bluuebunny/crossref_metadata_2025_split huggingface dataset:
Thrombomodulin: A marker for endothelial damage during orthotopic liver transplantation
Authors: Himmelreich G. , Riewald M. , Rosch R. , Blumhard G. , Neuhaus P. , Roissant R. , Riess H. 
Published: February 2007

AbstractThrombomodulin is a surface protein of vascular endothelial cells. A smaller form of thrombomodulln in blood and urine, the soluble (s) thrombomodulln, appears to be derived from injured endothelial cells or to be proteolytically cleaved from thrombomodulin by proteases. Several in vitro and in vivo studies have suggested s thrombomodulin as a marker of endothelial damage. In orthotopic liver transplantation, platelet and leukocyte activation as well as prothrombin activation are suspected of being caused by damaged endothelial cells in the graft liver. We determined s thrombomodulin antigen as well as thrombin‐antithrombin III complexes, protein C, and antithrombin III activities in the course of 23 orthotopic liver transplantations. Samples were taken at 7 different time‐points intraoperatively, as well as out of the perfusate released from the graft liver vein during the flushing procedure with arterial blood prior to the opening of the hepatocaval anastomosis. Levels of s thrombomodulin antigen and thrombin‐antithrombin III complexes showed a significant increase with reperfusion of the graft liver and levels in the perfusate were higher (both: P = 0.0001) than the corresponding systemic levels. In parallel, antithrombin III decreased significantly with reperfusion and perfusate levels of antithrombin III and protein C activities were lower in the systemic circulation (both: P − 0.0001). In conclusion, high levels of s thrombomodulin antigen in the early reperfusion phase and in the perfusate strongly indicate endothelial damage to the graft liver vascular bed, paralleled and followed by signs of prothrombin activation. © 1994 Wiley‐Liss, Inc.
The use and limitations of the INR system
Authors: Ts'ao C. , Neofotistos Demetra 
Published: February 2007

AbstractThis study was undertaken to answer some practical questions physicians and medical directors of clinical laboratories face when they contemplate replacing their high international sensitivity index (ISI) thromboplastins with low ISI ones and using international normalized ratio (INR) in place of prothrombin time ratio (PTR) for monitoring warfarin therapy. To the question of whether low‐ISI thromboplastins would produce a prolonged PT on normal patients, the answer is probably no. To the question of the extent of normalization of disparate PTs, determined by high and low ISI thromboplastins, of patients on oral anticoagulants upon the conversion of PTR to INR, the answer is a mixed one. For those whose PTs were 14–20 sec, conversion of PTR to INR would markedly, but not completely, normalize the PTR values. In other words, there would be a lessening of disparity of the PTR after the conversion. For patients whose PTs were >20 sec, conversion of PTR to INR could even widen the disparity seen with the PTR. Finally, when PTs were assayed on different coagulation devices with the same reagent, highly congenial results were obtained. © 1994 Wiley‐Liss, Inc.
Anti‐annexin V antibody in systemic lupus erythematosus patients with lupus anticoagulant and/or anticardiolipin antibody
Authors: Matsuda Juzo , Saitoh Noriko , Gohchi Kengo , Gotoh Moritaka , Tsukamoto And Miyo 
Published: February 2007

AbstractWe investigated anti‐annexin V antibody (aANX) in patients with systemic lupus erythe‐matosus (SLE), and correlated to positivity with lupus anticoagulant (LA)/anticardiolipin antibody (aCL). aANX was positive in 12/47 SLE patients (26%), including 7 with β2‐glycoprotein 1 (GPl)‐dependent aANX. The positivity of aANX was higher in patients with aCL (19%) and LA/aCL (50%) than in those without LA/aCL (10%). From these results, it is concluded that aANX is an autoantibody closely related to LA/aCL, and can be a possible new risk marker for thrombosis. © 1994 Wiley‐Liss, Inc.
Trends in the incidence of polycythemia vera among olmsted county, Minnesota residents, 1935–1989
Authors: Anía Basilio J. , Suman Vera J. , Sobell Janet L. , Codd Mary B. , Silverstein Murray N. , Melton L. Joseph 
Published: February 2007

AbstractTo investigate the suggestion that the incidence of polycythemia vera has increased in recent decades, we ascertained secular trends in the incidence of polycythemia vera in Olmsted County, Minnesota, over the 55‐year period, 1935–1989. The inpatient and outpatient medical records of all potential cases of polycythemia vera in Olmsted County residents were reviewed and the diagnostic criteria of the Polycythemia Vera Study Group were applied. We found no indication of an increase in the age‐ and sex‐adjusted incidence of polycythemia vera, which averaged 1.9 per 100,000 person‐years (95% C.I., 1.4–2.5) over the study period. Incidence rates increased with age, and age‐adjusted incidence rates were greater for men (2.8 per 100,000 person‐years; 95% C.I., 1.8–3.9) than for women (1.3 per 100,000 person‐years; 95% C.I., 0.7–1.9), with the highest incidence rate (23.5 per 100,000 person‐years) among men aged 70–79 years. Survival was reduced in this inception cohort of 50 cases, compared to that expected for individuals of like age and sex (P < 0.0001); median survival following diagnosis was 7.2 years. © 1994 Wiley‐Liss, Inc.
Inhibition of hemoglobin S polymerization by N‐terminal band 3 peptides: New class of inhibitors: Solubility studies
Authors: Danish Elizabeth H. , Lundgren David W. , Harris John W. 
Published: February 2007

AbstractTwo synthetic peptides corresponding to the N‐terminal amino acids (AA) of band 3 were designed to inhibit deoxyhemoglobin S (deoxy S) polymerization through two different mechanisms. Peptide I, an N:1–15AA fragment, was employed to bind to the 2,3‐diphosphoglycerate (2,3‐DPG) receptor locus of single deoxy S molecules with 5–7 AA extending internally and the remaining 10‐8 AA projecting external to hemoglobin (Hb) S, thereby inhibiting polymerization by steric hindrance. Peptide II consisted of two N:1‐8AA + K (lysine) sequences linked by a coupler through the lysine, and it was employed to bind to the 2,3‐DPG loci of two deoxy S molecules, tethering them together to form “binary hemoglobin complexes” incapable of entering the polymer chains. Decreased polymerization would result from reduction in effective concentration of deoxy S.Binding of peptides to the 2,3‐DPG receptor loci was demonstrated by a progressive rightward shift in the hemoglobin oxygen binding curves as a function of increasing peptide concentrations.Inhibition of deoxy S polymerization was studied by equilibrium solubility measurements of purified, stripped solutions of Hb S. Physiologically significant inhibition was demonstrated for both peptides with near‐maximum increases in solubility achieved by Peptide II at 1:1 peptide:Hb S ratios.These peptides represent a new class of inhibitors of deoxy S polymerization. © 1994 Wiley‐Liss, Inc.
Acute spontaneous tumor lysis syndrome
Authors: Jasek Andrzej M. , Day H. James 
Published: February 2007

AbstractAn 83‐year‐old woman with no previous history of malignancy was admitted to our institution with weakness and anemia and subsequently developed acute tumor lysis syndrome secondary to newly diagnosed Burkitt's leukemia/lymphoma. This syndrome has been previously described in patients with hematologic malignancies; however, its development has been related to the administration of chemotherapy, steroids, or radiotherapy. The spontaneous occurrence of tumor lysis syndrome has not been previously reported; however, Cohen et al. [Am J Med 58:486–491, 1980] report 8 of 37 patients with “clinically insignificant pretreatment derangements” of serum potassium, phosphate, and calcium. © 1994 Wiley‐Liss, Inc.
Hereditary hemochromatosis: A prevalent disorder of iron metabolism with an elusive etiology
Authors: Conrad Marcel E. , Umbreit Jay N. , Moore Elizabeth G. , Parmley Richard T. 
Published: February 2007

AbstractHereditary hemochromatosis is a prevalent inherited disorder with an estimated frequency of homozygosity of 0.2 to 0.45% in Caucasians. The disease is characterized by progressive iron overload until a massive accumulation of body iron occurs. Undetected, the disorder eventually can produce either cirrhosis, diabetes mellitus, cardiac disease, arthritis, or hepatocellular carcinoma or a combination of these manifestations. Early diagnosis and treatment prevents organ damage and normalizes life expectancy. Screening studies to detect hemochromatosis are most effectively accomplished by measurement of the serum iron and total iron binding capacity. Treatment is most effectively performed by frequent phlebotomy until body stores are empty and then 3 to 4 times yearly for life. The basic defect of hemochromatosis appears to increase iron absorption, decrease iron excretion, and produce preferential deposit of iron in hepatic parenchymal cells rather than Kupffer cells. The genetic abnormality of hemochromatosis is located on chromosome 6 in close association with the gene for HLA antigens. Recent speculation postulates that tumor necrosis factor may be involved in the etiology of this disease because of its location on chromosome 6 and its effect upon iron transport. © 1994 Wiley‐Liss, Inc.
Bone marrow necrosis
Authors: Ranaghan L. , Morris T. C. M. , Desai Z. R. , Markey G. M. 
Published: February 2007

AbstractBone marrow necrosis is most frequently diagnosed at postmortem examination. Antemortem diagnosis is still uncommon. In a recent review of world literature, we have found 133 cases of bone marrow necrosis diagnosed during life. It has been observed during the course of a wide variety of diseases, most commonly in association with acute and chronic leukemia, carcinoma, malignant lymphoma, infections, and sickle cell disease.We report two intravitally diagnosed cases of bone marrow necrosis occurring in two patients with high‐grade B‐cell lymphoproliferative disease. These cases are unusual in that both patients had a triad of bone marrow necrosis, high‐grade B‐cell lymphoproliferative disease, and hypercalcemia. Despite chemotherapy, both cases ultimately proved fatal, with progressive involvement of the central nervous system. © 1994 Wiley‐Liss, Inc.
African‐American factor VII‐deficient variants in Georgia (FVII variants)
Authors: Krauss Jonathan S. , Matthews Anthony , Oliver Joyce , Lightsey Alton , Jonah Mary H. , Pantazis Cary G. 
Published: February 2007

AbstractWe studied African‐American Factor (FVII)‐deficient variants and carriers in Georgia by measuring their levels of FVII antigen (FVIIAG) and FVII procoagulant (FVIIC). Factor VIIAG was determined using enzyme‐linked immunoassay (ELISA), whereas FVIIC was measured in two ways: 1) by fibrin clotting methods that employed human recombinant (HRFVIIC), human placental (HPFVIIC), rabbit brain (RBFVIIC), and bovine brain (BBFVIIC) thromboplastins; and 2) by an amidolytic method (AMFVIIC). Prothrombin time tests (PT) were also performed by standard methods. These 4 FVII‐deficient patients and 3 carriers demonstrated the following results: PT: 18.2 ± 6.5 sec; FVIIAG: 73.0 ± 14.9; HRFVIIC: 30.6 ± 20.3%; HPFVIIC: 30.5 ± 21.4%; RBFVIIC: 25.3 ± 21.4%; BBFVIIC: 30.6 ± 17.5%; AMFVIIC: 44.1 ± 18.3. We conclude that a group of clinically mild African‐American FVII‐deficient variants exists in Georgia. This group is characterized by the presence of FVIIAG and decreased FVIIC, using a variety of thromboplastins; an excellent correlation was noted for both human thromboplastins. © 1994 Wiley‐Liss, Inc.
High expression of CD56 (N‐CAM) in a patient with cutaneous CD4‐positive lymphoma
Authors: Adachi Masaaki , Maeda Kazuo , Takekawa Mutsuhiro , Hinoda Yuji , Imai Kohzoh , Sugiyama Sadao , Yachi Akira 
Published: February 2007

AbstractCutaneous lymphoma is a disease characterized with massive skin infiltration of lymphoid malignant cells. They commonly express some T‐cell markers, such as CD2, CD3, CD4, and CD7, and thus termed as CTCL (cutaneous T cell lymphoma). Here, we present a case with CD56/N‐CAM‐positive cutaneous lymphoma, which appears lymphocytic morphology and expresses CD4, but does not express CD2, CD3, CD8, CD14, CD16, CD57, and CD20. The most malignant cells contained no distinctive azurophilic granules in the cytoplasm. Southern blot analysis revealed that T cell receptor‐β, γ, and immunoglobulin heavy chain genes in the cells were in germ‐line configurations. Electron microscopic examination showed characteristics of lymphoid cells with higher nucleocytoplasmic ratio and lacked structures typical of other cell types (i.e., epithelial cells, neuroendocrine cells, and mesenchymal cells). Thus, the cells are likely to be immature lymphoid cells. Histological analysis revealed the cells infiltrate mainly into the dermis with angiocentric growth pattern. The clinical course was aggressive, with rapid involvement of bone marrow and central nervous system. These striking features of the patient may represent a novel fraction (CD2−, CD4+, and CD56+) of cutaneous lymphoma. © 1994 Wiley‐Liss, Inc.
Erythrocyte membrane vesicles and irreversibly sickled cells bind protein S
Authors: Lane Peter A. , O'Connell Jacquie L , Marlar Richard A. 
Published: February 2007

AbstractPlasma levels of free protein S, a vitamin K‐dependent anticoagulant, are decreased in persons with sickle cell anemia, but the etiology of the low levels is unknown. Protein S binds to phosphatidylserine‐containing phospholipids in a calcium‐dependent manner. Other studies have indicated that phosphatidylserine may be abnormally present on the outer surface of the membrane lipid bilayer of sickle cells and of the spectrin‐depleted vesicles they shed in vivo. We studied the binding of purified, radiolabeled protein S to spectrin‐depleted erythrocyte membrane vesicles and to density‐separated fractions of sickle and normal erythrocytes. Calcium‐dependent binding of protein S occurred with vesicles and with well‐aerated dense irreversibly sickled cells, but not with well‐aerated sickle discocytes or with normal erythrocytes. These data provide further evidence that phosphatidylserine is abnormally present on the outer surface of spectrin‐depleted vesicles and of irreversibly sickled cells. In addition, protein S binding to such sickle membranes in vivo may be responsible, in part, for the decreased levels of free protein S in sickle cell plasma. © 1994 Wiley‐Liss, Inc.
A functional factor X deficiency
Authors: Sun Paul , Hata Jane , Bauer John , Haibach Carolyn , Campbell Doug , Farhangi Medhi , Smith Dan 
Published: February 2007

AbstractA functional factor X deficiency is described which caused pronounced reduction in the in vitro activation of the extrinsic system while marginally affecting the in vitro activation of the intrinsic pathway. All studies were normal with the exception of a prolonged PT, an elevated factor X antigen, and low factor X activity. Western blot analysis revealed the presence of two factor X species. The abnormal molecule was of higher molecular weight. Interestingly, there was no bleeding associated with this deficiency. The biochemical basis of this defect is currently under investigation. ©1995 Wiley‐Liss, Inc.
Genetic heterogeneity of β‐thalassemia in southeast sicily
Authors: Schilirò G. , di Gregorio F. , Samperi P. , Mirabile E. , Liang R. , Cürük M. A. , Ye Z. , Huisman T. H. J. 
Published: February 2007

AbstractIn this study we have defined the spectrum of the β‐thalassemia mutations, the β‐thalassemia haplotypes, and the genotype‐to‐phenotype correlations in a large number of patients with different β‐thalassemia conditions. Seventeen different (β‐thalassemia mutations were detected which included one chromosome each with Hb Dhonburi and Hb Lepore. Five alleles, namely, codon 39 (C→T), IVS‐l‐110 (G→A), IVS‐l‐6 (T C), IVS‐ll‐745 (C→G), and IVS‐l‐1 (G→A), account for 90% of all β‐thalassemia mutations in 846 thalassemic chromosomes studied.Haplotyping for a large number of subjects showed that the five common mutations are linked to a few haplotypes. The presence of milder mutations, mainly IVS‐l‐6 (T C), in about 19% of our patients explains some of the clinical variables. Among the 37 patients with thalassemia of intermediate severity, only 6 were homozygous or compound heterozygous for two severe alleles. The type of β‐thalassemia is the main factor responsible for differences in the phenotypic expression of the disease in patients with Hb S‐β‐thalassemia; patients with Hb S‐β+‐thalassemia are less severely affected than those with Hb S‐β°‐thalassemia. The five most frequent mutations have comparable distributions all Over Sicily. ©1995 Wiley‐Liss, Inc.
Combination Therapy With Recombinant Human Granulocyte Colony‐Stimulating Factor and Erythropoietin in Aplastic Anemia
Authors: Imamura Masahiro , Kobayashi Masanobu , Kobayashi Sumiko , Yoshida Kohki , Mikuni Chikara , Ishikawa Yorikazu , Matsumoto Shuzo , Sakamaki Sumio , Niitsu Yoshiro , Hinoda Yuji , Yachi Akira , Kudoh Tohru , Chiba Shunzo , Kasai Masaharu , Oka Toshiaki , Okuno Akimasa , Maekawa Isao , Sakurada Keisuke , Miyazaki Tamotsu 
Published: February 2007

AbstractRecombinant human granulocyte colony‐stimulating factor (rhG‐CSF) and erythropoietin (rhEPO) were used to treat patients with aplastic anemia (AA). In terms of effects on erythrocyte recovery, the combined use of rhG‐CSF and rhEPO showed a favorable response in 6 of 14 (42.9%) patients with moderate AA following 10 weeks treatment and in 3 of 14 (21.4%) patients thereafter. However, the response was poor in patients with severe AA (3/13). A favorable response in severe AA was observed in 1 of 13 (7.7%) patients following 10 weeks treatment and in 2 of 13 (15.4%) patients thereafter. The overall effect on erythrocytes was observed in 44.4% patients. A dose of 400 μ/m2 G‐CSF was sufficient to cause an increase in neutrophil count and 100 IU/kg rhEPO appeared to be sufficient to cause an increase in erythrocyte count. In 6 of 27 (22.2%) patients, a trilineage response was observed. Interestingly, a delayed and long‐lasting effect was obtained in 5 of 27 (18.5%) patients. These results suggest that rhG‐CSF can synergize with rhEPO in erythrocyte response, especially in patients with moderate AA.
Hyperaggregation of platelets in intestinal tuberculosis: Role of platelets in chronic inflammation
Authors: Sarode Ravindra , Bhasin Deepak , Marwaha Neelam , Roy Pallab , Singh Kartar , Panigrahi D. , Garewal Gurjeevan , Mehta S. K. 
Published: February 2007

AbstractEighty‐eight percent (38/43) patients of intestinal tuberculosis showed significant hyperaggregation of platelets (P < 0.001). Serum and plasma from 15 patients when incubated with normal platelets caused hyperaggregation. Gel filtered platelets from 2 patients suspended in normal plasma showed hyperaggregation of platelets with arachidonic acid. Tubercular protein had no effect on platelet aggregation. A role of hyperactive platelets in chronic inflammatory response is discussed. ©1995 Wiley‐Liss, Inc.
Simultaneous assessment of cell kinetics and programmed cell death in bone marrow biopsies of myelodysplastics reveals extensive apoptosis as the probable basis for ineffective hematopoiesis
Authors: Raza Azra , Mundle Suneel , Iftikhar Amna , Gregory Stephanie , Marcus Bridget , Khan Zaineb , Alvi Sairah , Shetty Vilasini , Dameron Sherry , Wright Vonda , Adler Solomon , Loew Jerome M. , Shott Susan , Ali Syed Nadir , Preisler Harvey 
Published: February 2007

AbstractDespite hypercellular bone marrows (BM), peripheral cytopenias are the rule in patients with myelodysplastic syndromes (MDS). This study examined the roles played by cell birth and cell death rates in generating this paradox. Cell kinetics from BM biopsies of 35 MDS patients were measured using intravenous infusions of either iododeoxyuridine or bromodeoxyuridine, or both. Degree of apoptosis or programmed cell death (PCD) was estimated using in situ end‐labeling of DNA directly from BM biopsies, which were simultaneously double‐labeled for proliferation/PCD. MDS were found to be highly proliferative disorders with large numbers of myeloid, erythroid, and megakaryocytic cells synthesizing DNA. Median cycling time (Tc) of myeloblasts was more rapid than that of patients with acute myeloid leukemia (44.1 hr vs. 56.0 hr). Interestingly, most marrow cells of all three lineages in 32 of 34 evaluable cases were undergoing PCD. In 19 of 32 patients, greater than 75% cells were apoptotic. Surprisingly, large numbers of S‐phase cells were found to be simultaneously undergoing PCD, as were stromal cells of the BM microenvironment. We conclude that the extensive apoptosis in hematopoietic cells effectively cancels the high birth rate resulting in ineffective hematopoiesis and accounting for deficient bone marrow function. ©1995 Wiley‐Liss, Inc.
Abnormal platelet von willebrand factor (vWF) as a marker of abnormal function in megakaryocytic dysplasia
Authors: de Cataldo F. , Baudo F. , Redaelli R. , Corno A. R. 
Published: February 2007

AbstractThe myelodysplastic syndromes (MDS) are neoplastic disorders of the hemopoietic system; multilineage involvement is also evidenced by specific cellular dysfunctions. The von Willebrand factor (vWF), synthesized and processed in the megakaryocytes (MK), is stored in the α granules of the platelets. The platelet vWF multimeric pattern was studied in 18 patients with MDS, and in 4 with pernicious anemia (PA), to investigate whether the processing of vWF is abnormal in the megakaryocytic dysplasia. An abnormal multimeric pattern was observed in 10/18 MDS and 4/4 PA patients. The abnormality of this specific protein is the discrete expression of the basic disorder, and is reversible when hemopoiesis is normalized. Although the data do not allow any conclusion, abnormal synthesis is the likely explanation of the abnormality. ©1995 Wiley‐Liss, Inc.
Lupus anticoagulant in children with thrombosis
Authors: Manco‐Johnson Marilyn J. , Nuss Rachelle 
Published: February 2007

AbstractNineteen children who presented with thromboses over a 7‐year period were found to have a lupus anticoagulant (LA). The initial thrombosis was proximal deep vein thrombosis (DVT) in six children, central nervous system (CNS) in five, primary pulmonary in four, distal DVT in two, central venous in one, and proximal arterial in one. Five children were diagnosed with systemic lupus erythematosus (SLE), including two children for whom thrombosis was the presenting sign of SLE. The remaining 14 children were diagnosed with the antiphospholipid antibody (APA) syndrome. The APA syndrome was manifest by venous or arterial thrombosis in association with a positive LA; positive anticardiolipin antibodies and a fine, speckled antinuclear antibody (ANA) pattern were additionally found in the majority of children. Approximately one‐half of the children with SLE or the APA syndrome had a pulmonary embolus, and one‐half developed recurrent thrombosis. Oral anticoagulation with coumadin to achieve an INR of >2.0 prevented thrombosis recurrence. The recognition of a LA in children with thrombosis necessitates evaluation for SLE, APA, and other autoantibodies. © 1995 Wiley‐Liss, Inc.