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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Iron deposits in ethionine induced aortic siderosis of rats, in splenic deposits in sickle cell anemia and siderocalcific vessels in cerebral arteriosclerosis were completely removed by exposure to 20 percent hydrochloric acid for 30 min. This contrasted with idiopathic hemochromatosis and idiopathic pulmonary hemosiderosis in which the iron containing organs had to be exposed to 40 percent hydrochloric acid for two hours. The more soluble iron appeared colorless in unstained tissues, purplish blue with hematoxylin and eosin, turquoise blue with Perls' stain, violet blue with gallocyanin and dark-drown with sodium rhodizonate. The less soluble iron was golden yellow in unstained tissues. It appeared golden yellow with hematoxylin and eosin and sodium rhodizonate, but it stained greenish blue with Perls' method and dark brown with gallocyanin. Lead and copper were capable of deposition in some tissues in vitro in the presence of iron and/or calcium but not when these minerals were removed. This phenomenon may be of importance in certain pathological conditions, e.g. hemochromatosis, where on preexisting tissue-iron-complexes there is a secondary deposition of copper.
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PMID:Staining of minerals and solubility of iron in tissues. 6 77

Systemic salmonellosis is a recognized complication of sickle cell anemia (SCA). In our initial study of SCA host defences against salmonella, we evaluated the bactericidal activity of serum against Salmonella typhimurium. When compared to controls, sera from eight out of nineteen SCA patients were deficient in bactericidal function. Levels of factor B, haemolytic complement and agglutinating antibody were similar in SCA and control sera. However, abnormalities that might theoretically account for the decreased antibacterial activity were observed in many SCA sera. These abnormal findings included: (a) defective function of the alternative complement pathway (decreased bacterial killing in the presence of Mg EGTA); (b) low serum C3 concentration; and (c) decreased total iron-binding capacity (TIBC), with a resultant increase in per cent saturation of iron-binding capacity. Of these deficiencies only the abnormal alternative pathway function was significantly associated with decreased serum bactericidal activity. A suggested function of serum bactericidal activity is prevention of bacteraemia by susceptible organisms. Thus diminished serum bactericidal capacity may increase the risk of Salmonella bacteraemia in some individuals with sickle cell disease.
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PMID:Deficiency of serum bactericidal activity against Salmonella typhimurium in sickle cell anaemia. 34 54

Serial microscopic immunodiffusion assays of F cells, i.e., erythrocytes that contain fetal hemoglobin (HbF), in four individuals recovering from anemia demonstrate initial increases in the percentage of circulating reticulocytes that contain HbF (F reticulocytes) and subsequent increases in the percentage of mature erythrocytes that contain HbF (F erythrocytes). In one individual responding to therapy for iron-deficiency anemia, the average percentage of F reticulocytes increased from 4.8+/-1.1 to 16.0+/-2.8% (mean+/-SD), while the mean level of F erythrocytes increased from 3.5+/-0.7 to 7.2+/-0.6%. Two normal children with transient erythroblastopenia exhibited F reticulocyte percentages of 71.3+/-6.7 and 41.5+/-1.5%, respectively, when erythropoiesis resumed. With recovery these values fell to finally measured values of 33.7+/-4.7 and 12.6+/-1.1%, respectively. In an adolescent with sickle cell anemia, F-reticulocyte percentages fluctuated between 0.6+/-1.1 and 34.0+/-2.8% and paralleled the rise and fall of total reticulocytes associated with therapy for a nasopharyngeal carcinoma. Such findings suggest that first, the production of F cells and non-F cells are separately regulated. Second, F-cell production is preferentially stimulated during escape from erythropoietic suppression and selectively depressed at the start of suppression. Third, during escape from erythropoietic suppression, F-cell production in vivo resembles that reported for in vitro cultures of erythroid stem cells. Fourth, individuals with sickle cell anemia, like individuals without hemoglobinopathies, can change their relative level of F-cell production.
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PMID:Production of erythrocytes that contain fetal hemoglobin in anemia. Transient in vivo changes. 42 49

Splenic uptake of 99mTc-diphosphonate was found on the bone scan of a patient with sickle cell disease. No splenic activity was demonstrated on 99mTc-sulfur colloid liver-spleen scans and no evidence of splenic calcification was seen on plain abdominal radiographs. A computerized transaxial tomographic scan, however, did demonstrate a small spleen denser than other abdominal organs. The splenic accumulation of 99mTc-diphosphonate and the increased density on computerized transaxial tomography could be attributed to microscopic splenic calcifications and/or iron deposits in the spleen.
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PMID:Splenic uptake of 99mTc-diphosphonate in sickle cell disease associated with increased splenic density on computerized transaxial tomography. 61 7

Iron chelation with deferoxamine was studied in ten patients with sickle cell anemia who had received 2 to 37 liters of red blood cells. Urinary excretion of iron in response to 1.5 gm of deferoxamine administered intravenously ranged from 5.9 to 28.7 mg/24 hours and was closely related to the amount of iron acquired from transfusions. Administration of ascorbic acid did not improve deferoxamine-induced excretion of iron. Urinary excretion of iron in response to 0.75 gm of DFO intramuscularly was 4.7 to 6.9 mg/24 hours in three patients who had received 15 to 37 liters of red cells. The data indicate that measurement of DFO-induced excretion of iron is of value in detecting increased iron stores in children with sickle cell anemia who have received repeated transfusions and that chelation therapy will retard the accumulation of iron.
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PMID:Excretion of iron in response to deferoxamine in sickle cell anemia. 63 13

Total body iron burden was estimated by two indirect methods in 23 patients with sickle cell anemia. Concentrations of serum ferritin correlated directly and significantly with age of the patients. Eleven of 15 patients under 20 years of age had normal levels of serum ferritin. Deferoxamine-induced urinary excretion of iron was considerably less than that reported in patients with thalassemia major who were receiving regular blood transfusions. These data imply that patients with sickle cell anemia generally do not acquire excessive iron burdens during the first two decades of life. The risks of transfusional hemosiderosis in patients with sickle cell anemia who are included in hypertransfusion programs are discussed.
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PMID:Iron burden in sickle cell anemia. 63 16

In a group of 35 children with sickle cell anaemia serum ferritin concentration ranged from 70 to 2460 microgram/l (mean 367, median 180 microgram/l). This was significantly higher than the ferritin levels (range 8-101, mean 34, median 30 microgram/l) in a group of 63 normal control children of the same age group. 30 (86%) of the sickle cell children showed serum ferritin levels greater than 101 microgram/l, and 2 (6%) levels greater than 1000 microgram/l. 7 of the patients had not been transfused before this study. Their serum ferritin levels were all raised and showed a significant correlation with age but not with haemoglobin level. In the remainder of the patients the serum ferritin bore no significant correlation with age, haemoglobin level, or number of units of blood transfused. 2 children with HbSC disease had levels within the control range. Since patients with sickle cell anaemia have an increasing chance of long survival, we suggest that serial estimations of their iron status be made by means of serum ferritin assay in order to determine which patients are accumulating excessive iron.
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PMID:Value of serum ferritin estimation in sickle cell anaemia. 64 43

Globin synthesis studies are useful in the analysis of thalassemia syndromes. We have applied globin synthesis and free alpha-chain pool studies of peripheral blood to characterize hematologic disorders where alpha- or beta-thalassemia was present in combination with HbS or HbC. In 60 non-thalassemic controls, the beta/alpha specific activity ratio was 1.01 +/- 0.06 (SD). In three patients with HbS-beta0-thalassemia, the (betas + gamma)/alpha ratios were 0.48-.067. In four patients with HbSS-alpha-thalassemia, the (BETAS/ALPHA RATIO was 1.26 +/- 0.18 (1.13-1.53). The radioactive free alpha-chain pool in three patients with HbS-beta0-thalassemia was elevated (35.1%-53.0%), while three patients with HbSS-alpha-thalassemia had decreased free radioactive alpha-chain pools (3.2%-6.4%); both were significantly different from the mean (15.1% +/- 2.6%) of the 17 iron-sufficient controls. Simultaneous studies of the fraction of newly synthesized alpha chain contained in the free alpha-chain pool in peripheral blood and bone marrow demonstrated that this fraction was larger in peripheral blood than in marrow, and that the differences between thalassemia patients and controls previously found in bone marrow using these methods were also present in peripheral blood. The results indicate that even when family studies are not possible, patients with HbS in combination with alpha- or beta0-thalassemia can be differentiated from those with homozygous sickle cell disease by globin synthesis and free alpha-chain pool studies using peripheral blood.
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PMID:Detection of sickle alpha- or beta0-thalassemia by studies of globin biosynthesis. 85 59

The variability in the clinical expression of sickle cell anemia led us to study factors which might influence the course of this disease. We examined erythrokinetics, blood volumes, and variables which influence hemoglobin function in a group of adults with sickle cell anemia of varying degrees of clinical severity. We were unable to correlate any single measurement with the clinical course; however, our patient sample was small and the data suggested areas for further study. An expansion of plasma volume noted in all patients. This made it difficult to predict red cell mass from the hemoglobin level, which consistently underestimated its magnitude. The red cell production index and iron turnover values indicated that there is often a suboptimal erythropoietic response to anemia in sickle cell disease.
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PMID:Sickle cell anemia: erythrokinetics, blood volumes, and a study of possible determinants of severity. 86 65

Many complex forces are at work during adolescence which can contribute to the occurrence of anemia. Careful consideration of the entire patient should enable a physician to identify the unusual as well as the more common causes of anemia in this age group. Anemia in teenage girls is primarily due to menstrual iron loss. In boys, borderline diets and the demands of rapid growth predominate as causative factors. Hemoglobinopathies (thalassemia, sickle cell disease), G6PD deficiency, infectious mononucleosis, and illicit drug use account for small proportions of cases.
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PMID:Anemia in adolescence. 2. Hemoglobinopathies and other causes. 110 36


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