bluuebunny/crossref_metadata_2025_split huggingface dataset:
Überwachung der Heparintherapie bei Akutdialysen
Authors: Molitor B. , Klingel R. , Hafner G. 
Published: March 2018

ZusammenfassungDie Antikoagulation im Rahmen einer Nierenersatztherapie ist notwendig, um die Thrombosierung der Filter zu verhindern und den Blutfluss zu erhalten. Ausnahmen bilden Patienten mit akutem Nierenversagen und Begleiterkrankungen (z. B. Sepsis, Multiorganversagen), bei denen eine drohende Blutung das extrakorporale Verfahren auch ohne Antikoagulation erfordern kann.Am häufigsten wird unfraktioniertes Heparin als Antikoagulanz eingesetzt. Mit niedermolekularen Heparinen liegen ebenfalls positive Erfahrungen vor. Zur sicheren Therapieführung ist ein Monitoring der Antikoagulation notwendig. Die aktivierte Vollblut-Gerinnungszeit (ACT), die aktivierte partielle Thromboplastinzeit (aPTT) und die Anti-Faktor-Xa-Bestimmung mit chromogenen Substraten stehen als Routine- und Point-of-Care-Tests zur Verfügung. Zum Monitoring der niedermolekularen Heparine (NMH) kann nur die Anti-Faktor-Xa-Messung eingesetzt werden. Die spezifischste und valideste Methode für das Therapiemonitoring der Heparine ist der Anti-Faktor-Xa-Test mit Hilfe chromogener Substrate. Da wenig kontrollierte Studien zur Antikoagulanzientherapie und Monitoring mit dem Anti-Faktor-Xa-Test in der akuten Nierenersatztherapie vorliegen, beruhen die Empfehlungen auf den Erfahrungen mit der chronischen Nierenersatztherapie.
Ecarin Chromogenic Assay
Authors: Olschewski A. , Nowak G. , Bucha E. , Lange U. 
Published: March 2018

ZusammenfassungDer ECA (ecarin chromogenic assay) wurde zur quantitativen Bestimmung von direkten Thrombininhibitoren entwickelt. Er ist eine Weiterentwicklung der ECT (ecarin clotting time) und basiert wie diese auf der Prothrombinaktivierung durch Ecarin, einem Schlangengiftenzym aus Echis carinatus. Durch die entstehenden Aktivierungsprodukte Meizothrombin und Meizothrombin-Des-Fragment 1 wird im ECA ein chromogenes Substrat gespalten, während im Gerinnungsassay ECT plasmatisches Fibrinogen zu Fibrin umgesetzt wird.Die Aktivität von Meizothrombin und Meizothrombin-Des-Fragment 1 wird konzentrationsabhängig durch direkte Thrombininhibitoren gehemmt. Der ECA kann als ECA-H zur quantitativen Hirudinbestimmung und als ECA-T zur Bestimmung von synthetischen Thrombinhemmstoffen eingesetzt werden. Am Beispiel von Hirudin, Argatroban und Melagatran erwies sich der ECA als äußerst präzise und sensitive Methode, die die Vorteile der ECT mit denen chromogener Tests verbindet. Im Vergleich zu aPTT und ECT weist der ECA sehr geringe interindividuelle Schwankungen auf. Er wird weder von der Prothrombin-noch von der Fibrinogenkonzentration im Plasma beeinflusst.
Tumorassoziierte Prognosefaktoren der Plasminogenaktivator-Familie
Authors: Mengele K. , Harbeck N. , Reuning U. , Magdolen V. , Schmitt M. 
Published: March 2018

ZusammenfassungProteolysefaktoren aus der Plasminogenaktivator-Familie (Plasmin, t-PA, u-PA, u-PAR, PAI-1, PAI-2), die an Blutgerinnung und Abbau von Blutgerinnseln beteiligt sind, kommen in gesunden und Krebszellen (z. B. der Niere, Lunge, Leber, Brust, Prostata, des Ovars, Gehirns und Magen-Darmtrakts) vor und sind dort an Neuronalentwicklung, Blutgefäßausbreitung und Wundheilung sowie der Plazentabildung und Embryonalentwicklung beteiligt. Diese Proteolysefaktoren sind im malignen Gewebe oft in größerer Menge als im Normalgewebe der befallenen Organe anzutreffen. Bei Brustkrebspatientinnen geht ein hoher Gehalt des Plasminogenaktivators u-PA, des Plasminogenaktivator-Inhibitors PAI-1 und des u-PA-Rezeptors u-PAR (CD87) im Tumorgewebe mit erhöhter Tumoraggressivität und schlechter Prognose einher, ein erhöhter Gehalt des Plasminogenaktivators t-PA und des Inhibitors PAI-2 jedoch mit einer guten Prognose. Zur klinisch relevanten Bestimmung dieser Proteolysefaktoren im Tumorgewebeextrakt werden enzymimmunometrische ELISA-Tests empfohlen. Die enzymatische und zymographische Aktivität dieser Faktoren wird nur im Rahmen experimenteller, vorklinischer Untersuchungen bestimmt.
Verletzungen des ulnaren Seitenbandes am Daumengrundgelenk
Authors: Zichner Ludwig 
Published: March 2018

ZusammenfassungDie Ruptur des ulnaren Seitenbandes am Daumengrundgelenkist eine häufige Verletzung der Hand. Sie kommt besonders beim Skifahren (Skidaumen), aber auch anderen Sportartenvor. Diegenaue Kenntnis derAnatomie ist erforderlich, um in der klinischen Diagnostik das Verletzungsausmaß zu eruieren. Eine Röntgenaufnahme ist unabdingbar. Liegt eine komplette Ruptur oder ein knöcherner Ausriss vor, so ist die Operation das Mittel der Wahl. Die postoperativen Ergebnissesind in aller Regel sehrzufriedenstellend.
Desmopressin parenteral bei VWD 1, VWD 2A und Thrombozytopathie
Authors: Pollmann H. , Siegmund B. 
Published: March 2018

SummaryDesmopressin (DDAVP, Minirin® parenteral), which induces the release of von-Willebrand factor from endogenous stores, is indicated in von Willebrand disease type 1 (VWD 1). In the present study effectiveness of DDAVP was tested and side effects were recorded in patients with VWD 1, von Willebrand disease type 2 (VWD 2) or thrombocytopathy (TCP).
Patients, methods Subjects were analysed prior to and after Minirin parenteral infusion (0.4 μg/kg body weight (b.w.) over 60 minutes) for partial thromboplastin time (PTT, seconds), ADP/epinephrine triggered plateletfunction analyzer (PFA-100) occlusion time (seconds), factor VIII activity (FVIII, %), VWF as ristocetin cofactor activity (VWF:RCo, %) and VWF antigen (VWF:Ag, %). Side effects of DDAVP during operative interventions were recorded per questionnaires by the patients.
Results The mean ± standard deviation dose (n = 165 patients) of Minirin parenteral administered was 0.37 ± 0.02 μg/kg b.w., most often upcoming dental operations (57%) necessitated testing. Coagulation parameters of patients with VWD 1 or TCP normalised in almost all patients, but only in approximately 50% of patients with VWD 2 respectively. Appraisal of effectiveness of Minirin parenteral as good was 96% in case of VWD 1 and 95 % in case of TCP. During minor surgeries (n = 23) in 91% of the patients no complications and in 2 patients (9%) postoperative haemorrhages without need for further interventions occurred, but 83% of the patients reported adverse reactions in the questionnaires, although Minirin parenteral was well tolerated by all patients during DDAVP efficacy tests.
Conclusion Desmopressin is well tolerated and affective in patients with VWD 1 and thrombocytopathy.
Inhibitor-Immunology-Study
Authors: Wermes C. , Eifrig B. , Holstein K. , Pollmann H. , Siegmund B. , Eberl W. , Kemkes-Matthes B. , Bidlingmaier C. , Kurnik K. , Lischetzki G. , Nimtz-Talaska A. , Eisert R. , Bogdanova N. , Doerk T. , Sykora K.-W. , Wieland I. 
Published: March 2018

SummaryThe development of inhibitors in haemophilia B is one of the most important complications of replacement therapy, affecting mortality and morbidity. Inhibitor development is based on complex immunological factors, and to date, only little is known about its underlying mechanisms. Here, we present first results of the haemophilia B group of our Inhibitor-Immunology study.
Patients, methods So far we have analysed 15 patients with haemophilia B. Four of them developed a high titre inhibitor; the remaining 11 had no inhibitor. We evaluated 9 SNPs in 8 genes (CD40, CTLA-4, IL-1β, IL-10, TLR2, TLR4, TLR9, TNF-α). We compared the distribution of these alleles between inhibitor and non-inhibitor haemophilia B patients and between haemophilia B patients and a normal male control population. HLA typing was performed in all patients.
Results, discussion There appears to be a trend towards a skewed distribution of TLR 9, IL-10 and CTLA4 alleles in haemophilia B patients. Due to the limited number these differences are, however, not statistically significant.The t-test of all patients with inhibitor versus without inhibitor was significant for HLA-A*03 and DPB1*0401 and borderline for DRB1*0201.
Therapy refractory menorrhagia as first manifestation of Hermansky-Pudlak syndrome
Authors: Gehrisch S. , Tauer J. T. , Knöfler R. , Lohse J. 
Published: March 2018

Summary
Introduction Oculocutaneous albinism (OCA) in combination with a platelet function defect caused by a disturbed release reaction from platelet δ-granules (storage pool defect – SPD) is typical for the autosomal recessive inherited Hermansky-Pudlak syndrome (HPS).
Case report A girl (age: 13 years) with OCA was hospitalized with transfusion-requiring menorrhagia. The suspicion of HPS was confirmed by results of lumi-aggregometry. Suspecting a disorder in primary haemostasis treatment with tranexamic acid (10 mg/kg body weight every 8 h i. v.), desmopressin (0.3 μg/kg body weight every 8 to 12 h) and hormonal therapy (norethisterone) was started but the menorrhagia persisted. Clinical response was finally achieved by a single injection of 100 μg/kg body weight recombinant factor VIIa (rFVIIa).
Conclusion The diagnosis of HPS should be suspected in patients with OCA and bleeding symptoms and is confirmed by the proof of SPD. In case of absent clinical response to desmopressin the application of rFVIIa should be considered. Hormones and antifibrinolytics are useful options in the treatment of extensive menorrhagia.
Patients with isolated prolonged in vitro bleeding time
Authors: Stein M. , Scholz K. , Llugaliu B. , Asmelash G. , Miesbach W. , Alesci S. 
Published: March 2018

SummaryIn patients with isolated prolonged in vitro bleeding time there is no standardised treatment concept. With this study we characterized the extent of bleeding symptoms.
Patients, methods All diagnoses known to cause prolonged in vitro bleeding time (PFA-100) (epinephrine-cartridge >160 s, ADP-cartridge > 120 s) have been excluded, such as platelet function disorders, effects of medications, nutrition or von Willebrand disease. 75 patients (77%, n = 58 women; 23%, n = 17 men, median age 46 (16–81) years were included. All bleeding symptoms have been stored in a databank with help of a comprehensive questionnaire.
Results 78% (n = 54) of all patients reported of having had an operation, 69.8% (n = 37) of them described postoperative bleedings (p = 0.0373). 13.5% (n = 5) of the 54 could remember having been randomly treated by the administration of a transfusion and only 2.7% (n = 1) were treated by substitution of von Willebrand factor. 71% (n = 51) patients indicated haematoma (p = 0.8116). About 33.8% (n = 24) patients had gum bleeding and 40.8% (n = 29, p = 0.7808) patients reported bleeding after the dentist. 41.4% (n = 29) patients suffered under frequent epistaxis (p = 0.0212). There was no correlation between prolonged epinephrine bleeding time to VWF : Ag (rho = 0.16) nor to VWF : RCo (rho = 0.12) nor between prolonged epinephrine and ADP bleeding time (rho = 0.01) nor to ROTEM® analysis.
Conclusion Patients with isolated prolonged PFA are mainly women and can be affected by all kinds of bleedings while haematoma is the main symptom. VWD might not be causal
Haemophilia Registry of the Medical Committee of the Swiss Haemophilia Society
Authors: von der Weid N. 
Published: March 2018

SummaryThe Haemophilia Registry of the Swiss Haemophilia Society is currently more than 12 years old. We present here the data as from October 31st, 2012. Registered are patients with haemophilia A and B, von Willebrand disease with VWF : R-Co < 10% and other rare factor deficiencies. For this latter group, inclusion in the Registry depends on the clinical relevance of the bleeding disorder, not on the factor level. Data come directly from the Swiss haemophilia reference and treatment centers and should be updated once a year. Currently 967 patients are registered, the majority (587) presenting with haemophilia A. Disease severity is graded according to ISTH criteria. Basic epidemiological findings are similar to those from larger registries in Europe, Canada or the USA.More that 60% of persons with haemophilia in Switzerland are treated on-demand, with the exception of young patients (<20 years) who present an 80 to 90% rate of prophylactic therapy. Nevertheless, global use of factor concentrates went continuously up over the last decade and reaches now 5.52 Units per capita, still a low value compared to other high-income European countries. A recent survey of the Registry shows that treaters’ compliance with yearly data updates is insufficient; measures will be undertaken in 2013 to enhance data quality.
Morbidity and mortality of haemophilia patients in Germany
Authors: Rieger A. , Schramm W. 
Published: March 2018

SummarySince 1982, the multicenter study of the epidemiology of patients with haemophilia is carried out annually with the support of all sizes of haemophilia centers and treatment facilities. The long period of investigation has not only enabled the reporting of up-to-date mortality and morbidity status, but also of retrospective observations. The participating centers have made anonymized data available for patients with Haemophilia A, B and von Willebrand’s disease (total counts for treated patients, type and severity of the disease, HIV status and causes of death). For 2011–2012 3331 patients were reported with haemophilia A or B from 32 centers. Although the mortality from HIV/AIDS in haemophilia patients continues to decline, HIV infection remains clinically relevant since an HIV/HCV coinfection can raise the risk of severe liver disease. The data from the multicenter survey were, together with the anonymized data from the Foundation for Humanitarian Aid for Persons Infected with HIV though Blood Products, comparatively descriptively compared and the temporal progression illustrated by a so-called moving average. Expectedly, haemophilia patients were shown to be under-represented by the data from the multicenter survey. Otherwise, the data are consistent.
Neue Daten aus dem Deutschen Hämophilieregister
Authors: Haschberger B. , Heiden M. , Seitz R. , Schramm W. , Hesse J. 
Published: March 2018

SummaryThe German Haemophilia Registry records online data from patients with haemophilia A, haemophilia B, von Willebrand`s disease and other coagulation factor deficiency disorders since 2009. Patient´s pseudonymised data will only be enrolled in the German Haemophilia Registry if the patient signs an informed consent. Without the informed consent, only aggregated data according to §21 German Transfusion Law are reported. These data include the number of persons with congenital haemostasis disorders classified to type of disease and severity as well as patients’ age, and the consumption of clotting factor according to each group. Results: The highest number of patients with haemophilia was reported in 2010: 3375 patients with haemophilia A and 614 with haemophilia B respectively; the highest number of patients with von Willebrand`s disease was 1473, reported in 2011. Conclusion: In comparison to data from registries in Austria and Switzerland it can be assumed that most of the patients with severe haemophilia are registered in the German Haemophilia Registry whereas patients with moderate and mild forms are still missing.
Selection and characterisation of FVIII-specific single chain variable fragments
Authors: Naumann A. , Scherger A. K. , Neuwirth J. , Orlowski A. , Kahle J. , Schwabe D. , Königs C. 
Published: March 2018

SummaryThe development of inhibitory anti-FVIII antibodies is currently the most severe complication in the treatment of haemophilia A patients. Inhibitor eradication can be achieved by immune tolerance induction (ITI). Recent findings suggest a correlation between the FVIII-specific IgG subclass distribution and the duration or outcome of ITI. To quantify FVIII-specific IgG subclasses in patients’ plasma FVIII-specific IgG standards are required. Here, the isolation of FVIII-specific single chain variable fragments (scFvs) from synthetic phage display libraries and the characterisation of their FVIII domain specificity are described. The isolated scFv 1G10, which binds to the FVIII A2 domain, was cloned into the context of the four human IgG (hIgG) subclasses and expressed in mammalian cells. Purified 1G10-hIgG1, -hIgG2, -hIgG3 and -hIgG4 are used as standards to determine the absolute amounts and relative contribution of the different FVIII-specific IgG subclasses in future studies. The results from these studies will eventually add to understanding the role of the FVIII-specific IgG subclass distribution as prognostic factor for the outcome of ITI.
Überlegungen zum schmerzhaften Fuß
Authors: Senn Edward 
Published: March 2018

ZusammenfassungAnhand von zwei eindrücklichen Fallbeispielen wird die Entwicklung äußerst schmerzhafter Gewebe bzw. Strukturen von Füßen jeweils nach einem Fußtrauma dargestellt. Die Betonung liegt dabei auf der langjährigen Progredienz mit einer sukzessiven Ausbreitung der Beschwerden und Weichteilbefunde entlang des Beins bis zum Beckengürtel. In beiden Fällen führte die operative Korrektur, die bildgebend indiziert war, zu einer wesentlichen Verschlechterung des regionalen Schmerzzustandes sowie der Belastbarkeit. Das Fehlen der typischen fleckförmigen Osteoporose sowie besonders ausgeprägter vegetativer und dystropher Zeichen ließ immer wieder Zweifel an der Diagnose eines CRPS („Complex Regional Pain Syndrome“, Morbus Sudeck) aufkommen. Zur begrifflichen Erfassung eines derartigen regionalen Schmerzzustandes mit nur unvollständig manifest werdenden sudeckoiden Symptomen wird der Begriff des „regionalen Irritationszustandes” mit seiner zentralnervösen Verankerung vorgeschlagen.
Management der lumbalen Spinalkanalstenose
Authors: Actor B. , Paus Ch. 
Published: March 2018

ZusammenfassungDie lumbale Spinalkanalstenose ist eine häufige Wirbelsäulenerkrankung des höheren Alters. Meist wird sie mit belastungsabhängigen Schmerzen in den Beinen symptomatisch. Die Diagnose beruht hauptsächlich auf der Anamnese und den klinischen Beschwerden; mittels Bildgebung wird sie dann verifiziert. In leichten bis mittelschweren Fällen sollte die konservative Behandlung bevorzugt werden. Bei Versagen der konservativen Behandlung und in schweren Fällen ist die Zuweisung an einen Wirbelsäulenchirur-gen zur Beurteilung der Operationsindikation angebracht.
Degenerative Deformitäten der Lendenwirbelsäule
Authors: Kleinstück F. 
Published: March 2018

ZusammenfassungMit zunehmendem Alter der Bevölkerung nehmen degenerative Deformitäten an Bedeutung zu. Ihre Behandlung ist dank fortgeschrittener Operationsverfahren und Anästhesiemöglichkeiten heute einfacher als noch vor einigen Jahren – und dadurch nicht beschränkt auf konservative The-rapiemethoden. Ziel der Operation – auch beim älteren Menschen – ist ein gutes Ergebnis und eine verbesserte Lebensqualität. Von Bedeutung ist, eine für jeden Patienten angepasste individuelle Lösung zu finden. Diese kann in der Regel nur in Zusammenarbeit mehrerer Disziplinen mit einem Wirbelsäulenspezialisten gefunden werden.
Thyroid Disorders and the Distribution of Orally Administered Radioiodine
Authors: Bagwe B. A. , DaCosta H. 
Published: March 2018

Summary128 control, thyrotoxic, hypothyroid and goitrous euthyroid children were administered 1–2 μCi Na 131I orally and studied in a shadow-shield whole-body counter. 48-hr whole-body counts permitted the differentiation of the first 3 groups (p < 0.005). Ratio of extrathyroid:excreted 131I was 3 times greater in thyrotoxics than in goitrous euthyroids, but the thyroid:extrathyroid ratio was 3 times greater in the latter group. The whole-body profile scan obtained at 48 hr on an attached strip chart recorder, revealed activity not only in the thyroid but even lower down in the vicinity of the knees and feet.
Influence of PET/CT-introduction on PET scanning frequency and indications
Authors: Bockisch A. , Eschmann S. M. , Krause B. J. , Rödel R. , Tiling R. , Weckesser M. , Stergar H. 
Published: March 2018

Summary
Aim: To evaluate the influence of the introduction of combined PET/CT scanners into clinical routine. This investigation addresses the quantitative changes between PET/CT and stand alone PET. Methods: The study included all examinations performed on stand alone PET- or PET/CTscanners within 12 month prior to and after implementation of PET/CT. The final data analysis included five university hospitals and a total number of 15 497 exams. We distinguished exams on stand alone tomographs prior to and after installation of the combined device as well as PET/CT scans particularly with regard to disease entities. Various further parameters were investigated. Results: The overall number of PET scans (PET and PET/CT) rose by 146% while the number of scans performed on stand alone scanners declined by 22%. Only one site registered an increase in stand alone PET. The number of exams for staging in oncology increased by 196% while that of cardiac scans decreased by 35% and the number of scans in neurology rose by 47%. The use of scans for radiotherapy planning increased to 7% of all PET/CT studies. The increase of procedures for so-called classic PET oncology indications was moderate compared to the more common tumors. An even greater increase was observed in some rare entities. Conclusions: The introduction of PET/CT led to more than a doubling of overall PET procedures with a main focus on oncology. Some of the observed changes in scanning frequency may be caused by a rising availability of new radiotracers and advancements of competing imaging methods. Nevertheless the evident increase in the use of PET/CT for the most common tumour types demonstrates its expanding role in cancer staging. The combination of molecular and morphologic imaging has not only found its place but is still gaining greater importance with new developments in technology and radiochemistry.
Eine Methode zur Erfassung akuter Plasmavolumsveränderungen
Authors: Auerswald W. , Höfer R. , Vetter H. , Zekert F. 
Published: March 2018

ZusammenfassungEs wird eine Methode beschrieben, die es ermöglicht, kurzdauernde P.V.-Veränderungen mittels einer einmaligen Injektion einer Indikatorsubstanz nach dem Verdünnungsprinzip zu messen.Nach Injektion von etwa 45 bis 105 μC J131-Albumin wird zunächst in üblicher Weise das P.V. bestimmt. Nach Einstellung des Äquilibriums wird dann eine Infusion vorgenommen und aus den folgenden Änderungen der J131-Plasma-konzentration die P.V.-Zunahme errechnet.Nach Wiedereinstellung des Äquilibriums können solche Untersuchungen so lange wiederholt werden, als die noch verbleibende Aktivität ausreichend genaue Messungen erlaubt.
The Adjuvant Therapy of Bronchogenic Carcinoma Following Pneumonectomy or Lobectomy with the Intrathoracic Introduction of Silver-Coated Radioactive Colloidal Gold
Authors: Hahn P. F. 
Published: March 2018

SummaryIn previous investigation it was observed that silvercoated Au198-colloid is removed from the thoracic cavity by lymph nodes at the same speed as pure silver colloid. The percentage uptake of radioactivity in the lymph nodes is sufficiently high to ensure that, despite extremely variable distribution between the various lymph nodes, those which incorporate the smallest amounts will still receive a sufficiently high radiation dose.Since 40% of the patients with bronchogenic carcinoma develop metastases in liver and adrenals, it is recommended to have the administration of the silver-coated gold colloid into the pleural cavity to be followed by an intravenous injection of 50 mC of ordinary radioactive colloidal gold in order to apply to the liver an evenly distributed radiation dose of about 3500 rad. Subsequently, the patient should be carefully watched and blood transfusions should be given in the event of marked leucopenia and thrombocytopenia. To facilitate a clinical trial on a large number of patients, a number of hospitals collaborate to ensure identical treatment of 600 patients with lung resection.