Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the cases of 38 children with transfusion-associated HIV infection: 18 hemophiliacs and 2 patients with Von Willebrand disease, 6 with hemoglobinopathies, 8 with malignant diseases or aplastic anemia, 2 transfused during neonatal period and 2 during a surgical operation. Two groups with a different prognosis were found: In group A [hemophilia and Von Willebrand disease (n = 20)] 17 patients were asymptomatic or only with lymphadenopathy; 3 reached stage IV and none died. In group B [Others (n = 14)] 2 patients were asymptomatic, 4 reached stage IV and 8 died, 4 of them directly from AIDS. The difference between both groups was statistically significant. Prognosis of HIV infection is particularly severe in patients with leukemia, malignant tumors and aplastic anemia.
 ...
PMID:[Infection by the HIV virus and transfusions. Study of 38 pediatric cases]. 259 49

Bone marrow transplantation is increasingly used to treat a spectrum of diseases in man, including immune and genetic disorders, hematological diseases, and cancer. Approximately 11,000 transplants have been performed worldwide since 1970. About two-thirds of these transplants have involved donors, including related and unrelated individuals, and in the remaining third the patient's bone marrow has been used in the form of an autotransplant. In some disorders and under carefully defined circumstances, bone marrow transplantation appears to be the preferred therapy; these diseases include aplastic anemia, acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, and selected immune and genetic disorders. In other circumstances, the value of bone marrow transplantation is less well defined. Diseases in which bone marrow transplantation may be of benefit include Hodgkin's and non-Hodgkin's lymphoma, other cancers, thalassemia, hemoglobinopathies, genetic disorders, and possibly multiple myeloma. It has been difficult to precisely identify the role of bone marrow transplantation in many of these diseases. Prospective randomized controlled clinical trials have sometimes shown an advantage for bone marrow transplantation, but in most circumstances a benefit is as yet unproven. In the U.S. the annual incidence of individuals with diseases in which bone marrow transplantation is thought to be of proven benefit is approximately 5,400, and an additional 15,000 individuals annually have diseases in which bone marrow transplantation is thought to be of possible benefit. This study reviews data available from both controlled and uncontrolled clinical trials indicating the potential role of bone marrow transplantation in the treatment of human diseases.
 ...
PMID:Clinical trials of bone marrow transplantation. 352 45

A 27 year old male with aplastic anemia developed a high fetal hemoglobin, a low hemoglobin A2, a decreased beta/alpha synthetic ratio, and an increased G gamma/A gamma synthetic ratio. This acquired hemoglobinopathy resembling delta beta-thalassemia was recognized at the onset of acute erythroleukemia. Certain features of this abnormal globin synthetic pattern resemble those of the normal fetus and thus appear to provide another example of gene expression by malignant cells resembling that of an earlier stage of the organism's development.
 ...
PMID:Erythroleukemia manifesting delta beta-thalassemia. 618 18

It has been recommended that red blood cell transfusions to patients with hemoglobinopathy or aplastic anemia be matched for antigens other than ABO and Rho(D). We studied 1,010 patients with disorders that often lead to repetitive transfusion. The frequency of transfused patients with clinically important antibodies was not significantly different among the disease groups except for those with lymphocytic leukemia. The frequency of multiple red cell antibodies was about 3% overall. Most antibodies (71%) developed early in the transfusion course, before the 15th transfusion. From the standpoints of frequency of alloimmunization, multiplicity of antibodies, and time course of antibody development, patients with hemoglobinopathy and aplastic anemia were not significantly different from other transfused patients. Matching for antigens other than ABO or Rho(D) might increase costs in our hospital by 40,000-370,000 dollars per year for these patients. Because morbidity or mortality due to these antibodies is rare, antigen matching for other than ABO and Rho(D) is not cost-effective.
 ...
PMID:Should chronic transfusions be matched for antigens other than ABO and Rho(D)? 643 96

Patients receiving chronic transfusion for aplastic anemia or hemoglobinopathy are believed to be at high risk for developing red blood cell alloantibodies, while those undergoing chemotherapy for leukemia are believed to be at low risk. To test this hypothesis, we studied the acquisition of new alloantibodies in 703 transfused patients. While none of 99 patients with lymphocytic leukemia made new antibodies, patients with myelogenous leukemia (16%), hemoglobinopathy (29%), aplastic anemia (11%), gastrointestinal bleeding (11%) or renal failure (14%) made antibodies at statistically similar rates. Lymphocytic leukemia or its treatment is characterized by a lack of immunologic response to transfusion. Patients with hemoglobinopathy or aplastic anemia do not appear at statistically significant greater risk of alloimmunization than many other patients requiring chronic transfusion. Neither intensive chemotherapy for myelogenous leukemia nor the uremia of renal failure significantly suppress the formation of blood group antibodies.
 ...
PMID:Immune response to chronic red blood cell transfusion. 660 81

Our experience at the Ramathibodi Hospital with 20 infants and children who had Aeromonas septicemia is reviewed. Their ages were from 1 day to 14 years. Eighteen patients had underlying diseases: leukemia, 5; aplastic anemia, 4; cirrhosis, 2; thalassemia/hemoglobinopathy, 3; renal failure, 1; ileal perforation, 1; marasmus, 1; and cavernous hemangioma with thrombocytopenia, 1. Blood cultures yielded Aeromonas hydrophila in all patients, and four patients had polymicrobial bacteremia. Fifteen episodes of septicemia were community-acquired and five were hospital-acquired. The clinical manifestations of these patients were similar to septicemia due to other Gram-negative enteric bacilli. Two patients each had ecthyma gangrenosum, necrotizing fasciitis and meningitis. Antibiotic treatment included penicillins, cephalosporins, aminoglycosides and sulfamethoxazole-trimethoprim. The overall case fatality rate was 50%; eight of the nine patients with acute leukemia or aplastic anemia died. With the exception of one child the blood cultures were sterile in all patients before death. Aeromonas septicemia is an uncommon but severe infection which occurs predominantly in compromised hosts.
 ...
PMID:Aeromonas septicemia in infants and children. 672 2

In the years 1991 and 1992, 203 teams in 26 European countries performed 11026 BMT procedures: 4841 allografts and 6185 autografts; 10263 (93%) were performed in those 12 countries which did at least 100 transplants in total and five transplants per 1 million inhabitants per year during this 2-year period. Indications for these 10263 BMT were leukemia in 5080 patients (3294 allo-BMT, 1786 auto-BMT), lymphopro-liferative disorders in 3177 patients (329 allo-BMT, 2848 auto-BMT), solid tumors in 1236 patients (18 allogeneic-BMT), 1218 autologous-BMT), aplastic anemia in 305 patients and hemoglobinopathies or inborn errors of disease in 465 patients (456 allogeneic-BMT, 9 autologous-BMT). The proportion of allogeneic- and autologous-BMT, the main indications and the timing (first complete remission versus later stages) were compared between these 12 countries. Differences and similarities can be observed in all three aspects. They underline previously described differences in Europe which can partially be ascribed to different patient populations but may also be caused by divergent medical opinions.
 ...
PMID:EBMT survey on bone marrow transplant activity in Europe: regional differences. 792 Feb 92

The diverse manifestations of human parvovirus B19 infection have been well established. Erythema infectiosum, fetal hydrops, adult arthropathy, and aplastic anemia in patients with hemoglobinopathies or underlying immunocompromise have been described. Recently we successfully treated a patient who, after heart transplantation, had fever, rash, and pneumonia with respiratory failure caused by human parovirus B19. Human parovirus B19 has not been reported previously as a pathogen causing pulmonary disease after pediatric heart transplantation, and we wish to report it at this time.
 ...
PMID:Severe pneumonia after heart transplantation as a result of human parvovirus B19. 803 18

The characteristic spectrum of infections in patients with aplastic anemia, chronic neutropenic diseases, sickle cell disease, thalassemia, and other hemoglobinopathies are described. The major risk factor for infection in patients with bone marrow failure is the degree of neutropenia and monocytopenia. In patients with aplastic anemia, invasive fungal infections emerge as the major causes of mortality. Life-threatening infections are rare in patients with chronic neutropenic diseases; however, necrotizing enterocolitis due to Clostridium species may be an exception. Bacterial infections, predominantly with encapsulated bacteria, are the most common cause of death in patients with sickle cell disease, especially those who are younger than 5 years of age. Patients with thalassemia and other hemoglobinopathies are particularly susceptible to life-threatening infections with Yersinia enterocolitica as a result of iron overload or of the chelating therapy with desferrioxamine.
 ...
PMID:Approach to management of fever and infection in patients with primary bone marrow failure and hemoglobinopathies. 835 59

The past year's important advancements in the ocular manifestations of cardiovascular and hematologic diseases are reviewed. Systemic changes related to blood pressure and carotid artery disease are commonly manifested on the ophthalmic examination. Ocular ischemic changes have been observed in patients with unusual aortic or congenital cardiac disorders. The ophthalmologist plays an important role in the detection of patients who are at risk for cardiovascular and cerebrovascular events. The hematologic disorders affecting the eye are subdivided into benign and malignant categories. A spectrum of diseases that include sickle cell hemoglobinopathy, aplastic anemia, leukemia, lymphoma, and multiple myeloma are discussed. With increased patient survival time after bone marrow transplantation, secondary ophthalmic complications are being identified. Although the ocular findings in the hematologic maladies are diverse, they may be an indication of serious systemic complications and have significant visual consequences.
 ...
PMID:Ocular manifestations of cardiovascular and hematologic disorders. 1015 Aug 35


1 2 3 Next >>