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

The red cell distribution width index (RDW) was determined in 103 normal children, 69 iron-deficient (ID) patients, 73 with the thalassaemia trait, and 71 with other haemoglobinopathies. Elevated RDW values were found in anaemic patients, the highest values in ID anaemia, sickle thalassaemia, sickle cell anaemia, and beta-thalassaemia trait in decreasing order. The normal RDW in children was 13.2 +/- 0.9 and an elevated RDW reflects active erythropoiesis. The RDW was elevated and the MCV low in all 69 patients with ID anaemia. The RDW was also elevated in 11/13 children with sickle cell anaemia, in 25/29 patients with sickle cell-thalassaemia, and in all patients with thalassaemia major. The ID anaemia could be differentiated from the thalassaemia trait by the markedly elevated RDW in ID anaemia (mean = 20.7 +/- 3.2) and the mildly elevated RDW (mean 15.4 +/- 1.4) in thalassaemia trait. The RDW index discriminated better than Mentzer's index, discriminant function or the Shine Lal index. Thus the RDW is a good indicator of anisocytosis, and a good screening index especially for ID anaemia and the thalassaemia trait.
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PMID:The value of red cell distribution width in the diagnosis of anaemia in children. 279 25

Fourteen pregnant women with sickle cell disease (11 SS and 3 SC) were randomized into two groups to receive routine antenatal supplementation either with ferrous gluconate or with placebo tablets. Their hemoglobin levels and bone marrow iron content were determined prenatally and 6 weeks post partum. The fetal weights and the incidence of pain crisis in both groups were recorded. Using an iron content grading from 0 to 5, no marrow of any subject showed iron depletion. The placebo group showed an aggregate postnatal loss of 4 grades of iron repletion while the iron supplemented group showed an aggregate gain of 2 grades. There were no significant differences between the birth weight or the incidence of pain crises in both groups. We conclude that routine iron supplementation is not justified in pregnant women with sickle cell disease, as it would tend to increase already adequate or excessive iron body stores. We recommend that a clear need for iron should be established before iron supplementation is prescribed to them.
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PMID:Antenatal iron supplementation in sickle cell disease. 289 99

Non-transferrin-bound iron (NTBI), a potentially toxic compound, is increased in the serum of patients with iron overload and fully saturated transferrin. We found markedly elevated NTBI levels in 16 children (including nine with sickle cell disease and five with beta-thalassemia) with iron overload secondary to prolonged transfusion therapy. During iron chelation with subcutaneous desferrioxamine infusion, NTBI levels decreased to normal, but became elevated within 2 to 4 hours after discontinuation of desferrioxamine. NTBI causes hepatic and cardiac toxicity in experimental systems, but our patients lacked sufficient organ dysfunction for this association to be made. The use of continuous 24-hour chelation to maintain NTBI at low levels may prevent progressive iron toxicity in patients who first received chelation therapy at an older age or who already have evidence of cardiac damage.
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PMID:Non-transferrin-bound iron in long-term transfusion in children with congenital anemias. 308 76

Even though the development of the membrane abnormalities in sickle cell erythrocytes is due to the presence of a mutant gene product, sickle cell anaemia is in a sense a membrane disease. In particular, the presence of iron-containing breakdown products of haemoglobin in the sickle cell membranes provides a source of continued oxidative damage. An understanding of the primary causes of these membrane abnormalities may be useful in the development of effective therapies.
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PMID:Role of membrane-bound haemoglobin products in oxidative damage in sickle cell membranes. 312 Apr 54

The relationship between the number of units of blood transfused and indicators of iron status in 37 patients with sickle cell anaemia (Hb SS), SC disease (Hb SC) or S beta-thalassaemia has been studied. The correlation coefficient between serum ferritin and the number of units transfused was good (r = 0.86), provided that ferritin samples taken within one week following a crisis were excluded. The relationship of transfusion history to serum ferritin in the steady state showed a similar relationship to that previously observed for other multiply transfused patients. The serum ferritin taken within 7 days of a painful crisis was significantly greater than the serum ferritin from the same patients in the steady state (p less than 0.025). The serum alanine transaminase did not rise as consistently as the serum ferritin during crises; it correlated with the serum ferritin but not the transfusion burden in the steady state. Transferrin iron saturation correlated less clearly with transfusion history than serum ferritin (r = 0.62). Patients who had received exchange transfusions were less likely to be iron-overloaded (ferritin increment per unit of blood = 9.9 +/- 3.8 micrograms/l) than patients who had received an equivalent number of units by conventional transfusion (ferritin increment per unit of blood transfused = 25.1 +/- 2.42 micrograms/l).
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PMID:Transfusion and exchange transfusion in sickle cell anaemias, with particular reference to iron metabolism. 312 Apr 72

This paper reviews the factors governing the rate of iron loading and iron toxicity in the thalassaemia syndromes and sickle cell disease. It outlines the main determinants of iron mobilization by the iron-chelating drug, desferrioxamine, together with the effects of this drug in clinical practice.
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PMID:Iron overload and iron chelation therapy in thalassaemia and sickle cell haemoglobinopathies. 312 Apr 73

Children and young adults with hemolytic anemias requiring frequent transfusions develop increased liver iron content. We evaluated 15 chronically transfused children with sickle cell disease to determine whether spin-echo magnetic resonance (MR) imaging was useful in assessing the degree of iron overload. Quantitative MR parameters were correlated with liver biopsy iron determinations and serum ferritin levels. The best predictor of liver iron was the ratio of the intensities between the liver and paraspinal musculature on somewhat T1 weighted sequence (repetition time 0.5 s, echo time 28 ms), R2 = 0.58. Magnetic resonance was able to separate those patients with liver iron levels greater than 100 micrograms/mg (intensity ratios approximately 0.4), from those with levels less than 100 micrograms/mg (intensity ratios near 1). However, MR was unable to quantitate liver iron in patients with values ranging from 100 to 400 micrograms/mg since similar intensity ratios were present in this range. Thus, MR provides a qualitative rather than quantitative assessment of liver iron overload.
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PMID:MR evaluation of liver iron overload. 333 77

The adequacy of tissue iron supply was examined with ferrokinetic techniques in subjects with decreased plasma iron concentration and in subjects with a normal plasma iron concentration but with increased tissue iron requirements. The competition by transferrin receptors for diferric vs monoferric transferrin was measured in eight normal persons and eight with iron deficiency. There was a highly significant (P less than 0.001) decrease in receptor preference for diferric transferrin in subjects with iron deficiency, indicating an insufficient amount of iron-bearing transferrin to saturate tissue receptors. The adequacy of the plasma iron supply was also examined by determining the number of iron-bearing transferrin molecules with receptors at normal and elevated plasma iron concentrations. Significant increases were found at the higher plasma iron concentration, not only in patients with iron deficiency, but also in patients with sickle cell anemia and thalassemia. Furthermore, the increase in the latter two groups was shown to be proportional to the degree of erythroid hyperplasia. These data indicate that tissue iron supply must be evaluated in terms of both plasma iron supply and erythropoietic requirements and that a relative iron deficiency is frequent in patients with erythroid hyperplasia.
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PMID:Adequacy of iron supply for erythropoiesis: in vivo observations in humans. 368 Nov 15

A patient with sickle cell disease and multiple alloantibodies required frozen storage of autologous red cells (RBC) to provide the RBC volume equivalent of 6 units of normal donor blood for an orthopedic surgical procedure. Eleven 600-ml donations were made at 4-week intervals, without significant change in hemoglobin concentration, hematocrit, or reticulocyte count, and with only small decreases in serum iron and ferritin concentrations. Minor modifications of a glycerolization and deglycerolization procedure described recently were used. We report in vitro recoveries of deglycerolized RBC from the 11 units following frozen storage for 27 to 391 days. Despite use of a single donor and adherence to a standardized processing protocol, considerable variation in the percent in vitro RBC recovery was observed (51-81%). In vivo survival of an aliquot following frozen storage for 27 days was identical with fresh autologous RBC (100% at 1 hr, 88% at 24 hr, one-half disappearance of 7.5 days). Autologous transfusion of RBC recovered from 7 units after storage for as long as 126 days was uneventful.
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PMID:Frozen storage of 11 units of sickle cell red cells for autologous transfusion of a single patient. 372 9

As a clinical entity, sickle cell anemia (SCA) is known to be relatively rarer in Africans than in the African-American population of the United States. Paradoxically, sickle cell trait (SCT), the non-anemic, heterozygous condition, is about three times more common among indigenous Africans than in African-Americans. The ratio of SCA to SCT is 1:50 for African-Americans, and less than 1:1,000 for tropical Africans. This etiological disparity is attributed to an anti-sickling agent, thiocyanate, (SCN-) found abundantly in staple African foods, such as the African yam (Dioscorea sp) and cassava (Manihot utilissima). Staple American foods have negligible SCN-concentrations. Nonstaple foods in the American diet, such as carrots, cabbage, and radishes, have SCN- levels far below the African yam and cassava. This finding explains the high incidence of SCA among African-Americans and its rarity in Africans.The author concludes that SCA is a congenital deficiency anemia, ameliorable by prophylactic diets of foods with high SCN- contents. Thus, "thiocyanate deficiency anemia" is nutritionally a more correct clinical status for those born with the homozygous sickle hemoglobin genome. Just as any iron undernourished person can suffer from iron deficiency anemia, sickle hemoglobin homozygotes suffer from "thiocyanate deficiency anemia" when they subsist on SCN-deficient foods. This article reviews the role of dietary SCN- in SCA control.
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PMID:Anti-sickling effect of dietary thiocyanate in prophylactic control of sickle cell anemia. 379 84


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