Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with sickle cell anemia was hospitalized because of an acute erythropoietic aplastic crisis. 111In chloride bone marrow scintigraphy performed during the aplastic crisis showed markedly impaired uptake of the radionuclide and simultaneous 59Fe clearance T 1/2 studies were prolonged (420.9 min). Following recovery from the aplastic crisis 111In scintigraphy revealed an expanded marrow and the radioiron clearance T 1/2 was accelerated (20.9 min). The clinical course, hemograms and bone marrow aspirations correlated with marrow scintigraphy. 111In appears to be a valuable marrow scanning agent for assessing the presence of intramedullary erythropoietic activity.
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PMID:111In chloride bone marrow scintigraphy and ferrokinetic studies in a case of sickle cell anemia with transient erythroid aplasia. 11 5

The survival characteristics of human sickle (SS) erythrocytes (RBCs) transfused to intact chimpanzees were determined. The mean post-transfusion recovery of 51Cr-labelled SS RBCs in four chimpanzees was 30.5% +/- 15.2 SD, and the half-life survival was 4.2 h +/- 0.8 SD. The recovery of control (hemoglobin AA) human red cells in five chimpanzees was complete and their mean intravascular T 1/2 was 22.3 h. Shorter survival of sickle erythrocytes was also shown by transfusing chimpanzees with mixtures of human cells such as 51Cr AA RBCs and 59Fe SS RBCs, or 51Cr SS RBCs and non-labelled fetal (cord blood) erythrocytes. The difference in survival of AA and SS RBCs resembles that in human recipients and was probably caused by sickling of SS cells in the chimpanzee circulation. These primate animals could, therefore, be used as a model for in vivo studies of sickle cell disease.
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PMID:Human sickle erythrocytes: survival in chimpanzees. 713 31

An alloantibody to a high-incidence antigen, associated with multiple other alloantibodies, made it impossible to supply antigen-negative red blood cells (RBCs) for a chronically transfused sickle cell anemia patient. Anti-Cra,-E,K,-S, -Fya, -Fyb, as well as anti-M reactive at 37 degrees C and in the antiglobulin phase of testing, were identified in the patient's serum. An extensive search of rare donor files at the American Red Cross and at the American ASsociation of Blood Banks (AABB) failed to identify Cr(a-),M-,E-,K-,S-, Fy(a-b-) donors. Various studies were performed to predict the clinical significance of the anti-Cra and anti-M. Results of 51chromium survival studies showed 91.8 percent survival at 10 minutes and 87.2 percent survival at 60 minutes with Cr(a +),M-, K-,S-,Fy(a-b-) donors. Various studies were performed to predict the clinical significance of the anti-Cra and anti-M. Results of 51chromium survival studies showed 91.8 percent survival at 10 minutes and 87.2 percent survival at 60 minutes with Cr(a +),M-,E -,K-,S-,Fy(a-b-) red cells, suggesting that immediate destruction of transfused CrCa+) red cells would he unlikely. However, further analysis revealed diminished long-term survival of the donor's red cells with only 60.1 percent recovery at six days (T 1/2 = 12 days) and 10.8 percent at 14 days (T 1/2 = 4.5 days). A monocyte- monolayer assay (MMA) indicated that both the anti-Cra (5.9%) and anti-M (18%) would probably be clinically significant (normal value 0-3%). Mass screening continues at several blood centers for Cr(a-),M-, E-,K-,S-,Fy(a-b-) donors. However, if no suitable donors are found, the results of the 51chromium survival studies and the MMA support the decision to transfuse this patient with Cr(a+),M-,F(a- b-),S-,K- ,E- red cells, if necessary.
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PMID:Assessing the clinical significance of anti-Cra and anti-M in a chronically transfused sickle cell patient. 1594 61