bluuebunny/crossref_metadata_2025_split huggingface dataset:
Comparative Transcriptome Analysis Reveals Common and Specific Tags for Root Hair and Crack-Entry Invasion inSesbania rostrata
Authors: Capoen Ward , Den Herder Jeroen , Rombauts Stephane , De Gussem Jeroen , De Keyser Annick , Holsters Marcelle , Goormachtig Sofie 
Published: June 2007

AbstractThe tropical legume Sesbania rostrata provides its microsymbiont Azorhizobium caulinodans with versatile invasion strategies to allow nodule formation in temporarily flooded habitats. In aerated soils, the bacteria enter via the root hair curling mechanism. Submergence prevents this epidermal invasion by accumulation of inhibiting concentrations of ethylene and, under these conditions, the bacterial colonization occurs via intercellular cortical infection at lateral root bases. The transcriptome of both invasion ways was compared by cDNA-amplified fragment length polymorphism analysis. Clusters of gene tags were identified that were specific for either epidermal or cortical invasion or were shared by both. The data provide insight into mechanisms that control infection and illustrate that entry via the epidermis adds a layer of complexity to rhizobial invasion.
Discovery of antibacterial cyclic peptides that inhibit the ClpXP protease
Authors: Cheng Lin , Naumann Todd A. , Horswill Alexander R. , Hong Sue‐Jean , Venters Bryan J. , Tomsho John W. , Benkovic Stephen J. , Keiler Kenneth C. 
Published: June 2007

AbstractA method to rapidly screen libraries of cyclic peptides in vivo for molecules with biological activity has been developed and used to isolate cyclic peptide inhibitors of the ClpXP protease. Fluorescence activated cell sorting was used in conjunction with a fluorescent reporter to isolate cyclic peptides that inhibit the proteolysis of tmRNA‐tagged proteins in Escherichia coli. Inhibitors shared little sequence similarity and interfered with unexpected steps in the ClpXP mechanism in vitro. One cyclic peptide, IXP1, inhibited the degradation of unrelated ClpXP substrates and has bactericidal activity when added to growing cultures of Caulobacter crescentus, a model organism that requires ClpXP activity for viability. The screen used here could be adapted to identify cyclic peptide inhibitors of any enzyme that can be expressed in E. coli in conjunction with a fluorescent reporter.
In vivo recovery and early half‐life of infused factor VIII in haemophilia A
Authors: Kasper Carol K. 
Published: June 2007

SummaryBetween 1980 and 1994, 169 in vivo recovery studies were performed in one haemophilia centre on casually‐recruited outpatients with severe haemophilia A, and 149 early‐phase half‐life studies were performed on patients scheduled for elective surgery. Average recoveries improved from 80% to 97% of the expected values over the study period. No relationship of recovery to level of product purification was seen. In the disappearance studies, plasma factor VIII levels fell to half the peak levels by 3.25h after infusion, on average.
Immune tolerance therapy in paediatric haemophiliacs with factor VIII inhibitors: 14 years follow‐up
Authors: Kreuz W. , Ehrenforth S. , Funk M. , Auerswald G. , Mentzer D. , Joseph‐Steiner J. , Beeg T. , Klarman D. , Scharrer I. , Kornhuber B. 
Published: June 2007

SummaryWe report our clinical experience in the immune tolerance (IT) therapy of 21 paediatric haemophiliacs with FVIII inhibitor: high responders (16HR) received initially FVIII twice daily at a dosage of 50–300 U/kg/day, 11/16 received a concomitant treatment with activated prothrombin complex concentrate (100–200 U/kg/day). Low responders (five LR) received 20–100 FVIII U/kg every second or third day. Inhibitor elimination was achieved in 19/21 patients in a median time of 4 months in HR and 1.5 months in LR. The outcome and length of time needed to induce IT was significantly correlated with FVIII exposure between the first inhibitor detection and onset of IT therapy and to interruption of IT therapy. For a rapid elimination of FVIII inhibitors it is important to start continuous administration of high‐dose FVIII (≥ 100 FVIII U/kg/day) before repeated exposure to FVIII, in order to prevent rebooster effects, prolongation of elimination time, and to reduce expense.
Effect and side‐effects of alpha interferon treatment in haemophilia patients with chronic hepatitis C
Authors: Mauser‐Bunschoten E. P. , Bresters D. , Reesink H. W. , Roosendaal G. , Chamuleau R. A. F. M. , Haan E. , Jansen P. L. M. , van den Berg H. M. 
Published: June 2007

SummaryTo evaluate the effect and side‐effects of alpha interferon 2B (IFN) treatment in haemophilia patients with chronic hepatitis C (positive HCV antibody test, positive HCV‐RNA test and ALT levels >2.5 × upper limit of normal) eight HIV and HBs‐ag negative haemophilia patients were treated with IFN for 26 weeks in a dosage of 5 mega units (MU) daily for 2 weeks, 2.5 MU daily for 4 weeks and 1.5 MU 3 times a week for 20 weeks. Patients who were transient or non‐responders after 26 weeks of IFN therapy were treated for 2.5 years with 5 MU IFN three times a week. At the end of 3 years follow‐up, four patients were considered complete responders (HCV‐RNA negative) with complete and sustained ALT normalization and disappearance from HCV‐RNA from blood, two patients only showed a transient response and two patients were non‐responders.All patients experienced the common side‐effects of IFN treatment. Two patients complained about feelings of depression and impotence. For both this was the reason to stop IFN treatment. In one patient, IFN treatment was stopped 90 weeks after start of therapy because of persistent fatigue. In another patient the dosage was adjusted because of a decrease in platelet count.No effect of IFN on bleeding frequency and chronic arthopathy was seen.We conclude that the clinical effects and side‐effects of IFN therapy in patients with haemophilia and chronic hepatitis C are comparable with those of non‐haemophilia patients with chronic hepatitis C. Haemophilia patients can be treated for chronic hepatitis C infection in the same way as patients without haemophilia.
The feasibility of using concentrates containing factor IX for continuous infusion
Authors: SCHULMAN SAM , GITEL SANFORD , ZIVELIN ARIELA , KATSAROU OLGA , MANDALAKI TITIKA , VARON DAVID , MARTINOWITZ URI 
Published: June 2007

SummaryWe have investigated the feasibility of continuous infusion of undiluted factor IX (F IX) over several days using minipumps. The stabilities of seven different reconstituted F IX products were substantially better than those declared by the manufacturers. Several concentrates maintained factor activities 80% of baseline for the entire period of 4 weeks at 4‐8d̀C as did one product at 20–23d̀A. At 37d̀C the latter concentrate was stable for at least 1 week. The stability seemed to correlate with the purity of the product. Analysis of two prothrombin comples concentrates by gel electrophoresis demonstrated degradation of prothrombin to prethrombin‐1 and fragment 1 at 37d̀C and in one of the concentrates also at 20–23d̀C. In two F IX concentrates the corresponding analysis did not reveal any degradation. Four patients were treated with continuous infusion with a pure F IX concentrate (Mononine™, Armour) after surgery or for serious haemorrhage (two each) with good haemostatic effect, an initial progressive decrease of the F IX clearance, and no side‐effects. Continuous infusion with F IX, using a minipump and undiluted reconstituted factor, is therefore feasible and effective, and can be conveniently prepared for several days at a time. Pure F IX products are more stable and probably safer for this purpose.
Seroprevalence of parvovirus B19, cytomegalovirus, hepatitis A virus and hepatitis E virus antibodies in haemophiliacs treated exclusively with clotting‐factor concentrates considered safe against human immunodeficiency and hepatitis C viruses
Authors: FLORES G. , JUÁREZ J. C. , MONTORO J. B. , TUSELL J. M. , ALTISENT C. , JUSTE C. , JARDÍ R. 
Published: June 2007

SummaryClotting‐factor concentrates (CFC) are a potential source of transmission of blood‐borne viruses. Newer physical and chemical methods (pasteurization, wet‐heating, solvent/detergent treating) developed to inactivate viruses are effective against HIV, HBV and HCV. However, it is not clear if these methods protect against other pathogenic viruses such as parvovirus B19, cytomegalovirus (CMV), hepatitis A virus (HAV) and hepatitis E virus (HEV). To evaluate the safety of current CFC we have studied seroprevalence of parovirus B19, CMV, HAV and HEV antibodies in 22 HIV and HCV negative haemophiliacs who were treated exclusively with clotting‐factor concentrates considered safe with respect to HIV and HCV transmission, 22 healthy individuals served as controls. Neither HAV nor HEV antibodies were detected in haemophiliacs or controls.Two controls and two haemophiliacs were seropositive for CMV. Five controls (32% prevalence) and 15 haemophiliacs (77%) were positive to parovirus B19. No statistical differences can be established for seropositivity with CMV, HAV and HEV between haemophilic patients and controls. In the case of parvovirus B19 the differences are statistically significant (P= 0.0128). The relative risk of parvovirus B19 is 2.4 in the case of haemophiliacs. CFC considered safe against HIV and HCV are not safe against parvovirus B19, although they seem to be safe against CMV, HAV and HEV.
Didanosine treatment of haemophilic patients infected with HIV
Authors: Wilde Jonathan T. , McKernan Angela , Hay Charles R. M. 
Published: June 2007

SummaryTwenty‐six haemophilic patients with advanced HIV infection who had developed resistance or intolerance to zidovudine were treated with didanosine (ddI). 11 patients continue to take ddI at a median time of 14 months from commencement (range 7–18 months). Five of these patients showed an increase in CD4 lymphocyte count, reaching a maximum at a median time of 4 months. Four patients with HIV‐related symptoms improved clinically. In general, the CD4 count and clinical improvements were not sustained. 11 patients discontinued ddI after a median of 3 months (range 3 days to 10 months), most commonly due to gastrointestinal side‐effects. No case of pancreatitis or peripheral neuropathy was seen. Six patients, all with very advanced HIV disease, died. HIV‐infected haemophilic patients who become resistant or intolerant to zidovudine may derive benefit from ddI, although this is usually transient.
Communication about safer sex and serostatus disclosure in HIV‐positive adolescents with haemophilia
Authors: NUSS RACHELLE , Smith Peter S. , Cotton David , Kisker Thomas ,   the Hemophilia Behavioral Intervention Evaluation Project (HBIEP) Adolescent Elicitation Committee
Published: June 2007

Summary Objectives. To assess the attitudes, beliefs and feelings of adolscents and young men with severe haemophilia with respect to discussing safer sex and disclosing their human immunodeficiency virus (HIV) seropositivity to potential sex partners. Methods. Fifty‐nine males with haemophilia from throughout the US answered open‐ended questions. Results. Talking about avoidance of transmitting AIDS and disclosing one's seropositivity was beneficial, moral and wise. Nevertheless, this was exceedingly, difficult, unpleasant, and fraught with fear of rejection and alienation. Communication was approved by family, friends, and health‐care providers. Facilitators of communication were: knowledge and an accepting attitude about persons with HIV, a supportive person to assist with discussion, and environmental cues. Conclusion. This first report of HIV‐infected adolescents and young adults reveals that although they endorse discussing safer sex and disclosing their HIV seropositivity, they are painfully aware of the social and interpersonal risks of such extremely difficult communications.
A study to evaluate the effectiveness of ‘Air‐Stirrup’ splints as a means of reducing the frequency of ankle haemarthroses in children with haemophilia A and B
Authors: Buzzard Brenda M. , Heim Michael 
Published: June 2007

SummaryWith the introduction of readily available factor VIII and IX concentrates and programmes for home therapy and prophylaxis, most severe haemophilic arthropathies can be prevented. However, despite these programmes, bleeds into the ankle joints still occur during the years of growth. It is suggested that ankle haemarthroses may be caused by unrecognized inversion injuries by the patient. The history from a small child is not always accurate and when a child presents with a swollen ankle it is not uncommon for the medical team to make a diagnosis of haemarthrosis without really searching for the pathogenesis. This paper describes a clinical study using the ‘Air‐Stirrup’; (AS) ankle splint which is designed to restrict inversion. Children between the ages of 3 and 18 years with a history of recurrent ankle haemorrhages were entered into a 6‐month trial. Results showed a positive reduction in the number of ankle haemorrhages experienced by the children.
Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a reivesw of the literature
Authors: RAMSAHOYE B. H. , DAVIES S. V. , DASANI H. , PEARSON J. F. 
Published: June 2007

SummaryThe case records of 13 patients (24 pregnancies) with von Willebrand's disease (vWD) were studies rettospectively. The overall incidence of primary and secondary post‐partum haemorrhage (PPH) was 15.8% and 25% respectively, all primary PPH occurring in tyre 2 discase (3/14 deliveries, 21.4%). The risk of primary PPH in type 2 patients who did not receive prophylactic factor VIII was 37.5% (3/8 deliveries).Factor VIII coagulant activity (VIII:C) and von Willebrand factor antigen (vWF:Ag) rose above bascline values by a factor of at least 1.5 during the pregnancy in most case. More severely affected patients were less likely to benefit significatntly. A baseline VIII:C of <15 iu/dl (4/14 cases) was predictive of a third trimester level of <15 iu/dl. Improvements in the von Willebrand factor activity were less marked. The baseline von Willebrand factor activity was <15 iu/dl in all patients with serial data, none of whom achieved a third‐trimester von Willebrand factor activity of >50 iu/dl. The bleeding times were unaltered significantly in all but one of the cases, reflecting a general failure of the primary haemostatic defect to improve with pregnancy.The findings demonstrate that coagulation parameters do not universally improve in pregnancy in vWD, especially when preconception levels are low. The risk of primary PPH is generally higher in type 2 diseases. The level of factor VIII:C is not a good predictor of the risk of primary PPH in type 2 patients. Secondary PPH is a significatnt risk in both type 1 and type 2 patients.
Prevalence of inhibitor formation in a cohort of haemophilic children exposed to several products of various purities
Authors: ARONIS S. , PLATOKOUKI H. , KAPSIMALI Z. , ADAMTZIKI E. , KOLOKITHAS A. , MITSIKA A. 
Published: June 2007

Summary. To evaluate the frequency and potency of inhibitor formation based on the product used, we retrospectively reviewed the records of 99 children with various types and severity of haemophilia (haemophilia A 82, severe 46; haemophilia B 10, severe 6; vWD 7) treated for the last 20 years. After a mean observation period of 8 years an overall of 23 patients (23.2′/0) developed an inhibitor (haernophilia A 26.8%; severe 4O%, moderate 20%, mild 3.8%). None of the haemophilia B patients presented with an inhibitor, and only one child with bevere vWD (1/7, 14.3%) showed a transient inhibitor under cryoprecipitate therapy. Inhibitor titre was low (< 5 BU) in most cases (91.3%) and in only two patients (8.75%) was 6 and 8 BU respectively. Antibodies to FVIII were transient (detected only once) in four (17.4%) and intermittent in 19 patients (82.6%). By the age of 12 years, 17/23 patients (73.9%) had demonstrated an inhibitor. The inhibitor detection seemed to be higher in the groups of patients exposed to monoclonal (3115, 20%), SID‐treated (10159, 16.9%) or H/T FVIII concentrates (6/41, 14.6%), compared to groups of patients who received cryo/plasma (9.5%) or unmodified concentrates (5.1%); nevertheless the differences were not statistically significant.Surprisingly, none of the 52 patients who received a S/D + chromatography‐treated factor VIIl concentrate developed an inhibitor after a mean observation period of 1.7 years (range 0.2–2 years). The overall prevalence of inhibitor formation in previously untreated haemophiliacs was 14.3% (4/28), irrespective of the product used.Our data indicate that a high proportion of our haemophilic children exposed to several products of various purities have developed a low‐titre inhibitor which in most cases was transient or intermittent. However, despite the presence of the antibody, none of the patients needed a change in the mode of treatment.
Psychological status of men with haemophilia and HIV infection: two‐year follow‐up
Authors: MARSETTIN ELENA PASQUAL , CIAVARELLA NICOLA , LOBACCARO CATERINA , GHIRARDINI ALESSANDRO , BELLOCCO RINO , SCHINAIA NICOLA 
Published: June 2007

SummaryThis study aimed to assess the psychological status of men with haemophilia and HIV infection and to monitor changes in psychlogical status over time, in order to evaluate the need for psychological support. The study included 24 HIV seropositive men and a control group of 21 HIV seronegative men who attended the Haemophilia Centre in Bari (Italy). Subjects underwent psychological tests (STAl‐Y: State and Trait Anxiety Inventory; SDS: Self‐Rating Depression Scale) and completed a questionnaire on the emotional impact of AIDS. Assessment was repeated at 6‐monthly intervals over a 2‐year period. Contrary to expectation, HIV seronegative men with haemophilia had worse anxiety and depression scores, reported more confusion and fear, and had more reluctance towards the use of blood products (despite their present safety) than HIV seropositives. Possible reasons for these findings are considered, and their implications for clinical practice discussed.
Use of plasma‐derived factor VIIa in a child with inhibitors of FVIII for the placement of an implantable intravenous access device
Authors: TSUCHIYA HIROYUKI , IKEDA SHINJI , YOSHIOKA AKIRA 
Published: June 2007

SummaryWe treated a 20‐month‐old boy with severe haemophilia A who developed a high level factor VIII (FVIII) inhibitor. An implantable intravenous access device (IVAD) was safely placed on the lateral chest under anaesthesia using plasma‐derived factor VIIa (FVIIa). Implantation of an IVAD into a haemophilic child with an inhibitor requires considerable care, because the area of subdermal invasion is broader than in the implantation of a central venous line. However, placement of an IVAD provides very convenient access to the central venous line and is an appropriate tool for frequent injection, such as the induction of immunotolerance to FVIII.
Variation in factor VIII inhibitor reactivity with different commercial factor VIII preparations
Authors: BERNTORP E. , EKMAN M. , GUNNARSSON M. , NILSSON I.M. 
Published: June 2007

Summary. During treatment of a haemophilia A patient with a high‐responding inhibitor against factor VIII coagulant activity (VIII:C), we observed a difference in recovery of VIII:C depending upon which factor concentrate was infused. Inhibitor plasma samples or IgG fraction from seven patients were tested against a panel of seven different commercially available factor VIII concentrates of which five were plasma‐derived and two recombinant. In two of the plasma samples, inhibitor titres manifested a wide range of values depending upon which concentrate was used in the test system. Thus, inhibitor neutralization was less and VIII:C recovery greater when factor VIII concentrates containing large amounts of von Willebrand factor were used than when highly purified concentrates containing no von Willebrand factor or only trace amounts were used. In both of these two patients the inhibitor was directed against the light chain of factor VIII, and it is possible that the epitope of the light chain with which the inhibitor reacts is partly blocked by the von Willebrand factor.We conclude that inhibitors may differ in their reactivity with factor VIII molecules contained in clotting factor concentrates, and that there is factor VIII epitope variation between different concentrates. These findings have implications for the selection of concentrates for the treatment of inhibitor patients and the haemostatic effect may be improved if a concentrate giving the lowest inhibitor titre is chosen. Thus, in vitro testing of inhibitor reactivity with a panel of concentrates is recommended when treatment of inhibitor patients with factor VIII concentrates is considered.
Neisseria gonorrhoeae O‐linked pilin glycosylation: functional analyses define both the biosynthetic pathway and glycan structure
Authors: Aas Finn Erik , Vik Åshild , Vedde John , Koomey Michael , Egge‐Jacobsen Wolfgang 
Published: June 2007

Summary Neisseria gonorrhoeae expresses an O‐linked protein glycosylation pathway that targets PilE, the major pilin subunit protein of the Type IV pilus colonization factor. Efforts to define glycan structure and thus the functions of pilin glycosylation (Pgl) components at the molecular level have been hindered by the lack of sensitive methodologies. Here, we utilized a ‘top‐down’ mass spectrometric approach to characterize glycan status using intact pilin protein from isogenic mutants. These structural data enabled us to directly infer the function of six components required for pilin glycosylation and to define the glycan repertoire of strain N400. Additionally, we found that the N. gonorrhoeae pilin glycan is O‐acetylated, and identified an enzyme essential for this unique modification. We also identified the N. gonorrhoeae pilin oligosaccharyltransferase using bioinformatics and confirmed its role in pilin glycosylation by directed mutagenesis. Finally, we examined the effects of expressing the PglA glycosyltransferase from the Campylobacter jejuni N‐linked glycosylation system that adds N‐acetylgalactosamine onto undecaprenylpyrophosphate‐linked bacillosamine. The results indicate that the C. jejuni and N. gonorrhoeae pathways can interact in the synthesis of O‐linked di‐ and trisaccharides, and therefore provide the first experimental evidence that biosynthesis of the N. gonorrhoeae pilin glycan involves a lipid‐linked oligosaccharide precursor. Together, these findings underpin more detailed studies of pilin glycosylation biology in both N. gonorrhoeae and N. meningitidis, and demonstrate how components of bacterial O‐ and N‐linked pathways can be combined in novel glycoengineering strategies.