bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neonataler Morbus Behçet
Authors: Schuberth Katharina , Kniess Norbert , Johann Müller Matthias , Seeliger Stephan 
Published: July 2018

Zusammenfassung
Hintergrund Der Morbus Behçet ist eine Multisystemerkrankung mit entzündlichem Befall, v. a. der kleinen und mittleren Arterien und Venen. Histologisch zeigt sich das Bild einer leukozytoklastischen Vaskulitis. Die Ursachen des Morbus Behçet sind unbekannt. Genetische Faktoren werden in der Pathogenese diskutiert. Ein „klassischer“ Autoantikörper als Diagnostiktool ist nicht bekannt. Die Erkrankung betrifft normalerweise Patienten in der dritten Lebensdekade und ist in der Pädiatrie selten. Eine sehr seltene klinische Form des Morbus Behçet tritt bereits in der Neonatalperiode auf. Hier sind bei einer Manifestation lebensbedrohliche Verläufe beschrieben.
Patient/Methode Vorgestellt werden soll eine Familie mit bekannter Erkrankung der Mutter, bei der bereits das zweite Kind in der ersten Lebenswoche mit Ulzerationen im Schleimhautbereich auffiel. Die Frage einer Übertragung von Autoantikörpern, die dieses beim Kind triggert, muss in diesem Zusammenhang diskutiert werden. Dieser klinische Verlauf wird mit Verläufen aus der Literatur verglichen, um Empfehlungen zum therapeutischen Procedere ableiten zu können.
Ergebnis Bei unserem Patienten ließen sich vorübergehend ANAs, die mit den mütterlichen korrelierten, nachweisen. Trotzdem zeigte sich ein milder Verlauf, der keine weiteren Interventionen bedurfte. An mögliche potentielle lebensbedrohliche Symptome sollte aber immer gedacht werden. Hierbei scheinen Steroide das Mittel der Wahl zu sein.
Schlussfolgerung Im Zuge der zunehmenden Immigration schwangerer Morbus Behçet-Patientinnen aus dem Mittelmeerraum und aus Ländern mit höherer Prävalenz, wie z. B. auch Japan, nach Deutschland sollte bei der Betreuung von Neugeboren Morbus Behçet-positiver Mütter an die Möglichkeit eines transienten neonatalen Morbus Behçet gedacht werden.
Niedrigintensive Interventionen zur Reduktion depressiver Symptome vor Beginn einer ambulanten Psychotherapie
Authors: Grünzig Sasha-Denise , Bengel Jürgen , Göhner Wiebke , Violetta Krämer Lena 
Published: July 2018

ZusammenfassungPsychotherapeutische Praxen in Deutschland können dem Versorgungsbedarf nur mit langen Wartezeiten nachkommen. Eine Möglichkeit der Versorgungslücke zu begegnen ist der Einsatz niedrigintensiver Interventionen. Ziel des Reviews ist es, niedrigintensive Interventionen zur Reduktion depressiver Symptome zu systematisieren und deren Wirksamkeit und Akzeptanz vor Beginn einer Psychotherapie zu beurteilen. Es wurden 22 Studien identifiziert, deren Interventionen sich den Intensitätsstufen aktives Warten, Selbsthilfe, begleitete Selbsthilfe, kurze fokussierte Intervention und niedrigintensive psychotherapeutische Behandlung zuordnen lassen. Die Evidenz zur Wirksamkeit einzelner Interventionen ist begrenzt; die Akzeptanz schwankt zwischen den Interventionen. Die resultierende Systematik veranschaulicht innovative Interventionen, die je nach Ressourcen und Bedürfnissen eingesetzt werden können. Versorgungsmodelle für die Bereitstellung niedrigintensiver Interventionen werden diskutiert.
Constitutive Plasminogen Activator Inhibitor 1 (PAI-1) Biosynthesis in Human Hep G2 Hepatoma Cells Is Maintained by an Autocrine Factor
Authors: Bergonzelli Gabriela E , Kruithof Egbert K O 
Published: July 2018

SummaryWe studied the production of PAI-1 by human Hep G2 hepatoma cells. When culturing these cells in fresh medium (FM), PAI-1 accumulation rate was not linear: PAI-1 antigen after 24 h was 200 ng/106 cells while in subsequent 24 h periods, it was on average 1,000 ng/106 cells. Culture of Hep G2 cells in regular changes of a 12 h conditioned medium (CM) obtained from other Hep G2, instead of in FM, resulted in a 2-fold higher PAI-1 accumulation. In cells incubated in FM, PAI-1 mRNA declined rapidly after medium change and returned to basal levels after 24 h. In contrast, PAI-1 mRNA remained relatively stable when CM was used. The acute phase mediator interleukin 6 (IL-6) was not responsible for the autocrine stimulation of PAI-1: neither IL-6 nor antibodies to IL-6 altered the observed variations in PAI-1 expression. Our studies suggest the presence of an unknown PAI-1 stimulating factor.
CD4 Cells in HIV-1 Infected Hemophiliacs: Effect of Factor VIII Concentrates
Authors: Goldsmith JoAnne M , Deutsche Joann , Tang Mary , Green David 
Published: July 2018

SummaryThe goal of this study was to determine the long-term clinical outcome of Human Immunodeficiency Virus (HIV) infection in a group of HIV-seropositive hemophiliacs for whom the dates of seroconversion were known and to investigate whether the use of monoclonal antibody purified factor (high purity) concentrate and treatment with zidovudine may alter the effect of HIV infection in seropositive hemophiliacs.Twenty-eight hemophiliacs were followed for up to 9 years after seroconversion. In addition, 13 seropositive patients who elected to receive (high purity) factor VIII concentrate for up to 1.5 years were compared to a contemporaneous concurrent control group of 8 seropositive patients treated with intermediate purity factor VIII concentrate, and then both groups were followed for an additional 1.5 years while receiving zidovudine.The acquired immunodeficiency syndrome (AIDS) developed in 9 of 28 patients (33%) from 1 to 9 years following seroconversion. CD4 cell count declined at a rate of 13.5% per year for the cohort but for persons 25 years and above the rate was significantly higher (17.5 ± 9.2% per year vs. 9.5 ± 4.7%, mean ± SD, p <0.05) than in those under 25. All three patients who had undergone splenectomy developed AIDS.Subjects treated with high purity concentrates, with the exception of one patient who developed the nephrotic syndrome secondary to amyloidosis, had stable CD4 cell counts. On the other hand, all patients receiving intermediate purity concentrates had a decline in CD4 cells, and the difference from initial levels was highly significant (p = 0.01). The CD4 cell concentrations in these patients were stable after treatment with zidovudine.We conclude that the incidence of AIDS in seropositive hemophiliacs more than 9 years after seroconversion is similar to that seen in other seropositive groups. CD4 cell counts declined more rapidly in those patients who were older at the time of seroconversion. Splenectomy may adversely affect the outcome of HIV infection.Individuals receiving high purity concentrate may have less immunosuppression than those receiving intermediate purity concentrates. Randomized controlled trials are needed to confirm these observations.
The Role of von Willebrand Factor in Pre-Eclampsia
Authors: Bergmann Frauke , Rotmensch Siegfried , Rosenzweig Bruce , How Helen , Chediak Juan 
Published: July 2018

SummaryThe von Willebrand factor (vWF) has gained considerable interest in recent years as a marker of endothelial cell activation or insult and by virtue of its interactions with platelets and vessel walls. Altered patterns of vWF multimers were found to occur frequently in patients with thrombotic thrombocytopenic purpura in the acute and chronic stages. This disorder shares some clinical and laboratory findings with pre-eclampsia, including thrombocytopenia. Recent studies have also suggested that abnormalities of endothelial cell metabolism play a central role in the pathophysiology of pre-eclampsia. In order to determine if vWF could be instrumental in the disease process and the thrombocytopenia of pre-eclampsia we analyzed the ante- and postpartum structural and functional distribution of vWF. This data was correlated with hematological parameters such as platelet counts and the clinical severity of the disease. We found no consistent changes of vWF in association with thrombocytopenia or clinical severity. However, functional vWF was lower in postpartum samples of severely affected pre-eclamptics as compared to normal controls. This finding may reflect endothelial cell exhaustion after stimulation or cellular injury. Elevated titers of fibrin split products and thrombocytopenia were evident in severe pre-eclampsia, as seen in DIC, despite factor VIII coagulant levels within the normal range. Our data is consistent with the hypothesis of endothelial cell dysfunction in pre-eclampsia. However, the mechanism of thrombocytopenia in this disorder does not appear to be related to alterations in the structure or biological function of vWF.
Effect of Heparin on the Activation of Factor XII and the Contact System in Plasma
Authors: Pixley Robin A , Cassello Anita , De La Cadena Raul A , Kaufman Nathan , Colman Robert W 
Published: July 2018

SummaryWe examined in purified systems and in human plasma whether heparin serves as a contact system activating compound. Purified human factor XII zymogen was not activated by heparin through an autoactivation mechanism, but was activated in the presence of purified prekallikrein. Zn2+ (12 εM) did not support autoactivation by heparin. The activation of factor XII and the contact system by heparin in plasma anticoagulated with citrate or with hirudin (not chelating ions) was examined by the cleavage of 125I-labeled factor XII and high molecular weight kininogen (HK). Heparin at 1.6 and 16 USP U/ml was not able to produce activation, in contrast to dextran sulfate (20 εg/ml) which supported activation of both factor XII and HK. This study indicates that heparinized plasma does not support activation of the contact system mediated through activation of factor XII. It is not expected that heparin anticoagulant therapy will contribute to activation of the contact system.
Discordant Expression of Tissue Factor Antigen and Procoagulant Activity on Human Monocytes Activated with LPS and Low Dose Cycloheximide
Authors: Walsh John D , Geczy Carolyn L 
Published: July 2018

SummaryThe mechanisms underlying the superinduction of procoagulant activity by cycloheximide (CHX) on LPS-activated human monocytes have been investigated. Tissue factor (TF) activity of intact, viable cells was quantitated with a plasma recalcification assay and assays using chromogenic substrates specific for thrombin and factor Xa (FXa). TF antigen was measured simultaneously by immunocytochemical staining and immunoblotting with an anti-TF monoclonal antibody (MAb). Peripheral blood mononuclear cells (PBMC) activated with LPS in the presence of low dose CHX expressed more TF activity (approx. 100% increase) than cells activated with LPS alone. However, TF antigen levels were decreased approximately 70% by CHX. This discordant relationship was due primarily to differences in rates of activation of factor X (FX); LPS/CHX-treated PBMC activated nearly twice as much FX as LPS-treated cells (2.19 ± 0.37 versus 1.10 ± 0.21 ng FXa/106 PBMC/min, respectively). These studies indicate that TF cofactor activity on LPS/CHX-treated monocytes was approximately 7 times greater than that present on LPS-treated cells. Increased TF functional activity may be due to CHX-induced alterations in the type and content of phospholipids (PL) in the cell membrane. Results showed that exogenous mixed PL markedly increased TF activity on LPS-activated monocytes, but not on LPS/CHX-activated cells, without increasing TF antigen levels or altering cell viability. Membrane alterations may occur on monocytes in certain pathological or iatrogenic conditions resulting in a highly active form of TF in vivo.
Functional Ca2+ Channel Produced by Purified Platelet Membrane Glycoprotein IIb-IIIa Complex Incorporated into Planar Phospholipid Bilayer
Authors: Fujimoto Tetsuro , Fujimura Kingo , Kuramoto Atsushi 
Published: July 2018

SummaryThe ability of the platelet membrane glycoprotein (GP) IIb-IIIa complex to function as a Ca2+ channel was investigated by electrophysiological methods. The GPIIb-IIIa complex was purified with an electrically silent detergent, CHAPS, and reconstituted into liposomes. After incorporation of these liposomes to a planar phospholipid bilayer, Ba2+-permeable channel currents were detected. Since neither residual detergent nor dissociated GPIIb and IIIa produced any currents, the observed channel currents were attributed to the GPIIb-IIIa complex. These channel currents showed similar electrical properties with the Ca2+ channel previously described in platelet plasma membrane; a single channel conductance of approximately 10 pS in 53 mM Ba2+ solution and voltage-independency. It was concluded that the purified GPIIb-IIIa complex can act as a divalent cation-permeable channel in the artificial phospholipid bilayer.
The Action of a Synthetic Pentasaccharide on Thrombin Generation in Whole Plasma
Authors: Béguin S , Choay J , Hemker H C 
Published: July 2018

SummaryWe investigated the effect on thrombin generation in plasma of the pentasaccharide that represent the AT II/binding site in heparin. This compound has no effect on the breakdown of thrombin in plasma. It dose-dependently inhibits the formation of thrombin in both the intrinsic and the extrinsic pathway. If coagulation is triggered by the complete prothrombinase complex (phospholipid – factor Va – factor Xa) under conditions in which the large majority of factor Xa is bound to the complex, the inhibition of prothrombinase activity is only minor. If no factor Va is present or if the prothrombinase activity is triggered by adding complete tenase (PL-FVIIIa-FIXa) or incomplete tenase (PLFIXa) to the plasma the inhibition by pentasaccharide is of the same magnitude as that in the intrinsic or extrinsic system.We conclude that the pentasaccharide inhibits blood coagulation by katalysing the inactivation of free factor Xa. In contrast to classical heparin it does inhibit the peak of thrombin formation in platelet rich plasma, probably because it is less subject to inactivation by heparin binding proteins from platelets than classical heparin is.
Involvement of 5-Hydroxytryptamine in Platelet Aggregation In Vivo in Rats and Guinea Pigs
Authors: Smith G M 
Published: July 2018

SummaryIn this study, 5-hydroxytryptamine (5-HT) caused a dose- dependent fall in the circulating platelet count suggesting that 5-HT receptors are activated in rat platelets to cause platelet adhesion and aggregation. When low doses of adenosine diphosphate (ADP) were simultaneously injected with 5-HT, there was a significant potentiation of the responses to ADR Ketanserin significantly reduced the potentiated responses. When higher doses of ADP were infused with bolus injections of 5-HT there was no potentiation and ketanserin did not reduce these responses. Ketanserin did not inhibit the collagen-induced fall in circulating platelet count, but did significantly increase the rate of return to the basal platelet count compared with control. 5-HT did not cause a fall in platelet count in guinea-pigs
Characterization of N,N’-bis(3-Picolyl)-4-Methoxy-Isophtalamide (Picotamide) as a Dual Thromboxane Synthase Inhibitor/Thromboxane A2 Receptor Antagonist in Human Platelets
Authors: Gresele P , Deckmyn H , Arnout J , Nenci G G , Vermylen J 
Published: July 2018

SummaryPicotamide (G137 or N,N’-bis[3-picolyl]-4-methoxy-iso- phtalamide), a drug which has shown platelet inhibitory effects in vitro and ex vivo, was investigated for its mechanism of action on human platelets in vitro. This compound suppresses the aggregation of human platelets induced by arachidonic acid (IC50: 1.8 × 1(T5 M), low-dose collagen (IC50: 3.5 × 10-4 M), U46619 (IC50: 1.4 × 10-4 M) and by authentic TxA2 (IC50: 1 × 10-4 M), without affecting the aggregation induced by A23187 or primary aggregation by ADP. Picotamide inhibits dose-dependently TxA2 synthesis by platelets (IC50: 1.5 × 10-4 M) and enhances the formation of PGE2. Picotamide-treated platelets also favour the formation of PGI2 by aspirinated endothelial cells; in addition, the drug appears to exert a direct stimulatory effect on PGI2- synthesis, at least at high concentrations. Finally, in platelet-rich plasma stimulated with arachidonic acid, picotamide increases intraplatelet cAMP while no effects on cAMP are detected in unstimulated platelets.In conclusion, picotamide is a dual thromboxane-synthase inhibitor/thromboxane-receptor antagonist in human platelets and introduces a new class of agents potentially useful in antithrombotic therapy.
Studies on a Subcellular System for Vitamin K- Dependent Carboxylation
Authors: Wallin R , Prydz H 
Published: July 2018

SummaryThe subcellular localization of acarboxyprothrombin and the vitamin K-dependent carboxylation system has been studied using livers from vitamin K-deficient rats and found to be mainly in the membranes of the rough microsomal fraction. Bovine acarboxyprothrombin or fragment 1 thereof were inactive as substrates in the rat carboxylating sytem.
Unbalanced Coagulation-Fibrinolysis Potential during L-Asparaginase Therapy in Children with Acute Lymphoblastic Leukaemia
Authors: Semeraro N , Montemurro P , Giordanol P , Schettini F , Santoro N , De Mattia D , Giordano D , Conese M , Colucci M 
Published: July 2018

SummaryTreatment of acute lymphoblastic leukaemia (ALL) with L-asparaginase (L-asp) may be associated with thrombotic complications, but the pathogenetic mechanisms of thrombus formation and persistence remain unclear. We studied the procoagulant activity (PCA) of peripheral blood mononuclear cells and some components of the plasma fibrinolytic system in L0 children with ALL undergoing remission induction therapy which includes L-asp. Mononuclear cells obtained 14 days after starting L-asp treatment generated significantly higher amounts of PCA (identified as tissue factor) than cells isolated before the first dose of L-asp and 7 days after the cessation of L-asp administration (p <0.01). Augmented PCA coincided with an increase in the plasma D-dimer. The plasma levels of type 1- plasminogen activator inhibitor were found signiticantly elevated during L-asp therapy (p <0.05), whereas plasminogen levels were markedly decreased (p <0.05). These findings suggest that, during the course of L-asp treatment, the coagulation-fibrinolysis balance is shifted towards promotion of fibrin formation and deposition. Although it remains to be conclusively established whether Lasp per se or the concurrent administration of multiple chemotherapeutic agents is responsible for these changes, the latter could contribute to the thrombotic complications associated with remission induction therapy for ALL.
Monoclonal Antibodies Specific for a Conformationally Altered State of Fibrinogen
Authors: Zamarron Concepcion , Ginsberg Mark H , Plow Edward F 
Published: July 2018

SummaryFour monoclonal antibodies were selected by their ability to discriminate surface-bound from soluble fibrinogen. These antibodies reacted with insolubilized fibrinogen but not other immobilized proteins and their reaction with surface-bound fibrinogen was not diminished by a 100-fold excess of sotuble fibrinogen. The antibodies reacted with the same or spatially proximal epitopes, and the recognized epitope(s) resided within the gamma chain segment of the D domain of fibrinogen. Fab fragments of the antibodies inhibited fibrin polymerization in a dose dependent manner, suggesting that the epitope(s) was also exposed by the conversion of fibrinogen to fibrin. These data indicate that adsorption of fibrinogen onto a surface induces conformational changes and that similar changes are also evoked when fibrinogen is converted into fibrin.
Azidothymidine (AZT) in the Treatment of Symptomatic HIV-1-Infected Hemophiliacs
Authors: Eichinger S , Pabinger I , Hartl H , Stain C , Mayerhofer S , Schweiger C , Kier P , Schwarzinger I , Kyrle P A , Lechner K 
Published: July 2018

SummaryTwenty-one immunodeficiency virus 1 (HIV 1)-positive hemophilic patients were treated with Azidothymidine (AZT) for symptomatic HIV infection. The median observation period was 20.5 months.At 25 months the probability of survival was 82%, the probability of progression of disease from CDC III or IV C2 to IV C1 (AIDS) was 20% in patients on continuous AZT treatment and 50% in patients with intermption of treatment. Three patients developed severe leukopenia and 3 patients severe anemii during AZT treatment. In 1 patient a dose-dependent striking increase of transaminases during AZT treatment was observed. In 7 patients treatment was intermpted, in 1 patient because of anemia, in 1 because of pruritus and in 5 patients because of noncompliance.No signiticant changes in the consumption of clotting factor concentrates and number of bleeding episodes before and during AZT treatment were noted.We conclude, that both hematological and non-hematological side effects of AZT in HIV 1-infected hemophilic patientr ur. comparable to those seen in other risk groups . AzT does not increase the bleeding tendency in this patient group.
Solvent Extraction of Test Plasmas for Improved Recovery of Lupus Anticoagulant Activity
Authors: Exner Thomas , Papadopoulos George , Sahman Nilgun , Koutts Jerry 
Published: July 2018

SummaryPhospholipid procoagulant material mainly derived from platelets interferes or “bypasses” the more specific tests for lupus anticoagulants. Such material in test plasmas can be inactivated with recovery of lupus anticoagulant activity by simple extraction with chloroform. This solvent treatment damages mainly factors VIII, V, VII and IX. Ether and various other solvents were less damaging to these clotting factors but not quite as effective as chloroform in the recovery of lupus anticoagulant activity.
Plasminogen Activator Inhibitors Type 1 and Type 2 and Plasminogen Activators in Amniotic Fluid during Pregnancy
Authors: Estellés Amparo , Gilabert Juan , Andrés Cristina , España Francisco , Aznar Justo 
Published: July 2018

SummaryPlasminogen activator inhibitor activity (PAI), PAI-1 and PAI-2 antigen, and other fibrinolytic parameters were evaluated in amniotic fluid during normal pregnancy and compared with that obtained in plasma of pregnant women. The results indicate the presence of both PAI-1 and PAI-2 in amniotic fluid during normal pregnancy. In amniotic fluid, PAI-1 antigen levels increased from 194 ± 109 ng/ml (first trimester) to 640 ± 396 ng/ml (third trimester) and PAI-2 antigen levels increased from 72 ± 57 ng/ml to 173 ± 97 ng/ml. In contrast, a decrease in tissue-type plasminogen activator (t-PA) antigen level was observed during pregnancy. The PAI-1 levels in amniotic fluid were significantly higher than the PAI-1 levels in plasma of women at a similar gestational age. However, PAI activity, measured using single chain t-PA, was lower in amniotic fluid than in plasma of normal pregnant women. The PAI activity/PAI-1 antigen ratio in amniotic fluid after activation by a denaturing agent increased from 0.003 ± 0.004 to 0.059 ± 0.018. These results indicate that high levels of PAI-1 are present in amniotic fluid and suggest that this PAI-1 is present in a latent form that can be reactivated, at least partially, by a denaturing agent.