[Hereditary factor V deficiency and factor V inhibitor without bleeding. Rare causes of pathological screening tests of coagulation]. / Hereditärer Faktor-V-Mangel und Faktor-V-Inhibitor ohne Blutungsneigung. Seltene Ursachen pathologischer Globaltests.

Isolated reduction in factor V activity either occur in form of a hereditary deficiency of factor V as an acquired inhibitor against factor V. Diagnosis can not be made by bleeding alone because in both cases it can occur or not occur. Two patients were investigated showing pathological screening tests of coagulation without bleeding. A hereditary and an acquired deficiency of factor V were proved.

[Modified platelet aggregation test in patients on ASA and/or clopidogrel]. / Kontrolle der Thrombozytenaggregations-hemmung unter Therapie mit Azetylsalizylsäure und/oder Clopidogrel mit einem modifizierten Thrombozytenaggregationstest.

Therapy with acetylsalicylic acid (ASA) and/or clopidogrel is used to achieve prophylactic inhibition of platelet aggregation in patients with arterial thrombosis. We examined if aggregometry can be used to see the effect of antiplatelet drugs (ASA 30, 50, 100, 300 mg/d, clopidogrel 75 mg/d or ASA 100 + clopidogrel 75 mg/d). A modified platelet aggregation test was used to investigate maximum aggregation in response to ADP, collagen, adrenalin and arachidonic acid. Reference values were established based on healthy individuals. We devised a simple scoring system for detection of inadequate platelet inhibition. Compared with the control group, we detected a significant delay of maximum aggregation in response to all agonists in patients on ASA and combination therapy ASA + clopidogrel. Patients on clopidogrel alone were found to have prolonged aggregation when induced with ADP, collagen and arachidonic acid. The failure rate to achieve adequate platelet inhibition on 100 mg/d ASA, 75 mg/d clopidogrel or combination therapy was 27%, 26% and 7%, respectively. Our results demonstrate that platelet inhibition in aggregometry is inadequate in many patients with arterial thrombosis.

[Storage stability of 14 plasma proteins in ACD-AG banked blood]. / Untersuchungen zur Lagerungsstabilität von 14 Plasmaproteinen in ACD-AG-Blutkonserven.

In summarizing the results it can be stated that, when stored, the proteins haemopexin, alpha 1-antichymotrypsin, alpha 1-antitrypsin, which are rich of neuraminic acid, and IgA are predominantly characterized by a diminution of electrophoretic mobility and thus of concentration in electro-immunodiffusion, with the neuraminic acid content decreasing at the same time. These changes are probably caused by bacteria and/or virus neuraminidase. The decrease of pre-albumin concentration to be observed in radial-immunodiffusion and electro-immunodiffusion is caused by other mechanisms because pre-albumin contains no neuraminic acid. In accordance with these findings the most important changes of single proteins which can be measured by our equipment concern the percentage of non-proteins, whereas the antigen binding places will remain relatively stable during storage (ep. findings of radial immunodiffusion).

[Protein analytic studies in children with mastoiditis]. / Eiweissanalytische Untersuchungen bei Kindern mit Mastoiditis.

Protein analysing examinations were carried through in children--fit to be compared to their age--suffering from inflammable ear diseases (acute middle ear inflammation, mastoiditis) and those who were healthy as to their ears. The obtained values statistically analysed resulted in significant differences as to the acute phase and those of closely associated proteins in the sense that haptoglobin, C3c and C1-inactivators in patients with a mastoiditis and partially in those who suffered from an acute middle ear inflammation were increased but prealbumin and transferrin in the same group were decreased. Subsequently the correlative connections of the parameters with singled-out clinical findings in the mastoiditis-group were examined. A factor of the acute phase, a factor of the subacute phase and the immunoglobulin factor could have been marked off, whereby the two first-mentioned are significant for mastoiditis, a fact that will help to facilitate the decision on further therapeutical practice. Presently there are no specific proteins for diagnostics of 'mastoiditis', so that to clinical expertness and experience of the therapeutist will come up the most significant role for the ill child.

[A simple and fast enzyme immunoassay for the quantitative determination of C-reactive protein]. / Ein einfacher und schneller Enzymimmunoassay zur quantitativen Bestimmung des C-reaktiven Proteins.

The principle is based on the competitive binding of the enzyme-labelled CRP (enzyme: alkaline phosphatase) and the serum CRP to solid phase-bound antibodies. The detection limit was 10 micrograms/l. The precision in the series in the border-line "normal" to "pathological" (5 mg/l) was 2.9%; the precision from day to day at the concentration of 5 mg/l was 4.5%. Only 2 h are required for the whole test.

[Polybrene thrombin time--a simple new method for monitoring a fibrinolytic therapy]. / Die Polybrenthrombinzeit--eine neue einfache Methode zur Uberwachung einer fibrinolytischen Therapie.

The thrombin time is essential to control the fibrinolytic therapy with streptokinase. But it is not efficient to use the thrombin time to estimate the fibrinolytic activity if heparin is applied simultaneously. After addition of heparin antidote polybrene the results are comparable with the reptilase time (so-called polybrene thrombin time). In the case of a fibrinolytic therapy it is recommended to use the thrombin time according to AB-D.L. (heparin-sensitive method) and the polybrene thrombin time (heparin-insensitive method).

[Pre- and postpartum hemostasis in type II Willebrand-Jürgens syndrome]. / Prä- und postpartale Hämostase beim von Willebrand-Jürgens-Syndrom Typ II.

In a female patient with a von Willebrand's disease type II (ristocetin cofactor less than 20%) shortly before and after birth of a son the factor VIII-related antigen and the ristocetin cofactor showed considerable increases, which some weeks later again decreased to the original values. Despite additional thrombocytopenia only on the 4th day post partum an easily controllable uterine haemorrhage was to be established. The risk of haemorrhage in von Willebrand's disease during pregnancy, birth and puerperium seems to be insignificant; nevertheless on the basis of the heterogeneity of this disease a peripartal coagulation-analytic control is regarded necessary.

[Hereditary dysprothrombinemia with a mild bleeding tendency (prothrombin Magdeburg)]. / Hereditäre Dysprothrombinämie mit geringer Blutungsneigung (Prothrombin Magdeburg).

Seven members of one family over three generations were found to have a characteristic reduction to about 0.5 of the ratio between factor II clotting activity and factor II concentration, which was not seen when measuring prothrombin after activation with staphylocoagulase. In addition to the "normal" prothrombin, two abnormal prothrombins, with higher molecular weights and lower isoelectric points, were found by SDS-polyacrylamide gel electrophoresis. This is an autosomal hereditary dysprothrombinemia, the affected persons being heterozygotes. Five of the seven persons had a slightly increased bleeding tendency which manifested itself especially in more marked or prolonged posttraumatic and postoperative bleedings.

[Thrombogenicity of prothrombin complex concentrates]. / Zur Thrombogenität von Prothrombinkomplexkonzentraten.

PCC's were isolated either by adsorption to DEAE-Sephadex A-50 (particle size 40-120 microns; Pharmacia Uppsala) or by adsorption to Molselect DEAE-50 (particle size 100-320 microns; Reanal Budapest) from human citrated plasma after separation of factor VIII by cryoprecipitation. The two products obtained (without heparin) were both compared by in vitro (NAPTT, TGt50) and in vivo (venous stasis) model in rats acc. to WESSLER) tests. The agent prepared by adsorption to DEAE-Sephadex A-50 showed a significantly higher thrombogenicity than that prepared by Molselect DEAE-50. ED50 values for thrombus formation to amounted 15 Factor IX U/kg for the first product and to 210 factor IX U/kg for the second.

Fibrin monomer complexes (SFMC) after substitution of antithrombin III (AT III) in consumption coagulopathy.

The report refers to substitution therapy of 33 patients who suffered consumption coagulopathy. Various blood coagulation and fibrinolytic variables were measured. After successful AT III donation to patients suffering DIC, soluble fibrin monomer complexes (SFMC) disappeared within 0.5-12 hours. If AT III decreases SFMC proves positive again. In addition to analysis of AT III we recommend to analyse SFMC to detect thrombin induced consumption reaction (DIC). Furthermore we found the fibrin split product D-dimer was a particularly sensitive indicator of DIC in case of hyperfibrinolysis (D-dimer was analysed in six patients). The reactions of fibronectin and SFMC proved inversely proportional.