bluuebunny/crossref_metadata_2025_split huggingface dataset:
Adenosine Triphosphatase Activity of the Red Cell Membrane in Paroxysmal Nocturnal Haemoglobinuria
Authors: Brabec V. , Mirčevová L. , Palek J. 
Published: March 2008

Summary:The activity of the ATPase complex of red cell ghosts as well as the activity of the ATPase components, Mg2+ ATPase and Mg2+, Na+ and K+ ‐dependent ATPase was studied in patients with paroxysmal nocturnal haemoglobinuria (PNH). In ghosts prepared by osmotic haemolysis a significantly higher ATPase activity was found in PNH cells than in normal red cells. Ghosts exposed to the effect of saponin showed a significant increase of ATPase activity both in normal and PNH red cells; however, the increase of activity was markedly higher in PNH ghosts than in ghosts of normal red cells. Both Mg2+‐dependent and Mg2+, Na+ and K+‐dependent ATPase contribute to this increase. The protein content in osmotic ghosts of PNH red cells was higher than normal; but after treating these ghosts with saponin, no marked difference in protein content was found.By comparison with the results of ATPase activity obtained using red cells from non‐splenectomized individuals with hereditary spherocytosis with similar reticulocyte values it is assumed that the increase of ATPase activity is not due to younger cells in the blood but is a further manifestation of the abnormality of the cell membrane in PNH. ATPase activity is present mostly in the inactive form in the red cell membrane and is activated (unmasked) by structural damage of the cell membrane. It is suggested that the increased ATPase activity in the PNH red cell membrane represents a manifestation of abnormal structural arrangement which is more susceptible to damage.The deviations found in the content and metabolism of organic phosphates are probably caused by the younger population of red cells in PNH.
Experience with a Simplified Method of Radio‐isotopic Assay of Serum Vitamin B12
Authors: Britt R. P. , Bolton F. G. , Cull A. C. , Spray G. H. 
Published: March 2008

Summary:A simplified method for the assay of serum vitamin B12 by isotopedilution is described. Results obtained by this method correlated well with those obtained by microbiological assay using Lactobacillus leichmannii; reproducibility was of the same order and the range of values obtained in normal subjects fell within the accepted range. There was no difficulty in detecting clinical vitamin B12 deficiency.
Combined Factor‐V and Factor‐ VIII Deficiency: Report of Four Cases
Authors: Seligsohn U. , Ramot B. 
Published: March 2008

Summary:Three sisters, descendants of a consanguineous marriage, with combined Factor‐V and Factor‐ VIII deficiency, enuresis until early adulthood, and psychopathological manifestations have been reported. The family study, which included three generations, revealed several clinical and laboratory patterns: mild bleeding with normal levels of Factors V and VIII; mild bleeding with mild deficiencies of Factors V and VIII, mild Factor‐V and Factor‐VIII deficiencies without bleeding; neither bleeding nor blood clotting abnormalities. The mode of inheritance of the combined deficiency of Factors V and VIII seemed to be autosomal with complete expression in the homozygous state and partial or no expression in the heterozygous state. This conclusion is in accordance with previous reports. The association of the blood coagulation defect with psychopathological manifestations and enuresis in the present kindred appeared coincidental.A fourth woman with similar clotting defects but with normal personality is reported from another family. Her parents were also related.Infusion of fresh haemophilia A plasma to one of the patients in the first family had no effect on Factor‐V and Factor‐VIII levels, but physical effort had a remarkable effect, the levels rising after 24 hours from 16 per cent to 41 per cent and from 0.7 per cent to 32 per cent respectively. In the patient from the other family, only the Factor VIII rose after exercise (from 2.3 to 18.8 per cent), but there was a similar delay in the response.The technical assistance of Mrs. E. Goldberg is gratefully acknowledged. We are also grateful to Dr. J. Caen for plasma from a known case of Factor‐VII deficiency.
The Free Globin in Human Erythrocytes. I
Authors: Winterhalter Kaspar H. , Heywood J. David , Huehns Ernst R. , Finch Clement A. 
Published: March 2008

Summary:A method of measuring free globin has been described. Human erythrocytes are shown to contain free globin and levels have been established for a group of haematologically normal individuals. Storage experiments indicate the importance of performing the free globin assay on fresh blood. Evidence is presented that this free globin is probably a mixture of α‐β apoprotein and intermediate α2‐β2 tetramers partially saturated with haem.
The Haematological Effects of Glucose‐6‐Phosphate Dehydrogenase Deficiency and Thalassaemia Trait: Interaction between the Two Genes at the Phenotype Level
Authors: Piomelli S. , Siniscalco M. 
Published: March 2008

Summary:The haematological effects of glucose‐6‐phosphate dehydrogenase (G6PD) deficiency and thalassaemia trait were evaluated in a field study of 317 individuals in an isolated Sardinian village, where both traits were present at high frequency. G6PD deficiency was diagnosed with rigid genetic criteria. Thalassaemia trait was diagnosed on the basis of abnormal osmotic fragility.Complete G6PD deficiency resulted in mild anaemia with macrocytosis, a consequence of mild chronic haemolysis. Partial G6PD deficiency had a similar, but less marked effect. Thalassaemia trait resulted in mild anaemia, with marked microcytosis and moderate hypochromia.The haematological effects of the combination of both traits were equal to the sum of the independent effect of each. The G6PD activity of the individual red cells was similar in thalassaemic and normal individuals. Because of the microcytosis, the G6PD activity per gram of haemoglobin per unit volume of red cells or of whole blood appeared to be elevated in thalassaemia trait.
Alpha‐Thalassaemia in Chinese: Cord Blood Studies
Authors: Todd David , Lai Michael C. S. , Braga Carolina A. , Soo H. N. 
Published: March 2008

Summary:In an unselected series of 500 umbilical cord blood samples from Southern Chinese infants 15 were found to have from 3.1 to 9.8 (mean 6.5) per cent haemoglobin Bart's. Seven of the 15 were re‐investigated 8–19 months after birth; in six, haemoglobin Bart's was no longer detected while in the remaining one the amount had markedly decreased. Haematological studies, carried out also in some of the parents, indicate that these infants are probably heterozygous for α‐thalassaemia. In the same series one stillborn infant with haemoglobin Bart's hydrops foetalis was encountered.
Nature of the Inhibition of Prothrombin Consumption by Lysed Fibrinogen
Authors: Triantaphyllopoulos D. C. , Chen C. , Triantaphyllopoulos E. 
Published: March 2008

Summary:The derivatives of the fibrinolytic digestion of fibrinogen which precipitate between 25 and 50 per cent ammonium sulphate saturation inhibit the prothrombin consumption of blood. Disintegration of the platelets by freeze‐thawing or sonication or addition of purified platelet factor 3 did not normalize prothrombin consumption. Normalization occurred after addition of an excess of platelet factor 3 or brain phospholipid. These findings suggest that lysed fibrinogen inhibits the action rather than the release of platelet factor 3. The anticoagulant activity varied neither with the degree of digestion of the parent fibrinogen solution nor with the electrophoretic fraction of the fibrinogen derivatives in contradistinction to the antithrombic effect, which has been shown to depend on these parameters. In purified systems the 25–50 per cent ammonium sulphate fraction of the fibrinogen derivatives retarded the degradation of prothrombin by thrombin and more thrombin activity was found in the two‐stage assay.
Antibody Activity against Hog Intrinsic Factor in Pernicious Anaemia
Authors: Ungar Berta 
Published: March 2008

Summary. The sera of 100 adult patients with Addisonian pernicious anaemia not previously exposed to hog intrinsic factor were tested for antibody activity against hog intrinsic factor by the charcoal method: 55 sera containing antibody to human intrinsic factor showed antibody activity against hog intrinsic factor; 45 sera had no demonstrable antibody activity against human or hog intrinsic factor. A high degree of cross reactivity between antibody to human intrinsic factor and hog intrinsic factor was thus demonstrated in vitro.Hog intrinsic factor was equally effective in potentiating vitamin B12 absorption to normal levels in pernicious anaemia patients with and without circulating antibody active against hog intrinsic factor. Antibody to intrinsic factor circulating in the blood was therefore considered to have no effect on hog intrinsic factor in the human gastrointestinal tract. The possibility of a direct effect of circulating antibodies on the production and/or release of endogenous intrinsic factor by the gastric mucosa could not be excluded.
Hereditary Erythroblastic Multinuclearity Associated with a Positive Acidified‐Serum Test: a Type of Congenital Dyserythropoietic Anaemia
Authors: Crookston J. H. , Crookston Marie C. , Burnie Katherine L. , Francombe W. H. , Dacie J. V. , Davis J. A. , Lewis S. M. 
Published: March 2008

Summary.In five patients (including two sisters) an unusual anaemia was characterized by erythroblastic multinuclearity, ineffective erythropoiesis and a positive acidified‐serum test. Unlike PNH, the sugar‐water test was always negative in these patients. The lysis of their cells by acidified normal sera indicated their abnormal sensitivity to an agglutinating and complement‐binding antibody present in some normal subjects. The patients cells gave high agglutination scores with anti‐i, and were unusually susceptible to lysis by anti‐i and anti‐I. The disorder is apparently inherited as an autosomal recessive character.
A Comparative Electron Microscopic Study of Refractory and Alcoholic Sideroblastic Anaemia
Authors: Grasso Joseph A. , Hines John D. 
Published: March 2008

Summary.A comparative electron microscope study of refractory and alcoholic sideroblastic anaemia has been performed. In all cases the mitochondria were infiltrated by dense clumps of iron which were located between the cristae in the matrix. Resolution of the clumps revealed the presence of a finely granular, threadlike precipitate. In alcoholic patients iron in the form of ferritin was found in cytoplasmic heterogeneous bodies. The latter, being rather numerous in one case, contribute to the positive Perls reaction. Based on their morphological diversity and resemblance to hepatic dense and lytic residual bodies, it is assumed that these bodies may be lysosomes involved in autophagy. Coated vesicles and smooth thick vesicles, with or without ferritin, were seen in all cases, as were ferritin aggregates.
Iron Absorption, Iron Loss and Iron Retention in Man: Studies after Oral Administration of a Tracer Dose of 59FeSO4 and 131BaSO4
Authors: Boender C. A. , Verloop M. C. 
Published: March 2008

Summary.The absorption of iron administered as ferrosulphate was studied in normal subjects, in patients with iron deficiency and in patients with idiopathic and secondary haemosiderosis. By using a test dose combining radioactive iron (59Fe) with radioactive barium sulphate (131Ba) as inert indicator, it was possible to determine how much iron was initially absorbed and whether a proportion of this was later excreted in the faeces.The (initial) iron absorption, the iron loss as a percentage of the iron absorbed, and the (ultimate) iron retention 2 weeks after administration of the test dose were calculated.In normal subjects and patients with secondary haemosiderosis, a large proportion of the initially absorbed iron later returned to the intestinal lumen. In patients with iron deficiency and those with idiopathic haemosiderosis, however, nearly all the initially absorbed iron was retained in the organism. In differential diagnosis between idiopathic and secondary haemosiderosis, therefore, determination of the iron absorption and iron retention percentages from a test dose may be important.In patients with secondary haemosiderosis there seemed to be a negative correlation between the degree of haemosiderosis and the amount of iron from the test dose ultimately retained in the organism.
Effects of Thrombocytopenia on Megakaryocytopoiesis
Authors: Junr. Odell T. T. , Jackson C. W. , Friday T. J. , Charsha Deborah E. 
Published: March 2008

Summary.Megakaryocytopoiesis was studied in rats made thrombocytopenic by daily injections of platelet‐specific antiserum. Tritiated thymidine was injected 24 hours after the first antiserum and rats were killed at intervals up to 72 hours after the thymidine. Megakaryocyte number and mitotic index were determined on sections of bone marrow; the labelling index (per cent of cells labelled) and frequency of megakaryocytes in morphologic compartments were determined in autoradiograms of marrow smears. Thrombocytopenia caused an increase in the numbers of megakaryocytes, beginning about a day after the first injection of antiserum, with young forms becoming more frequent in the recognizable population. At about the same time, there was an increase in the mitotic index (endomitosis) of megakaryocytes. All of these results indicated an increased influx of cells into the recognizable megakaryocyte population. Later the frequency of older forms increased, revealing that a cohort of cells was passing through the maturation process. The changes in labelling index suggested that megakaryocytes may mature more rapidly in thrombocytopenic rats than in controls. The results suggest that a regulatory agent may act on a diploid precursor cell in responding to the thrombocytopenic state.
Defective Synthesis of an Unstable Haemoglobin: Haemoglobin Koln (β98Val‐Met)
Authors: White J. M. , Brain M. C. 
Published: March 2008

Summary. The in vitro synthesis of haemoglobin A and the unstable haemoglobin, haemoglobin Köln, has been studied using reticulocytes from a patient heterozygous for these two haemoglobins.The proportions of the two haemoglobins synthesized were: 80% haemoglobin A and 20% haemoglobin Köln. The small amount of haemoglobin Köln synthesized has been found to be due to a deficiency in the synthesis of βKoln‐chains. The deficiency of βKoln‐chains results in an excess production of α‐chains by the reticulocytes, which can be detected as a free α‐chain pool. The excess α‐chains are available to combine with βA‐chains to synthesize haemoglobin A.The overall result is a preferential synthesis of haemoglobin A over haemoglobin Köln. The deficiency in the synthesis of the unstable haemoglobin probably explains the comparatively mild degree of haemolysis found in haemoglobin Köln disease.
Simultaneous Fibrinogen and Platelet’ Survival with [75Se]Selenomethionine in Man
Authors: Brodsky Isadore , Siegel Norman H. , Kahn S. Benham , Ross Evelyn M. , Petkov Goldie 
Published: March 2008

Summary. Simultaneous fibrinogen and platelet survivals were determined with a cohort label, [75Se]selenomethionine, in patients with hepatocellular disease and abnormal serial thrombin times (STT) and in control patients. The fibrinogen survival curves were divided into an anabolic and catabolic phase. In a control group maximum labelling of fibrinogen (anabolic phase) occurred within 24 hr and mean fibrinogen survival (catabolic phase) was 7.8 ± 2.8 days. Patients with hepatocellular disease fell into two groups, those with short fibrinogen survivals and those with normal fibrinogen survivals. In general, the more abnormal the STT the shorter the fibrinogen survival. The anabolic phase of the curve was also abnormal in that there was delay in incorporation of label in these patients.Average platelet survival in control patients was 10.6 ± 3.3 days and similar results were obtained in the patients with hepatocellular disease. In the latter no correlation was noted between platelet survival and fibrinogen survival.The data are consistent with the hypothesis that in hepatocellular disease fibrinogen anabolism is abnormal and short fibrinogen survival, when present, is due to either the production of an abnormal fibrinogen or defibrination or both.
Abnormal Kinetics of Red Cell Membrane Cholesterol in Acanthocytes: Studies in Genetic and Experimental Abetalipoproteinaemia and in Spur Cell Anaemia
Authors: McBride J. A. , Jacob H. S. 
Published: March 2008

Summary. Abnormalities in the content and kinetics of red cell membrane cholesterol are described in several syndromes associated with spurring of red cells (‘acanthocytosis’). In all (hereditary abetalipoproteinaemia, ‘spur cell anaemia’ of severe hepatic failure, and in a lipid‐deprived infant with acquired hypobeta‐lipoproteinaemia) red cell membrane cholesterol was 15–50% increased. Free cholesterol normally flows bidirectionally between red cell membranes and plasma betalipoproteins, but plasmas from affected patients with acanthocytosis were uniformly inefficient in accepting cholesterol from red cells. Stagnation of the sterol on red cells resulted. Plasma from rats in which abetalipoproteinaemia was experimentally produced by prolonged feeding of orotic acid, demonstrated similar abnormalities. Thus, red cells from treated animals became acanthocytic within I mth; with the shape change an increase in membrane cholesterol of about 20% and a significant decrease in osmotic fragility occurred. 14C‐cholesterol flux from rat red cell membranes was 30–40% decreased into the experimentally‐abetalipoproteinaemic, relative to betalipoprotein‐replete, rat plasmas.We conclude that stagnation and, less importantly, accumulation of red cell cholesterol may be associated with conformational changes in the structural lipoproteins of the red cell membrane leading to spurring. Regardless of mechanism, this shape change causes acanthocytes to become abnormally rigid and resistant to flow, documented in the present studies by their increased viscosity in cone‐plate viscosimeters and by their diminished filterability through micropore filters. Both features are probably involved in the diminished survival of these cells in the circulation.
The Haematological and Histological Findings in 18 Patients with Clinical Features Resembling those of Myelofibrosis
Authors: Pegrum G. D. , Risdon R. A. 
Published: March 2008

Summary. The presentation and haematological abnormalities in 18 patients with splenic and hepatic enlargement and a leukaemoid or leucoerythroblastic blood picture have been reviewed. The clinical features in each case were consistent with a diagnosis of myelofibrosis. Histological examination of the bone marrow showed myeloid proliferation of various types for which no underlying cause was apparent. On the basis of the bone marrow histology, it was possible to divide the 18 patients in this study into two groups.The first group of four patients presented with a splenomegaly and a marked leucocytosis. Bone marrow aspirate and biopsy specimens showed marked granulocytic proliferation akin to chronic myeloid leukaemia, from which these cases were separated by the findings of a normal or raised leucocyte alkaline phosphatase score. Other distinguishing features these patients had in common were that they were all elderly and their illness appeared to follow a prolonged course, with little need of active treatment.The second larger group of 14 patients were less uniform. The histological and haematological appearances were variable. Excess bone marrow reticulin was present in each case, and marrow fibrosis was noted in eight. Prominent aggregations of large primitive stem cells were a histological feature in all 14 instances. Correlation between the clinical, haematological and histological features was poor and in particular the marrow aspirate gave little indication of the marrow morphology as found in the bone marrow sections.
Excretion of Endogenous Non‐haem Iron by the Gastrointestinal Tract in Human Subjects and its Influence on the Size of Body Store of Iron with Particular Reference to Coeliac Disease
Authors: Singh A. K. 
Published: March 2008

The present study demonstrates that there is continuous loss of endogenous non‐haem iron by the gastrointestinal tract in man, and underlines its importance in patients with coeliac disease and in those with iron overload. The findings suggest that this loss occurs mainly as a result of desquamation of villous epithelium. The faecal loss of endogenous non‐haem iron is markedly increased in patients with coeliac disease in whom the cell turnover of the small intestinal mucosa is increased and it seems that it could be mainly responsible for the causation of iron deficiency amongst a considerable number of these patients.In subjects with normal mucosal structure of the small intestine this loss increases progressively with iron load at high levels of labile iron store and thus reduces the total iron store in the body.
The Relationship between Body Iron Stores and Ferritin Turnover in Rat Liver and Intestinal Mucosa
Authors: Cumming R. L. C. , Smith J. A. , Millar J. A. , Goldberg A. 
Published: March 2008

A single dose of oral or parenteral iron is known to stimulate ferritin synthesis in the liver and small intestine in experimental animals. A study has been carried out on the ferritin content and ferritin turnover in rat liver and intestinal mucosa in iron deficient, normal and iron loaded animals. [I‐14C]DL‐leucine incorporation into ferritin was used as a measure of its synthesis.A significantly greater ferritin accumulation was found in the intestinal mucosa of the group of iron loaded animals compared with normal or iron deficient rats (P < 0.001). In the liver the ferritin protein, ferritin iron and labelled ferritin content was significantly different between all three groups (P < 0.001), the amounts being higher in the iron loaded animals and lower in the iron deficient animals when compared with the normal group. These results may explain how the absorption of iron in the small intestine is controlled by body iron stores.
Specificity of Acquired Haemolytic Anaemia Autoantibodies and their Scrological Characteristics
Authors: Vos G. H. , Petz L. , Fudenberg H. H. 
Published: March 2008

Summary In a study of 24 red cell eluates obtained from patients with acquired haemolytic anaemia of the ‘warm’ type, it was established that some eluates possessed autoantibody of only one specificity either for ‘normal’ (‘anti‐nl’) or ‘Rhnull’ (‘anti‐dl’) red cells, whereas others contained autoantibodies of both specificities. Combined autoantibodies were often better recognized by different testing procedures than by the same method. Thus the ability of these autoantibodies to strongly sensitize ‘normal’ red cells by the ficin test and ‘Rhnull’ cells by the antiglobulin test was frequently found, whereas the occurrence of strong ficin‐reactive ‘anti‐nl’ and strong ficin‐reactive ‘anti‐dl’ was rare.Additional studies using red cell eluates revealed that no direct correlation could be established between the presence of complement components and the intensity of the indirect antiglobulin test using anti‐IgG serum. On the other hand, the simultaneous presence of ‘anti‐nl’ and ‘anti‐dl’ autoantibodies was often associated with the presence of complement components in the red cell eluates. Fixation of complements by Rh isoantibodies of multiple specificity has been reported, and thus the presence of complement components in association with ‘anti‐nl’ and ‘anti‐dl’ may be analogous to complement fixation by multiple Rh isoantibodies. The formation of ‘anti‐nl’ with ‘anti‐dl’ which tends to result in complement components being fixed by the antibody may be considered as a natural progression of antibody synthesis associated with an advanced state of autoimmunization.