bluuebunny/crossref_metadata_2025_split huggingface dataset:
Polymerized Monoclonal IgA in Two Patients with Myelomatosis and Hyperviscosity Syndrome
Authors: Virella G. , Preto R. Valadas , Graça Felicidade 
Published: March 2008

Summary. Two cases of multiple myeloma with IgA gammopathy and the syndrome of serum hyperviscosity are reported. A common feature of both patients was the production of large amounts of polymeric IgA (more than 50% of the monoclonal proteins existed as dimers or larger forms). However, similar large amounts of polymeric IgA were present in sera with only slightly raised viscosity, suggesting that factors such as the molecular configuration of the polymers or their sub‐units may play a role in determining serum viscosity.The association of plasmapheresis and chemotherapy produced remarkable improvements of the symptoms attributable to increased serum viscosity, and 16 months after diagnosis one of the patients has survived relatively free of symptoms. The hyperviscosity syndrome is being diagnosed with increasing frequency in association with IgA multiple myeloma, and should be investigated in all cases with monoclonal proteins exceeding a concentration of 3.0 g/dl.
Foetal Erythropoiesis in Steel Mutant Mice
Authors: Chui David H. K. , Loyer Brian V. 
Published: March 2008

Summary A rapid and reliable method of definitively identifying mutant (Sl/Sld) and wild type (+/+) mouse embryos in segregating litters is described, based on the mean cell volume determination of circulating foetal erythrocytes by an electronic particle counter. The mean cell volumes of yolk sac derived nucleated erythrocytes from +/+ and Sl/Sld embryos are similar, whereas the foetal liver derived non‐nucleated red blood cells are much larger in Sl/Sld than +/+ embryos. There is no significant retardation in the growth of mutant embryos in spite of the severe anaemia which is macrocytic and normochromic. Evidence is also presented that the proportion of haemopoietic stem cells among hepatic erythroblasts, assayed by the macroscopic spleen colony technique, is higher in the mutant embryos, even though the total number of these progenitor cells present in each mutant foetal liver is less than the normal. Furthermore, these stem cells undergo active proliferation in Sl/Sld foetuses during development.The data indicate that the mutant Sl genes do not affect the primitive erythroid cell lineage derived from the yolk sac blood islands, but seriously interfere with the differentiation of the definitive erythroid cell lineage of foetal liver origin. It is further suggested that the mutant foetal liver fails to support or interferes with the normal rate of differentiation from immature precursor cells into haemoglobinized erythroblasts. The reduction of total haemopoietic stem cells in mutant embryos may be secondary to this defect in cellular differentiation.
Factor VIII and Human Platelet Aggregation
Authors: Vermylen J. , Bottecchia D. , Szpilman H. 
Published: March 2008

SUMMARY When highly purified human factor VIII is submitted to agarose gel chromatography in the presence of 0.5 M CaCl2, the procoagulant activity (low molecular weight factor VIII, LMW‐F VIII) is separated from the void volume protein (Vo‐VIII). Upon incubation of human factor VIII with purified neuraminidase, a very stable platelet aggregating activity develops in the ‘Vo‐VIH’fraction, not in the ‘LMW‐F VIII’part. Evidence is provided that the generated aggregating activity is a property of the ‘carrier protein’for LMW‐F VIII.Desialylated factor VIII retains its antigenic reactivity, its procoagulant or ristocetin cofactor properties and the capacity of its subunits to dissociate and recombine. Neuraminidase‐treated human factor VIII, in contrast to intact bovine factor VIII or intact human factor VIII in the presence of ristocetin, does not induce aggregation of EDTA‐platelet rich plasma, of congenitally afibrinogenaemic platelet rich plasma, nor of washed platelets.
Cell Proliferation and Protein Synthesis in Human Leukaemic Myeloblasts after Cytosine Arabinoside Therapy
Authors: Wantzin G. Lange , Karle H. , Killmann S.‐A. 
Published: March 2008

The effect of a single intravenous bolus injection of cytosine arabinoside (ARA‐C) on the cytokinetics and 3H‐leucine uptake of leukaemic myeloblasts in bone marrow and blood was studied at intervals up to 96h after ARA‐C in five patients with previously untreated acute myelogenous leukaemia. An early decrease in mitotic index and in 3H‐thymidine labelling was observed in four of five patients and pretreatment values were reached again within the observation period. Changes suggesting synchronization were not observed.ARA‐C induced a marked decrease in protein synthesis in the leukaemic myeloblasts as estimated from either a decrease in the 3H‐leucine labelling index or the mean grain count of 3H‐leucine labelled cells. It is suggested that ARA‐C is incorporated also by cells which are not in S‐phase and has a prolonged biochemical effect on the leukaemic cells.
Radioimmunoassay Studies of the Cross‐reacting Antibody of Human Group O Sera
Authors: Holburn A. M. 
Published: March 2008

Cross‐reacting antibody was obtained from three recently stimulated group O donors. The biological reactions of cross‐reacting antibody were investigated by a study of the binding characteristics of 125I‐labelled purified IgG fractions with red cells and by the reactions of whole antiserum or eluates with 125I‐labelled blood group glycoproteins.The bulk of cross‐reacting antibody activity is restricted to the IgG fraction. Experiments with 125I‐labelled IgG and with the radioimmunoassay system revealed that cross‐reacting antibody has a higher binding constant than specific anti‐A or anti‐B.In the radioimmunoassay system complete inhibition of cross‐reacting antibody was obtained either with D‐galactose or with N‐acetyl‐D‐galactosamine, but the same concentrations of monosaccharides gave only partial inhibition of specific anti‐A and anti‐B antibodies. The results indicate that cross‐reacting antibody binds to a smaller determinant than specific antibody. Investigations with other monosaccharides did not, however, reveal a common inhibitory structure. The ability of cross‐reacting antibody to bind to both group A and B antigens may, therefore, be a property of a ‘Polyfunctional’ antibody binding site.
Clinical and Haematological Data in 254 Cases of Beta‐Thalassaemia Trait in Italy
Authors: Mazza U. , Saglio G. , Cappio F. Caligaris , Camaschella C. , Neretto G. , Gallo E. 
Published: March 2008

Summary.The haematological and clinical data in 254 Italian subjects with β‐thalassaemia trait are reported. 46% of the patients were anaemic, 40% complained of weakness, 19% showed enlargement of the spleen and 10% enlargement of the liver. The haemoglobin levels ranged from 8 to 15.5 g/dl with a normal distribution and a mean of 12.73 for males, 10.93 for females and 11.34 for children (4–15 years). Reticulocyte counts and serum bilirubin levels were slightly increased and both showed a statistically significant relationship with haemoglobin levels. The serum iron level was increased in 27% and decreased in 6% of the cases. Haemoglobin A2 concentrations ranged from 3.5 to 8% with a normal distribution and a mean of 5.37; Hb F values were less than 1% in 36% and varied from 1 to 14% in the remainder. Red cell osmotic fragility was decreased in all but 6% of the subjects: low MCV, MCH and MCHC values were observed in 75%, 86% and 10% respectively. A comparison is made between the data and those obtained by other workers.
The Availability of Folates in Man: Effect of Orange Juice Supplement on Intestinal Conjugase
Authors: Tamura T. , Shin Y. S. , Buehring K. U. , Stokstad E. L. R. 
Published: March 2008

Summary.The mechanisms of the low availability of endogenous folates in orange juice and the inhibitory effects on the availability of pteroylheptaglutamate (PteGlu7) caused by orange juice supplements were investigated in man by the use of simulated orange juice (containing the principal components: citric acid, malic acid, etc.) with several different pHs.The endogenous folates in orange juice were identified as mainly 5‐methyl reduced derivatives of pteroylpolyglutamates (40–45% pentaglutamate, 10–15% tetraglutamate, 30–40% monoglutamate, and the rest were tri‐ and diglutamates).The availability of PteGlu7 is decreased to 54% by supplementation with 600 g concentrated orange juice (pH 3.7) and to 39% and 66% by the supplement of 24 g and 12 g citric acid (pH 3.7), respectively. Neutralization of 12 g citric acid to pH 6.4 largely reverses the inhibitory effect. On the other hand, the availability of pteroylmonoglutamate (PteGlu) is not influenced by a supplement of orange juice or citric acid.The results suggest that the low availability of PteGlu7 is due to the inhibition of intestinal conjugase caused by the low pH. These observations suggest that pH of foodstuffs may be an important factor in the absorption of polyglutamate forms of folate.
Analysis of Treatment in Childhood Leukaemia
Authors:   Medical Research Council's Working Party on Leukaemia in Childhood, MacLennan I. C. M. , Kay H. E. M. , Festenstein M. , Smith P. G. 
Published: March 2008

Summary. The first and second Medical Research Council UKALL trials have shown that alteration in the timing of methotrexate and 6‐mercaptopurine maintenance therapy for the treatment of acute lymphoblastic leukaemia can markedly change drug induced toxicity. Maintenance chemotherapy in both trials used a similar total dosage of these drugs but the timing of their administration was different in the two schedules.Serial analysis of leucocyte count showed that the UKALL I regimen caused considerably more neutropenia than UKALL II but UKALL II was associated with prolonged lymphopenia which was not characteristic of UKALL I. Analysis of deaths in remission in these two trials showed some correlation with these findings. Deaths in complete remission were compared with the known prognostic features of age and presenting white cell count. Of the 350 patients achieving remission 63% had the good prognostic features of being less than 13 years of age and having a white cell count below 20 × 109/1. at presentation. Twelve out of the 26 deaths in this group occurred in patients in first remission. In the remaining 128 patients who lacked one or both of these good prognostic features only one out of 56 deaths recorded to date occurred during remission.
Inhibitory Effect of Eggs on Vitamin B12 Absorption: Description of a Simple Ovalbumin 57Co‐Vitamin B12 Absorption Test
Authors: Doscherholmen A. , McMahon J. , Ripley D. 
Published: March 2008

Summary. Ovalbumin and egg yolks, mixed separately in vitro with radiocyanocobalamin (57Co‐vitamin B12), were served to normal volunteers in a cooked form. Ovalbumin, and to a lesser degree, egg yolks were observed to inhibit vitamin B12 absorption. This observation explains the rather poor assimilation of vitamin B12 from eggs labelled in vivo with 57Co‐vitamin B12.The assimilation of vitamin B12 from the ovalbumin 57 Co‐vitamin B12 mixture was also measured in patients with structural alterations and functional disorders of the stomach. In contrast to the absorption test using crystalline radio‐vitamin B12, this simple food‐vitamin B12 absorption test successfully distinguished between the controls and subjects who had simple gastric achlorhydria and patients who had undergone a Billroth I or II operation or a successful vagotomy with pyloroplasty. Furthermore, this ovalbumin 57Co‐vitamin B12 test successfully separated patients with pernicious anaemia from those with simple gastric achlorhydria. The ovalbumin 57Co‐vitamin B12 test, in contrast to that using crystalline 57Co‐vitamin B12, showed a positive correlation with the serum vitamin B12 concentration. This ovalbumin57 Co‐vitamin B12 test, being more physiologic and informative than the method using crystalline radio‐vitamin B12, is the first simple model for food‐vitamin B12 absorption to be proposed for research and for clinical purposes.
Relationship of Factor VIII‐like Antigen (VIII AGN) and Clot Promoting Activity (VIII AHF) as Measured by One‐and Two‐Stage Assays in Patients with Liver Disease
Authors: ROGERS J. S. , EYSTER M. ELAINE 
Published: March 2008

Summary. We recently observed an increase in factor‐VIII clot promoting activity as measured by a one‐stage assay (VIII AHF1) in a haemophiliac with hepatitis. However, VIII AHF as measured by a two‐stage assay (VIII AHF2) was 0.013 u/ml at a time when VIII AHF1 measured 0.38 u/ml.We then studied seven non‐haemophiliacs with liver disease, and attempted to correlate the levels of VIII AHF1 and VIII AHF2 with factor VIII‐like antigen (VIII AGN) as measured by quantitative immunoelectrophoresis. In four of the seven patients, disproportionate elevations of VIII AHF2 compared to VIII AHF1 were found. Furthermore, VIII AHF2 values correlated well with VIII AGN values. No such discrepancy was apparent in four normal control subjects.These findings emphasize the necessity for performing two‐stage assays in haemophiliacs as well as non‐haemophiliacs with liver disease to assess factor‐VIII levels. In addition, they suggest that confirmation of the diagnosis of haemophilia may not be possible in the haemophiliac with hepatitis unless VIII AHF2 determinations are performed. The reason for the disparity between VIII AHF1 and VIII AHF2 levels is not apparent. However, the correlation of VIII AGN and VIII AHF2 levels in the non‐haemophiliacs with liver disease provides further support for the concept that VIII AGN and VIII AHF are closely related or identical molecular entities.
Neutrophil Function in Bone Marrow Transplant Recipients
Authors: Territo Mary C. , Gale Robert P. , Cline Martin J. ,   The UCLA Bone Marrow Transplantation Team
Published: March 2008

The neutrophil function of seven patients receiving allogeneic bone marrow transplantation was studied. Five of the patients had been transplanted for aplastic anaemia and two for acute leukaemia. Determinations were made of neutrophil phagocytosis, chemotaxis, random migration, and microbicidal activity for Candida albicans and Staphylococcus aureus. One patient showed a decreased ability to kill C. albicans at a time when she had active pneumonia due to Pneumocystis carinii. The remainder of the studies showed normal neutrophil functions. No differences were observed in the patients who had graft versus host disease (GvH) from those without GvH.These studies suggest that defects in phagocytic neutrophil function do not contribute significantly to the impaired host defenses in recipients of bone marrow transplantation.
Haemophilia Treatment in the United Kingdom from 1969 to 1974
Authors: Biggs Rosemary 
Published: March 2008

A study has been made by the Haemophilia Centre Directors of the United Kingdom and Northern Ireland. From 1969 to 1974 2600 patients with haemophilia A and 388 with haemophilia B attended Haemophilia Centres for treatment.Of these patients, 71 are known to have died in the survey period. A record is presented of the amounts and types of therapeutic materials used each year during this time. The incidence of jaundice and anti‐factor VIII and anti‐factor IX antibodies was recorded.
Red Cell Metabolic and Membrane Features in Haemolytic Anaemia of Alcoholic Liver Disease (Zieve's Syndrome)
Authors: Goebel Klaus M. , Goebel Frank D. , Schubotz Rolf , Schneider Jürgen 
Published: March 2008

Zieve's syndrome (ZS), which consists of transient haemolytic anaemia, jaundice, hyperlipoproteinaemia, and alcohol‐induced liver disease, was studied in male patients during the acute (n= 20) and the remittent (n= 10) phase. Chronic alcoholics (n= 10) without haemolysis and healthy male persons (n= 10) served as controls.Erythrocytes were separated into old and young cells by means of density‐layer centrifugation. Those fractions which contained older red cells disclosed a pyruvate‐kinase instability which resulted in impaired metabolism. Changes in membrane lipid composition as indicated by increased cholesterol and polyunsaturated fatty acids (PUFA) were also detected in patients during the acute phase of ZS. Alcohol‐induced red‐cell vitamin‐E deficiency with a decrease in PUFA levels may provoke an oxidation of reduced red‐cell glutathione which in turn results in the enzyme instability. This study lends further support to the hypothesis that the putative role of the red‐cell metabolic injury in the origin of haemolysis in ZS cannot be envisaged without introducing membrane‐linked and extracellular cofactors.
Characterization of Marrow Granulocyte Development: Changes in Response to Inflammatory Reactions
Authors: Scott R. B. , Eanes R. Z. , Cooper Laverne W. , Higgins Linda L. , Eastment Christine A. 
Published: March 2008

Summary. Studies of marrow granulocyte differentiation require separation of precursors into populations of varying stages of maturity. This has been accomplished by isopycnic sedimentation of marrow cells in density gradients of Ficoll and Hypaque under conditions in which erythrocyte precursors are virtually eliminated and lymphocyte contamination is small. Immature cells have lower buoyant density and predominate in the top of the gradients and cells of increasing maturity are found as the density level of the gradient increases. Distinct differences were found between control marrows and those of animals with an intense inflammatory response. The inflammatory stimulus depleted the marrow of many mature granulocytes and the immature cell populations were shown to be enriched. Peritoneal exudate cells (mostly mature granulocytes) were shown to have altered buoyant density properties compared to mature granulocytes in marrow. Activity of alkaline phosphatase (a granule‐associated enzyme) increased progressively as cells matured, while lactic dehydrogenase activity (a cytosol enzyme) remained stable.Protein synthesis in control and stimulated marrows showed two‐ to five‐fold greater synthesis in immature cells compared to nearly mature marrow cells. Successful separation of varying stages of development in granulocytes permits varied studies of cell properties involved in the process of differentiation.
Distribution of Rhesus Antigens on Red Cell Surfaces
Authors: Knox E. G. 
Published: March 2008

Summary. Two pieces of evidence suggest different conclusions about the arrangement of the rhesus antigens on the surfaces of red cells. Indirect evidence suggests an ordered arrangement; electron‐microscopic evidence has been interpreted visually as essentially random.Re‐analysis of electron‐microscopic material, using a form of computer spectral analysis, suggests that the visual interpretation was mistaken, and that the pattern is orderly. The material suggests that the maternally derived and paternally derived proteins are linked into couplets, and that the couplets may be regularly linked or packed, at least at shorter ranges. The material does not at present illuminate the question of longer range periodicity.
Assay and Characterization of the Factor in Porcine and Bovine Plasma which Aggregates Human Platelets
Authors: Evans R. J. , Austen D. E. G. 
Published: March 2008

Summary. A sensitive and objective bioassay has been developed to measure the factor present in porcine or bovine plasma which aggregates human blood platelets. The technique is based on direct measurement of platelet aggregation using an electronic particle counter.Using this assay it has been established that platelet aggregating activity is present in both bovine and porcine plasma and in therapeutic concentrates of factor VIII prepared from these materials. Platelet aggregating activity was found to be a function of the factor‐VIII‐related antigen concentration and independent of factor‐VIII coagulant activity. The activity is absent in the plasma from pigs with von Willebrand's disease. It is concluded that factor‐VIII‐related antigen and the platelet aggregating factor are closely related and may represent one protein species.
Cyclic AMP Maintenance of Rabbit Reticulocyte Haem and Protein Synthesis in the Presence of Ethanol and Benzene
Authors: Freedman Michael L. , Spieler Paul J. , Rosman Judith , Wildman Joseph M. 
Published: March 2008

SUMMARY.Haemin is necessary for maximal protein synthesis in intact reticulocytes and their cell‐free lysate preparations. Under conditions of haemin deficiency a translational protein repressor of initiation (HCRA) forms from a non‐inhibitory form of the same protein (HCR1) and protein synthesis ceases. It has recently been suggested that this repressor is a protein kinase and that high concentrations of 3′,5’cyclic AMP could substitute for haemin in maintaining reticulocyte lysate cell‐free protein synthesis. The present study was undertaken, therefore, to investigate if cAMP had a similar function in intact rabbit reticulocytes. The cells were incubated with eidier epinephrine (10‐6 M), isoproterenol (10‐4 M), theophylline (10‐4 M) or dibutyryl cAMP (10‐4 M). All these compounds elevated the intracellular concentration of cAMP within the first 2 min of incubation, as measured by a specific competitive binding assay. This indicates that rabbit reticulocytes contain both adenyl cyclase and phosphodiesterase systems. The cells were incubated with an inhibitor of haem synthesis (either 0.1 M ethanol or 0.1 M benzene) to form HCRA prematurely and inhibit protein synthesis. When the cells were incubated simultaneously with any one of these inhibitors and either epinephrine, isoproterenol, theophylline or dibutyryl cAMP, there was no inhibition of haem or protein synthesis and no demonstrable HCRA. Furthermore, dibutyryl cAMP reversed the inhibition of both haem and protein synthesis induced by ethanol or benzene. Dibutyryl cAMP relieved the inhibition of haem synthesis prior to relieving the inhibition of protein synthesis, which indicates that sufficient haemin must be present before maximal protein synthesis may resume. In contrast, elevation of cyclic AMP levels did not protect against iron deficiency inhibition of either haem or protein synthesis. This indicates that in intact cells cAMP does not directly convert HCRA to HCR1On the basis of these findings, we postulate that in intact reticulocytes (in contrast to cell‐free systems) a major role of cAMP is maintenance of maximal haem synthesis, even in the presence of inhibitors. Intracellular haemin concentration appears to be a major control mechanism of protein synthesis via prevention and reversal of HCRA. This model assures a balance between haem and globin synthesis in erythroid precursors.
Quantitation of the Reticuloendothelial System Clearance of Soluble Fibrin
Authors: Sherman L. A. , Lee J. , Jacobson A. 
Published: March 2008

SUMMARY.Phagocytosis of particulate fibrin has been previously established as a function of the reticuloendothelial system (RES). More recently, reticuloendothelial cells have been shown to bind soluble fibrin/fibrinogen (f/F) complexes in vitro. To quantitate RES clearance us microthrombus formation, varying doses (0.1‐6 mg/kg) of 125I‐soluble f/F was injected into rabbits. One hour later the animals were killed, at which time 48 $pM 8% of the 125I f/F had been cleared from the blood. Homogenized organ samples were separated into insoluble pellet, soluble protein bound, and free 125I. Treatment of other samples with plasmin prior to homogenization differentiated the insoluble 125I into RES cleared (intracellular‐plasmin resistant) vs microthrombi (plasmin sensitive pellet 125I). 125I‐f/F was chiefly found in liver and spleen. Injection of low f/F concentrations resulted in no plasmin sensitive pellet 125I. 3 mg/kg f/F caused small, variable amounts of plasmin sensitive pellet 125I, chiefly in the kidney. With 6 mg/kg, 21‐50% of the insoluble 125I in all organs was plasmin sensitive, and occasional 1–2 mm thrombi were found. The data indicate complete and rapid RES clearance of small amounts of soluble f/F from the blood, without microthrombi being formed. The RES was acutely saturated at 1.5–3.0 mg f/F/kg, which is equivalent to immediate conversion to fibrin of 1–2% of the intravascular fibrinogen pool.