bluuebunny/crossref_metadata_2025_split huggingface dataset:
Studies on the Energy Metabolism of Human Leukocytes II. Mechanism of the Pasteur Effect in Human Leukocytes
Authors: BAIERLEIN JEAN L. , FOSTER JOHN M. 
Published: October 2019

Abstract
1. The control of glycolysis by respiration in normal human leukocytes was studied under conditions in which a Pasteur effect was present and under conditions in which this effect was lost.
2. There was a 20-50 per cent Pasteur effect in leukocytes isolated by dextran sedimentation of heparinized blood and suspended in isologous serum. Crossover point analysis of glycolytic intermediates indicate that phosphofructokinase becomes rate-limiting for aerobic glycolysis and that this enzyme is primarily responsible for the Pasteur effect.
3. Studies on the activity of PFK in leukocyte homogenates showed that this enzyme is competitively inhibited by ATP and reactivated by ADP and Pi. The concentrations of these compounds which significantly effect PFK activity in homogenates fall within the range found in intact cells. The differences in PFK activity in intact cells under anaerobic and aerobic conditions can therefore be attributed to allosteric effects caused by an increase in the concentration of ATP on transition to aerobic conditions, with concomitant decreases in the levels of ADP and Pi.
4. Leukocytes isolated by the same procedure but suspended in an artificial medium rather than serum did not show a Pasteur effect. There were no differences between the aerobic and anaerobic concentrations of ATP, ADP, and Pi, and there was no control point at PFK. It is suggested that a change in respiratory function is responsible for the altered control of glycolysis in leukocytes suspended in nonserum media.
A Study of Hematological Complications Occurring in Patients with Polycythemia Vera Treated with 32P (Based on a series of 296 patients)
Authors: TUBIANA M. , FLAMANT R. , ATTIE E. , HAYAT M. 
Published: October 2019

Abstract
Three hundred and three patients with polycythemia vera have been treated with 32P more than five years ago. Seven patients have been excluded because they have been treated by 32P elsewhere before their first visit. Of the 296 other patients, 269 have been followed up regularly for more than 5 years. The median survival after hematologic diagnosis is 14.5 years. It is shorter in older patients and in those patients who had the shortest remissions after the initial 32P treatment. Of 145 patients who had died, death was due in 53 cases to hematologic complications (29 acute leukemia, 4 chronic leukemia, 16 myeloid metaplasia or myelofibrosis, 4 aplastic anemia). The latter complications were seen most commonly in those patients who had received the highest total dose of 32P, those with a high initial white count and those in whom the spleen was palpable at least 2 cm. beneath the costal margin.
The influence of the quantity of 32P given is very significant for equivalent clinical situations, the influence of white cell count and spleen size is significant for equal doses of 32P. This suggests that these two types of factors favor the early occurrence of hematologic complications.
Degradation of Membrane Phospholipids and Thiols in Peroxide Hemolysis: Studies in Vitamin E Deficiency
Authors: JACOB HARRY S. , LUX SAMUEL E. 
Published: October 2019

Abstract
To understand more clearly the hemolytic anemia associated with administration of certain oxidant drugs, the mechanism by which H2O2 causes hemolysis in rat red cells, deficient in vitamin E was investigated. It was demonstrated that the locus of attack by H2O2 was the red cell membrane, in which one phospholipid, i.e., phosphatidyl ethanolamine, was specifically destroyed prior to the onset of hemolysis. No perturbation of intracellular components or metabolism was noted during peroxidative hemolysis. E-deficient red cells incorporated 14C-labelled fatty acids into this phosphatide at nearly twice the rate that in E-supplemented cells, reflecting the continual tendency of phosphatidyl ethanolamine to be destroyed. Young red cells were especially active in this regard and concomitantly were less vulnerable to damage by H2O2 both in vitro and when circulating in rats exposed to hyperbaric oxygenation. If, however, replacement of fatty acids in phosphatidyl ethanolamine was prevented by inhibition of metabolism or if fatty acids were enzymatically removed by a phospholipase-A, H2O2 hemolysis was potentiated. Hemolysis was also associated with, and potentiated by, loss of membrane sulfhydryl activity. It is suggested that hemolytic anemia may occur in patients with vitamin E deficiency (i.e., with steatorrhea) if oxidant drugs capable of generating H2O2 and oxidizing membrane thiols are administered. Two such cases are under investigation.
Regulation of Erythropoiesis
Authors: KUBANEK BERNHARD , FERRARI LUIGI , TYLER WILLIAM S. , HOWARD DONALD , JAY SUSAN , STOHLMAN FREDERICK 
Published: October 2019

Abstract
The effect of hypoxia on red cell production and stem cells, as measured by CFU, was studied in two strains of mice. One of these strains, the CAF1, has been shown to produce relatively little erythropoietin in response to hypoxia; the other, the CF1, produces substantial amounts of erythropoietin. In the studies reported herein the CAF1 mice had an increase of ∼ 30 per cent in red cell mass after 23 days of exposure to either discontinuous (16 hrs./day) or continuous hypoxia equivalent to 23,000 feet of altitude. The CF1 mouse doubled its red cell mass after similar exposure. The splenic CFU decreased to as low as 17 per cent in the poorly responding CAF1 but remained at or near control levels in the CF1. It is suggested that the dissociation between erythroid response and changes in the CFU reflect a secondary effect of hypoxia not directly related to the erythropoietin-induced increase in red cell production.
The Erythropoietin/Hematocrit Relationship in Normal and Polycythemic Man: Implications of Marrow Regulation
Authors: ADAMSON JOHN W. 
Published: October 2019

Abstract
The relationship between erythropoietin excretion and change in hematocrit has been determined in normal and polycythemic subjects. Erythropoietin was measured by a method involving the assay of urinary concentrates in polycythemic protein-depleted mice. Basal erythropoietin excretion in normal subjects ranged from 2.8 to 7.5 Standard B units per day. Studies in subjects made anemic by bleeding demonstrated an inverse relationship between hematocrit and the log of erythropoietin excretion. Patients with hypoxia-induced erythrocytosis had increased levels of urinary erythropoietin when their hematocrit was reduced to normal levels by phlebotomy. Three patients whose erythrocytosis was associated with tumors had an erythropoietin excretion which remained constant over a wide range in hematocrit. In polycythemia vera, erythropoietin output was absent or markedly decreased at high hematocrit levels, but measurable erythropoietin appeared in the urine when the hematocrit was reduced to normal or anemic levels by bleeding.
Analytic Review: Von Willebrand’s Disease-Diagnostic Criteria
Authors: WEISS HARVEY J. 
Published: October 2019

Abstract
Our present criteria for diagnosing von Willebrand’s disease are summarized below and illustrative examples of recently studied patients are shown in Table 1.
A) Prolonged bleeding time and decreased AHG:
When these abnormalities, inherited as an autosomal dominant, are found in several family members, they are considered diagnostic for these patients and for their clinically affected relatives, even though the latter may now show both defects (Case 10). In the absence of family studies, the diagnosis would also be made in individual subjects if a history of bleeding in relatives was consistent with autosomal inheritance (Cases 3,13,18). When family studies are negative (sporadic cases) or no history is attainable, a diagnosis of typical von Wille-brand’s disease would also be made (Cases 1,2,4,14,15,16) unless intrinsic platelet defects were found (see below) or plasma transfusion failed to produce a progressive increase in AHG activity.
B) Prolonged bleeding time, normal AHG:
The diagnosis would be considered "probable" in such a patient if the following criteria were met:
1. There was no evidence of an "intrinsic" platelet abnormality, as indicated by normal values for platelet aggregation, PF-3 availability and ADP release by connective tissue and kaolin
and
2. Platelet adhesiveness, by the modified Salzman test, was abnormal and/or the patient’s bleeding time was shortened by transfusion of plasma in a dosage of 13-15 ml. per Kg. body weight.
C) Normal bleeding time, decreased AHG (Case 17):
A diagnosis of probable von Willebrand’s disease would be made if:
1. An unequivocal pattern of autosomal dominant inheritance, by history, ruled out hemophilia.
2. The Salzman test was abnormal.
3. Transfusion of hemophiliac, as well as normal plasma resulted in a progressive increase in plasma AHG concentration.
D) Normal bleeding time, normal AHG:
The diagnosis would be considered as "possible" if:
1. There was no evidence of intrinsic platelet abnormality (see B 1).
2. The Salzman test was abnormal.
The above criteria are tentative and, undoubtedly, will not satisfy all investigators. To confuse matters even more, some patients who appear to have the "classic" syndrome may also have abnormal platelet factor 338 or fail to "synthesize" AHG after plasma transfusion.13,38 Whether even the "classic" syndrome represents a spectrum of disorders remains to be determined.
Brief Report: Effect of Acute Administration of Acetysalicylic Acid on the Prothrombin Activity of Bishydroxycoumarin-Treated Rats
Authors: COLDWELL BLAKE B. , ZAWIDZKA ZOFIA 
Published: October 2019

Abstract
The administration of a single oral dose of acetylsalicylic acid (100 mg./Kg.) to bishydroxycoumarin-treated male albino rats significantly decreased the one-stage prothrombin time of blood collected 20 hr. after administration of the analgesic. This antagonistic effect of acetylsalicylic acid resulted in a mean increase of 12 per cent in the prothrombin activity of rats given the anticoagulant intraperitoneally compared to an increase of 6 to 7 per cent in those taking it orally. In the latter animals the intensity of the effect seems to be related to the time that elapses between administration of the two drugs.
Porphyrin Synthesis and Heme Synthetase Activity in Pyridoxine-Responsive Anemia
Authors: VOGLER WILLAM RALPH , MINGIOLI ELIZABETH S. 
Published: October 2019

Abstract
Previous in vitro studies had shown that reticulocytes from a patient with pyridoxine-responsive anemia (PRA) incorporated glycine-2-14C into heme at 11-25 per cent of the normal rate. Incorporation of the intermediate compound, δ-aminolevulinic acid-4-14C (ALA), varied from 46 to 81 per cent of normal. These findings suggested two defects in the synthesis of heme: a major one prior to formation of ALA, probably ALA synthetase, and a minor one somewhere between ALA and heme. This minor defect was investigated in a patient with PRA during a period of repeated phlebotomies resulting in a 30 gram reduction in iron stores. Porphyrin synthesis from ALA was measured in vitro and found to be normal. Heme synthetase activity in particulate fractions of reticulocytes measured by radioiron incorporation into heme was 50 per cent less than normal when the iron stores were high and significantly improved when the iron stores were reduced to normal. Repeat studies showed that incorporation of ALA-4-14C into heme by intact reticulocytes equaled the normal rate. Glycine incorporation was still reduced. Concomitant with the improvement in heme synthetase activity was an improvement in anemia. These results suggest that excess iron is a significant cause of the reduced heme synthetase activity observed in this patient with pyridoxine-responsive anemia.
Genetics of the Bombay Phenotype
Authors: YUNIS EDMOND J. , SVARDAL JANET M. , BRIDGES ROBERT A. 
Published: October 2019

Abstract
Seven individuals with the Bombay phenotype have been found among the thirty-three members of an Indian family spanning three generations. This is the first report of children resulting from the union of an individual of the Bombay phenotype hh and an individual heterozygous Hh at the Bombay locus. This family again demonstrates the effective suppression of the A-B-O phenotype by the Bombay genotype. Three Bombay individuals of this family were shown to be heterozygous at the Lewis locus. This had not been determinable in previously reported cases. This is consistent with the concept that there are two kinds of Bombay genotypes. We propose that the Lewis gene has evolved from a duplication of the H gene. Such duplication predisposes to both higher orders of duplication and to deletion. Deletions in this system would then provide the genetic basis for the Lewis-negative and the Bombay phenotype. In order to explain the rarity of the Lewis-negative Bombay phenotype by the proposed mechanism, we postulate that it would only arise by crossing over at the position determining the active site of the transferase enzymes which determine the Lewis and the H specificities.
Hemolytic Transfusion Reaction Occurring in a Patient with Acute Renal Failure
Authors: HOFFSTEN PHILLIP E. , CHAPLIN HUGH 
Published: October 2019

Abstract
An acute hemolytic transfusion reaction due to ABO-incompatibility occurred in a patient during the fifth week of oliguria secondary to post-traumatic acute renal failure. Resort to "plasma-exchange" transfusion to reduce the high level of free hemoglobin in the circulation was considered and rejected. Sequential measurements of free and haptoglobin-bound hemoglobin documented rapid disappearance of both components from the patient’s plasma in the absence of hemoglobinuria. Within one week, the patient entered the diuretic phase, and ultimately stabilized at normal levels of urine volume, with a creatinine clearance of 53 ml per minute. The implications of free hemoglobinemia in the oliguric phase of acute renal failure are discussed.
Paroxysmal Nocturnal Hemoglobinuria with Acute Leukemia
Authors: HOLDEN DOROTHY , LICHTMAN HERBERT 
Published: October 2019

Abstract
In a case of paroxysmal nocturnal hemoglobinuria, documented since 1955, the features of acute leukemia became evident in 1962, leading to quick death. Between 1960 and 1962, indications of PNH became greatly diminished and a slight, nonspecific leukocytosis developed. No apparent etiologic agent either for the PNH or the acute leukemia could be elicited.
Studies in Chronic Lymphocytic Leukemia: Further Studies of the Proliferative Abnormality of the Blood Lymphocyte
Authors: RUBIN ARNOLD D. , HAVEMANN KLAUS , DAMESHEK WILLIAM 
Published: October 2019

Abstract
Peripheral lymphocytes from patients with chronic lymphocytic leukemia were exposed to phytohemagglutinin in culture. Quantitative assessment of the proliferative response provided an in vitro test of lymphocyte function. The results were correlated with several clinical parameters, both hematologic and immune. Lymphocytes from patients with modestly elevated lymphocyte counts and mild disease manifested slightly delayed and depressed reactions to PHA. All immunoglobulin levels in these patients were also moderately reduced. Further delay and depression of the PHA response showed a rough correlation with progressive lymphocytosis and reduction in circulating immunoglobulins.
Kinetic and autoradiographic analyses have suggested that late-reacting lymphocytes formed distinct populations of abnormal cells which did not seem to be appreciably contaminated with normal lymphocytes, even in instances of mild disease. Only in a patient with splenic lymphosarcoma were multiple circulating populations of normal and late reacting lymphocytes detected simultaneously. In that case, at least some of the late-reacting lymphocytes appeared to originate in parenchymal lymphoid masses.
A definition of CLL could well include, not only the findings in the blood and bone marrow, but a statement of the reaction of the lymphocytes to phytohemagglutinin and of the immunoglobulin characteristics.
Food Iron Absorption: A Comparison of Vegetable and Animal Foods
Authors: LAYRISSE M. , COOK J. D. , MARTINEZ C. , ROCHE M. , KUHN I. N. , WALKER R. B. , FINCH C. A. 
Published: October 2019

Abstract
Iron absorption measurements have been made in 131 individuals relating the absorption of nine different foods tagged biosynthetically with radioiron. Relatively low absorption, ranging from 1.7-7.9, was found with wheat, corn, black beans, lettuce and spinach. Higher values of from 15.6-20.3 were observed with soybeans, fish, veal and hemoglobin. When these values were related to the absorption of ferrous ascorbate determined simultaneously in each subject, food iron absorption could be predicted over the spectrum of normal to iron deficient states.
The Effects of Radio-Opaque Contrast Media on the Structure, and Solubility of the Fibrin Clot
Authors: VEREBELY KARL , KUTT HENN , TORACK RICHARD M. , MCDOWELL FLETCHER 
Published: October 2019

Abstract
Radio-opaque contrast media interfered with the normal process of fibrin and plasma clot formation. The morphologic changes included a decrease of the fibrin fiber diameter and an increase of protein aggregates. These appeared amorphous in the light microscope and showed a linear relationship with the concentration of contrast agents in the reaction mixture. Above a threshold concentration, which was different for each contrast media, no clot formed. With increasing concentrations of contrast medium between 3.5 x 10-3M and 2.0 x 10-2M, the optical density of the clots decreased rapidly while the incorporation of fibrin monomers into the polymer structure of the clots stayed within normal limits. This indicated that the spatial arrangements of the clots was altered. Electron micrographs confirmed that the fibrin fibers were reduced by 70 per cent to 90 per cent of the control fiber diameter in the presence of 6.1 x 10-2M sodium diatrizoate. The fibrin stabilizing enzyme (FSF, LLF or Factor XIII) catalyzed covalent cross links despite the structural changes in the clot.
Erythrocyte Volume, Plasma Volume, and Lean Body Mass in Adult Men and Women
Authors: RETZLAFF JOHN A. , TAUXE W. NEWLON , KIELY JOSEPH M. , STROEBEL CHARLES F. 
Published: October 2019

Abstract
Erythrocyte volume, plasma volume, hematocrit, lean body mass (from total body water), skinfold thickness (at three sites), arm circumference, height, and weight were measured in 40 normal males, 38 normal females, and 12 obese females. From these data on the normal subjects, equations for estimating erythrocyte and plasma volumes were derived. Equations utilizing combined height-weight, surface area, height-weight-skinfold thickness, or lean body mass were found to be the most accurate for predicting erythrocyte and plasma volumes in normal and in obese subjects. The body:venous hematocrit ratio (BH:VH) and the variability of this ratio in our subjects were determined and discussed. Errors in indirect estimates of blood volumes based on this ratio are presented. Erythrocyte and plasma volume standards based on height-weight regression equations or surface area ratio equation are suggested for use in clinical laboratories. Tables and a nomogram based on these equations have been prepared and are available on request.
G-6-PD Bangkok: A New Variant Found in Congenital Nonspherocytic Hemolytic Disease (CNHD)
Authors: TALALAK PHUMARA , BEUTLER ERNEST 
Published: October 2019

Abstract
A new variant of G-6-PD was found in a Thai boy who had typical clinical and hematologic findings of congenital nonspherocytic hemolytic anemia. This mutant has normal electrophoretic mobility, normal affinity for G-6P and NADP, and is very labile to heat. It has an activity of about 5 per cent of normal and shows increased utilization of 2-deoxyglucose-6-phosphate and NAD and decreased utilization of deamino NADP. It is different from previously described mutants, and is named G-6-PD Bangkok.
In Vitro Detection of Platelet Antibody in Patients with Idiopathic Thrombocytopenic Purpura and Systemic Lupus Erythematosus
Authors: KARPATKIN SIMON , SISKIND GREGORY W. 
Published: October 2019

Abstract
Two technics have been developed, which make possible the in vitro detection of platelet antibody in patients with idiopathic thrombocytopenic purpura (ITP), and systemic lupus erythematosus (SLE). The dextran-platelet agglutination technic takes advantage of the enhancement of platelet agglutination in the presence of dextran which brings about a threefold increase in simple agglutination. The Platelet-Factor-3 test measures the ability of platelet antibody to injure platelets and in so doing, make Platelet Factor 3 available to the coagulation cascade, thus accelerating the fibrin clotting time. This technic, when compared to the simple platelet agglutination test, increases the detection sensitivity of antiplatelet factor(s) twenty-fivefold.
The antiplatelet factor(s) as measured by these technics was found to be an immunoglobulin of the IgG class. The antiplatelet factor(s) was present in the sera of 85 per cent of patients with ITP and 88 per cent of patients with SLE. This antiplatelet factor(s) was absent in 25 consecutive healthy controls and in 22 out of 26 patients with thrombocytopenia not due to ITP or SLE.
Although 88 per cent of patients with SLE had demonstrable antiplatelet activity in their serum only 2 out of 33 were thrombocytopenic. These observations suggested that a compensated thrombocytolytic state was present in these patients. This conclusion was consistent with the good correlation between an elevated large platelet index and the presence of antiplatelet factor in these patients.
The Heterogeneity of the High Molecular Weight B12 Binder in Serum
Authors: LAWRENCE CHRISTINE 
Published: October 2019

Abstract
The binding of vitamin B12 by serum proteins was studied by separating Co57B12-enriched serum by Sephadex gel filtration, column chromatography with DEAE-cellulose, and paper electrophoresis. Each method of separation yielded two discrete B12-binding fractions. However, the analysis of each serum by all three separation technics indicated that one of the fractions was, in each case, bipartite.
The "high" molecular weight B12-binding fraction defined by Sephadex gel filtration consisted of transcobalamin I and just part of the transcobalamin II fraction. The remaining portion of transcobalamin II was eluted from Sephadex gel in a "low" molecular weight fraction. Thus, transcobalamin II, equivalent to the β-globulin B12-binder, consisted of both "high" and "low" molecular weight components.
This suggests that there are at least three serum proteins that can bind vitamin B12: two β-globulins, together comprising the transcobalamin II fraction and differing in molecular weight; and transcobalamin I.
Polycythemia Rubra Vera With Pernicious Anemia Some Observations on Vitamin B12 Metabolism
Authors: SAGE ROBERT E. 
Published: October 2019

Abstract
A patient with polycythemia rubra vera developed symptoms and signs of pernicious anemia three years after institution of therapy for the polycythemia. This is the first satisfactorily documented report of this sequence. Strict criteria utilized make both diagnoses unequivocal. Autoantibody studies, the first performed in such a patient, show the presence of parietal cell and intrinsic factor autoantibodies but no antibodies to other tissues. Vitamin B12 requirements have been excessive despite high or high normal serum levels and extreme marrow sensitivity to withdrawal of the Vitamin is evident in the presence of these high serum levels. Studies on Vitamin B12 binding proteins showed an elevation in α binding globulin with reversal of the α to β ratio. It is suggested that this protein is physiologically abnormal. The literature is briefly reviewed regarding the pathogenesis of the two disorders with the conclusion that their appearance in the one patient represents purely a chance phenomenon.