Richmond, CA) therapy for chronic lymphocytic leukemia (CLL). PATIENTS AND METHODS: Data on 24 patients who developed HA related to fludarabine therapy were collected from the ...
deHoll J D - - 1998
Herein we describe the use of intracavernous methylene blue (MB), a guanylate cyclase inhibitor, or internal pudendal artery embolization for the treatment of priapism. Eleven patients with priapism were treated from 1993-1996. Etiologies of priapism included PGE1/papaverine (3), trazodone (2), and sickle cell disease (1), in the other five cases ...
Moore C - - 1998
This study examined the presence of a persistent state of low-grade inflammation in sickle cell anemia patients by measuring circulating sHLA-I heterodimers and C-reactive protein during the steady state and after recent crises. Thirty-nine pediatric sickle hemoglobinopathy patients were studied during the steady state and 11 patients were evaluated within ...
Teach S J - - 1998
OBJECTIVE: To assess factors related to compliance with penicillin prophylaxis among patients with sickle cell disease. DESIGN: Prospective case series. SETTING: Urban pediatric medical center where universal penicillin prophylaxis is recommended for all patients with any sickle cell hemoglobinopathy independent of age. PARTICIPANTS: Eligible patients with sickle cell hemoglobinopathies were ...
Reinhardt D - - 1998
Reactivation of fetal hemoglobin synthesis in adulthood can be seen in hematological disorders affecting the erythropoietic system. The objective of the present study was to evaluate the incidence and prognostic significance of increased hemoglobin F in patients with myelodysplastic syndrome. Hemoglobin F concentrations and Ggamma/Ggamma + A gamma-globin chain ratios ...
Schönhofer B - - 1998
We report a case series of five anaemic patients (haemoglobin: 8.7 +/- 0.8 g.dl-1) with chronic obstructive lung disease in whom trials of weaning from the ventilator were unsuccessful. After transfer to our regional weaning centre, blood was transfused to increase the haemoglobin value to 12 g.dl-1 or higher. Subsequently, ...
Hoontrakoon S - - 1998
OBJECTIVE: To compare the efficacy of exchange transfusion as the adjunct to quinine treatment (21 patients) with quinine therapy alone (29 patients). METHOD: A retrospective study of 50 patients with severe falciparum malaria was conducted at Chumphorn Hospital, Southern Thailand. RESULTS: Clinical characteristics in both treatment groups were not significantly ...
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