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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three siblings of a Kurdish Jewish family with clinical and hematologic findings compatible with congenital dyserythropoietic anemia (CDA) are described. All patients presented with mild anemia, marked hyperbilirubinemia and splenomegaly. The bone marrow morphology and ultrastructure of the normoblasts was typical of CDA type II and there was strong agglutination of the patients' red blood cells by anti-i serum. These patients displayed two features that were not characteristic of CDA type II, namely, the acidified serum lysis test was negative on more than 10 occasions, and high levels of Hb A2 were observed in two siblings. In one of the siblings, abnormal globin-chain synthesis was found and alpha-chain production exceeded beta-chain production, as in beta-thalassemia minor. In the light of the above findings, our patients are perhaps best classified as having aberrant CDA with features of thalassemia.
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PMID:Aberrant congenital dyserythropoietic anemia with negative acidified serum tests and features of thalassemia in a Kurdish family. 75 May 40

This article on microcytic anemia is the first of several on laboratory investigation of anemia. Microcytic anemia, characterized by a mean corpuscular volume of less than 80 cu mu, is usually due to iron deficiency or chronic disease but may signify thalassemia minor. Exact identification of the cause is important, since inappropriate iron therapy may be useless or even dangerous.
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PMID:Investigation of microcytic anemia. 76 90

The clinical hematologic change in 2 groups of progeny from mice carrying radiation-induced strain SEC alpha-chain deficiencies was found to be similar to the hematologic alterations in persons with alpha-thalassemia. The heterozygous deletion or inactivation of the alpha-chain gene in mice caused an anemia similar to alpha-thalassemina minor in persons. The alpha-chain deficiency in mice created an erythrocytosis, reticulocytosis, and microcytic, hypochromic anemia comparable with the changes in human alpha-thalassemia minor resulting from deletion of the alpha-chain gene. These mouse mutants are the only known animal models of human thalassemia. A comparison of hematologic values obtained from progeny possessing an alpha-chain gene deficiency and from progeny possessing a beta-chain duplication suggested that the deficiency of alpha-chain synthesis, rather than a simple imbalance between the amounts of alpha- and beta-chains produced, was primarily responsible for the altered hematologic characteristics in these alpha-thalassemic mice.
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PMID:Radiation-induced alpha-thalassemia in mice. 87 56

A study was made of the routine electronic measurements of erythrocyte size and hemoglobin concentration in blood samples from 122 patients with decreased transferrin saturation and 66 patients with elevated levels of hemoglobin A2 or F. The medical histories of these patients were reviewed to identify 52 cases of uncomplicated iron-deficiency anemia and 39 cases of uncomplicated thalassemia minor. Four decision functions were compared for separating these two disorders. The functions evaluated were: D.F'. = MCV--[5 X Hb]-RBC; ratio MCV/RBC; ratio MCH/RBC, and RBC. The rules performed better in the uncomplicated cases than in the routine laboratory defined cases. Only minor differences in the performances of the various decision functions were observed. None was sufficiently accurate for final diagnosis, but they should have value in screening patients and in determining which additional test should be considered.
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PMID:Routine erythrocyte measurements in diagnosis of iron-deficiency anemia and thalassemia minor. 98 94

The absolute hemoglobin A2 concentration in mg. per 100 ml. of blood was calculated from the hemoglobin level in Gm. per 100 ml. and hemoglobin A2 percentage for 38 patients with documented iron deficiency, 37 patients with proven beta-thalassemia minor, 26 patients with simple chronic anemia and 40 normal control laboratory workers. The mean hemoglobin A2 concentration (mg. per 100 ml.) in the control group was 459 plus or minus 60 (2 S.D.) and that in the beta-thalassemia group, 766 plus or minus 99. However, in the iron deficiency group it was 229 plus or minus 58, while in the simple chronic anemia group it was 315 plus or minus 39. The mean corpuscular volume (M.C.V.) in cu. mu was 90 plus or minus 8 (2 S.D.) in the normal controls, 68 plus or minus 10 in beta-thalassemia, 69 plus or minus 9 in iron deficiency, and 90 plus or minus 15 in secondary anemia. It is proposed that the absolute hemoglobin A2 level in mg. per 100 ml. of blood taken in conjunction with the M.C.V. is of value in establishing the diagnosis of iron deficiency.
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PMID:Hemoglobin A2 level. A proposed test for confirming the diagnosis of iron deficiency. 111 45

Thalassaemia minor has been diagnosed in 12 members of two Norwegian families. Haematological data from 23 family members are reported. The diagnosis was based on estimation of Hb A(2) by polyacrylamide gel disc electrophoresis. All patients having high Hb A(2) levels had confirmatory evidence of thalassaemia minor (hypochromic anaemia, marked variation in size and shape of the red cells, target cells, basophilic stippling, decreased osmotic fragility, normal or high serum iron level). The majority of the patients had previously been treated with iron without effect.
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PMID:Thalassaemia minor. Twelve patients in two Norwegian families. 112 58

Shear stress is a potential cause of erythrocyte fragmentation and hemolysis in flowing blood. In this study, the response of abnormal human erythrocytes to shear stress in virto was evaluated using a concentric cylinder viscometer. Compared to normal red cells, deoxygenated erythrocytes from persons with sicle cell anemia were particularly susceptible to fragmentation and hemolysis by shear stress. Oxygenation of sicke cell blood improved the resistance of those red cells to shear stress; they remain, however, more susceptible to shear stress than normal erythrocytes. Erythrocytes from patients with iron deficiency, thalassemia minor, and erythrocyte pyruvate kinase deficiency showed fragmentation and hemolysis at threshold shear stresses intermediate between those ovserved for blood from patients with sickle cell anemia and normal persons. Blood samples from patients with hereditary spherocytosis were more resistant to shear stress than normal blood. These results indicate that there are important differences in the response of various red cells to shear stress.
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PMID:Fragility of abnormal erythrocytes evaluated by response to shear stress. 114 31

Thalassemic syndromes are produced by a quantitative defect in the synthesis of globin chains of hemoglobin. They are classified according to the severity of the clinical picture and to the type of globin chain that is affected. Physiopathology, clinical picture, and treatment of thalassemias are discussed in this work. Thalassemia minor does not create, in general, anesthetic problems. In cases of thalassemia major one should consider not only problems derived from the severity of the anemia it self, but also those related to transfusional therapy, and to bony malformations that may disturb tracheal intubation. Discussion on the management of homozygotic thalassemia during the pre, per, and postoperative phase completes this revision.
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PMID:[Thalassemic syndromes and anesthesia]. 141 Jul 32

A preliminary baseline survey was conducted to estimate the prevalence of anaemia in a group of 391 children aged 6-60 months, randomly selected from three urban slums of Karachi. Haemoglobin and the red cell indices including haematocrit, MCV, MCH, MCHC, RBC and red cell distribution width (RDW) were estimated for each of the selected children. Ferritin estimation was done on 354 (91%) children to assess the iron storage status. According to WHO criteria, the accepted cut-off point for anaemia screening in children is set at 11 gm/dl, 70 fl and 20 pg for haemoglobin, MCV and MCH respectively. Following these criteria, 118 (30%) children were classified as normals (Hg = greater than 11 gm/dl) and 273 (70%) as anaemic (Hg = less than 11 gm/dl). Of the 354 ferritin estimations, 225 (64%) children had ferritin levels lower than normal (less than 11 ng/ml) and 128 (36%) had ferritin levels within normal limit (11-120 ng/ml). From this group, a total of 61% (214/354) children were classified as microcytic hypochromic (MIH) and 11% (39/354) of which had normal ferritin levels suggesting the presence of thalassemia minor trait. The overall results obtained indicate that iron deficiency anaemia is highly prevalent among these children.
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PMID:Prevalence of iron deficiency anaemia in children of the urban slums of Karachi. 150 88

The most prevalent hemoglobinopathies in the world, thalassemias are hereditary microcytic anemias. Each year, 100,000 children throughout the world are born with thalassemia major, a fatal blood disease. Thalassemia minor is the carrier state for this catastrophic disease. Health practitioners can easily identify patients who may have thalassemia minor through evaluation of routine red-blood-cell indices, and diagnosis can be confirmed by Hgb A2 evaluation. Once individuals know they have thalassemia minor, a lifetime of unnecessary treatment for anemia can be avoided. Patient education should also include information about preconception counseling and partner screening. Recognition of the potential for congenital diseases and subsequent screening or referral are the responsibilities of all health care practitioners who see clients before and during their reproductive years.
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PMID:Thalassemias: simple screening for hereditary anemias. 154 63


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