bluuebunny/crossref_metadata_2025 huggingface dataset:
Therapy refractory menorrhagia as first manifestation of Hermansky-Pudlak syndrome
Authors: Gehrisch S. , Tauer J. T. , Knöfler R. , Lohse J.
Published: March 2018

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

In 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

The 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

Since 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

The 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

The 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.
Erfahrungen mit Rivaroxaban bzw. Dabigatran
Authors: Boxberger N. , Heim M. U. , Hartung K.-J. , Aumann V.
Published: March 2018

Die Behandlung mit Phenprocumon und Protein C bot einer Patientin mit schwerem angeborenem Protein-C-Mangel lange Zeit sicher den sichersten Schutz vor thromboembolischen Ereignissen. Die nachlassende Wirksamkeit von Phenprocumon führte zu Purpura-fulminans-ähnlichen Erscheinungen. Rivaroxaban und Dabigatran wurden deshalb alternativ eingesetzt. In normaler Dosierung konnten sie die Patientin nicht ausreichend schützen. Aktuell erfolgt ihre Behandlung mit Protein C und Enoxaparin.
Überlegungen zum schmerzhaften Fuß
Authors: Senn Edward
Published: March 2018

Anhand 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

Die 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

Mit 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.
Bewegungsprogramm gegen Osteoporose, Sarkopenie, Stürze und Frakturen
Authors: Runge Marti
Published: March 2018

Das Ziel jeder Osteoporosetherapie ist das Verhindern von Frakturen. Da in der Pathogenese der überwiegenden Anzahl der sogenannten osteoporotischen Frakturen der Sturz eine kausale Rolle spielt, ist neben der Verbesserung der Knochenfestigkeit die Senkung des Sturzrisikos ein vorrangiges Therapieziel. Damit rückt der Muskel in den Mittelpunkt.
The Evaluation of Noise Reduction and Resolution Degradation in Scintigraphic Images Due to the Smoothing Procedure
Authors: Ewa A. , Skrzypczak T. , Tołwiński J. R. , Gwiazdowska Barbara
Published: March 2018

Es wurden die Herabsetzung der räumlichen Auflösung und der Verminderung des statistischen Fehlers als Ergebnis von Glättungsverfahren mittels “Einheits”-Matrizen analysiert und eine quantitative Beziehung zwischen dem Auf-lösungsverschlechterungsfaktor kR und dem Produkt aus dem Fehlerherabsetzungsfaktor und der linearen Größe des Bildelementes (m · kf) erhalten. Die numerischen Werte für kR für den Fall einer Einzelglättung mit „Einheits“-Matrizen jeglicher Größe wurden mit jenen für n-fache Glättungen verglichen. Es wurde gezeigt, daß die letztere Methode vorteilhafter ist, d.h. sie ergibt niedrigere Werte für den Faktor kR bei gleichen Werten von (m · kf).
Thyroid Disorders and the Distribution of Orally Administered Radioiodine
Authors: Bagwe B. A. , DaCosta H.
Published: March 2018

128 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.
Yttrium-90 zur Schmerztherapie von Knochenmetastasen
Authors: Dähnert W. , Schreyer T. , Grimm W. , Brod K. H. , Becker M. , Kutzner J.
Published: March 2018

Im fortgeschrittenen Tumorstadium hat sich bei Ausbildung von schmerzhaften Skelettmetastasen die Applikation von betastrahlenden knochenaffinen Isotopen bewährt. Die Anwendung von 89Sr bietet aufgrund der langen physikalischen Halbwertzeit erhebliche Strahlenschutzprobleme. Durch tierexperimentelle Untersuchungen mit Yttrium konnte nachgewiesen werden, daß bei Verwendung eines Y-Zitratkomplexes eine hohe Knochenaffinität besteht und Y sich damit kalziumähnlidh verhält. 90Y läßt sich nach dem beschriebenen Verfahren mit hoher Reinheit trägerfrei von einer 90Sr-Säule eluieren. Retentionsmessungen mit einem Ganzkörperzähler ergaben bei Tumorpatienten mit ausgedehnten Knochenmetastasen eine Gesamtretention von Yttrium von über 80%. Die ersten Ergebnisse therapeutischer Anwendung bei der Knochenmetastasenbehandlung zeigen den gleichen analgetischen Effekt wie 89Sr, wobei aufgrund der kurzen Halbwertzeit von 64 Std. die Strahlenschutzprobleme gering sind. Auch kostenmäßig bietet die Anwendung von 90Y gegenüber 89Sr Vorteile.
Quantitative Radiokardiographie zur Differentialdiagnose und Verlaufskontrolle von Herzkrankheiten
Authors: Oberhausen E. , Wilhelm H. , Brill G.
Published: March 2018

Bei über 400 Patienten wurde eine quantitative Herzbinnenraumszintigraphie durchgeführt und hierbei die regionale Motilität und mittels Fourieranalyse die folgenden Funktionsparameter ermittelt: Ejektionsfraktion, maximale Ejek-tionsrate und deren Zeitpunkt, maximale Füllungsrate und deren Zeitpunkt. Zusätzlich ergeben sich durch das bei der ersten Passage verwendete Kalibrierungsverfahren die Ventrikelvolumina und das Herzminutenvolumen. Die Veränderungen der verschiedenen Funktionswerte bei unterschiedlichen Herzerkrankungen werden im einzelnen diskutiert. Hierbei zeigt sich, daß sich aus den Meßwerten wesentliche differentialdiagnostische Hinweise ergeben. Die Untersuchungen vor und nach chirurgischen Eingriffen erlauben eine weitgehende Beurteilung des Therapieerfolges.
201T1-Myocardial Scintigraphy: Current Status in Coronary Artery Disease, Results of Sensitivity/Specificity in 3092 Patients and Clinical Recommendations
Authors: Kanemoto N. , Hör G.
Published: March 2018

This review is concerned with qualitative and quantitative sectorial 201Tl-redistribution analysis of exercise myocardial scintigraphy (EMS). In 3092 cases the sensitivity (specificity) was on average 83 (90) %, the average CAD prevalence being 71%. Sensitivity (Se) for EMS (ExECG) increased from 73 (43) % in single vessel disease through 83 (69) % in double vessel to 90 (77) % in triple vessel disease (n = 879); average Se was 77% for LAD-, 79% for RCA- and 65% for LCX-stenosis. Se for detection of the real extension of CAD conversely decreased from 59% in SVD through 41% in DVD down to 33% in TVD. Clinical recommendations for EMS and rest scans are outlined in CAD (atypical angina, follow-up after bypass-surgery, percutaneous transluminal angioplasty), in non-coronary artery disease (non-ischemic cardiomyopathies, right ventricular hypertrophy) and in pediatric cardiology.
Ossäre Metastasierung des Prostata-Karzinoms in Abhängigkeit von Tumorgröße und Geschwulstdifferenzierung
Authors: Wegner G. , Distelmaier W. , Krause U. , Biersack H.
Published: March 2018

Bei 153 Patienten mit Prostata-Karzinom wurde eine Ganzkörper-Skelettszintigraphie durchgeführt. In 101 Fällen waren Knochenmetastasen auszuschließen, bei 46 Patienten hingegen nachzuweisen. Sechs Patienten mit fraglichem Befund wurden in die weiteren Untersuchungen nicht mit einbezogen. Es zeigte sich, daß die ossäre Metastasierungstendenz mit zunehmender Entdifferenzierung der Geschwulst deutlich ansteigt: so wiesen Patienten mit Prostata-Karzinom von geringem Malignitätsgrad in 7,3%, von mittlerem Malignitätsgrad in 40% und von hohem Malignitätsgrad in 38,5% Absiedlungen im Skelett auf. Auch die Turmorgröße korrelierte mit dem Metastasennachweis: von Patienten mit dem Tumorstadium T1 zeigten lediglich 7,1%, mit T2 19,3%, mit T3 39,2% und mit T4 bereits 65% ossäre Fernmetastasen. Die Entdifferenzierung der Geschwulst nahm mit der Tumorgröße ebenfalls zu. Die erhobenen Befunde beweisen die Notwendigkeit der Früherkennung eines Prostata-Karzinoms.
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

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

Es 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

In 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.
The Differential Diagnosis of the Polycythaemias by Plasma Iron Turnover Determination
Authors: Telfer Nancy , Schiffman Norman
Published: March 2018

We have demonstrated the value of the red blood cell mass and plasma iron turnover determinations in differentiating among the three polycythaemias. We have proposed a simple, clinically practical test, and a mathematical means of expressing it, in which, by use of one serum iron determination and one injection of Fe59, both the RBC mass and the PIT can be determined.

Two corrections are necessary in the blood volume calculations if one is to use the Fe59 plasma labelling method:

1. Twenty per cent must be subtracted from the blood volume value determined.

2. The total body haematocrit (0.91 × Hctv) is used.

The significance of the PIT is determined by comparing the observed PIT with the PIT predicted to be necessary to maintain the patient’s RBC mass; this value is termed the Polycythaemia Index. It was shown that in polycyth-aemia vera the PIT is in excess of that necessary to maintain the red cell mass,

whereas in the other polycythaemias the PIT is just adequate to maintain the red cell mass.

The results obtained in patients with primary polycythaemia, secondary polycythaemia, and relative polycythaemia are compared with the results in a group of controls, and the value of the procedure demonstrated.
Ein neues, einfaches Gerät zur Isotopen-Nephrographie
Authors: Höfer R. , Egert H. , Roszuczky A.
Published: March 2018

Unsere bisherigen Erfahrungen haben gezeigt, daß mit dieser Methode im wesentlichen die gleichen Ergebnisse erzielt werden können, wie mit der konventionellen Nephrographie. Die Notwendigkeit auf Informationen aus der ersten (Durchblutungs-)Phase des Nephrogramms verzichten zu müssen, dürfte den diagnostischen Wert der Methode nicht wesentlich einschränken. Dagegen bedeutet die Verwendung nur eines Szintillationskopfes neben der relativen Billigkeit den Vorteil, daß naturgemäß die Notwendigkeit wegfällt, zwei Szin-tillationszähler in ihrer Empfindlichkeit aufeinander abzustimmen. Die Einstellung des Detektors im Verhältnis zum Nierenparenchym ist durch die Verwendung der angegebenen Kollimation und durch die Tatsache, daß das Parenchym beider Nieren in einer Richtung überwandert wird, relativ einfach. Die Tatsache, daß die Kurven für beide Nieren gleichzeitig von einem Schreiber aufgezeichnet werden, bringt für die Interpretation gewisse Vorteile.
Gamma-Ray Spectrometric Analysis of Some Trace Elements in the Human Articular Disc of the Mandible
Authors: Bergman Bo , Söremark Rune
Published: March 2018

By means of neutron activation and gamma-ray spectrometry the concentrations in the human mandibular articular disc of the following elements have been determined: Na, Mn, Cu, Zn, Rb, Sr, Cd, W, and Au. The discs were obtained at necropsy from seven men and nine women, ranging in age from 56 to 71 years.

The activation was carried out in a thermal neutron flux of about 1.7 XlO12 neutrons × cm−2 × sec.−1 for about 20 hours. A chemical group separation

was performed before the gamma-ray spectrometry. Quantitative data based on the dry weight of the cartilage samples were obtained by comparing the photo-peak area of the identified elements with those of appropriate standards.
Zur Methodik des 131J-Trijodthyronin-in-vitro-Tests
Authors: Moll E. , Romen W. , Börner W.
Published: March 2018

Eingangs wird das Prinzip eines 131J-Trijodthyronin-in-vitro-Tests (131JT3-Test), der den Kationenaustauscher Amberlite CG 50 II benützt, beschrieben. Methodische Untersuchungen zu diesem Test hatten folgende Ergebnisse:

1. Auf eine Vorinkubation des Serum-131 JT3-Gemisches kann nicht verzichtet werden. 15 min sind hierfür ausreichend.

2. Das Alter von verschlossenen, kühl gelagerten Seren beeinflußt den Test während der beiden, auf die Blutabnahme folgenden Tage nicht. Am 3. und 6. Tag danach wird eine leichte Erhöhung der Werte beobachtet.

3. Die Abhängigkeit der 131JT3-Aufnahme vom pH-Wert des Blutes wird untersucht. Im physiologischen pH-Bereich wird die Schwankungsbreite der Wasserstoffionenkonzentration durch eine Pufferung des Inkubationsgemisches nur unwesentlich verringert. Deshalb verzichten wir auf die Verwendung einer Pufferlösung.

4. Bei Durchführung des 131JT3-Tests im offenen System tritt in Fällen von CO2-Retention ein allmählicher CO2-Ausgleich und damit eine Eliminierung dieses Störfaktors ein.

5. Eine mehr oder minder starke Anlagerung des 131JT3 an Glaswaren läßt sich kaum vermeiden. Bei der Durchführung des Tests in der von uns angegebenen Weise tritt hierdurch jedoch keine Verfälschung der Ergebnisse ein.
A Simple Pulse Interval Analyzer for Scintillation Scanning Technical Note
Authors: Erban J. , Hušák V. , Wiedermann M.
Published: March 2018

A simple pulse interval analyzer as an attachment to a scanner was developed which efficiently suppresses the background of scintiscans. The analyzer was used both for mechanical and optical records, the latter exploiting a television cathode-ray tube. Electronic details of the apparatus and examples of its use are given.
Thrombin generation before and after multicomponent blood collection
Authors: Cimenti C. , Sipurzynski S. , Gallistl S. , Rosenkranz A. , Hiden M. , Leschnik B. , Schallmoser K. , Lanzer G. , Muntean W. E.
Published: March 2018

IThe development of apheresis technology has increased efficiency in donor blood use by collecting specific blood components in several combinations. The question of donor safety raised by the contact of donor blood with foreign, only in part biocompatible surfaces remains. The aim of this study was to estimate the effect of multicomponent blood collection on thrombin generation performing an overall function test of coagulation. Donors, methods: 26 blood donors were included. Per apheresis two units of platelets and one unit of RBCs were collected by two cell separators (Amicus® and Trima Accel®). Each donor underwent the procedure on both apheresis systems. Samples were collected before, immediately after, and 48 hours after apheresis. Thrombin generation was measured by means of calibrated automated thrombography (CAT). Results: CATdata changed only slightly and no significant changes were seen before, immediately after, and 48 hours after apheresis. The parameters did not differ significantly between the two different apheresis devices. Conclusion: No change in parameters of continuous thrombin generation occurred, suggesting that apheresis did not lead to severe alterations in the haemostatic system.
Nicht-viraler Gentransfer führt zu therapeutischen Faktor-IX-Spiegeln im Hämophilie-B-Mausmodell
Authors: Milanov P. , Roth S. , Seifried E. , Tonn T. , Schüttrumpf J.
Published: March 2018

In ersten klinischen Gentherapiestudien mit viralen Vektoren sind Bedenken hinsichtlich des Risikos der Entstehung von Malignomen sowie bezüglich unerwarteter Immunantworten gegen virale Proteine aufgekommen. Im Gegensatz dazu standen nicht virale Therapien bisher eher im Abseits. In unserer Studie untersuchen wir daher einen nicht-viralen Ansatz zur Behandlung der Hämophilie B. Methodik: Zuerst wurde ein Expressionsplasmid für eine starke leberspezifische Faktor-IX(FIX)-Expression konstruiert. Um den Vektor zu testen, wurden Gruppen von FIX-Knockout-Mäusen zwei Vektordosierungen mittels hydrodynamischer Injektionstechnik appliziert. Ergebnis: Bereits eine einmalige Behandlung führte zu FIX-Expression oberhalb 100% normaler Plasmaspiegel. Das FIX-Protein war voll funktionsfähig, inhibitorische Antikörper gegen FIX wurden nicht beobachtet, Expressionsspiegel waren abhängig von der Vektordosis. Schlussfolgerung: Die hier vorgestellten FIX-Expressionsspiegel im Mausmodell geben Hoffnung auf die Weiterentwicklung nicht viraler Gentherapieansätzen zur Behandlung der Hämophilie B auch am Menschen.
Cutaneous ulcers following hydroxyurea therapy
Authors: Hartmann K. , Nagel S. , Erichsen T. , Rabe E. , Grips K. H. , Bieber T. , Pannier-Fischer F.
Published: March 2018

Hydroxyurea (HU) is usually a well tolerated antineoplastic agent and is commonly used in the treatment of chronic myeloproliferative diseases. Dermatological side effects are frequently seen in patients receiving longterm HU therapy. Cutaneous ulcers have been reported occasionally.

We report on four patients with cutaneous ulcers whilst on long-term hydroxyurea therapy for myeloproliferative diseases. In all patients we were able to reduce the dose, or stop HU altogether and their ulcers markedly improved. Our observations suggest that cutaneous ulcers should be considered as possible side effect of long-term HU therapy and healing of the ulcers can be achieved not only by cessation of the HU treatment, but also by reducing the dose of hydroxyurea for a limited time.
Rezidivvarizen: Progression der Varikosis
Authors: Hofer T.
Published: March 2018

Ziel: Welche klinische Bedeutung hat das echte Magnakrossenrezidiv (MKR) im Kontext mit der natürlichen Progression der Varizenkrankheit in einem Kollektiv von Voroperierten (KVO)? Methode: Bei 221 Patienten mit erneuter Varizenbildung bei Zustand nach früher erfolgten Krossektomien mit oder ohne Stripping epifaszialer Varizen werden die MKR und die von ihnen ausgehenden Rezidivvarizen auf ihre klinische und hämodynamische Relevanz hin untersucht und den im Rahmen der Progression der Varikosis neu aufgetretenen Magnakrosseninsuffizienzen (nMKI) gegenübergestellt. Ergebnisse: Neben 112 nMKI bestehen 118 MKR, 46 von ihnen stehen in Verbindung mit einem noch in situ gelegenen Magnastamm (oder kalibermäßig vergleichbarem Gefäß). In 50% lässt sich in diesen Fällen photoplethysmographisch ein pathologischer T0-Wert <19 s nachweisen. 54 MKR, in Verbindung stehend mit unsystematischen Seitenastvarizen oder einer Vena saphena magna accessoria zeigen nur in 24% T0-Werte <19 s (p = 0,014). 39% der MKR erfordern eine erneute operative Revision. Dies entspricht 20% der duplexsonographisch nachgewiesenen MKR und nMKI im KVO. Schlussfolgerung: Die klinische Relevanz der MKR bleibt bescheiden im direkten Vergleich mit der Progression der Varizenkrankheit.
Ätiologie sekundärer Lymphödeme onkologischer Genese
Authors: Neuhüttler S. , Kröll A. , Brenner E.
Published: March 2018

Neoplasien stellen eine wichtige Ursache für sekundäre Lymphödeme dar. Dabei kann der Tumor selbst (malignes Lymphödem) oder seine Therapie das Lymphödem auslösen. Die wichtigsten Neoplasien im Hinblick auf Lymphödeme betreffen – in absteigender absoluter Lymphödem-Inzidenz – die Mamma, die Prostata, die Haut (Melanom), das Ovar und die Harnblase. Jedoch können auch zahlreiche andere Tumore direkt oder indirekt ein sekundäres Lymphödem auslösen. Ganz klar erkennbar ist, dass mit der Radikalität der Therapien die Inzidenz von Lymphödemen zunimmt; Daten für einen Zusammenhang mit dem Schweregrad fehlen jedoch. Bei zunehmenden Überlebensraten steigt daher die Prävalenz von Patentinnen und Patienten mit einem Lymphödem mit physischen und/oder psychischen Beschwerden und Einschränkungen im Erwerbsund/oder Privatleben.
Ambulatory and haemodynamic treatment of venous insufficiency by ultrasound-guided sclerotherapy (ESEC cure)
Authors: Bernardini E. , Piccioli R. , Bisacci C. , Pagliuca V. , Bisacci R. , De Rango P.
Published: March 2018

Optimal treatment for venous insufficiency is still conflicting. Aim of this study was to assess long term outcome of a new method of sclerosis for treatment of primary varicose veins. Methods: ESEC (Echo-Sclerosis haEmodynamic Conservative) technique is a new treatment for varicose veins that combines sclerotherapy and haemodynamic principles. Super-selective sclerosis of a saphenous branch or saphenous trunk interrupts venous pressure column (primary cause of venous stasis) over a reentry confluence. Advantageous new drainages into deep circulation through saphenous junctions and perforating veins follow. Results: A total of 146 ESECs (107 great, 24 small, and 15 accessory saphenous veins) with at least two years follow up were analysed. There was no treatment failure. At 14 years, 100% of visible varicosities were successfully clinically treated (complete absence, or minor varicosities or symptoms after treatment). Cosmetic outcome was assessed by the patients as good (excellent or fair cosmesis). Over 14 years, repeated treatment was required with a mean of 2.16/case. Mean vein diameter decrease was 52%. Conclusion: ESEC technique is efficient, safe, in-office manageable and easily repeatable. It might be considered as the primary treatment for most varicose patients. Large comparative study assessing cost-efficacy and patient satisfaction from a life-long point of view are needed.
Sex hormone receptors in varicose veins of women with premenstrual syndrome
Authors: Dzieciuchowicz L. , Kotwicka M. , Gabriel M. , Szczęśniak-Chmielecka A. , Krasińska B. , Oszkinis G. , Majewski W. , Krasiński Z.
Published: March 2018

The pathogenesis of premenstrual tension syndrome is not fully understood. It has been hypothesized that the interaction between sex hormones and target organs is the key event in its pathogenesis. Aim: The purpose of the study was to examine the differences in the prevalence of smooth muscle cells with sex hormones receptors in varicose veins of women with and without premenstrual tension syndrome (PS). Patients, methods: Samples of great saphenous vein were obtained from 50 women during varicose vein surgery. They were divided into group I (20 women with clinically diagnosed PS) and group II (30 women without PS). Estrogen and progesterone receptors were detected with an immunohistochemical method. Superficial densities of smooth muscle cells positive to estrogen and progesterone receptors were analyzed with densitometric program IM-AN and automatic image analyzer Magicall. The results were compared with t-Student test. Results: There were no differences in superficial density of smooth muscle cells positive to estrogen receptors between the groups. In contradiction to this, superficial density of smooth muscle cells positive to progesterone receptors was higher in group with PS than in patients without, 343 (±171) and 240 (±123), respectively. The difference was statistically significant (p < 0.05). Conclusion: The amount of cells with sex hormone receptors, not the level of hormones, could play a role in PS pathology. Our results show that progesterone is more important than estrogen in effector organs.
Effectivness and tolerability of bipolar radiofrequency-induced thermotherapy for the treatment of incompetent saphenous veins
Authors: Harnoss B. , Akkersdijk G. , Braithwaite B. , Hnatek L. , Roche E. , Santoro P. , Sarlija M. , Sezgin Y. , Nio D. , Ajduk M. , Koios D. , Camci M.
Published: March 2018

Endoluminal techniques such as laser therapy, radiofrequency therapy and sclerotherapy are increasingly replacing classic stripping for the treatment of incompetent saphenous veins. Interim results of the ongoing international, multicentre BRITTIV study to investigate the effectiveness and tolerability of the minimally invasive bipolar radiofrequency-induced thermal therapy (RFITT) are presented. Patients, methods: A flexible bipolar RFITT applicator is passed under ultrasound control into the affected vein of patients with varicosis of the great saphenous vein (GSV) or small saphenous vein (SSV). The vein is occluded by impedance-controlled release of radiofrequency energy as the RFITT applicator is slowly and smoothly withdrawn. The effectivness and tolerability of RFITT is checked over a one-year period by regular follow-ups. Results: To date a total of 345 saphenous veins (90% GSV, 10% SSV) in 271 patients (average age 50 years, 63% women, mean CEAP score 2.8) have been treated with RFITT in eight European hospitals. 72% of patients underwent general anaesthesia (18% spinal, 11% local) and tumescent anaesthesia was also used in 83% of the veins (mean length 45 cm). The average application time was 1.4 s/cm with a mean power output of 24 Watt. At the interim analysis after an average follow-up of 103 days, 90% of 335 treated veins were occluded. Differential analysis showed that partial (P) and complete (C) recurrences occurred significantly less often when longer application times had been used, whereby complete recurrences showed a greater reduction in frequency than partial. The total recurrence rate with an application time < 1 s/cm was 22% (P = 10%, C = 12%); with an application time between 1.0 and 1.4 s/cm it had already fallen to 9% (P = 6%, C = 3%) and with a time greater than 1.4 s/cm it was only 4% (P = 3%, C = 1%). According to subjective pain assessment on a visual analogue scale (VAS) of 0 to 10 (corresponding to no pain up to the strongest conceivable pain), by the first day after treatment already 74% of those questioned had only slight remaining pain or none at all (VAS 0–2). This generally low subjective pain sensation continued to fall at subsequent followups and at the 6-month follow-up, 99% of patients reported a VAS score of 0. Nearly all patients (99%) were satisfied with the treatment and would recommend it to others. Conclusions: With the same power output, even a minor increase in the application time to more than 1.4 s/cm can markedly reduce recurrence rate. In summary, the ongoing BRITTIV study illustrates the potential of RFITT for use in phlebology as it combines a spatially-defined obliteration of incompetent saphenous veins with overall moderate side effects and impressive results.
Ergebnisse der Radiojod-Behandlung autonomer Schilddrüsen-Adenome unter Berücksichtigung regionaler Jodkinetik und paranodulärer Speicherung
Authors: Moser E. , Büll U. , Tosch U.
Published: March 2018

Bei 110 Patienten mit dekompensiertem autonomen Adenom der Schilddrüse wurde der Erfolg einer Radiojod-Therapie mit unterschiedlicher Herddosis (300 Gy, 400 Gy) überprüft. Zusätzlich wurden die relativen paranodulären Speicherungswerte vor und nach Radio-Therapie in Abhängigkeit von der Therapiedauer verglichen. Bei kurzer Therapiedauer (5-8 Tage) sinkt die paranoduläre Speicherung ab, während sie bei langer Therapiedauer (9-20 Tage) zunimmt. Dadurch ist die bei langer Therapiedauer nachweisbare höhere Hypothyreoserate (14%) erklärbar. Die Ausschaltquote betrug bei einer Herddosis von 300 Gy 93,6%, bei 400 Gy waren es 95%. Dagegen fand sich bei einer Herddosis von 300 Gy eine geringere (3,2%) Hypothyreoseinzidenz als bei 400 Gy (8,8%). Trotzdem sollte zur Verminderung der Versagerquote die höhere Herddosis gewählt werden. Wegen des Ansteigens der Hypothyreoserate mit zunehmender paranodulärer Speicherung empfiehlt sich in Fällen von paranodulären Speicherungswerten zwischen 10 und 20% (sogenannter Übergangstyp) eine exogene Suppression mit Schilddrüsenhormon.
Clinical Significance of Endogenously Labelled Thyroid Hormones in the Diagnosis of Thyroidal Autonomy
Authors: Kutzim H. , Waters W.
Published: March 2018

The clinical value of the determination of 123I concentration in serum 48 hrs after tracer administration (123I)48 is investigated with special regard to thyroidal autonomy. Serum radioiodine concentration, thyroid radioiodide uptake at 4 and at 48 hrs were measured in 74 healthy subjects and patients with simple goiter, in 36 patients with thyroidal autonomy (diagnosis by thyroid suppression test), and in 20 hyperthyroid patients. 83% of the patients with elevated radioiodine concentration belonged to the group of thyroidal autonomy. The product of radioiodine concentration and thyroid radioiodide uptake is a much better parameter. 95% of the patients in which this product was elevated, belonged to the autonomy group (5 % diagnostic error). Also in the control group the diagnostic error was 5 %. The combination of (123I)48 with the result of the TRH-test is very useful in excluding thyroidal autonomy, if (l23I)48 is normal and the TRHtest is positive (100% of the patients have regulated thyroid glands). 94% of the patients having elevated (123I)48 and a negative TRH-test belonged to the group of thyroidal autonomy. A very useful combination for the diagnosis of borderline hyperthyroidism is the determination of the product of (123I)48 and the uptake48 together with the pulse rate or fine tremor of the fingers (or TRH-test). The results suggest that the determination of (123I)48 is a very good parameter of thyroidal autonomy beside the thyroid suppression test. It may be used alone for the diagnosis of thyroidal autonomy if the suppression test is contraindicated. In the diagnosis of borderline hyperthyroidism its determination makes the suppression test unnecessary in many instances.
Radioligands for imaging myocardial α- and β-adrenoceptors
Authors: Schäfers M. , Law M. P. , Wichter T. , Schober O. , Riemann B.
Published: March 2018

Alpha- and beta-adrenoceptors play an important role in the control of heart function. According to their molecular, biological, and pharmacological characteristics, they are subdivided into α1-, α2- and β1-, β2-, β3-, β4-adrenoceptors. In cardiac disease, there is often a selective downregulation of β1-adrenoceptors associated with a relative increase in β2- and α1-adrenoceptors. Functional imaging techniques like single-photon emission tomography (SPECT) and positron emission tomography (PET) provide the unique capability for non-invasive assessment of cardiac adrenoceptors. Radioligands with high specific binding to cardiac α- and β-adrenoceptors suitable for radiolabelling are required for clinical studies. The non-selective β-adrenoceptor antagonist [11C]CGP-12177 was used to quantify β-adrenoceptor density using PET in patients with heart disease. New non-selective ligands (e. g. [11C]CGP-12388, [18F]CGP-12388, [11C]carazolol and [18F]fluorocarazolol) are currently evaluated; β1-selective radioligands (e. g. [11C]CGP-26505, [11C]bisoprolol, [11C]HX-CH 44) and β2-selective radioligands (e. g. [11C]formoterol, [11C]ICI-118551) were assessed in animals. None of them turned out as suitable for cardiac PET.

Potential radioligands for imaging cardiac α1-adrenoceptors are based on prazosin. Whereas [11C]prazosin shows low specific binding to myocardium, its derivative [11C]GB67 looks more promising. The putative α2-adrenoceptor radioligand [11C]MK-912 shows high uptake in rodent myocardium but has not yet been evaluated in man.

A number of radioligands were evaluated for assessing cardiac adrenoceptors using PET. New radioligands are needed to provide more insight into cardiac pathophysiology which may influence the therapeutic management of patients with cardiovascular disease.
Quantitative accuracy of serotonergic neurotrans-mission imaging with high-resolution 123I SPECT
Authors: Kuikka J. T.
Published: March 2018

Aim: Serotonin transporter (SERT) imaging can be used to study the role of regional abnormalities of neurotransmitter release in various mental disorders and to study the mechanism of action of therapeutic drugs or drugs’ abuse. We examine the quantitative accuracy and reproducibility that can be achieved with high-resolution SPECT of serotonergic neurotransmission. Method: Binding potential (BP) of 123I labeled tracer specific for midbrain SERT was assessed in 20 healthy persons. The effects of scatter, attenuation, partial volume, mis-registration and statistical noise were estimated using phantom and human studies. Results: Without any correction, BP was underestimated by 73%. The partial volume error was the major component in this underestimation whereas the most critical error for the reproducibility was misplacement of region of interest (ROI). Conclusion: The proper ROI registration, the use of the multiple head gamma camera with transmission based scatter correction introduce more relevant results. However, due to the small dimensions of the midbrain SERT structures and poor spatial resolution of SPECT, the improvement without the partial volume correction is not great enough to restore the estimate of BP to that of the true one.
Tissue distribution of radioiodinated FAUC113
Authors: Hocke C. , Löber S. , Hübner H. , Gmeiner P. , Kuwert T. , Prante O.
Published: March 2018

Aim: Disturbances of the D4 receptor subtype have been implicated in the genesis of a broad range of psychiatric disorders. In order to assess the suitability of a radioiodinated analogue of the D4-selective ligand FAUC 113 for tracer studies in vivo, we investigated the in-vivo stability, biodistribution and brain-uptake of 7-131I-FAUC 113 in Sprague-Dawley rats. Methods: Radiolabelling was carried out with high radiochemical yield and specific activity. After intravenous injection, blood and tissue samples, taken at designated time intervals, were collected for analysis. Analyses of metabolites were performed by radiohplc and radio-tlc. For in-vivo evaluation, sagittal cryo-sections of the rat brain were investigated by in-vitro and exvivo autoradiography on a μ-Imager system. Results: 7-131I-FAUC 113 was rapidly cleared from blood. Highest uptake was observed in kidney (0.603±0.047% ID/g, n=4) and liver (0.357±0.070% ID/g, n=4) at 10 min p.i.; 7-131I-FAUC 113 displayed rapid uptake (0.21-0.26% ID/g) and fast clearance in various brain regions consistent with the determined logP-value of 2.36±0.15 (n=4). In-vivo stability of 7-131I-FAUC 113 was confirmed in the frontal cortex (>95%). Ex-vivo autoradiography revealed a frontal cortex-to-cerebellum ratio of 1.57±0.13 at 10 min p.i. (n=6). Coinjection with L-750667 could not suppress any putative specific binding of 7-131I-FAUC 113. In-vitro autoradiography using authentic 7-iodo-FAUC 113 or L-750667 failed to cause significant displacement of the radioligand. Conclusions: Radioiodinated FAUC 113 does not allow imaging of D4 receptors in the rat brain in vivo nor in vitro. Further work should aim at the development of selective dopamine D4 radioligands with improved tracer characteristics, such as receptor affinity and subtype selectivity, specific activity or blood-brainbarrier permeability.