bluuebunny/crossref_metadata_2025_split huggingface dataset:
Elevated urinary levels of thromboxane and prostacyclin metabolites in sickle cell disease reflects activated platelets in the circulation
Authors: Kurantsin‐Mills Joseph , Ibe Basil O. , Natta Clayton L. , Raj J. Usha , Siegel Robert S. , Lessin Lawrence S. 
Published: March 2008

SummaryThere is evidence for increased factor VII turnover and the associated increased thrombin generation and fibrinolytic activities in sickle cell disease (SCD) that may affect in vivo platelet and endothelial cell reactivity. We studied the release of specific eicosanoids that are indicative of in vivo platelet activation and endothelial cell injury. The circulating and urinary levels of 2,3‐dinor thromboxane B2(2,3‐dinor‐TxB2), TxB2, PGI2 [as 6‐keto‐PGFlα], and PGE2 were measured in 15HbSS patients, eight HbAA nonhaemolytic anaemic individuals and 12 healthy HbAA controls using specific RIAs. The mean urinary 2,3‐dinor‐TxB2 in the HbSS patients was significantly higher than in both the healthy HbAA and the anaemic controls. 6‐keto‐PGFlα was undetected in the urines of the healthy HbAA controls, but was measured insignificant amounts in the HbSS and the HbAA anaemic patients. The urinary concentrations of PGE2 and TxB2 in HbSS patients' samples were also significantly higher than those of both control groups (P < 0.05). PGE2 and TxB2 levels were below the detection limit in the plasmas of the HbAA subjects, but were measurable in the HbSS and HbAA anaemic plasmas. The plasma level of 6‐keto‐PGFlα in the HbSS patients was also significantly higher than in the control groups. The data indicates a persistent inflammatory process in the HbSS patients, and is consistent with the hypothesis that there is platelet and endothelial cell activation in SCD.
Anti β2 glycoprotein I antibodies: detection and association with thrombosis
Authors: Martinuzzo M. E. , Forastiero R. R. , Carreras L. O. 
Published: March 2008

Summary. It has been demonstrated that antiphospholipid antibodies (aPL) recognize epitopes formed by anionic phospholipids and protein cofactors. β2 glycoprotein I (β2GPI) is accepted as the cofactor of anticardiolipin antibodies (aCL). In the present study we explored the presence and clinical associations of anti β2GPI antibodies of IgG isotype (aβ2GPI‐IgG), measured by ELISA. We studied sera from 169 patients with aCL and/or lupus anticoagulant (LA), including 52 patients with systemic lupus erythematosus and 49 with primary antiphospholipid syndrome (PAPS). We found 31.9% positive sera for aβGPI‐IgG in the whole population and 48.6% in the aCL‐IgG(+) group. There was a good correlation between the titre of aCL‐IgG and the optical density for aβGPI‐IgG (r= 0.69, P <0.01). The presence of aβ2GPI‐IgG was associated with the presence of aCL‐IgG (P <0.0001) and LA (P <0.0005). However, none of 23 LA (+) patients without aCL had aβ2GPI‐IgG.We found a statistically significant association between the presence of aβ2GPI‐IgG and a history of venous thromboembolism (VTE) in our patients (P <0.005). This association was observed in PAPS (P <0.05) but not in secondary antiphospholipid syndrome (SAPS).Our study confirms that some aPL(+) sera react with β2GPI in special experimental conditions. In addition, the presence of these antibodies is associated with a history of VTE.
Acquired hypochromic and microcytic sideroblastic anaemia responsive to pyridoxine with low value of free erythrocyte protoporphyrin: a possible subgroup of idiopathic acquired sideroblastic anaemia (IASA)
Authors: TAKEDA YASUSHI , SAWADA HIROYOSHI , SAWAI HIROFUMI , TOI‐MATSUDA TOMOKO , TASHIMA MASARO , OKUMA MINORU , WATANABE SHIGEYA , OHMORI SEIICHI , KONDO MASAO 
Published: March 2008

Patients with idiopathic acquired sideroblastic anaemia (IASA) usually show macrocytic or normocytic anaemia and increased free erythrocyte protoporphyrin (FEP). The mean cell haemoglobin concentration is normal or slightly low. Here we report a pyridoxine‐responsive IASA patient with microcytic and hypochromic anaemia and low FEL level; these features are usually seen in cases of hereditary sideroblastic anaemia. Microcytosis increased during therapy.There may be a subgroup of IASA with microcytic and hypochromic anaemia, low normal FEP and some response to pyridoxine like hereditary sideroblastic anaemia.
Polyclonal haemopoieses associated with long‐term persistence of the AML1‐ETO transcript in patients with FAB M2 acute myeloid leukaemia in continous clinical remission
Authors: GUERRASIO ANGELO , ROSSO CLAUDIA , MARTINELU GIOVANNI , COCO FRANCESCO LO , PAMPINELLA MARIA , SANTORO ALESSANDRA , LANZA CARLO , ALLIONE BERNARDINO , RESEGOTTI LUIGI , SAGLIO GIUSEPPE 
Published: March 2008

Summary. The t(8;21) (q22;q22) translocation is a recurring chromosomal abnormality observed in about 20‐40% of AML patients with subtype FAB M2 (AML‐M2). The molecular facet of this translocation is represented by the formation of a new hybrid gene, the AML1‐ETO, which is regularly transcribed in a chimaeric mRNA and translated into a new fusion protein believed to have a key role in the pathogenesis of this type of leukaemia. We looked for the presence of AML1‐ETO transcripts, by RT‐PCR, in 49 unselected patients affected by AML‐M2 diagnosed at various Italian Institutions. A hybrid transcript was detected in 11 cases (23%). Minimal residual disease status was investigated in three patients in continuous complete remission (CCR) after a median follow‐up of 44 months; at least one sample from each subject was found positive for the AML1‐ETO transcript suggesting a long‐term persistence of t(8;21) leukaemic cells.In two female patients in CCR a‘clonality’analysis was performed on peripheral blood DNA by exploiting the × chromosome inactivation pattern of the human androgen‐receptor gene (HUMARA); in both cases the results were consistent with the presence of a polyclonal haemopoiesis.Our data confirm that the persistence of residual cells expressing the AML1‐ETO transcripts is a frequent occurrence even in patients with long‐term remission; on the other hand, clonality assays indicate that in t(8;21) leukaemias long‐term remission haemopoiesis is sustained by a polyclonal bone marrow reconstitution.
Comparative study of peripheral blood progenitor cell collection in patients with multiple myeloma after single‐dose cyclophosphamide combined with rhGM‐CSF or rhG‐CSF
Authors: DEMUYNCK HILDE , DELFORGE MICHEL , VERHOEF GREGOR , ZACHEE PIERRE , VANDENBERGHE PETER , BOOGAERTS MARC 
Published: March 2008

Summary. Patients suffering from high‐risk multiple myeloma (MM) were randomized to receive single high‐dose cyclophosphamide followed by either rhGM‐CSF or rhG‐CSF in order to harvest circulating peripheral blood progenitor cells. The safety of the procedure, the mobilization kinetics, the relative efficacy of rhGM‐CSF and rhG‐CSF to mobilize progenitor cells and their relative toxicity were studied. Special attention was paid to the antigenic profile of CD34+ progenitor cells.Group I patients (n=ll) were treated with cyclophosphamide 4 g/m2 i.v. followed by rhGM‐CSF at 10 /ig/kg/d by subcutaneous administration. Group II (n=ll) patients received rhG‐CSF s.c. at 10/zg/kg/d after the same dose cyclophosphamide. Both mobilization regimens appeared to be equally effective. No significant differences in absolute numbers of circulating progenitors, determined by CD34 expression or in yields of MNC, CFU‐GM, BFU‐E and CD34 subsets were observed. rhGM‐CSF administration resulted however in delayed haemopoietic recovery and an increased complication rate.We conclude that rhG‐CSF may be preferred because of its markedly lower toxicity and lower in‐hospital costs.
The effect of haemopoietic growth factors on the cell cycle of AML progenitors and their sensitivity to cytosine arabinoside in vitro
Authors: SMITH M. A. , SINGER C. R. J. , PALLISTER C. J. , SMITH J. G. 
Published: March 2008

Summary. This study examines the effect of pretreatment in liquid culture of acute myeloid leukaemic (AML) progenitors with recombinant human IL‐3 or G and GM‐CSF. Prior to and following cytokine priming, the sensitivity of cells to cytosine arabinoside (Ara‐C) at concentrations ranging from 10−12 to 1(T−4 M was assessed in clonogenic culture. In addition, the initial percentage of AML cells in S phase was assessed and their subsequent kinetic response to cytokine treatment evaluated by FACScan analysis. Light‐density marrow cells (LDMCs) from 19 AML patients were initially T‐cell and monocyte depleted in order to remove potential sources of endogenous cytokine production prior to in vitro investigation. LDMCs were incubated in liquid phase for 7 d in a chemically defined complete medium with or without cytokines.Clonogenic data from fresh AML LDMCs not pretreated with growth factors demonstrated a heterogenous response to Ara‐C. In only 4/15 marrows tested clonogenically was there any improvement in sensitivity to Ara‐C following cytokine priming. S‐phase data on all 19 marrows were similarly variable either before or after cytokine preincubation. There was no discernible correlation between clonogenic and kinetic data, nor could any relationship be established between in vitro findings and the FAB subtypes of patients or clinical outcomes.In summary, it would appear that the cell‐cycle status of AML cells is likely to be only one of many contributory factors governing the sensitivity of AML progenitors to Ara‐C. The clinical response of AML patients to cytokine therapy in association with cell‐cycle‐specific cytotoxic agents may therefore be variable and unpredictable.
Degradation of platelet glycoprotein Ib by elastase released from primed neutrophils
Authors: Aziz Khalil A. , Cawley John C. , Kamiguti Aura S. , Zuzel Mirko 
Published: March 2008

Summary Mutual interactions between neutrophils (PMN) and platelets are recognized to be important in modulating the respective functions of these two cell types. Here we show that primary granule secretion from appropriately‐stimulated PMN can lead to complete proteolytic removal of GPIb from the platelet surface.Thus, when the PMN in PMN/platelet mixtures were stimulated by FMLP, platelets lost GPIb as measured by ristocetin‐induced aggregation, flow cytometry and SDS‐PAGE analysis. This loss was most marked when PMN were primed by GM‐CSF, and could be inhibited by a specific elastase inhibitor. As expected, the al‐antiproteinase in plasma inhibited GPIb loss, but when PMN were strongly stimulated by FMLP and GM‐CSF in the presence of platelets this inhibition was incomplete or absent.It is concluded that joint priming of PMN with GM‐CSF and platelets can cause a previously unrecognized degree of primary granule secretion which, via elastase, leads to platelet GPIb loss. We suggest that this loss is likely to be of physiological and pathophysiological importance.
Increased platelet CD36 constitutes a common marker in myeloproliferative disorders
Authors: Thibert V. , Bellucci S. , Cristofari M. , Gluckman E. , Legrand C. 
Published: March 2008

Summary. The distribution of the major platelet membrane glycoproteins (GP), Ib, IX, IIb‐IIIa and IV (or CD36), which play important roles as receptors for adhesive molecules in haemostasis and thrombosis, was studied in 34 patients with myeloproliferative disorders (MPD): 13 had essential thrombocythaemia (ET), 12 had polycythaemia vera (PV) and nine had chronic myelogenous leukaemia (CML). Only occasionally were modifications of the numbers of GPIb or GPIIb‐IIIa measured using the binding of specific radiolabeled antibodies to platelets. In contrast, 2‐3‐fold increases of the total CD36 content and the surface CD36 expression were measured in almost all patients studied, using a radioimmunoassay and the direct binding of the radiolabelled antibody, FA6‐152, to the platelet surface, respectively. These results indicate that the abnormality affected both the external and internal CD36 pools. Therefore platelet CD36 may be a useful tool for the diagnosis and the follow‐up of MPD patients.Surface CD36 has been proposed as a platelet receptor for thrombospondin, an adhesive glycoprotein that is released from platelets upon activation and promotes aggregate formation. Despite a 2‐fold increase of CD36 molecules, resting and thrombin‐activated platelets from ET patients expressed the same amount of thrombospondin as normal platelets, suggesting that there is not a direct correlation between the CD36 expression and thrombospondin binding either spontaneously or after activation.
Role of Epstein‐Barr virus and soluble CD21 in persistent polyclonal B‐cell lymphocytosis
Authors: LARCHER CLARA , FEND FALKO , MITTERER MANFRED , PRANG NADJA , SCHWARZMANN FRITZ , HUEMER HARTWIG P. 
Published: March 2008

Summary. The expression of EBV proteins and immunological properties were studied in the first stable cell line (SM) established from a patient presenting with persistent polyclonal B‐cell lymphocytosis (PPBL).SM cells which represent a small population of EBV‐positive atypical cells found in the peripheral blood of the patient express the KI‐1 antigen (CD30) as well as the proto‐oncogene bcl‐2 product and cell surface markers of mature activated B lymphocytes. The cells harbour an EBV subtype A genome and contain EBNA2 protein. This argues against a transformation‐incompetent virus as the main cause of the chronic active EBV infection observed in our patient. Latent membrane protein (LMP1) was weakly expressed and found predominantly in a perinuclear localization, a location which could lead to decreased immunogenicity in vivo. Similar to the EBV‐transformed marmoset cell line B95‐8, SM cells were in part productively infected as transcription of the immediate early gene BZLF1 could be shown and in some cells high levels of EBV‐genome were detected by in situ hybridization with a BamHl W‐probe. Comparable to the atypical cells in the peripheral blood of the patient, EBV small RNAs were not detected with EBER‐specific probes.Of interest, we noticed a markedly increased production of soluble CD21 (sCD21) antigen by SM cells as compared to LCL‐type Burkitt's lymphoma cell lines. This could explain the elevated sCD21 levels observed in the serum of our PPBL patient and confirms our previous findings in patients with acute EBV infection. It also suggests a possible role of sCD21 in EBV‐mediated regulation of the immune response and provides a possible explanation for the dysregulation of the humoral immune system observed in PPB1 patients.
Absence of incorporation of plasma von Willebrand factor into porcine platelet a‐granules
Authors: ROUSSI JACQUELINE , DROUET LUDOVIC , SIGMAN JEFF , VAIMAN MARCEL , PIGNAUD GEORGES , BONNEAU MICHEL , MASSE JEAN‐MARC , CRAMER ELISABETH MARTIN 
Published: March 2008

Summary. In order to study the relationship between plasma and platelet von Willebrand factor (vWF), we used an experimental model of crossed bone marrow transplantation (BMT) between SLA immunocompatible normal and homozygous von Willebrand (vWD) pigs. A normal pig received bone marrow from a vWD pig and a second pig with vWD was engrafted with marrow from a normal pig.Each recipient, after total irradiation of 10 Grays, received by a central catheter 1010 monocellular bone marrow cells without immunosuppression. The animals were followed for 50 d and no graft rejection or graft‐versus‐host disease was observed. After aplasia occurring 3 weeks after BMT, white blood cells and platelets returned to normal.Before transplantation, in the vWD pig, vWFAg and vWF activity were not detected in plasma and in platelet and megakaryocyte a‐granules. After transplantation with normal marrow, platelet vWFAg and platelet vWF activity were normal and high molecular weight multimers and numerous tubular structures were present in a‐granules.Before transplantation, the normal pig had normal plasma and platelet vWFAg‐vWF activity, normal multimeric pattern, and the platelet and megakaryocyte a‐granules displayed many tubular structures, eccentrically located in one of their poles, coinciding with immunogold staining vWFAg. After transplantation with homozygous vWD marrow, platelet and megakaryocyte a‐granules lacked tubular structures. Alpha‐granule immunogold staining for vWF was consistently negative, although plasma vWF was at a normal level. In conclusion, this study shows that, unlike other plasma proteins such as fibrinogen, vWF endocytosis does not occur from plasma to the platelet a‐granules. Platelet and megakaryocyte vWF solely originates from megakaryocyte endogenous synthesis and is independent of plasma vWF.
Analysis of molecular breakpoint and m‐RNA transcripts in a prospective randomized trial of interferon in chronic myeloid leukaemia: no correlation with clinical features, cytogenetic response, duration of chronic phase, or survival
Authors: Shepherd Patricia , Suffolk Ruth , Halsey Jim , Allan Norman 
Published: March 2008

Two hundred and nineteen cases of Ph+ve CML and 15 Ph‐ve, BCR+ve CML cases have been analysed to determine the breakpoint site and its relationship to clinical features, cytogenetic response, duration of chronic phase and survival. 119 cases have had RNA analysis performed to determine the type of BCR/ABL transcript and have also been analysed in a similar way. Presenting features at diagnosis including age, sex, white‐cell count and platelet count showed no significant difference for those with 5’and 3’breakpoints and those with either b2a2 or b3a2 BCR/ABL transcripts. However, in a subgroup of patients whose presenting white‐cell count was <100×p109/1, those with b3a2 transcript did have a significantly higher platelet count.Analysis by Sokal risk grouping showed no difference for 5’or 3’breakpoints but a trend for lower stage among those with b2a2 transcripts. No correlation was found either for genomic breakpoint site or BCR/ABL RNA transcript in terms of duration of chronic phase or survival. When stratified by randomized therapy, either interferon‐α or standard chemotherapy, no difference was noted in relation to genomic breakpoint site or BCR/ABL transcript. Cytogenetic response was not related to the molecular findings.
Habitat heterogeneity overrides the species–area relationship
Authors: Báldi András 
Published: March 2008

AbstractAim  The most obvious, although not exclusive, explanation for the increase of species richness with increasing sample area (the species–area relationship) is that species richness is ultimately linked to area‐based increases in habitat heterogeneity. The aim of this paper is to examine the relative importance of area and habitat heterogeneity in determining species richness in nature reserves. Specifically, the work tests the hypothesis that species–area relationships are not positive if habitat heterogeneity does not increase with area.Location  Sixteen nature reserves (area range 89–11,030 ha) in central Hungary.Methods  Four‐year faunistic inventories were conducted in the reserves involving c. 70 fieldworkers and 65 taxonomists. CORINE 50,000 land‐cover maps were used for calculating the heterogeneity of the reserve landscape (number of habitat types, number of habitat patches and total length of edges).Results  Large reserves were less heterogeneous than small reserves, probably because large reserves were established in large blocks of unproductive land whereas small reserves tended to be in more fertile land. In total, 3975 arthropod species were included in the analysis. The slope of the species–area relationship was positive only for Neuroptera and Trichoptera. There was no significant relationship in the other nine taxa examined (Collembola, Acari, Orthoptera, Thysanoptera, Coleoptera, Araneae, Diplopoda, Chilopoda, Diptera). The density (number of species ha−1) of all species, however, showed a positive correlation with heterogeneity.Main conclusions  The general lack of fit of species–area relationships in this study is inconsistent with most previous published studies. Importantly, and unlike many other studies, habitat heterogeneity was not correlated with reserve area in the studied system. In the absence of this source of covariation, stronger relationships were identified that suggested a fundamental link between species richness and habitat heterogeneity. The results indicate that habitat heterogeneity rather than area per se is the most important predictor of species richness in the studied system.
The association between cheese consumption and cardiovascular risk factors among adults
Authors: Houston D. K. , Driver K. E. , Bush A. J. , Kritchevsky S. B. 
Published: March 2008

AbstractBackground  Although current dietary guidelines recommend limiting foods high in fat and saturated fat, such as high‐fat dairy, the effect of cheese consumption on body composition and cardiovascular risk factors is largely unknown.Methods  Participants from a US population‐based survey, NHANES III, aged 25–75 years who completed a food frequency questionnaire and had measures of body composition and cardiovascular risk factors were included (n = 10 872). Linear regression was used to compare anthropometrics, blood lipids, blood pressure and blood glucose across categories of cheese consumption (combined full and low‐fat).Results  In women, more frequent cheese consumption was associated with higher HDL‐C and lower LDL‐C (p for trend, < 0.05). However, in men, more frequent cheese consumption was associated with a higher body mass index (BMI), waist circumference, HDL‐C and LDL‐C, and diastolic blood pressure (p for trend, < 0.05). Men consuming 30 +  servings/month had significantly higher BMI, waist circumference, and diastolic blood pressure compared to nonconsumers (P < 0.05).Conclusions  More frequent cheese consumption was associated with less favourable body composition and cardiovascular risk profile in men, but with a more favourable cardiovascular risk profile in women. However, the type of cheese consumed by men and women may have differed resulting in opposing trends on body composition and cardiovascular risk factors.
Assessing dietary patterns in Barbados highlights the need for nutritional intervention to reduce risk of chronic disease
Authors: Sharma S. , Cao X. , Harris R. , Hennis A. J. M. , Wu S.‐Y. , Leske M. C. ,   for the Barbados National Cancer Study Group
Published: March 2008

AbstractBackground  The dietary habits of the Caribbean have been changing to include more fast foods and a less nutrient dense diet. The aims of this study are to examine dietary patterns in Barbados and highlight foods for a nutritional intervention.Methods  Four‐day food diaries collected from control participants in the population‐based, case–control Barbados National Cancer Study (BNCS).Results  Forty‐nine adult participants (91% response) completed the diaries providing 191 days of dietary data. Total energy intake was almost identical to data collected 5‐years earlier in the Barbados Food Consumption and Anthropometric Survey 2000, but the percent energy derived from fat was from 2.1% to 5.2% higher. Sugar intake exceeded the Caribbean recommendation almost four‐fold, while intakes of calcium, iron (women only), zinc and dietary fibre were below recommendations. Fish and chicken dishes were the two largest sources of energy and fat. Sweetened drinks and juices provided over 40% of total sugar intake.Conclusions  These data provide existing dietary patterns and strongly justify a nutritional intervention program to reduce dietary risk factors for chronic disease. The intervention could focus on the specific foods highlighted, both regarding frequency and amount of consumption. Effectiveness can be evaluated pre‐ and post‐intervention using our Food Frequency Questionnaire developed for BNCS.