bluuebunny/crossref_metadata_2025_split huggingface dataset:
G‐6‐PD Walter Reed: Possible insight into “structural” NADP in G‐6‐PD
Authors: Beutler Ernest , Hartman Kip , Gelbart Terri , Forman Linda 
Published: February 2007

AbstractA new Gd‐PD variant, Gd‐PD Walter Reed, causing hereditary nonspherocytic hemolytic anemia is characterized. This variant is unusual in that its stability requires the presence of high concentrations of NADP, while its Km for NADP is normal. This finding is consistent with the suggestion that Gd‐PD has two separate binding sites, a high affinity “structural” site and a lower affinity catalytic site. The mutation in G‐6‐PD Walter Reed, like that of the previously described variant, G‐6‐PD Torrance, may be due to a mutation of the “structural” site for NADP.
Acute renal infarction in sickle cell disease
Authors: Granfortuna James , Zamkoff Kenneth , Urrutia Enrique 
Published: February 2007

AbstractEnd organ failure in sickle cell disease has classically been attributed to changes in the microvasculature. A case is reported in which sudden and complete loss of blood flow to the left kidney occurred during a painful crisis in a woman with homozygous SS disease. The findings are most consistent with occlusion of the renal artery.
Serum markers for type IV collagen and type III procollagen in the myelofibrosis‐osteomyelosclerosis syndrome and other chronic myeloproliferative disorders
Authors: Hasselbalch H. , Junker P. , Lisse I. , Bentsen K. D. , Risteli L. , Risteli J. 
Published: February 2007

AbstractMyelofibrosis is characterized by excessive deposition of interstitial and basement membrane collagens in the bone marrow. In this study, specific radioimmunoassays for the aminoterminal propeptide of type III procollagen and for the 7S collagen domain of type IV (basement membrane) collagen were used to determine how this accumulation is reflected in serum.Of the 41 patients with chronic myeloproliferative disorders studied, the highest levels of both parameters were found in idiopathic myelofibrosis and in chronic myelogenous leukaemia associated with bone marrow fibrosis. Increasing degrees of bone marrow fibrosis were accompanied by increasing serum concentrations of both markers, except for osteomyelosclerosis, where notably low values were seen. Pathologically high values of one or both parameters were also found in a few patients with polycythaemia Vera or a transitional myeloproliferative disorder.The antigens related to type III procollagen and type IV collagen correlated significantly with each other and with the leucocyte count. These parameters should provide noninvasive means for following the accumulation of interstitial and basement membrane collagens in the bone marrow.
Excessive fibrinolysis in suspected amyloidosis: Demonstration of plasmin‐α2‐plasmin inhibitor complex and von Willebrand factor fragment in plasma
Authors: Takahashi Hoyu , Koike Tadashi , Yoshida Nobuhiko , Kitahara Osamu , Hanano Masaharu , Shibata Akira , Aoki Nobuo 
Published: February 2007

AbstractWe performed a hemostatic evaluation in detail in a patient with suspected amyloidosis who was suffering from several bleeding episodes. He had a shortened euglobulin clot lysis time, decreased alpha 2‐plasmin inhibitor (α2‐PI), decreased plasminogen, elevated tissue‐type plasminogen activator (t‐PA), elevated plasmin‐α2‐PI complex, and decreased ratio of ristocetin cofactor to von Willebrand factor (vWF) antigen. Fibrinogen and fibrin/fibrinogen degradation products levels fluctuated, with abnormal values on several occasions. On crossed immunoelectrophoresis, plasmin‐α2‐PI complex and vWF fragment were demonstrated in the patient plasma. These abnormal findings and bleeding symptoms improved following the administration of tranexamic acid. Discontinuation of tranexamic acid resulted in deterioration of these parameters. These observations indicate that pathologic fibrinolysis (continuous intravascular plasmin generation) characterized by the consumption of α2‐PI and plasminogen, formation of plasmin‐α2‐PI complex, and fragmentation of vWF contributed to the bleeding in this patient. It is important to recognize excessive fibrinolysis as the underlying cause of bleeding in these patients, since specific treatment with antifibrinolytic agents is effective in controlling the bleeding.
In vitro effects of gammaglobulin (IgG) on human monocyte Fc receptor function. I. Effect on monocyte membrane‐associated IgG and Fc receptor‐dependent binding of antibody‐coated platelets
Authors: Saleh Mansoor N. , Court Wayne S. , Lobuglio Albert F. 
Published: February 2007

AbstractIntravenous immunoglobulin (IV IgG) has been reported to be clinically beneficial for the treatment of immune thrombocytopenic purpura (ITP). The mechanism of effect still remains unknown. We examined the in vitro effects of two commercially available IV IgG preparations. Exposure of normal monocytes to IgG in vitro produced a significant increase in monocyte‐bound IgG. Prior treatment of the commercial IgG preparations by filtration through a 0.2‐μm Millipore filter or ultracentrifugation caused a dramatic decrease in IgG bound to the monocyte surface, indicating that IgG aggregates were responsible for this effect. Exposure of monocytes to IgG levels as high as 150 mg (Sandoglobulin) and 400 mg (Gamimune) did not result in a statistically significant inhibition of monocyte‐platelet interaction as examined by a morphologic rosetting assay. Thus, despite the ability of IV IgG preparations to cause substantial increments in monocyte surface IgG, impairment of Fc receptormediated monocyte binding of antibody‐coated platelets was not observed.
Increased fibrinolytic activity in patients undergoing cardiopulmonary bypass operation
Authors: Kucuk Omer , Kwaan Hau C. , Frederickson James , Wade Leonard , Green David 
Published: February 2007

AbstractThe influence of cardiopulmonary bypass on fibrinolytic activity was assessed by a sensitive clot lysis assay in eight patients undergoing coronary artery bypass operations. Fibrinolytic activity increased immediately after cardíopulmonary bypass was begun and remained elevated throughout the period of extracorporeal circulation. At the conclusion of the operative procedure, fibrinolytic activity returned to the normal range in all patients, and no subject had postoperative bleeding.
Kinetic analysis in single, intact cells by microspectrophotometry: Evidence for two populations of erythrocytes in an individual heterozygous for glucose‐6‐phosphate dehydrogenase deficiency
Authors: Ashmun Richard A. , Hultquist Donald E. , Schultz Jerome S. 
Published: February 2007

AbstractA microspectrophotometric technique was used to measure the kinetics of methemoglobin reduction in intact, unaltered human erythrocytes. Reduction was catalyzed by endogenous NADPH‐methemoglobin reductase in the presence of Nile Blue. The technique was applied to the study of erythrocytes from a female donor with decreased glucose‐6‐phosphate dehydrogenase (G‐6‐PD) activity. The individual was shown to be heterozygous for deficiency of G‐6‐PD. The kinetic study revealed two distinct populations of erythrocytes that were nearly equal in number. One cell population showed reduction rates between 0 and 25% of normal, whereas the second cell population displayed rates within the range seen for normal cells. Single‐cell indices of cell size, cell hemoglobin content, and ratio of cell hemoglobin to cell size did not correlate with single‐cell reduction rates and were not significantly different between the two populations. These results provide quantitative support for the X‐inactivation hypothesis in G‐6‐PD deficiency.
Pure red cell aplasia: Response to therapy with anti‐thymocyte globulin
Authors: Abkowitz Janis L. , Powell Jerry S. , Nakamura Jeffrey M. , Kadin Marshall E. , Adamson John W. 
Published: February 2007

AbstractPure red cell aplasia (PRCA) results from the failure of erythrocyte differentiation and may respond to immunosuppressive therapies. We have treated nine patients with PRCA refractory to steroids and/or cyclophosphamide with anti‐thymocyte globulin (ATG). Six patients had normal numbers of erythroid bursts (from erythroid burstforming units) or erythroid colonies (from erythroid colony‐forming units) detectable in vitro, and all responded to therapy with ATG. In vitro studies suggested T‐cell inhibition of erythropoiesis in four of these six patients and humorally mediated erythroid suppression in one. In three individuals, virtually no erythroid progenitors were detected in marrow culture. None of these patients responded to ATG. Myelo‐fibrosis, 5q‐ chromosomal abnormality, or the subsequent development of thrombo‐cytopenia in these individuals suggested that PRCA resulted from an intrinsic stem cell disorder. Our studies demonstrate that ATG is effective therapy for PRCA, and it may be especially useful in children or other patients in whom alkylating agents are not appropriate. We also confirm that erythroid growth in marrow culture predicts those patients who will respond to ATG or other immunosuppressive therapies.
Echinocyte‐stomatocyte transformation and shape control of human red blood cells: Morphological aspects
Authors: Reinhart Walter H. , Chien Shu 
Published: February 2007

AbstractRed cell morphology was studied after the induction of echinocytic transformation by metabolic depletion, Ca2+ loading, and salicylate and stomatocytic transformation with chlorpromazine. The results indicate that the red cell has an energy‐dependent shape control mechanism that allows it to counteract shape‐changing stimuli such as metabolic depletion. Albumin was found to induce stomatocytic transformation, whereas γ‐globulins induced echinocytic transformation. Loading of the red cell with calcium resulted in polymorphous membrane damages such as submembranous, “blister‐like” lesions, and membrane disintegration; the red cell age had no influence on this process. Conversely, the stomatocyte‐echinocyte transformation induced by chlorpromazine and salicylate was shifted towards echinocytes in density‐separated old red cells. Sphero‐stomatocytes were capable of echinocytic transformation with spicule formation within the red cell vacuoles, whereas sphero‐echinocytes were unable to undergo stomatocytic transformation without hemolysis. These observations may help to unravel the complexity of echinocyte‐stomatocyte transformation of red blood cells.
Clinical and molecular correlations in the sickle/β+‐thalassemia syndrome
Authors: Atweh George F. , Forget Bernard G. 
Published: February 2007

AbstractSickle/β thalassemia is a sickling disorder of varying severity which results from compound heterozygosity for sickle cell trait and β‐thalassemia trait. Clinical and genetic studies have shown an inverse correlation between the level of hemoglobin A and the severity of the disease. It has been suggested that the level of hemoglobin A may be a function of the severity of the β‐thalassemia defect. In this study, we use molecular biological techniques to test this hypothesis. We show that the interaction of the mildest of the β+‐thalassemia genes with the sickle gene results in a high level of hemoglobin A. However, the interaction in this case resulted in a severe sickling disorder in the absence of significant anemia. We hypothesize that a mild β+‐ thalassemia gene may have two opposite effects on the clinical course of sickle/β+ thalassemia: (1) A high level of hemoglobin A which probably confers a favorable antisickling effect and (2) decreased hemolysis leading to increased numbers of total circulating red cells, thereby increasing the blood viscosity and the propensity for sickling. The inheritance of heterozygous α thalassemia 2 in conjunction with the mild β+‐thalassemia gene and sickle gene in this patient may have further enhanced the latter effect and resulted in a severe sickling disorder.
Leu 7+, Leu 11a− acute T‐lymphoblastic leukemia having low K cell activity and NO NK cell activity
Authors: Falcäo Roberto P. , Ismael Sebastiäo J. 
Published: February 2007

AbstractThe phenotypic and functional features of the leukemic blasts from a child with Tacute lymphoblastic leukemia (T‐ALL) were studied. The leukemic cells lacked the usual markers of T‐cell lineage (T3−, T11−, E‐sheep−) although they displayed some T‐lymphocyte markers (T6+, T8+, T9+, T10+) and were Tγ−. Furthermore, these cells had a strong reaction with anti‐Leu 7 but were negative to anti‐leu 11a antibody and exhibited low K cell activity, no NK activity, and showed virtually no response to PHA. These leukemic cells probably represented the leukemic counterpart of the Leu 7+, Leu 11a− subset that has been demonstrated in the peripheral blood of normal individuals.
Normal pregnancy in a patient with a postpartum factor VIII inhibitor
Authors: Vicente V. , Alberca I. , Gonzalez R. , Alegre A. 
Published: February 2007

AbstractA patient who had developed a VIII:C IgG‐Kappa antibody after her first delivery became pregnant for a second time. At that time the titer of the inhibitor had increased fourfold, remaining at high levels throughout the rest of the pregnancy. The neonate exhibited an inhibitor with characteristics identical to that of the mother which could not be detected 3 months after birth. No hemorrhagic complications in either of the subjects were observed.
Restoration of impaired natural killer cell activity of B‐chronic lymphocytic leukemia patients by recombinant interleukin‐2
Authors: Kay Neil E. , Zarling Joyce 
Published: February 2007

AbstractNatural killer (NK) function in the majority of B‐cell chronic lymphocytic leukemia patients is markedly deficient. This study was undertaken to determine if the biological response modifier interleukin‐2 (IL‐2), which is a potent augmenter of normal individuals' NK activity, could augment the low NK activity in these patients. Peripheral blood lymphocytes (PBL), depleted of B‐cells, from most B‐CLL patients exhibited low natural killer activity against NK‐sensitive K562 cells and against herpes simplex virus (HSV)‐infected lymphoblastoid cell lines (LCL). Incubation of patients' B‐cell‐depleted PBL with recombinant IL‐2 resulted in augmentation of their NK activity against both K562 cells and HSV‐infected cells. Furthermore, whereas large granular lymphocytes (LGL) isolated from CLL patients are deficient in cytoplasmic granules, which are thought to play a role in NK‐cell‐mediated lysis, treatment of patients' LGL resulted in increased granulation by 4 hr after treatment with IL‐2 and showed a concomitant increase in lytic activity comparable to that of normal individuals.
Additional variant of type I von willebrand disease
Authors: Tavori S. , Tatarsky I. 
Published: February 2007

AbstractWe introduce a family with a von Willebrand subgroup that has not been described before. All of the eight subjects examined had normal levels of factor VIII coagulant activity (FVIII:C), a moderate reduction in the level of von Willebrand factor antigen (VWF:Ag), which resulted in a high FVIII: C/VWF: Ag ratio, and normal crossed immunoelectrophoresis, with normal multimeric pattern. The ristocetin cofactor in plasma and platelets was very low. Bleeding time was prolonged in two subjects without clearcut linkage to laboratory findings. In addition, an abnormality of platelet aggregation in response to ADP was observed: a decreased initial response was followed by marked disaggregation.
Prothrombin “Mexico city,” an asymptomatic autosomal dominant prothrombin variant
Authors: Valls‐de‐Ruiz Matilde , Rulz‐Arguelles Alejandro , Ruiz‐Arguelles Guillermo J. , Ambriz Raul 
Published: February 2007

AbstractA functionally normal but structurally abnormal prothrombin variant was found in a Mexican family. Immunoisoelectricfocusing studies revealed that this variant has a more acidic isoelectric point (4.01) than normal prothrombin (4.29), but it proved to have a normal molecular weight as assessed by sodium‐dodecyl‐sulphate polyacrylamide gel electrophoresis. Two‐dimensional immunoelectrophoresis studies showed an abnormal cleavage of the prothrombin molecule by factor Xa and Echis carinatus venom as well, despite the fact that both yield functionally normal thrombin molecules. Finally, the ability of the molecule to bind calcium ions as well as its overall antigenic structure were investigated and found to be normal. These results taken together suggest a simple (substitution or translocation) mutation at the fragment 2 level. Since this prothrombin variant is different from others described previously, the name “Mexico City” is proposed to identify it.
Incidence of inhibitors in patients with severe and moderate hemophilia a treated with factor VIII concentrates
Authors: Schwarzinger Iise , Pabinger Ingrid , Korninger Christian , Haschke Ferdinand , Kundi Michael , Niessner Herwig , Lechner Klaus 
Published: February 2007

AbstractRecent data published on the prevalence of inhibitors to factor VIII in hemophiliacs on treatment show great variations, with prevalence rates ranging from 3.6 to 14.2%. We have studied the cumulative risk of inhibitor development in a cohort of 62 patients with hemophilia A. All patients were born after 1960, were natives of the Vienna area, had a factor VIII activity of less than 5%, and were treated at least once. Using the method of Cutler and Ederer, the cumulative risk of inhibitor development was found to be 24% at the age of 25 years. Most inhibitors developed between the ages of 3 and 7 years. The current prevalence of F VIII inhibitors in the group of patients studied is 17.5%. It is concluded that prevalence data underestimate the true risk of inhibitor development.
Hematological aspect of Rh deficiency syndrome: A case report and a review of the literature
Authors: Nash R. , Shojania A. M. 
Published: February 2007

AbstractThe hematological aspects of the original case of Rhmod are reported. The subject, as in other reported cases, had a chronic hemolytic anemia characterized by stomatocytosis, reduced osmotic fragility, and abnormal autohemolysis correctable with the addition of glucose. The 51Cr red cell survival studies showed the spleen to be the preferential site of red cell destruction and splenectomy produced a dramatic improvement in red cell survival. The topic of Rh deficiency syndrome (Rhnull and Rhmod) is briefly reviewed with regard to the number of cases reported, to genetic aspects, to the hematological findings, and to the results of splenectomy.
Effectiveness of oral iron chelators assayed in the rat
Authors: Kim Byoung‐Kook , Huebers Helmut A. , Finch Clement A. 
Published: February 2007

AbstractA 4‐hour in vivo test for iron chelator activity in the rat is described. The amount of radioiron in the gut and urine that results from chelator‐induced excretion of previously injected radioiron labelled ferritin is measured. Hepatocyte localization and desferrioxamine‐induced radioiron mobilization from in vitro tagged homologous ferritin is shown to be similar to that from in vivo tagged ferritin. Non‐homologous ferritin preparations labelled in vitro proved unsatisfactory. Radioiron mobilization by chelator occurred regardless of the iron status of the animal. Employing this measurement, the effectiveness of three iron chelators, pyridoxal isonicotinoyl hydrazone, the dimethyl ester of ethylene diamine‐N, N′‐dis (3‐hydroxyphenyl acetic acid), and the dimethyl ester N, N′‐di (3‐hydroxybenzyl) ethylene‐1, ethylene‐1, 2‐diamine‐N, N′‐diacetic acid, given orally was determined. All three chelators, when given in comparable dosage, induced iron execretion similar in amount to that observed with parenteral desferrioxamine. In addition, pyridoxal isonicotinoyl hydrazone administered in the diet over a period of 4 weeks was shown to reduce hepatic and splenic iron of normal animals by about one third, providing further validation of this method of evaluating chelator effectiveness.
Cyclosporin treatment for aplastic anemia: A case report demonstrating a second response to second exposure to cyclosporin
Authors: Bern Murray M. , Roberts Mark S. , Yoburn David 
Published: February 2007

AbstractCyclosporin has been used in the treatment of aplastic anemia, often in combination with other drugs, making it difficult to make a clear distinction between the effects of cyclosporin and those of other treatments. This report demonstrates a clear response of a patient with aplastic anemia to single drug cyclosporin therapy.
Thrombotic thrombocytopenic purpura: Treatment with plasmapheresis
Authors: Blitzer Jonathan B. , Granfortuna James M. , Gottlieb Arlan J. , Smith J. Robert , Theodorakis Michael E. , Zamkoff Kenneth W. , Landaw Stephen A. , Goldberg Jack , Scalzo Anthony J. , Lamberson Harold 
Published: February 2007

AbstractTwenty‐one episodes of thrombotic thrombocytopenic purpura (TTP) were treated with plasmapheresis. Adjunctive agents included corticosteroids, aspirin, dipyridamole, and vincristine. There were 17 patients; 12 were female. The median age was 41 years. Most patients presented with neurologic symptoms. Thrombocytopenia was profound with a mean initial platelet count of 14,900/mm3. The mean hematocrit on presentation was 26.7% and the mean LDH 1300 IU/L. Eighteen episodes responded completely following plasmapheresis/plasma exchange (86%). Response was prompt, the initial rise in platelet count occurred after a mean of four exchanges, and complete response (a platelet count over 150,000/mm3) was obtained after a mean of nine exchanges. Four of the episodes treated were relapses that occurred in three patients. All responders are alive with a median duration of follow‐up of 20 months. The three patients who failed to respond have died. This report extends recent observations that the addition of plasmapheresis/plasma exchange to the therapy of TTP has significantly improved the outlook for patients with this disorder.
Clinical and laboratory changes induced by alpha interferon in chronic lymphocytic leukemia‐a pilot study
Authors: Talpaz M. , Rosenblum M. , Kurzrock R. , Reuben J. , Kantarjian H. , Gutterman J. 
Published: February 2007

AbstractTen chronic lymphocytic leukemia patients were treated with partially pure alpha interferon (IFN‐α) at doses ranging from 3‐9 × 106 units administered intramuscularly daily. Of these patients, three patients with disease stages 0, 1, and 3 (and prolymphocytic leukemia), respectively, responded with partial remissions lasting from 10 to 24+ months; four additional patients had minor responses. Among those responding, modulation of B cell chronic lymphocytic leukemia, surface immunoglobulins, and surface antigens were observed in four patients. These changes included a decline in the proportion of B cells bearing surface immunoglobulin without change in the number of the B cells. Likewise, a decline in the proportion of B cells bearing T‐1 antigen was observed in three patients. Increase in the percentage of T cells, primarily the T helper cells, was seen in two of the responding patients. Specific binding of α interferon was demonstrated prior to therapy in CLL cells of all patients. This binding declined rapidly following treatment with IFN α.Induction of the enzyme 2′ 5′ oligoisoadenylate synthetase was examined in CLL patients prior to and during therapy with IFN‐α. Variable levels of the enzyme were induced in all patients on therapy; however, only minimal induction was observed in two of three patients failing therapy.