bluuebunny/crossref_metadata_2025_split huggingface dataset:
Characteristics of enzymes of erythrocytes from newborn infants and adults: Activity, thermostability, and electrophoretic profile as a function of cell age
Authors: Mohrenweiser H. W. , Fielek S. , Wurzinger K. H. 
Published: February 2007

AbstractThe level of enzyme activity, the enzyme thermostability profile, and the isozyme electrophoretic pattern were determined in young and old erythrocytes from newborn infants and adults and in samples from adult individuals with increased reticulocyte counts. Cord blood samples had higher levels of enzymatic activity for 12 of the 14 enzymes measured, adenylate kinase and phos‐phoglucomutase being the exceptions. The largest differences in activity between newborns and adults were for glutamic oxaloacetic transaminase, hexo‐kinase, glucose 6‐phosphate dehydrogenase, and glutathione reductase, while glutamic oxaloacetic transaminase and pyruvate kinase showed the largest differences between young and old cells. The levels of activity of glutathione reductase, adenylate kinase, phosphoglucomutase, lactate dehydrogenase, phos‐phoglycerokinase, and glucose phosphate isomerase in cord blood samples suggest the regulation of expression of these enzymes is different in fetal erythrocytes than in erythrocytes from an adult. Differences in the thermostability profile of enzymes from cells from different sources and/or of different ages were noted for 5 of 9 enzymes. No unique electrophoretically identifiable fetal isozymes were observed, although differences in isozyme distribution and staining intensity associated with cell source and/or cell age were noted for many of the 23 enzymes examined. Many of these differences in enzyme characteristics have the potential to be confused with genetic alterations in enzyme structure and function.
Immune complexes in hemophilia
Authors: Poskitt Thomas R. , Poskitt Paula K. F. , Bean Cheryl A. , Arkel Yale S. 
Published: February 2007

AbstractCirculating immune complexes (ICs), assayed by the L1210 enzyme‐linked immunoassay, were detected in 48% of patients with hemophilia A, 50% of patients with von Willebrand's disease, and in none of our patients with hemophilia B. Eighty‐five % of the hemophilia A and B patients had mild to moderate disease with only one patient demonstrating a circulating inhibitor. No correlation was found between IC levels and hepatitis B infection, SGOT, disease severity, total quantity of factor VIII or IX infused, time interval from last infusion, or rheumatoid factor positivity. Although the nature of the ICs is not known, the similarity of IC levels between hemophilia A and von‐Willebrand's disease is discussed with regard to antibodies generated to non‐procoagulant portions of the factor VIII molecule.
Effect of endotoxin on arachidonic acid release and thromboxane B2 production by human platelets
Authors: Stuart Marie J. 
Published: February 2007

AbstractPlasma thromboxane A2, which is elevated during endotoxemia, has previously been shown to be a major factor contributing to the mortality and morbidity that occurs in endotoxin shock in the experimental animal. Using a minimal dose of Escherichia coli endotoxin (1 μg/ml), we have demonstrated that the preincubation of human platelets with endotoxin induces changes in platelet arachidonic acid release and the subsequent conversion of the released arachidonic acid to thromboxane B2, the stable end product of thromboxane A2. In paired experiments, in the presence of endotoxin, the addition of the aggregating agent thrombin (0.5 U/ml) caused human platelets to release 29.1 ± 3.4% of 14C‐arachidonic acid from prelabeled platelet phospholipids. This value was significantly elevated (P < 0.02) when compared with the release of 14C‐arachidonic acid from platelets in the absence of endotoxin (21.9 ± 3.6%). Similarly, comparison of the results of the conversion of the released arachidonic acid to platelet thromboxane B2 (TxB2) revealed that TxB2 production was significantly increased (P < 0.01) when human platelets were preincubated with endotoxin prior to the addition of thrombin (6.1 ± 0.6%) when compared with TxB2 formation observed in the absence of endotoxin (3.4 ± 0.5%). The absolute amount of released arachidonic acid that was converted to TxB2 in the presence of endotoxin (1.8 ± 0.3%) was also significantly higher (P < 0.01) than the value observed in its absence (0.8 ± 0.2%). This study suggests that one of the tissue sources of the proaggregatory vasoconstrictor thromboxane A2 during endotoxemia is the platelet.
Morphologic and functional heterogeneity of chronic neutropenia of childhood with normal neutrophil colony formation in vitro
Authors: Komiyama A. , Yamazaki M. , Yoda S. , Saitoh H. , Morosawa H. , Akabane T. 
Published: February 2007

AbstractIn order to obtain further knowledge of chronic neutropenia of childhood, we studied nine neutropenic infants six to ten months of age by in vitro techniques, including bone marrow culture, electron microscopy, and chemotaxis assay. Eight of the nine patients had a benign clinical course and the bone marrow aspirates showed a reduced number of segmented neutrophils. The ninth patient had a moderately severe course and the bone marrow showed maturation arrest at the promyelocyte stage. Bone marrow cultures demonstrated that the in vitro neutrophil colony formation and production of colony‐stimulating activity were normal in all of the eight patients studied. Neutrophils from one of the nine patients had ultrastructural abnormalities such as a decrease in number of primary and secondary granules and the presence of myelin figures in primary granules. Neutrophil chemotaxis was defective in three of the nine patients. All of the six patients in whom the neutrophil colony formation in agar, the ultrastructure of neutrophils, and neutrophil chemotaxis were normal recovered from the neutropenia between 11 and 30 months of age. These in vitro parameters appear to be useful for evaluating chronic neutropenia of childhood.
Standardization of platelet function tests
Authors: Roper‐Drewinko Pamela R. , Drewinko Benjamin , Corrigan Gail , Johnston Dennis , McCredie Kenneth B. , Freireich Emil J. 
Published: February 2007

AbstractMethods for the standardization of several currently used platelet function tests (bleeding time, capillary fragility, platelet retention, platelet aggregation, platelet factor 3, and platelet volume profiles) are presented. Different variables that may interfere with the reproducibility of the results of each assay were identified and standardized. Using the standardized techniques, the range of normal values for each test was determined in a large population of normal volunteers and used to identify disease states by comparing patient results with those of the normal population. A format for presenting the entire profile of platelet function parameters is proposed.
Evidence for the generation of hydroxyl radical during arachidonic acid metabolism by human platelets
Authors: Singh Daljeet , Greenwald James E. , Bianchine Joseph , Metz Earl N. , Sagone Arthur L. 
Published: February 2007

AbstractReactive oxygen species, probably hydroxyl radicals (OH·), have been suggested to be generated during arachidonic acid (AA) metabolism and, once released, these species can modify the rate and extent of various reactions involved in AA metabolism. We have studied this phenomenon in washed human platelets. OH· generation was quantitated using 14C‐benzoic acid as a specific trap in a continuous ionization chamber system. Resting platelets did not produce any detectable signal, whereas addition of AA resulted in gradual OH· production with peak values detected at approximately 20 min. Similar studies conducted under nitrogen or after boiling the platelets almost abolished OH· generation. Aspirin had no significant effect, whereas 5,8,11,14‐eicosatetraynoic acid decreased the signal by > 90%, thus suggesting that OH· is produced primarily through the lipoxygenase pathway. Superoxide dismutase (SOD) and catalase had no effect and, as expected, phenol and mannitol decreased OH· production considerably, by > 50% and 90%, respectively. Azide and cyanide also reduced the OH· generation by about two‐thirds. We conclude that OH· is generated during AA metabolism by human platelets. It is primarily produced via the lipoxygenase pathway and may require a heme‐dependent peroxidase. This highly reactive oxidant may play an important role in normal and abnormal hemostasis.
Evidence that circulating immune complexes remove transfused platelets from the circulation
Authors: Kutti Jack , Zaroulis Charles G. , Safai‐Kutti Soodabeh , Dinsmore Robert E. , Day Noorbibi K. , Good Robert A. 
Published: February 2007

AbstractFifteen recently diagnosed patients with acute leukemias admitted for induction chemotherapy were selected for study. When thrombocytopenic (venous platelet count <20 × 109/1) these patients received prophylactic platelet transfusions. A total of 67 platelet transfusion therapies were administered and evaluated. Using the Raji cell radioimmunoassay, the serum concentrations of circulating immune complexes (CIC) were measured immediately before and 10–12 hr after each platelet transfusion. In 36 instances, elevated values of CIC were present in the recipient's pretransfusion samples, the corresponding posttrans‐fusion values being significantly lower (P < 0.05). Furthermore, in those 36 instances the mean percentage for the posttransfusion platelet increment was significantly lower (P < 0.001) than in the remaining 31 instances in which normal pretransfusion values of CIC were measured. We conclude that CIC were an important factor in rapidly removing transfused platelets from the circulation, thereby, adversely affecting the benefit of platelet transfusions.
Differences in synthesis of membrane proteins by leukemic cells from spleen and peripheral blood indicate distinct subsets of malignant cells in a patient with prolymphocytic leukemia
Authors: Spiro Robert C. , Ansell Jack , Katayama Isao , Muralidharan Kamala , Sullivan John L. , Humphreys Robert E. 
Published: February 2007

AbstractMorphological and biochemical differences were demonstrated between prolymphocytic leukemia cells obtained from the spleen and peripheral blood of one patient. Peripheral blood prolymphocytes had consistently smaller nuclear‐cytoplasmic ratios than did splenic prolymphocytes. Percoll gradient‐purified prolymphocytes from the spleen synthesized abundant amounts of some membrane proteins which were hardly expressed by peripheral blood prolymphocytes. Peripheral blood prolymphocytes did not change their expression of membrane proteins during three days in culture. These findings are consistent with the view that prolymphocytic leukemia cells from the spleen exist, on the average, at an earlier stage of differentiation than do circulating leukemic cells, and that peripheral blood leukemic cells are frozen at a specific phase of differentiation.
Problems in measurement of erythrocyte calcium
Authors: O'Rear Ed. A. , Udden Mark M. , McIntire Larry V. , Lynch Edward C. 
Published: February 2007

AbstractAs calcium has increasingly been the object of study in erythrocyte physiology, we reviewed the current methodologies for determination of calcium by atomic absorption spectrometry. The published normal values for erythrocyte calcium vary from 5 to 500 μmol/liter of packed cells. A method based on Harrison and Long's determination of calcium is presented and shows normal red cell calcium concentration to be 0.0149 ± 0.0023 μmol/ml of packed red cells. The influence of temperature and type of crucible used in ashing red cells is assessed. The method of additions is employed to corroborate our results.
Primary splenic hairy cell leukemia — remission for 21 years following splenectomy
Authors: Myers Thomas J. , Ikeda Yasuo , Schwartz Stanley , Pharmakidis Angelo B. , Baldini Mario G. 
Published: February 2007

AbstractA patient with primary splenic hairy cell leukemia is reported. This patient presented with massive splenomegaly and pancytopenia due to hypersplenism. Exploratory laparotomy failed to demonstrate any disease outside the spleen and splenic hilar lymph nodes. Splenectomy was the only form of treatment. During the following 21 years, no recurrent hairy cell leukemia has been found. This case allows speculation that hairy cell leukemia may originate in the spleen and that prolonged survivial or cure of the disease after Splenectomy alone may be due to removal of stem cells in the spleen.
Diagnosis and treatment of anticoagulant‐related adrenal hemorrhage
Authors: Anderson Kenneth C. , Kuhajda Francis P. , Bell William R. 
Published: February 2007

AbstractAnticoagulant‐related adrenal hemorrhage, as a cause of adrenal insufficiency, is rarely suspected and diagnosed during life. We report three patients in whom prompt diagnosis allowed successful treatment with replacement doses of corticosteroids. In all three patients, a hypotensive episode preceded the signs and symptoms of acute adrenal insufficiency. The symptoms and signs of adrenal hemorrhage were nonspecific and variable, making clinical diagnosis difficult. In none of our patients was the prothrombin time, partial thromboplastin time, or Lee White clotting time prolonged beyond the therapeutic range, and in none were excessive doses of anticoagulant therapy employed. Characteristic serum chemistry abnormalities, serum ACTH stimulation tests, and daily urinary collections for free cortisol, 17‐OH ketosteroids, and 17‐ketosteroids confirmed the clinical suspicion of primary adrenal insufficiency in all cases. Diagnosis was aided in one patient by abdominal computerized axial tomographic scan. In all patients, response to replacement doses of corticosteroids was prompt and gratifying, underlining the importance of suspecting this complication of anticoagulant therapy and instituting prompt lifesaving therapy.Adrenal hemorrhage is a complication of anticoagulant therapy that has been diagnosed during life in only 11 reported cases. We describe three patients in whom early diagnosis allowed prompt medical therapy and favorable outcome. The clinical presentation, diagnostic laboratory features, and therapeutic modalities are reviewed. Prolongation of prothrombin time, high levels of stress, and hypotension predispose to this complication in a patient maintained on anticoagulant therapy.
Two cases of red cell aldolase deficiency associated with hereditary hemolytic anemia in a japanese family
Authors: Miwa Shiro , Fujii Hisaichi , Tani Kenzaburo , Takahashi Keisuke , Takegawa Susumu , Fujinami Noboru , Sakurai Minoru , Kubo Masako , Tanimoto Yasuo , Kato Tomoaki , Matsumoto Noboru 
Published: February 2007

AbstractTwo cases of red cell aldolase deficiency associated with congenital nonspherocytic hemolytic anemia are reported. The proband is a fourteen‐month‐old Japanese boy. Consanguineous marriage was not proven but probable in this family, as the parents were born in the same small island. The proband had moderate to mild anemia aggravated by upper respiratory infections, 1 cm hepatomegaly and 2.5 cm splenomegaly, but was unremarkable in other respects and has thus far not shown mental or growth retardation. He did not have dysmorphic features. The red cell aldolase activity was 6% of the normal mean. The enzyme was unstable with respect to heat, and Km for fructose 1,6‐diphosphate (F‐1,6‐DP) was high. The parents and other heterozygotes showed intermediate activity between that of the proband and that of normal subjects. Red cell F‐1,6‐DP concentration in this case was remarkably increased. Red cell glucose consumption, and lactate formation, as well as hexose monophosphate shunt activity, were decreased as compared with a comparable reticulocyte‐rich hereditary spherocytosis patient. Hexose monophosphate shunt activity was assumed to be decreased due to inhibition of glucose 6‐phosphate dehydrogenase by a high concentration of F‐1,6‐DP in his red cells.As a result of family study, another homozygous aldolase deficiency case associated with hemolytic anemia was found. He is 13 years old and a nephew of the proband's paternal grandmother. His hemolytic anemia also is moderate to mild and aggravated by upper respiratory infections. He does not seem to have mental or growth retardation, nor does he possess dysmorphic features.
Studies of nicotinamide adenine dinucleotide methemoglobin reductase activity in a jewish population
Authors: Moore Michael R. , Conrad Marcel E. , Bradley Edwin L. , Prchal Josef T. 
Published: February 2007

AbstractIn the original study of the deleterious effects of oxidant drugs on persons heterozygous for nicotinamide adenine dinucleotide methemoglobin reductase (NADH MetHb R) deficiency, several Jewish physicians used as controls had decreased enzyme activity. We collected blood samples from 555 Jewish persons to 1) estimate the heterozygote frequency for NADH MetHb R deficiency; and 2) determine if external variables such as age, gender, and ingested medications may alter the NADH MetHb R activity and thereby alter estimates of heterozygosity. Two persons possibly heterozygous for the deficiency were identified (carrier incidence = 1/277; calculated homozygote incidence = 1/309,000 Jewish births). A difference in enzyme activity was found between males and females (P < 0.03). We have examined the influence of the mean red cell age on the activity of erythrocyte NADH MetHb R and found it not to be materially influenced by mean red cell age; thus the possible intersex differences of mean red cell age cannot explain this observation. No significant difference (P > 0.1) was seen between persons ingesting the following drugs and those not: Dilantin; antidepressants/sedatives; cardiac, antineoplastic, or antigout/antiinflammatory drugs; thyroid supplements, estrogen‐containing preparations, and antihistamines. NADH MetHb R activity did not correlate with age or spontaneous abortion. We conclude that heterozygosity for NADH MetHb R deficiency is not prevalent among Ashkenazic Jewish persons, and that external variables should be considered to ensure accurate interpretation of NADH MetHb R activity for correct estimation of heterozygosity.
Existence of tartrate‐resistant acid phosphatase activity in differentiated lymphoid leukemic cells
Authors: Usui Tadao , Konishi Hiroshi , Sawada Hiroyoshi , Uchino Haruto 
Published: February 2007

AbstractAcid phosphatase (AcP) in neoplastic cells from various lymphoid leukemias was examined. In the cytochemical studies, tartrate‐resistant AcP (T‐rAcP) activity was observed in the neoplastic cells from well‐differentiated lymphoid leukemias such as adult T‐cell leukemia (ATL), B‐cell chronic lymphocytic leukemia (B‐CLL), T‐cell chronic lymphocytic leukemia (T‐CLL), and hairy‐cell leukemia (HCL). T‐rAcP activity was also detected in a small number of leukemic cells obtained from T‐cell acute lymphoblastic leukemia (T‐ALL), while it was not detected in the neoplastic cells from null‐ALL, macroglobulinemia, and multiple myeloma (MM). In the electrophoretical studies, fraction 1 (F‐1), F‐3, F‐3b, and F‐4 were completely tartrate‐sensitive, while F‐2 was partially resistant and F‐5 was completely resistant. T‐rAcP activity (F‐5) was observed in ATL cells, B‐CLL cells, and HCL cells, while it was not detected in ALL cells, macroglobulinemia cells, and MM cells. The present study indicates that T‐rAcP activity is observed not only in HCL cells but also in the well‐differentiated lymphoid cells such as ATL cells, B‐CLL and T‐CLL cells except the most highly differentiated forms of B‐cells of MM and macroglobulinemia.
The effect of a low‐phosphate diet on hematocrit and oxygen transport in uremic rats
Authors: Connelly Thomas J. , Caro Jaime , Erslev Allan J. , Silver Ruth 
Published: February 2007

AbstractDietary restriction of phosphate was found to prevent the development of anemia in partially nephrectomized rats. In an attempt to examine the reason for this beneficial effect, hematologic and nephrologic studies were carried out on normal and on partially nephrectomized rats fed either a normal or a low‐phosphate diet. It was first found that a low‐phosphate diet ameliorates the degree of renal impairment found four weeks after partial nephrectomy. Nevertheless, it did not eliminate it, and the degree of uremia actually observed should have been associated with a significant reduction in hemoglobin and red cell mass. However, it did reduce serum phosphate and red cell 2,3‐diphosphoglycerate (2,3 DPG) levels and increase hemoglobin affinity for oxygen to a degree that should impair oxygen transport to the tissues. That the low phosphate actually caused tissue hypoxia with increased stimulation of the bone marrow was furthermore suggested by the observation that normal rats fed a low‐phosphorous diet developed a significant increase above normal in hemoglobin concentration and red cell mass. It was concluded that the effect of a low‐phosphate diet on the anemia of uremia is caused by a combination of reduced renal failure and increased tissue hypoxia.
Morphologic observations of contact‐induced lysis of EBV‐infected B lymphocytes by autologous hand mirror T cells
Authors: Thomas William J. , Duval‐Arnould Bertrand , Creegan William J. , Schumacher Harold R. , Forman David S. , Strong Douglas M. 
Published: February 2007

AbstractTo study the possible immunologic role of hand mirror lymphocytes (HML) in the control of Epstein‐Barr virus (EBV)‐infected B lymphocytes in patients with infectious mononucleosis (IM), we studied the in vitro interactions of these cells by time‐lapse video light microscopy. Peripheral blood T lymphocytes isolated from three patients in the early recovery phase of IM were mixed with their autologous EBV‐infected B cells. Motile lymphocytes with their characteristic hand mirror shape were observed to attach by their uropods to the B cells. The HML remained attached for variable periods ranging from 45‐75 min. Following detachment, B cells that were in contact with HML underwent lysis. Mixtures of T cells from healthy donors and EBV‐infected cell lines exhibited only rare uropod formation with no attachment or lysis of B cells. The present experiment indicates that contact‐induced lysis of EBV‐infected B lymphocytes is operative in IM and that this process is mediated by the HML.
The uptake of etiocholanolone by human bone marrow immature granulocytes
Authors: Minguell José J. , Lee S. , Helmer R. E. , Gardner F. H. 
Published: February 2007

AbstractA cell fraction enriched in immature granulocytes was obtained after fractionation of human bone marrow by a three‐step procedure. This fraction sediments at an average density of 1.073 and has a high content of myelocytes and metamyelocytes. It showed the highest specific uptake of etiocholanolone when compared to other bone marrow cell fractions. Autoradiographic studies demonstrated a higher number of silver grains over myelocytes and metamyelocytes than in other cell types. These results suggest the presence in human bone marrow of a target cell for etiocholanolone, located in the late mitotic or postmitotic pool or granulocytes.