bluuebunny/crossref_metadata_2025_split huggingface dataset:
Stimulation of in Vitro Granulocyte‐Macrophage Colony Formation by Mouse Heart Conditioned Medium
Authors: Byrne Peter V. , Heit Wolfgang , Kubanek Bernhard 
Published: March 2008

Summary. Medium conditioned by mosue heart tissue was found to contain colony‐stimulating factor(s) (CSF) which prodduced in vitro colonies of granulocytes and/or macrophages with bone marrow cells of CBA mice. The action of this CSF type (CSFMHCM) was compared with that of post‐endotoxin mouse serum (CSFES) on colony formation by density subpopulations of colony‐forming cells (CFU‐c), separated by isopycnic centrifugation in a continous albumin density gradient. The number, size and morphology of colonies were examined.Mouse heart conditioned medium produced slightly more colonies adn of larger size than endotoxin serum but the density distribution of their CFU‐c were similar. Low density CFU‐c produced predominatly pure granulocytic colonies and high density ones predominately pure macrophage colonies with both types of CSF. There was no apparent relationship between colony size and CFU‐c density.
Organelle Pathology in Primary and Secondary Haemochromatosis with Special Reference to Lysosomal Changes
Authors: Seymour Carol A. , Peters T. J. 
Published: March 2008

Summary. The organelle pathology of liver biopsy specimens from patients with either primary or secondary haemochromatosis was investigated by analytical subcellular fractionation in combination with enzymic microanalysis. The most striking changes were found in the lysosomes. Increased total activities but decreased latent activities of enzymes selectively localized to the high density population of lysosomes was demonstrated in the iron overloaded biopsies. Depletion of the iron, where possible, by venesection was accompained by a return to normal of these changes.The other subcellular organelles, plasma membrane, endoplasmic reticulum, biliary canaliculi, mitochondria, peroxisomes and the low density population of lysosomes appear to be relatively unaffected. The minor changes demonstrated are similar to those seen in other forms of chronic liver disease. It is suggested that iron mediated lysosomal disruption is implicated in the pathogenesis of the tissue damage in haemochromatosis.
Iron Deficiency Anaemia in Newborn sla Mice: a Genetic Defect of Placental Iron Transport
Authors: Kingston P.J. , Bannerman Catherine E. M. , Bannerman R. M. 
Published: March 2008

Summary. . Newborn mice with X‐linked anaemia (gene symbol sla) have lower haemoglobin levels at birth than normal and carrier mice but there is considerable overlap. Serial observations showed that the haemoglobin values of segregating male mice separate into a bimodal distribution by 42 d of age, and 50 d values were used to assign genotypes retrospectively. The anaemia in newborn sla mice is attributable to iron deficiency, since their total body iron is lower than in normal newborn mice, while their birth weights are almost identical.Haemoglobin levels at birth in normal, anaemic and carrier mice are also influenced by the mother's genotype and phenotype, and the haemoglobin value was progressively lower according to the sla gene dose of the mother.Materno‐fetal iron transfer was examined by labelling pregnant carrier females with radioiron in various ways. When given as single or intermittent doses by injection no clearcut differences emerged in apparent iron transfer to anaemic as compared to non‐anaemic fetuses. However, when radioiron was administered continuously in food a significant reduction in iron transfer to anaemic fetuses was demonstrated.The sla gene is already known to have a major effect in reducing iron transport in the small intestine. The present studies provide evidence of an analogous defect in placental iron transport.
A Case of Atypical Polyagglutinability due to Tk‐Transformation
Authors: Mullard G. W. , Haworth Clare , Lee D. 
Published: March 2008

Summary. A case of acquired transient polyagglutinability in a group O, Rh(D) positive female patient, Wa, is described. An unusual finding in the preliminary serological investigation was the non‐reaction of the cells with peanut (Arachis hypogaea) extract. The patient's red cells were strongly agglutinated by normal human and rabbit sera from which anti‐T had been removed. Electrophoretic mobility studies and tests with polybrene indicated that their sialic acid content was normal. The use of a specific antiserum raised in a rabbit to Wa cells and cross‐absorption tests showed that the patient's erythrocytes shared similar antigenic determinants to red cells treated with supernatants from pure cultures of Bacteroides fragilis. The in vitro‐modified red cells reacted strongly with BS II lectin prepared from Bandeiraea simplicifolia seeds thus providing confirmatory evidence for Tk transformation on Wa cells.It is concluded that the Tk receptor is probably a more frequent feature of acquired transient polyagglutinability than has hitherto been recognized.
Erythroblast Kinetics in Homozygous and Heterozygous Beta‐Thalassaemia
Authors: And P. Dörmer , Betke K. 
Published: March 2008

SummaryThe kinetics of erythroblast proliferation have been investigated in four patients with homozygous and two patients with heterozygous β‐thalassaemia. One case was studied prior to and following splenectomy. Another patient was included in the study in whom an unstable haemoglobin was assumed according to the clinical data. The cell‐kinetic technique is based on short‐term in vitro incubation of suspended bone marrow cells with [14C]thymidine and 5‐fluorodeoxyuridine. By means of quantitative autoradiography using 14C‐referencc sources, the rates and time of DNA synthesis were determined in individual cell compartments classified according to morphological criteria. Cell loss was estimated by comparing the ratios of the relative rates of cell production in successive cytological compartments with the corresponding ratios during normal erythropoiesis.In both homozygous and heterozygous β‐thalassaemia the erythroblasts proceed through all morphological compartments at a reduced rate. This kinetic impairment is considered to be independent of intracellular precipitation of excess α‐chains. A normal or increased rate of proliferation in the early compartments in the case of haemoglobinopathy underlines the significance of the findings in β‐thalassaemia, which become the more prominent the more the erythroblasts approach the non‐proliferating stage. In the homozygous disease only 15–30% of these cells escape destruction during their proliferative life. In heterozygous β‐thalassaemia intramedullary cell death seems to be confined to the non‐proliferating stages. On the basis of the kinetic parameters accessible through the method applied, the heterozygous disorder is largely comparable to iron deficiency anaemia.
Variations in Globin Synthesis in Delta‐Beta‐Thalassaemia
Authors: Kinney T. R. , Friedman Sh. , Cifuentes E. , Kim H. C. , Schwartz E. 
Published: March 2008

SummaryPeripheral blood globin synthesis studies were done in 11 patients with δβ‐thalassaemia trait, Hb S‐δβ‐thalassaemia or δβ/βº‐thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the diagnosis. In addition, four unrelated non‐thalassaemic patients with elevated Hb F levels also had peripheral blood globin synthesis studies. The β/α specific activity globin synthesis ratios in the three blacks with δβ‐thalassaemia trait were 0.60–1.04. In the four Caucasians with δβ‐thalassaemia trait, the β/α ratios were 0.58–1.02. These results demonstrate a wide range of ratios overlapping those of normal controls (0.99 ± 0.06). The βs/α ratios in three blacks with Hb S‐δβ‐thalassaemia ranged from 0.66 to 1.00, similar to those of patients with δβ‐thalassaemia trait. In the black patient with δβ/βº‐thalassaemia, the γ/α ratio was 0.67. The β/α peripheral blood ratios in the four non‐thalassaemic patients with elevated Hb F ranged from 1.00 to 1.1, similar to those of normal controls. These studies indicate that a decreased β/α ratio is not an invariable finding in δβ‐thalassaemia in blacks or Caucasian patients and that globin synthesis data alone is insufficient to diagnose definitively heterozygotes for δβ‐thalassaemia or to distinguish this trait from non‐thalassaemic haematological disorders associated with a normal percentage of Hb A2 and an elevated level of Hb F.
Surface Properties of Rat Reticulocytes during Maturation as Measured by Partition: Membrane Charge‐Associated Changes Discerned prior to Membrane Lipid‐Related Changes
Authors: And Harry Walter , Krob Eugene J. 
Published: March 2008

SummaryPartition of cells in dextran‐polyethylene glycol aqueous phases is an extremely sensitive method for separating cells and for tracing subtle changes in the cells’membrane surface as a function of differentiation, maturation and ageing. By appropriate manipulation of polymer concentration and ionic composition and concentration one can select, to a great extent, the membrane properties (e.g. charge‐associated, lipid‐related) that will determine the cells’partition coefficient. Combining radioiosotopic labelling and cell countercurrent distribution techniques we have previously reported rapid increases in charge‐associated partition coefficients during rat reticulocyte maturation followed by slower decreases in the partition coefficients during the entire life span of the mature erythrocyte. In the present experiments red blood cell populations containing radioisotopically labelled cells of different and distinct ages have been subjected to countercurrent distribution in phase systems reflecting either membrane charge‐associated or lipid‐related characteristics. It was found that: (a) membrane lipid‐related surface properties change rapidly during reticulocyte maturation; (b) membrane charge‐associated changes during reticulocyte maturation are discerned prior to those that are lipid‐related; and (c) lipid‐related membrane changes continue slowly throughout the entire life‐span of the erythrocyte.The sensitivity with which red cell membrane lipid‐related differences or changes can be detected and measured by cell partition suggests great usefulness of this simple method in the study of abnormalities in red cell lipids.
Identification of the Hairy Cells of Leukaemic Reticuloendotheliosis by an Esterase Method
Authors: Higgy K. E. , Burns G. F. , Hayhoe F. G. J. 
Published: March 2008

SummaryA distinctive pattern of alpha‐naphthyl butyrate esterase positivity was observed in all of a series of 14 patients with HCL. This pattern was not observed in a range of other haematological malignancies including nine cases of CLL, two cases of Sézary's syndrome, six cases of null cell ALL, two cases of Schilling‐type monocytic leukaemia and one case of lymphosarcoma cell leukaemia. The potential diagnostic value of this simple cytochemical test is discussed in relation to existing methods.
Efficacy of Platelets Collected by Semi‐continuous Flow Centrifugation (Haemonetics Model 30)
Authors: Slichter Sherrili J. 
Published: March 2008

SummaryThe viability of both autologous and donor platelets collected by means of a semi‐continuous flow centrifuge (Haemonetics Model 30) was measured. Radiochromium labelled pooled platelets harvested from six passes and reinfused into their normal donors showed recoveries and survivals of 58%± 8 and 9.6 d ± 0.8 compared with normal values of 65%± 5 and 9.5 d ± 0.6, respectively. When the viability in aplastic thrombocytopenic recipients of platelets collected from normal donors by the Haemonetics Model 30 was compared with that of platelets collected from the same donors by multiple bag plateletpheresis the results were very similar: recoveries of 82%± 18 and 80%± 25 and survivals of 3.9 d ± 2.0 and 3.8 d ± 1.3, respectively. The in vitro function of the collected platelets in response to aggregating agents was normal when measured within 60 min of collection at pH 7.4. The relationship between bleeding time and post‐transfusion platelet count in aplastic, thrombocytopenic patients showed that in vivo function was maintained.
Kinetics of Red Cell Membrane Phosphorylation: Altered Affinity of HS Membrane Protein Acceptors
Authors: Ali S. A. , Gordon‐Smith E. C. , Selhi H. S. 
Published: March 2008

Summary. Membrane ghosts were prepared from red blood cells of normal and hereditary spherocytosis (HS) subjects. Time dependent phosphorylation studies of the membrane (in the presence of γ labelled AT32P) showed that the HS membrane appeared to incorporate less phosphate than the normal membrane but the results were not significantly different. Using the initial linear rate of phosphorylation and varying the ATP concentration, it was found that the Michaelis constant (Km) for the normal membrane was 24.8 μM ATP and Vmax was 0.120 nmol phosphate bound/mg membrane protein/min. These values for the HS membrane were found to be 39.0 and 0.118 respectively. In one family the affected mother and son each showed a positive cooperativity effect for similar reciprocal plots. This indicates that phosphorylation of various HS membrane proteins may be an ordered rather than random process and suggests biochemical heterogeneity of the HS condition. The constants, Km and Vmax, however, were found to be similar to the rest of HS subjects studied.Crude extracts of the enzyme protein kinase, which catalyses the membrane phosphorylation, showed no significant difference in the Km and Vmax value in HS compared with that from normal red cells. The kinetic difference in phosphorylation is probably due to abnormalities in the membrane protein(s) which accept the phosphate from protein kinase in HS red cells.
Variations in Granulocyte Colony Forming Cell Numbers in Adult Blood
Authors: Barrett A. J. , Faille A. , Ketels F. 
Published: March 2008

Summary. Blood granulocyte colony forming units (CFU‐C) were studied in normal adults to establish: (i) a normal range; (ii) variability due to the culture technique; (iii) variability of blood CFU‐C within individuals. Thirty men studied had 98 (range 8–300) CFU‐C × 103/1, and 28 women studied had 44 (range 0–260) CFU‐C × 103/1. This difference was significant (P<0.001). There was also a significant sex difference in the total number of cells forming colonies and clusters per litre; and in the incidence of colony formers and of cluster formers in buffy coat and mononuclear cell blood fractions. CFU‐C were assayed in four subjects over a 10 week period. When buffy coat cells were used as a source of colony stimulation the week to week variation in the combined growth of the four subjects was wide (±36%) but with conditioned medium the variation was smaller (±14%).In all subjects colony and cluster growth varied in the same way (r=0.77, P=0.001) but there was no correlation with the total leucocyte count. A 3–4 week cyclical change in CFU‐C/1 was found which was independent of the variation inherent in the technique. The physiological significance of the sex difference and the apparent cyclical changes in blood CFU‐C are not explained, but the results emphasize the wide fluctuations in CFU‐C that may occur in normal individuals.
Determinants of Haemoglobin Level in Sickle Cell‐Haemoglobin C Disease
Authors: Bannerman R. M. , Serjeant Beryl , Seakins M. , England J. M. , Serjeant G. R. 
Published: March 2008

Summary. The determinants of steady state haemoglobin levels in sickle cell‐haemoglobin C (SC) disease were investigated by measuring routine haematological and biochemical indices, red cell survival, oxygen affinity, pitted erythrocytes, and red cell and plasma volumes in 31 adult patients (15 male; 16 female).Red cell survival was shortened in all subjects, and was positively correlated with haemoglobin level. However, many haemoglobin values were within the normal range, especially in male subjects. Palpable splenomegaly, which occurred in 53% of patients, did not appear to affect haemoglobin level, red cell survival, plasma volume, or red cell volume, but was associated with lower platelet counts and decreased pitted red cells. Sex related differences were found in total haemoglobin, packed cell volume, conductivity cell volume, red cell count, and in the blood volume measurements.Red cell, plasma and total blood volumes in patients varied with weight and cube of height in manner observed in normal subjects, although red cell volumes were lower and plasma volumes were greater than in normal subjects of given height and weight. Anaemia in SC disease is related to the haemolytic rate but the major determinant appears to be an inappropriate increase in plasma volume.
Variables Involved in Cryoprecipitate Production and their Effect on Factor VIII Activity
Authors: Gunson H. H. 
Published: March 2008

Summary. The Regional Transfusion Centres in England and Wales have studied the following variables in the preparation of cryoprecipitate: blood group of the donor, choice of anticoagulant (ACD or CPD), conditions of freezing, thawing and centrifugation of the plasma and age of the starting plasma.Mean factor VIII activity in cryoprecipitates derived from group A plasma was significantly higher than from group O plasma. CPD may lead to increased potency of factor VIII when freshly collected plasma is used to prepare cryoprecipitates; rapidity of thawing may also be advantageous.None of the other variables studied produced significant effects on the factor VIII activity of the cryoprecipitates and particular attention has been drawn to the results of experiments concerned with the age of starting plasma.
Prostaglandins as Inhibitors of Human Platelet Aggregation
Authors: Minno G. Di , Silver M. J. , Gaetano G. de 
Published: March 2008

Summary. The potencies of prostaglandins (PG) I2, PGD2 and PGE1 as inhibitors of human platelet aggregation induced by threshold concentrations of four aggregating agents were determined in platelet‐rich plasma from normal individuals who had not ingested aspirin. The order of activity against ADP, adrenaline and collagen was always PGI2 > PGD2 > PGE1. However, PGD2 and PGE1 were almost equipotent with PGI2 when tested against arachidonic acid (AA). The threshold inhibitory effects of PGD2, PGE1 and PGI2 could be overcome by increasing the concentrations of the aggregating agents AA, collagen or ADP. Adrenaline was found to be different from the other aggregating agents. It could overcome inhibition of platelet aggregation by PGD2 but could not overcome inhibition by PGI2 or PGE1. These facts support the hypothesis that platelet receptors for PGI2 and PGE1 are similar to each other and different from the receptor(s) for PGD2.PRP obtained from normal subjects after the ingestion of aspirin exhibited only one wave of aggregation in response to ADP, adrenaline or collagen. PGI2, PGD2 and PGE1 were all powerful inhibitors of this single wave of aggregation. The inhibitory activity of all three prostaglandins at threshold concentrations was overcome by increasing the concentration of ADP or collagen but not by increasing the concentration of adrenaline.
Interaction of Alpha and Beta Thalassaemia Genes in Two Sardinian Families
Authors: Furbetta M. , Galanello R. , Ximenes A. , Angius A. , Melis M. A. , Serra P. , Cao A. 
Published: March 2008

Summary. Our paper describes two Sardinian families with α‐β thalassaemia interaction. In the first (family S), the propositus, whose haemoglobin pattern at birth consisted of about 25% Hb Bart's and 75% Hb F, successively developed a clinical and haematological picture typical of Cooley's anaemia. Haematological and globin chain synthesis studies together with these findings suggest that he is homozygous for β0 thalassaemia and heterozygous for α thalassaemia‐1 and α thalassaemia‐2. This conclusion is further substantiated by the finding of various combination of α and β thalassemia among his family members.In the P family two twins whose haemoglobin pattern and synthesis data at birth were similar to those of the proband of family S died in the neonatal period. The mother was assumed to be a compound heterozygte for α thalassaemia‐2 and β0 thalassaemia and the father for α thalassaemia‐1 and β0 thalassemia. The homozygous state for β0 thalassaemia in association with the α thalassaemia 1 and α thalassaemia 2 genes results in a severe clinical picture similar to that of a homozygous β0 thalassaemia. The interaction between the heterozygous state for β0 thalassaemia and the α thalassaemia 1 or α thalassaemia 2 genes, or the combination of both, results in a haematological picture similar to that of a β thalassaemia heterozygote.
In Vitro DNA Synthesis by Bone Marrow Cells and PHA‐stimulated Lymphocytes. Suppression by Nonradioactive Thymidine of the Incorporation of 3H‐Deoxyuridine into DNA: Enhancement of Incorporation when Inadequate Vitamin B12 or Folate is Corrected
Authors: Das Kshitish C. , Manusselis Cathy , Herbert Ictor 
Published: March 2008

Summary.Previous studies demonstrated that excess deoxyuridine (dU) added to short‐term bone marrow and PHA‐stimulated lymphocyte cultures, blocks the incorporation of radioactive thymidine into DNA via the salvage pathway. In the current study, we investigated the effects of added thymidine (TdR) in varying concentrations (10−6to 1 μmol) on the incorporation of 3H‐dU into thymine‐DNA, i.e. we executed ‘thymidine suppression tests.’Increasing concentrations of exogenous TdR caused progressive inhibition of 3H‐dU incorporation into DNA, and decreasing 3H‐dU incorporation was parallelled by increasing incorporation of added 14C‐TdR. These findings demonstrate reciprocity of the salvage and the de novo pathways of thymine‐DNA synthesis, presumably mediated by thymidine‐triphosphate (dTTP), the common end product of both pathways, via feedback inhibition. In patients with folate and/or vitamin B12 deficiency, the addition of appropriate vitamins to marrow and lymphocyte cultures enhanced the incorporation of 3H‐dU into DNA. As predicted, this was not observed in normal subjects. The enhancing effect of these vitamins on in vitro incorporation of 3H‐dU into DNA by deficient cell systems was similar to their correcting effect on abnormal dU suppression. These findings support the theoretical concept that the dU suppression test defines biochemical megaloblastosis due to deficiency of folate and vitamin B12.
γ Chain Composition in Five Italian Newborns Heterozygous for Hb F Malta Gγ117 His → Arg
Authors: Mazza U. , Meloni T. , David O. , Pich P. G. , Camaschella C. , Saglio G. , Ciocca Vasino M. A. , Guerrasio A. , Ricco G. 
Published: March 2008

Summary.The percentage of Hb F Malta Gγ117His	Arg and the γ chain composition have been evaluated at birth and at different times after birth in five Italian newborns heterozygous for this variant. The percentage of Hb F Malta ranged at birth from 24% to 31% of the total Hb F, while the average Gγ/Aγ chain ratio was about 7/3, overlapping the values observed in normal newborns. Tγ chains were detected in three out of five newborns, with a percentage of about 10% of the total Hb F. After birth the Hb F Malta declined faster than the total Hb F; thus, the Hb F decrease during the first 45 d of life is mainly due to the switch‐off of the Gγ locus containing the Hb F Malta gene.