Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During 1990 143 teams in 20 European countries performed a total of 4234 bone marrow transplants. Donor source was in 1802 cases an HLA-identical family donor, in 118 cases a non-identical family donor, in 36 cases a twin donor, in 181 an unrelated volunteer donor and in 2097 cases autologous bone marrow. Indications for the transplant were leukaemias in 2365 patients, lymphoproliferative disorders in 1104 patients, solid tumours in 382 patients, aplastic anaemia and thalassaemia in 281 patients, inborn errors in 58 patients and miscellaneous disorders in 44 patients. This survey of the European Group for Bone Marrow Transplantation on transplant activity clearly indicates that within the last decade bone marrow transplantation has become an established therapy in Europe. The results of this first complete survey provide a basis for planning therapeutic trials and health policy strategies.
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PMID:Bone marrow transplantation activity in Europe 1990. European Group for Bone Marrow Transplantation (EBMT) 151 Dec 60

Uric acid is the end-product of purine nucleotide metabolism in man. The renal handling of urate is a complicated process, resulting in a fractional clearance of 8.2-10.3%. The anhydrous form is thermodynamically the most stable uric acid crystal. Uric acid is a weak acid that ionizes with a Pka at pH 5.75. At the normal acidic region, uric acid solubility is strongly increased by urinary pH. The prevalence of uric acid stones varies between countries, reflecting climatic, dietary, and ethnical differences, ranging from 2.1% (in Texas) to 37.7% (in Iran). The risk for uric acid stone formation correlates with the degree of uric acid supersaturation in the urine, depending on uric acid concentration and urinary pH. Hyperuricosuria is the major risk factor, the most common cause being increased purine intake in the diet. Acquired and hereditary diseases accompanied by hyperuricosuria and stone disease include: gout, in strong correlation with the amount of uric acid excreted, myelo- and lymphoproliferative disorders, multiple myeloma, secondary polycythemia, pernicious anemia and hemolytic disorders, hemoglobinopathies and thalassemia, the complete or partial deficiency of HGPRT, superactivity of PRPP synthetase, and hereditary renal hypouricemia. A common denominator in patients with idiopathic and gouty stone formers is a low urinary pH. Uric acid nephrolithiasis is indicated in the presence of a radiolucent stone, a persistent undue urine acidity and uric acid crystals in fresh urine samples. A radiolucent stone in combination with normal or acidic pH should raise the possibility of urate stones.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Uric acid nephrolithiasis. 778 6

In the year 1992, 203 teams in 26 countries in Europe performed a total of 6065 bone marrow transplantations (BMT). Transplant source in 2666 cases (44%) was an allogeneic donor, in 2171 cases an HLA-identical sibling donor, in 170 a non-identical family donor, in 29 a twin donor and in 296 cases an unrelated volunteer donor. There were 3399 autologous transplants (56%): 2494 autologous BMT, 644 autologous peripheral blood stem cell transplants and 261 combined autologous BM and peripheral blood stem cell transplants. Indications for transplant were leukemias in 2963 patients (49%; 1987 allogeneic, 976 autologous), lymphoproliferative disorders in 1890 patients (31%; 201 allogeneic, 1689 autologous), solid tumors in 739 patients (12%; 10 allogeneic, 739 autologous), aplastic anemia in 194 patients (3%; 193 allogeneic, 1 autologous), thalassemia in 128 patients (2%; allogeneic), inborn errors in 115 patients (2%; allogeneic) and miscellaneous disorders in 36 patients (32 allogeneic, 4 autologous). Compared with an EBMT survey 2 years ago, there was an increase in the number of participating institutions and in the number of transplants performed from all sources. If the 142 teams reporting in 1990 and 1992 are compared alone, there is an increase in unrelated allogeneic BMT and in autologous BMT. There was an increase in autologous transplants for myeloma and lymphoma. These data confirm a continuing trend to apply BMT as a therapeutic modality.
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PMID:Bone marrow transplantation activity in Europe 1992: report from the European Group for Bone Marrow Transplantation (EBMT). 801 52

Today, bone marrow transplantation (BMT) is an established therapy. This statement is best verified by the number of BMTs performed. Between January 1990 and December 1992, 172 European teams in 26 countries carried out a total of 14,334 transplants. There were 6642 allogeneic transplants: 5513 BMT from an HLA-identical sibling donor, 370 from a non-identical family member, 88 from an identical twin donor and 671 from an unrelated volunteer donor. There were 7692 autologous transplants: 6577 autologous bone marrow, 777 peripheral-blood stem-cell and 338 combined bone-marrow and peripheral-blood stem-cell transplants. Indications were: leukaemias in 52% (7479), lymphoproliferative disorders in 29% (4125), solid tumours in 11% (1540), aplastic anaemia and thalassaemia in 3% (487) and inborn errors an miscellaneous disorders in the remaining 5% (703). The results of these transplants are not yet known. From previous analyses it can be expected that more than 50% of patients will be alive and well 10 years after BMT. The main factors influencing outcome are known; they depend on type, sub-type, stage of disease at time of transplant, the time from diagnosis to transplant and the conditioning regimen for all transplants. For allogeneic BMT, donor source, donor and recipient age, sex, donor/recipient sex combination, donor and recipient viral status, graft-versus-host disease prevention method and region are additional factors. Knowledge of these factors enables us today to estimate the potential risk and adjust the therapy for an individual patient.
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PMID:Bone marrow transplantation today. 815 55

In 1991 171 teams in 21 European countries performed 4976 bone marrow transplants. There were 1829 transplants from an HLA-identical sibling donor, 101 from a nonidentical family member, 30 from a twin, 217 from an unrelated volunteer donor and 2799 autologous transplants. Indications for transplants were leukaemias in 2569 (52%), lymphoproliferative disorders in 1472 (30%), solid tumours in 549 (11%), aplastic anaemia and thalassaemia in 261 (5%), inborn errors in 97 and miscellaneous disorders in 28 patients. There are marked differences between the participating European countries. They relate to absolute numbers, indications and techniques. Less than 10 transplants per 10-million inhabitants are performed in Eastern European countries. Ten to fifty transplants per 10-million inhabitants are done in two, 50-100 transplants per 10-million inhabitants in five, 100-200 transplants per 10-million inhabitants in eight countries and more than 200 per 10-million inhabitants in one country (P < 0.01). The number of transplant teams ranges from less than one to twelve per 10-million inhabitants (P < 0.01). For the continent it becomes 3.3 per 10-million inhabitants. The reasons for these differences are not explained by this survey. The most likely explanation for the differences in transplant activity is availability of transplant beds, trained staff and resources. Bone marrow transplants are expensive. However, the demand is increasing and will increase further with the routine availability of unrelated volunteer donors. Clearly, criteria are required in Europe to define the indications and solutions to meet the legitimate requirements for transplantation in the various regions of Europe.
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PMID:Bone marrow transplantation in Europe: major geographical differences. The European Group for Bone Marrow Transplantation [EBMT]. 846 62

The European Group of Blood and Marrow Transplantation (EBMT) is collecting information on incidence of bone marrow transplantation in Europe. 203 teams in 26 European countries performed in 1992 a total of 6065 bone marrow transplants (BMT). There were 2666 (44%) allogeneic transplants from 2171 HLA identical sibling donors, 170 non-identical family donors, 29 twin donors and 296 unrelated volunteer donors. There were 3399 autologous transplants (56%): 2494 autologous BMT, 644 autologous peripheral blood stem cell transplants and 261 combined autologous bone marrow peripheral blood stem cell transplants. Transplant indications were leukemias in 2963 patients (49%) (1987 allogeneic, 976 autologous), lymphoproliferative disorders in 1890 patients (31%) (201 allogeneic, 1689 autologous), solid tumors in 739 patients (12%) (10 allogeneic, 739 autologous), aplastic anemia in 194 patients (3%) (193 allogeneic, 1 autologous), thalassemia in 128 patients (2%) (all allogeneic), inborn errors in 115 patients (2%) (all allogeneic) and miscellaneous disorders in 36 patients (32 allogeneic, 4 autologous). Main differences were observed in the number of transplanting teams, and in the number of allogeneic and autologous transplants performed in the different European countries compared to the number of inhabitants.
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PMID:Incidence of bone marrow transplantation in Europe. Report from the European Group for Blood and Marrow Transplantation. 874 19

A review of the literature during the past year on rheumatic manifestations in hematologic diseases supports the idea that 80% of the hemorrhage in hemophilia occurs within the joints, with knees, elbows, and ankles being the most affected joints in adults. In contrast, the ankle is the target joint in children. Septic arthritis in hemophilic patients is becoming more important due to the advent of HIV infection. Radioactive synoviorthesis in hemarthrosis has the same rate of success as surgical synovectomy, but with far lower costs. A new study documents the association of arthritis and vasculitis in patients with myelodysplasic syndromes and lymphoproliferative disorders. An increased incidence of scoliosis in patients with beta-thalassemia has been noted. Finally, the effects of bone marrow transplantation in patients with previous autoimmune diseases is reviewed. Progression of rheumatoid arthritis after bone marrow transplantation is documented in a patient with 13 years of follow-up. Hematologic disorders in rheumatic diseases are not the topic of this review.
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PMID:Rheumatic manifestations of hematologic disorders. 944 95

Hematopoietic stem cell transplantation (HSCT) is a highly effective treatment strategy for lymphoproliferative disorders and bone marrow failure states including aplastic anemia and thalassemia. However, its use has been limited by the increased treatment related complications, including acute kidney injury (AKI) with an incidence ranging from 20% to 73%. AKI after HSCT has been associated with an increased risk of mortality. The incidence of AKI reported in recipients of myeloablative allogeneic transplant is considerably higher in comparison to other subclasses mainly due to use of cyclosporine and development of graft-versus-host disease (GVHD) in allogeneic groups. Acute GVHD is by itself a major independent risk factor for the development of AKI in HSCT recipients. The other major risk factors are sepsis, nephrotoxic medications (amphotericin B, acyclovir, aminoglycosides, and cyclosporine), hepatic sinusoidal obstruction syndrome (SOS), thrombotic microangiopathy (TMA), marrow infusion toxicity, and tumor lysis syndrome. The mainstay of management of AKI in these patients is avoidance of risk factors contributing to AKI, including use of reduced intensity-conditioning regimen, close monitoring of nephrotoxic medications, and use of alternative antifungals for prophylaxis against infection. Also, early identification and effective management of sepsis, tumor lysis syndrome, marrow infusion toxicity, and hepatic SOS help in reducing the incidence of AKI in HSCT recipients.
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PMID:Acute Kidney Injury in Hematopoietic Stem Cell Transplantation: A Review. 2788 40