bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mast cell tryptase: a review of its physiology and clinical significance
Authors: Payne V. , Kam P. C. A. 
Published: June 2004

SummaryMast cells, which are granulocytes found in peripheral tissue, play a central role in inflammatory and immediate allergic reactions. β‐Tryptase is a neutral serine protease and is the most abundant mediator stored in mast cell granules. The release of β‐tryptase from the secretory granules is a characteristic feature of mast cell degranulation. While its biological function has not been fully clarified, mast cell β‐tryptase has an important role in inflammation and serves as a marker of mast cell activation. β‐Tryptase activates the protease activated receptor type 2. It is involved in airway homeostasis, vascular relaxation and contraction, gastrointestinal smooth muscle activity and intestinal transport, and coagulation. Serum mast cell β‐tryptase concentration is increased in anaphylaxis and in other allergic conditions. It is increased in systemic mastocytosis and other haematological conditions. Serum β‐tryptase measurements can be used to distinguish mast cell‐dependent reactions from other systemic disturbances such as cardiogenic shock, which can present with similar clinical manifestations. Increased β‐tryptase levels are highly suggestive of an immunologically mediated reaction but may also occur following direct mast cell activation. Patients with increased mast cell β‐tryptase levels must be investigated for an allergic cause. However, patients without increased mast cell tryptase levels should be investigated if the clinical picture suggests severe anaphylaxis.
A comparison of the intubating laryngeal mask airway and the Bonfils intubation fibrescope in patients with predicted difficult airways*
Authors: Bein B. , Worthmann F. , Scholz J. , Brinkmann F. , Tonner P. H. , Steinfath M. , Dörges V. 
Published: June 2004

SummaryTracheal intubation with the intubating laryngeal mask airway or the Bonfils intubation fibrescope was performed in 80 patients with predicted difficult airways. Mallampati score, thyromental distance, mouth opening and mobility of the atlanto‐occipital joint were used to predict difficult airways. The overall success rate, time to the first adequate lung ventilation and time taken for the successful placement of the tracheal tube were recorded, as well as a subjective assessment of the handling of the device and the incidence of postoperative sore throat and hoarseness. The median [range] time to the first adequate ventilation was significantly shorter with the intubating laryngeal mask airway than with the Bonfils intubation fibrescope (28 [6–85] s vs. 40 [23–77] s, p < 0.005). Tracheal intubation was significantly slower with the intubating laryngeal mask airway than with the Bonfils intubation fibrescope (76 [45–155] s vs. 40 [23–77] s, p < 0.0001. Patients in the Bonfils group suffered less sore throat and hoarseness than those in the other group.
Evaluation of a local anaesthesia regimen following mastectomy
Authors: Talbot H. , Hutchinson S. P. , Edbrooke D. L. , Wrench I. , Kohlhardt S. R. 
Published: June 2004

SummaryBreast surgery can be emotionally distressing and physically painful. Acute pain following surgery is often related mainly to the axillary surgery and is aggravated by arm and shoulder movement. We conducted a prospective double‐blind, randomised, placebo‐controlled trial to determine the influence of local anaesthetic irrigation of axillary wound drains on postoperative pain during the first 24 h following a modified Patey mastectomy (mastectomy with complete axillary node clearance). The treatment group received bupivacaine irrigation through the axillary wound drain 4‐hourly for 24 h postoperatively. Controls received irrigation with normal saline. Morphine via a patient controlled analgesia pump was used for postoperative analgesia. Morphine consumption, visual analogue and verbal rating pain scores were recorded. There were no statistical differences in morphine requirements or pain scores between the two groups, nor were there differences in anti‐emetic or supplemental analgesic consumption. Bupivacaine irrigation used in this manner does not appear to offer an effective contribution to postoperative analgesia.
Guidelines for the use of fresh‐frozen plasma, cryoprecipitate and cryosupernatant
Authors:   British Committee for Standards in Haematology, Blood Transfusion Task Force (J. Duguid, Chairman), O'Shaughnessy D. F. , Atterbury C. , Bolton Maggs P. , Murphy M. , Thomas D. , Yates S. , Williamson L. M. 
Published: June 2004

The indications for transfusing fresh‐frozen plasma (FFP), cryoprecipitate and cryosupernatant plasma are very limited. When transfused they can have unpredictable adverse effects. The risks of transmitting infection are similar to those of other blood components unless a pathogen‐reduced plasma (PRP) is used. Of particular concern are allergic reactions and anaphylaxis, transfusion‐related acute lung injury, and haemolysis from transfused antibodies to blood group antigens, especially A and B. FFP is not indicated in disseminated intravascular coagulation without bleeding, is only recommended as a plasma exchange medium for thrombotic thrombocytopenic purpura (for which cryosupernatant is a possible alternative), should never be used to reverse warfarin anticoagulation in the absence of severe bleeding, and has only a very limited place in prophylaxis prior to liver biopsy. When used for surgical or traumatic bleeding, FFP and cryoprecipitate doses should be guided by coagulation studies, which may include near‐patient testing. FFP is not indicated to reverse vitamin K deficiency for neonates or patients in intensive care units. PRP may be used as an alternative to FFP. In the UK, PRP from countries with a low bovine spongiform encephalopathy incidence is recommended by the Departments of Health for children born after 1 January 1996. Arrangements for limited supplies of single donor PRP of non‐UK origin are expected to be completed in 2004. Batched pooled commercially prepared PRP from donors in the USA (Octaplas) is licensed and available in the UK. FFP must be thawed using a technique that avoids risk of bacterial contamination. Plastic packs containing any of these plasma products are brittle in the frozen state and must be handled with care.
Is fresh frozen plasma clinically effective? A systematic review of randomized controlled trials
Authors: Stanworth S. J. , Brunskill S. J. , Hyde C. J. , McClelland D. B. L. , Murphy M. F. 
Published: June 2004

SummaryRandomized controlled trials of good quality are a recognized means to robustly assess the efficacy of interventions in clinical practice. A systematic identification and appraisal of all randomized trials involving fresh frozen plasma (FFP) has been undertaken in parallel to the drafting of the updated British Committee for Standards in Haematology guidelines on the use of FFP. A total of 57 trials met the criteria for inclusion in the review. Most clinical uses of FFP, currently recommended by practice guidelines, are not supported by evidence from randomized trials. In particular, there is little evidence for the effectiveness of the prophylactic use of FFP. Many published trials on the use of FFP have enrolled small numbers of patients, and provided inadequate information on the ability of the trial to detect meaningful differences in outcomes between the two patient groups. Other concerns about the design of the trials include the dose of FFP used, and the potential for bias. No studies have taken adequate account of the extent to which adverse effects might negate the clinical benefits of treatment with FFP. There is a need to consider how best to develop new trials to determine the efficacy of FFP in different clinical scenarios to provide the evidence base to support national guidelines for transfusion practice. Trials of modified FFP (e.g. pathogen inactivated) are of questionable value when there is little evidence that the standard product is an effective treatment.
Haemostatic and inflammatory biomarkers in advanced chronic heart failure: role of oral anticoagulants and successful heart transplantation
Authors: Cugno Massimo , Mari Daniela , Meroni Pier Luigi , Gronda Edoardo , Vicari Francesco , Frigerio Maria , Coppola Raffaella , Bottasso Bianca , Borghi Maria Orietta , Gregorini Luisa 
Published: June 2004

SummaryAdvanced chronic heart failure (CHF) is associated with abnormal haemostasis and inflammation, but it is not known how these abnormalities are related, whether they are modified by oral anticoagulants (OAT), or if they persist after successful heart transplantation. We studied 25 patients with CHF (New York Heart Association class IV, 10 of whom underwent heart transplantation) and 25 age‐ and sex‐matched healthy controls by measuring their plasma levels of prothrombin fragment 1 + 2 (F1 + 2), thrombin–antithrombin (TAT) complexes, tissue plasminogen activator (t‐PA), plasminogen activator inhibitor‐1 (PAI‐1), D‐dimer, factor VII (FVII), fibrinogen, von Willebrand factor (VWF), tumour necrosis factor (TNF), soluble TNF receptor II (sTNFRII), interleukin 6 (IL‐6), soluble intercellular adhesion molecule‐1 (sICAM‐1), soluble vascular cell adhesion molecule‐1 (sVCAM‐1), endothelial‐selectin (E‐selectin) and thrombomodulin. CHF patients had higher plasma levels of TAT, D‐dimer, t‐PA, fibrinogen, VWF, TNF, IL‐6, sTNFRII, sVCAM‐1 (P = 0·0001), sICAM‐1 (P = 0·003) and thrombomodulin (P = 0·007) than controls. There were significant correlations (r = 0·414–0·595) between coagulation, fibrinolysis, endothelial dysfunction and inflammation parameters, which were lower in those patients treated with OATs. Heart transplantation led to reductions in fibrinogen (P = 0·001), VWF (P = 0·05), D‐dimer (P = 0·05) and IL‐6 levels (P = 0·05), but all the parameters remained significantly higher (P = 0·01–0·0001) than in the controls. Advanced CHF is associated with coagulation activation, endothelial dysfunction and increased proinflammatory cytokine levels. Most of these abnormalities parallel each other, tend to normalize in patients treated with OATs and, although reduced, persist in patients undergoing successful heart transplantation, despite the absence of clinical signs of CHF.
Analysis of IgVH gene mutations in B cell chronic lymphocytic leukaemia according to antigen‐driven selection identifies subgroups with different prognosis and usage of the canonical somatic hypermutation machinery
Authors: Degan Massimo , Bomben Riccardo , Bo Michele Dal , Zucchetto Antonella , Nanni Paola , Rupolo Maurizio , Steffan Agostino , Attadia Vincenza , Ballerini Pier Ferruccio , Damiani Daniela , Pucillo Carlo , Poeta Giovanni Del , Colombatti Alfonso , Gattei Valter 
Published: June 2004

SummaryCases of B‐cell chronic lymphocytic leukaemia (B‐CLL) with mutated (M) IgVH genes have a better prognosis than unmutated (UM) cases. We analysed the IgVH mutational status of B‐CLL according to the features of a canonical somatic hypermutation (SHM) process, correlating this data with survival. In a series of 141 B‐CLLs, 124 cases were examined for IgVH gene per cent mutations and skewing of replacement/silent mutations in the framework/complementarity‐determining regions as evidence of antigen‐driven selection; this identified three B‐CLL subsets: significantly mutated (sM), with evidence of antigen‐driven selection, not significantly mutated (nsM) and UM, without such evidence and IgVH gene per cent mutations above or below the 2% cut‐off. sM B‐CLL patients had longer survival within the good prognosis subgroup that had more than 2% mutations of IgVH genes. sM, nsM and UM B‐CLL were also characterized for the biased usage of IgVH families, intraclonal IgVH gene diversification, preference of mutations to target‐specific nucleotides or hotspots, and for the expression of enzymes involved in SHM (translesion DNA polymerase ζ and η and activation‐induced cytidine deaminase). These findings indicate the activation of a canonical SHM process in nsM and sM B‐CLLs and underscore the role of the antigen in defining the specific clinical and biological features of B‐CLL.
Leukaemia – a developmental perspective
Authors: Izraeli Shai 
Published: June 2004

SummaryLeukaemia is characterized by the accumulation of malignant haematopoietic precursors. Recent studies have revealed that acquired alterations in genes that regulate normal haematopoiesis are frequently detected in leukaemia. The progression to leukaemia depends on additional mutations that promote the survival of developmentally arrested cells. This review describes three examples of this general paradigm of leukaemogenesis: RUNX1 abnormalities in acute leukaemias, GATA1 mutations in the leukaemias of Down syndrome, and SCL and LMO2 ectopic expression in T cell acute lymphoblastic leukaemia.
Assessment of an immature platelet fraction (IPF) in peripheral thrombocytopenia
Authors: Briggs Carol , Kunka Stefan , Hart Dan , Oguni Shinichiro , Machin Samuel J. 
Published: June 2004

SummaryA new automated method to reliably quantify reticulated platelets, expressed as the immature platelet fraction (IPF), has been developed utilizing the XE‐2100 blood cell counter with upgraded software (Sysmex, Kobe, Japan). The IPF is identified by flow cytometry techniques and the use of a nucleic acid specific dye in the reticulocyte/optical platelet channel. The clinical utility of this parameter was established in the laboratory diagnosis of thrombocytopenia due to increased peripheral platelet destruction, particularly autoimmune thrombocytopenic purpura (AITP) and thrombotic thrombocytopenic purpura (TTP). Reproducibility and stability results over 48 h were good. An IPF reference range in healthy individuals was established as 1·1–6·1%, with a mean of 3·4%. Patients in whom platelet destruction might be abnormal, were studied and two of these patients followed serially during the course of treatment. The IPF was raised in several disease states. The most significant increases in IPF values were found in patients with AITP (mean 22·3%, range 9·2–33·1%) and acute TTP (mean 17·2%, range 11·2–30·9%). Following patients during treatment demonstrated that as the platelet count recovered the IPF% fell. These results show that a rapid, inexpensive automated method for measuring the IPF% is feasible and should become a standard parameter in evaluating the thrombocytopenic patient.
Response to desmopressin of factors XI, X and V in patients with factor VIII deficiency and von Willebrand disease
Authors: White B. , Lawler P. , Riddell A. , Nitu‐Whalley I. C. , Hermans C. , Lee C. A. , Brown S. A. 
Published: June 2004

SummaryDesmopressin [1‐deamino‐8‐d‐arginine vasopressin (DDAVP)] has been successfully used in the treatment of type 1 von Willebrand disease (VWD) and mild haemophilia A (MHA). Data suggest that DDAVP can increase factor XI (FXI) plasma levels and may represent an effective treatment for mild FXI deficiency. We assessed the DDAVP response of FXI coagulant activity (FXI:C), FXI antigen (FXI:Ag), factor V coagulant activity (FV:C), and factor X coagulant activity (FX:C) in 33 individuals with VWD or MHA. DDAVP did not produce a clinically significant increase in FXI:C, FXI:Ag, FX:C or FV:C in any patient. The mean ± SD FXI:C pre‐DDAVP (time 0) and at 1 h post‐DDAVP was 90·7 (±22·9) U/dl and 92·1 (±20·9) U/dl, respectively. The mean (±SD) FXI:Ag at time 0 and 1 h was 92·2 (±20·1) U/dl and 89·9 (±21·3) U/dl, respectively. There was a small reduction at 1 h post‐DDAVP in both FV:C, from 101·8 (±20·9) U/dl to 97·2 (±21·4) U/dl (P < 0·001), and FX:C from 103 (±19·5) U/dl to 98·8 (±18·7) U/dl (P < 0·001). No significant increase in FXI:C, FXI:Ag, FV:C or FX:C levels was seen at 4 h post‐DDAVP. This study failed to demonstrate a clinically significant increase in the levels of FXI, FX or FV following administration of DDAVP.
Blood dendritic cells in patients with acute lymphoblastic leukaemia
Authors: Mami Naira Ben , Mohty Mohamad , Chambost Hervé , Gaugler Béatrice , Olive Daniel 
Published: June 2004

SummaryMyeloid and plasmacytoid dendritic cells (MDCs, PDCs) play a key role in the initiation of immune responses. In this study, we show a severe reduction of MDCs and PDCs in patients with B lineage acute lymphoblastic leukaemia (B‐ALL; P = 0·01 vs. controls). DCs from patients with T lineage ALL (T‐ALL) were quantitatively and functionally comparable to healthy donors, as demonstrated by secretion of interleukin (IL)‐12p70 and interferon‐α. In vitro, the circulating CD34+ fraction of B‐ALL cases did not generate either CD1a+ MDCs or PDCs, suggesting that DC development is probably affected in B‐ALL, but not in T‐ALL.
Natural history of paroxysmal nocturnal haemoglobinuria using modern diagnostic assays
Authors: Moyo Victor M. , Mukhina Galina L. , Garrett Elizabeth S. , Brodsky Robert A. 
Published: June 2004

SummaryParoxysmal nocturnal haemoglobinuria (PNH) is an uncommon, acquired disorder of blood cells caused by mutation of the phosphatidylinositol glycan class A (PIG‐A) gene. The disease often manifests with haemoglobinuria, peripheral blood cytopenias, and venous thrombosis. The natural history of PNH has been documented in retrospective series; but there has only been one study that correlated the more sensitive and specific flow cytometric assays that have become available in the last decade with severe symptoms associated with PNH. In a retrospective analysis of 49 consecutive patients with PNH evaluated at Johns Hopkins, large PNH clones were associated with an increased risk for thrombosis as well as haemoglobinuria, abdominal pain, oesophageal spasm, and impotence. Of the 14 (29%) patients that developed thrombosis, nine died; six of these from complications related to thromboses. According to logistic regression modelling, for a 10% change in PNH clone size, the odds ratio for risk of thrombosis was estimated to be 1·64. No patient with <61% PNH granulocytes developed a thrombosis, whereas 12 of 22 patients (54·5%) with ≥61% PNH granulocytes manifested with thrombosis. These data not only confirm that the size of the PNH clone correlates with the risk for thrombosis, but they also suggest a correlation of PNH clone size to more symptomatic PNH.
Most cases of primary salivary mucosa‐associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection
Authors: Ambrosetti Achille , Zanotti Roberta , Pattaro Cristian , Lenzi Lorenza , Chilosi Marco , Caramaschi Paola , Arcaini Luca , Pasini Felice , Biasi Domenico , Orlandi Ester , D'Adda Mariella , Lucioni Marco , Pizzolo Giovanni 
Published: June 2004

SummarySalivary gland mucosa‐associated lymphoid tissue (MALT) lymphomas (SGML) are rare, as are data concerning their behaviour. We analysed clinical features at presentation, particularly the association with Sjoegren syndrome (SS) and hepatitis C virus (HCV) infection, and outcome in 33 cases of SGML diagnosed between March 1985 and April 2003. There were five males and 28 females, with a median age of 61 years. At presentation, 12/33 (36%) had multiple salivary glands or mucosal involvement and four had bone marrow infiltration. Ann Arbor stage was IE in 15 (46%), IIE in four (12%) and IV in 14 patients (42%). Fifteen patients had a history of SS (46%), two of other autoimmune diseases, seven of HCV infection. No case had both SS and HCV. Of the 29 treated patients, 17 received surgery or local radiotherapy; 69% achieved complete remission. Histological transformation occurred in four (12%). Five patients died (three of lymphoma, two of unrelated causes). The 5 year‐overall survival (OS), cause‐specific survival and progression‐free survival was 85 ± 8%, 94 ± 6% and 65 ± 10% respectively. Overall, the disease course was indolent, despite the advanced stage at diagnosis, and local therapy often appeared to be adequate. The only prognostic factors influencing OS were histological transformation and age. The close association of SGML with either autoimmune diseases or HCV infection in our series (73%) confirms their possible role in the pathogenesis of these lymphomas.
Improved extension of platelet storage in a polyolefin container with higher oxygen permeability
Authors: Yuasa Takeshi , Ohto Hitoshi , Yasunaga Reiko , Kai Takanori , Shirahama Noriaki , Ogata Takashi 
Published: June 2004

SummaryThis study evaluated a newly developed polyolefin bag, which has a 50%/m2 higher oxygen permeability to extend the shelf‐life of platelets. Single‐donor aphaeresis platelets were pooled, separated equally into two bags, PO‐80 (0·8 l capacity) and KBP‐PO (1 l capacity) for control, and stored in plasma for up to 7 d. Platelet biochemical and functional parameters were monitored in bags containing high (4·2 × 1011/250 ml/bag, n = 9) and low (2·0 × 1011/200 ml/bag, n = 3) concentrations of platelets over the storage period. After 7 d of storage, the PO‐80 bags containing high concentrations of platelets had a better pH (mean pH 6·74) than those stored in KBP‐PO (pH 6·32, P < 0·01); none of the nine PO‐80 bags with a high platelet concentration had a pH below 6·2, compared with four of nine controls (P < 0·05). Similarly, lactate values were 20·19 mmol/l and 28·09 mmol/l respectively (P < 0·05). Aerobic metabolism was maintained better with greater O2 consumption and less lactate generation in high‐platelet concentration PO‐80 bags than in the control bags. A significant difference was also found in pH, pCO2 and lactate levels between the two bags containing low concentrations of platelets during the 7‐d period. The in vitro characteristics of platelets declined less over 7 d when stored in a higher oxygen permeable container than in a marketed bag.
A mouse model to study organ homing behaviour of haemopoietic progenitor cells reveals high selectivity but low efficiency of multipotent progenitors to home into haemopoietic organs
Authors: Henschler R. , Fehervizyova Z. , Bistrian R. , Seifried E. 
Published: June 2004

SummaryTo study the homing behaviour of an enriched multipotent primitive haemopoietic progenitor cell (HPC) population in mice, undifferentiated murine factor‐dependent multipotent HPCs (FDCP‐mix), stably transfected with the green fluorescence protein gene, were intravenously injected into congenic mice. After 2 or 24 h, cell suspensions were prepared from bone marrow, spleen, lung, liver, muscle, colon, kidney, brain or blood of the mice and analysed by flow cytometry. Using direct quantifiable determination of total HPC numbers homed per organ and a method to estimate the degree of organ contamination by HPC that were present in blood vessels within the organs before preparation, the highest absolute numbers of HPC were detected in the liver and lungs at 2 h but this was sharply decreased at 24 h, whereas HPC selectively accumulated in the bone marrow and spleen at 24 h after transplantation. Only a few HPC were detected in other organs. The seeding efficiency of homed FDCP‐mix HPC to the bone marrow and spleen was approximately 1·5% and ranged between that of primary whole bone marrow cells and lineage‐depleted freshly isolated bone marrow cells. Pretreatment of HPC with inhibitors of signal transduction indicated that short‐term homing of multipotent HPC into haemopoietic organs is an active process requiring co‐ordinated intracellular signalling through Rho family small GTPases and protein kinases. Thus, short‐term homing of FDCP‐mix HPC into haemopoietic organs is of low efficiency but high selectivity, and provides a system to analyse the mechanisms and manipulation of primitive HPC which saves large numbers of donor animals.