bluuebunny/crossref_metadata_2025_split huggingface dataset:
Weaning infants with phenylketonuria: a review
Authors: MacDonald A. , Evans S. , Cochrane B. , Wildgoose J. 
Published: August 2011

How to cite this article
MacDonald A., Evans S., Cochrane B. & Wildgoose J. (2012) Weaning infants with phenylketonuria: a review. J Hum Nutr Diet.25, 103–110AbstractCareful weaning is particularly important in phenylketonuria (PKU). Dietary phenylalanine intake is severely restricted, and the diet is supplemented with phenylalanine‐free amino acids and special low protein foods. In PKU, there are no evidence‐based weaning guidelines and no studies assessing the introduction of solid foods. We critically review the literature and examine current UK weaning practices. Ideally, weaning in PKU should closely reflect the ‘model’ for healthy infants. However, the requirement for optimal blood phenylalanine control and the demands of diet therapy overshadow the social aspects of weaning. Solid food intake is established with very low protein foods first, and then 50 mg phenylalanine exchanges (equivalent to 1 g of intact protein) gradually replace breast/formula feeds. Introducing solids before the recommended 6 months of age may be advantageous because there is a less persistent neophobic food response, possibly leading to better food acceptance. Infants with PKU also require a special phenylalanine‐free protein substitute. Between 6 and 12 months, a second concentrated source of phenylalanine‐free protein substitute is required. This is commonly given as an additional liquid, although the prescribed volume may adversely affect appetite. Alternatively, a second‐stage protein substitute administered as a paste may better suit feeding development. Further research aiming to examine the weaning process in PKU with a focus on biological, maternal, infant, social and environmental factors is required. This will help provide evidence for the effect of protein substitute on appetite and help in the development of evidence‐based guidelines.
Dose‐dependent systemic exposure of albendazole metabolites in lambs
Authors: ALVAREZ L. , SUÁREZ G. , CEBALLOS L. , MORENO L. , LANUSSE C. 
Published: August 2011

Alvarez, L., Suárez, G., Ceballos, L., Moreno, L., Lanusse, C. Dose‐dependent systemic exposure of albendazole metabolites in lambs. J. vet. Pharmacol. Therap. 35, 365–372.The gastrointestinal absorption of most drugs follows a first‐order kinetics, whereby a constant fraction of the total drug is absorbed in each equal time interval. Although this related absorption principle is applicable to the most of the therapeutically used drugs, it remains unclear for poorly water‐soluble compounds such as the benzimidazole anthelmintics in ruminants. The goal of the current work was to characterize the albendazole (ABZ) metabolites plasma disposition kinetics after ABZ administration at different dosages to nematode‐infected lambs. Eighteen Corriedale lambs artificially infected with a resistant Haemonchus contortus strain were allocated into three groups and intraruminally treated with ABZ at either 5 (ABZ5), 15 (ABZ15) or 45 (ABZ45) mg/kg. Blood samples were collected up to 120 h post‐treatment, and the collected plasma was analysed by high‐performance liquid chromatography. The estimated pharmacokinetic parameters were statistically compared using parametric and nonparametric tests. None of the animals involved in the current trial showed any adverse events during the study. While ABZ parent drug was not recovered in the bloodstream, the area under the concentration vs time curve (AUC) of the active ABZ‐sulphoxide (ABZSO) metabolite increased significantly (P < 0.05) from 21.0 (ABZ5) up to 158.6 (ABZ15) and 389.7 μg·h/mL (ABZ45), which indicates some type of nonproportionality in the relationship between dose level and drug systemic exposure. The overall kinetic disposition of the inactive sulphone metabolite did not change after treatment at threefold the therapeutic ABZ dosage. However, significantly (P < 0.05) higher AUC, Cmax and mean residence time values were observed after the administration of the highest dosage level. The higher dosages accounted for a significantly (P < 0.05) enhancement of the ABZSO peak plasma concentration, which were obtained at delayed times post‐treatment. High correlations between AUC0‐LOQ and Cmax and nematode counts were observed, with Spearman’s coefficients of −0.83 and −0.84, respectively. The results obtained in the current experiment show that increasing the dose of ABZ in sheep is clearly associated with enhanced plasma ABZ metabolites exposure. The data showed a nonproportionality on the gastrointestinal absorption of ABZ in nematode‐infected lambs.
Microbial influence on erosion, grain transport and bedform genesis in sandy substrates under unidirectional flow
Authors: HAGADORN JAMES W. , MCDOWELL CONOR 
Published: August 2011

AbstractInterpreting the physical dynamics of ancient environments requires an understanding of how current‐generated sedimentary structures, such as ripples and dunes, are created. Traditional interpretations of these structures are based on experimental flume studies of unconsolidated quartz sand, in which stepwise increases in flow velocity yield a suite of sedimentary structures analogous to those found in the rock record. Yet cyanobacteria, which were excluded from these studies, are pervasive in wet sandy environments and secrete sufficient extracellular polysaccharides to inhibit grain movement and markedly change the conditions under which sedimentary structures form. Here, the results of flume experiments using cyanobacteria‐inoculated quartz sand are reported which demonstrate that microbes strongly influence the behaviour of unconsolidated sand. In medium sand, thin (ca 0·1 to 0·5 mm thick) microbial communities growing at the sediment–water interface can nearly double the flow velocity required to produce the traditional sequence of ripple→dune→plane‐bed lamination bedforms. In some cases, these thin film‐like microbial communities can inhibit the growth of ripples or dunes entirely, and instead bed shear stresses result in flip‐over and rip‐up structures. Thicker (ca≥1 mm thick) microbial mats mediate terracing of erosional edges; they also, foster transport of multi‐grain aggregates and yield a bedform progression consisting of flip‐overs→roll‐ups→rip‐ups of bound sand.
Nocardia infection in kidney transplant recipients: case report and analysis of 66 published cases
Authors: Yu X. , Han F. , Wu J. , He Q. , Peng W. , Wang Y. , Huang H. , Li H. , Wang R. , Chen J. 
Published: August 2011

X. Yu, F. Han, J. Wu, Q. He, W. Peng, Y. Wang, H. Huang, H. Li, R. Wang, J. Chen. Nocardia infection in kidney transplant recipients: case report and analysis of 66 published cases. Transpl Infect Dis 2011: 13: 385–391. All rights reservedAbstract: Nocardiosis is a rare but life‐threatening opportunistic infection, especially in immune compromised patients, including kidney transplant recipients. Primary pulmonary infection is the most common clinical pattern, and can easily result in disseminated Nocardia infection if treatment therapy is not adequate at the beginning. In this article, we report a new case of disseminated nocardiosis (lungs, skin, and pericardium) after renal allograft transplantation. We also review the English literature published from 1980 to 2010 and analyze the clinical characteristics of nocardiosis in kidney transplant recipients.
Insights into the Structure and Function of Membrane Polypeptides Carrying Blood Group Antigens
Authors: Cartron J.P. , Bailly P. , Van Kim C. Le , Cherif‐Zahar B. , Matassi G. , Bertrand O. , Colin Y. 
Published: August 2011

AbstractIn recent years, advances in biochemistry and molecular genetics have contributed to establishing the structure of the genes and proteins from most of the 23 blood group systems presently known. Current investigations are focusing on genetic polymorphism analysis, tissue‐specific expression, biological properties and structure‐function relationships. On the basis of this information, the blood group antigens were tentatively classified into five functional categories: (i) transporters and channels, (ii) receptors for exogenous ligands, viruses, bacteria and parasites, (iii) adhesion molecules, (iv) enzymes and, (v) structural proteins. This review will focus on selected blood groups systems (RH, JK, FY, LU, LW, KEL and XK) which are representative of these classes of molecules, in order to illustrate how these studies may bring new information on common and variant phenotypes and for understanding both the mechanisms of tissue specific expression and the potential function of these antigens, particularly those expressed in nonerythroid lineage.
Stem Cell Biology for the Transfusionist
Authors: Lansdorp P.M. 
Published: August 2011

AbstractThe limited life‐span of most blood cells requires continuous production of cells which in adults may exceed 1012 cells/day. This impressive production of cells (˜ 4.1015 cells over a life time) is achieved by the proliferation and differentiation of committed progenitor cells which themselves are derived from a population of pluripotent stem cells with self‐renewal potential. In adults, the large majority of stem cells are found in the bone marrow among cells with a CD34+CD38‐ phenotype. Interestingly, small but significant numbers of such cells can be found in the circulation. The frequency of circulating CD34+CD38‐ cells can be dramatically increased by treatment with certain compounds including cytokines. Such “mobilized” peripheral blood stem cells have become an important alternative to bone marrow in stem cell transplantation procedures primarily because engraftment is more rapid. The latter is almost certainly related to the increased numbers of primitive CD34+CD38‐ cells capable of engrafting the bone marrow in blood versus bone marrow stem cell grafts [1]. Paradoxically, the large majority of “candidate” stem cells in adult bone marrow are quiescent cells. One possibility is that stem cells, like other somatic cells, have only a limited replicative potential (<100 divisions). This hypothesis is supported by two key observations and the consideration that, in theory, 52 divisions can yield 4.1015 cells. First, it was shown that “candidate” stem cells purified from fetal and adult tissue display marked functional differences in turn‐over time and the ability to produce cells with stem cell properties [2]. Secondly, these functional differences were found to correlate with a measurable loss of telomere repeats [3], despite the presence of low but readily detectable levels of telomerase in all purified cell fractions [4,5]. In order to address questions about the role of telomeres in normal and malignant hematopoiesis, we developed quantitative fluorescence in situ hybridization [6]. With this technique the length of telomere repeats at individual chromosome ends can be reliably estimated using optical density measurements from digital images of metaphase chromosomes after fluorescence in situ hybridization with directly labeled (CCCTAA)3 ‐ Peptide Nucleic Acid probe [6,7]. Furthermore, we recently showed that this method can be adapted to measure the total telomere repeat content of cells by flow cytometry [8].Here some issues in studies of hematopoietic stem cells are discussed in relation to rapidly accumulating information about telomere biology.
Epidemiology of the Hepatitis C Virus
Authors: Ebeling F. 
Published: August 2011

AbstractAccording to WHO estimations, about 3 % of the world population may be infected with the hepatitis C virus. The relative prevalences of subtypes of this virus vary in different geographic areas. The main known routes of transmission are parenteral; intravenous drug abuse, contaminated injection devices and receipt of unscreened blood. Sexual, vertical, household and nosocomial transmissions may occur, but seem to be rare. The risk of screened blood or blood products is now almost eliminated, but unscreened blood is a considerable risk in areas where screening is economically not possible. The future impact of this virus is greatly dependent on the trends in intravenous drug use as well as the possible emergence of increased late morbidity among present asymptomatic carriers during the next decades.
In Vitro and in Vivo Properties of Various Types of Platelets
Authors: Rebulla P. 
Published: August 2011

AbstractThe most popular procedures used to prepare platelet concentrates (PC) can be categorized into methods using platelet rich plasma (PRP) obtained by soft centrifugation of whole blood units, methods using buffy coats (BC) obtained by hard centrifugation of whole blood units, and apheresis procedures. The main feature that differentiates apheresis PC from whole blood unit derived PC is that apheresis reduces the number of donors necessary to support a recipient, although the advantages related to this feature have not been conclusively documented. From the biochemical point of view, a limited number of comparative studies and a large series of non‐comparative studies indicate that differences in PC obtained with different protocols depend more on the performance of the production laboratory rather than the preparative approach – PRP, BC or apheresis – itself. Similarly, the clinical effectiveness of PC seems to depend more on PC age, storage modalities and recipient's conditions rather than primarily on the method of PC preparation. Despite the basic differences in the three preparative approaches, all methods share a number of key elements that are necessary pre‐requisites for a successful platelet transfusion therapy: minimization of the risk of bacterial contamination by careful donor skin disinfection and temperature control during PC production and storage; protection of aerobic metabolism during PC storage; laboratory quality control including at least pH and volume determinations, platelet and white cell counts and visual inspection of the swirling phenomenon at time of release. Future directions in the field of PC production include the development of new products such as infusible platelet membranes, thrombospheres, thrombo‐ erythrocytes and reconstituted freeze‐dried platelets.
Novel Diagnostic and Therapeutic Strategies with Genetically Engineered Human Antibodies
Authors: Ouwehand W.H. , Watkins N. 
Published: August 2011

AbstractHuman antibodies can be generated by recombinant technology. The human immunoglobulin repertoire can be tapped in an effective manner by the so called V gene phage display technology. Using this technique the genes encoding the variable domains of an antibody of interest can be captured from the B cell repertoire. With these V genes tailor‐made immune recognition molecules can be obtained by a ‘design and build’ strategy. These novel developments will lead to the introduction of a manifold of antibody based therapies into the clinic in coming decades. Recombinant antibody technology also provides unique opportunities to study the molecular structure of antibody variable domains against blood cell antigens. Such studies might possibly lead to the development of new therapies for antibody mediated blood cell destruction.
Complement Regulation
Authors: Meri S. , Jarva H. 
Published: August 2011

AbstractBecause of its strong potential for generating inflammation and causing tissue destruction the complement system has to be kept strictly under control. Cells of the host need special protection against the cytolytic complement system. This paper will describe how inappropriate activation of complement in the fluid phase is prevented and how viable human blood cells defend themselves against being destroyed and cleared away by the complement system. Since disturbances in complement regulation occasionally result in disease a brief reference will be made to two of the syndromes caused by complement regulator deficiency, hereditary angioedema (HAE) and paroxysmal nocturnal hemoglobinuria (PNH).
Complement and Biocompatibility
Authors: Mollnes T.E. 
Published: August 2011

AbstractBlood contact with foreign surfaces induces a certain degree of activation of the defence systems including complement. Recent data using complement specific inhibitors have shown that complement is not only associated with, but in fact contributes to the whole body inflammatory reaction seen as a complication of cardiopulmonary bypass (artificial surfaces) and is responsible for the hyperacute rejection of xenografts (foreign endothelium). Complement activation products, particularly the terminal SC5b‐9 complex (TCC), is a sensitive indicator of bioincompatibility. Modifications of the artificial surfaces by coating with heparin improves both coagulation and complement compatibility. The use of specific complement inhibitors in general improves biocompatibility both of artificial materials and foreign endothelium.
Use of HLA‐ and HPA ‐ Matched Platelets in Alloimmunized Patients
Authors: Kekomäki R. 
Published: August 2011

AbstractRefractoriness for platelet transfusion is mostly due to clinical factors but may also be caused by alloimmunization. Use of leukocyte‐depleted blood cells for transfusions of patients with hematological diseases has reduced if not eliminated HLA‐alloimmunization. HLA‐antibodies reduce the survival time of incompatible platelets complicating seriously the platelet transfusion support in at least 5% of patients. If consecutive transfusions of HLA matched platelets also fail without identifiable clinical causes, HPA‐alloimmunization may have occurred. Platelets from donors phenotyped for both HLA and HPA may produce good platelet count increments and allow optimal treatment of the basic disease despite broad spectrum alloimmunization. Additional crossmatching of phenotyped platelets with patient serum may be needed to circumvent platelet‐specific antibodies of unknown specificity.
A Comparison of in Vitro Tests for predicting the Severity of Haemolytic Disease of the Fetus and Newborn
Authors: Hadley A.G 
Published: August 2011

AbstractHaemolytic disease of the newborn (HDN) is characterized by the presence of IgG antibodies in the maternal circulation which cause haemolysis in the fetus by crossing the placenta and sensitizing red cells for destruction by macrophages in the fetal spleen. Numerous serological, quantitative and cellular assays have been developed to predict the severity of HDN. These assays all measure and/or characterize alloantibodies in the maternal circulation. Quantitative assays which accurately measure antibody levels correlate with disease severity better than serological assays which are inherently less precise. Nevertheless, high antibody levels are found in some cases of mild HDN and relatively low antibody levels are found in some severe cases. This suggests that disease severity is influenced by factors in addition to antibody concentration. These factors remain to be fully elucidated but may include the subclass and glycosylation of maternal antibodies, the structure, site density, maturational development and tissue distribution of blood group antigens, the efficiency of IgG transport to the fetus, the functional maturity of the fetal spleen, polymorphisms which affect Fc receptor function, and the presence of HLA‐related inhibitory antibodies. Cellular assays which are sensitive to factors affecting antibody function have therefore been developed in an attempt to improve the prediction of disease severity. Although these assays are cumbersome, there are now sufficient data to suggest that some cellular assays, when used as part of a structured approach to diagnostic testing, may provide clinically‐useful information to complement serological and quantitative assays.
Requirements for the Electronic Crossmatch
Authors: Judd W. John 
Published: August 2011

AbstractWith the integration of laboratory information systems into transfusion services, it is now possible to develop standard operating procedures (SOPs) for an electronic crossmatch (EXM) to replace the immediate‐spin crossmatch for detecting ABO incompatibility between the blood sample submitted for pre‐ transfusion testing and the donor unit selected for transfusion. Essential to the safety of an EXM are requirements that: 1) the computer contains logic to prevent assignment and release of ABO incompatible blood; 2) no clinically significant antibodies are detected in the recipient's serum/plasma and there is no record of previous detection of such antibodies; 3) there are concordant results of at least two determinations of the recipient's ABO type on record, one of which is from a current sample; 4) critical elements of the system have been validated on‐site; and, 5) there are mechanisms to verify the correct entry of data prior to release of blood.EXM procedures are in use in at least 10 North American facilities, Scandinavia, Hong Kong and Australia. Some provide blood at remote sites lacking laboratory services. Experience has shown that a combination of properly programmed computer software and carefully developed SOPs can provide a safe and efficient means of detecting ABO incompatibility without performing a serological crossmatch.
From Serological to Computer Crossmatching in Nine Hospitals
Authors: Georgsen J. , Kristensen T. 
Published: August 2011

AbstractIn 1991 it was decided to reorganise the transfusion service of the County of Funen. The aims were to standardise and improve the quality of blood components, laboratory procedures and the transfusion service and to reduce the number of outdated blood units. Part of the efficiency gains was reinvested in a dedicated computer system making it possible – among other things – to change the cross‐match procedures from serological to computer cross‐matching according to the ABCD‐concept. This communication describes how this transition was performed in terms of laboratory techniques, education of personnel as well as implementation of the computer system and indicates the results obtained.The Funen Transfusion Service has by now performed more than 100.000 red cell transfusions based on ABCD‐cross‐matching and has not encountered any problems. Major results are the significant reductions of cross‐match procedures, blood grouping as well as the number of outdated blood components.
The French Hæmovigilance System
Authors: Noel L. , Debeir J. , Cosson A. 
Published: August 2011

AbstractHæmovigilance was part of the reform of the French transfusion system. The hæmovigilance network is now operational with approximately 4600 transfusion incidents reported annually. Immediate incidents observed within 8 days after transfusion account for 85 % of the reports. A cause cannot be identified in 41% of these, usually concerning minor clinical incidents with transient fever and /or shivers. An allergic reaction is described in 31% of transfusion reactions. Immunological conflicts account for 18% and bacteria associated transfusion reactions for 6%. The importance of bacteria associated transfusion reactions, the first identified cause of death associated with transfusion is one of the findings of hæmovigilance. Improvement in the hæmovigilance systems aims at obtaining better descriptions of transfusion incidents, standardisation of severity and imputability assessment and definitions of denominators such as the actual number of recipients. Delayed incidents will ultimately provide a true vision of post transfusion immunisation and infection The improvement of hæmovigilance now considered as part of transfusion medicine practice is a continuous process.