[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.

[Severe acquired protein S deficiency with thrombophlebitis after febrile infection in a 7-year-old girl]. / Schwerer erworbener Protein S-Mangel mit Beinvenenthrombose nach fieberhaftem Infekt bei einem 7jährigen Mdchen.

The importance of the anticoagulant properties of protein S is illustrated by the high incidence of thromboembolic events in individuals with protein S-deficiency. A 7 year old girl was hospitalized with purpura-like bruises and lesions on both thighs after she had suffered from febrile infection. A subsequently developing thrombosis of the left V. femoralis was treated successfully with urokinase. Haemostaseological investigations showed no signs of disseminated intravascular coagulation. However, isolated severe degradation or all protein S-components due to the presence of a circulating autoantibody to protein S was found. After several months the antibody was detectable not any more, activity and antigens of protein S were normal.

[Activity and concentration determinations of coagulation factors in conventional and virus-inactivated coagulation-active plasma]. / Aktivitäts- und Konzentrationsbestimmungen von Gerinnungsfaktoren in konventionellen und virusinaktivierten gerinnungsaktiven Plasmen.

Deep-frozen conventional plasma without viral inactivation [fresh frozen plasma (FFP)] and virus-inactivated plasma units [solvent/detergent (SD) procedure, methylene blue/illumination (MB) procedure] were investigated with regard to their contents of particles and their activities and concentrations, respectively, of blood clotting factors. Particles could be proved in FFP and in MB-treated plasma, but not in SD-treated plasma. Results of the global tests prothrombin time, partial thromboplastin time and thrombin time as well as the activities of blood clotting factors were located in normal range in SD-treated plasma. However, pathological results were partly found in MB-treated plasma. Activities of protein S and alpha 2-antiplasmin were lessened in SD-treated plasma. FFP and MB-treated plasma showed interindividual variations of outcomes (single-donor plasma units) in contrast to SD-treated plasma (pooled donor plasma units).

[Determination of the activity of single coagulation factors in clinically healthy swine and cattle]. / Aktivitätsbestimmungen von Einzelfaktoren der Gerinnung bei klinisch gesunden Schweinen und Rindern.

Special details of animal haemostaseological investigations are insufficiently known as yet. This concerns as well the use of standardized laboratory methods as the differences between species and the normal ranges. Global tests and determinations of activity of single blood clotting factors (II, V, VII - XII, AT III) were carried out on healthy pigs and cattle. Quantitative evaluations were performed by using human or animal reference plasmas. Special attention was paid to the determinations of single blood clotting factors.

Mitochondrial respiratory function as indicator of the ischemic injury of the rat kidney.

The function of mitochondria was considered to be an indicator of ischemic injury of the rat kidney, and the respiration of isolated mitochondria was measured in different metabolic states. The respiratory control index (RCI) was chosen as a parameter of mitochondrial intactness. The results indicate that normothermic in vitro ischemia leads to a rapid decrease of RCI and an almost complete loss of respiratory control within 45 min. Hypothermia during ischemic exposure proved to be an effective protection against mitochondrial injury as indicated by a prolonged coupled respiration and an RCI of about 2, even after 24 h of ischemic kidney storage. The ischemia-induced decrease of RCI was mainly due to a progressive decrease of active-state respiration, whereas the alterations of resting-state respiration were only small. The decline of active-state respiration was paralleled by a decrease of an uncoupled respiration rate. After termination of in vivo ischemia the RCI increased very slowly. Normal RCI values were not obtained until 7 days after onset of blood reflow, which supports the assumption of long-term damage due to ischemia.

Involvement of intramitochondrial adenine nucleotides and inorganic phosphate in oxidative phosphorylation of extramitochondrially added adenosine-5'-diphosphate.

Possible direct, product/substrate mediated interactions between the phosphate carrier and/or the adenine nucleotide translocator and the mitochondrial H+-ATPase were investigated by tracer flux experiments. For this purpose the specific radioactivity of ATP synthesized was measured and compared with those of intra- and extramitochondrial precursors. Two experimental strategies were applied: 1) preloading of mitochondria with [32P]Pi before starting oxidative phosphorylation of external ADP; 2) simultaneous addition of tracer amounts of [3H]ADP and [32P]Pi to phosphorylating mitochondria under steady-state conditions. The observed participation of the intramitochondrial Pi pool in oxidative phosphorylation excludes a directly functional interaction between Pi carrier and H+-ATPase. At 5 degrees C an at least partial compartmentation of intramitochondrial ADP during oxidative phosphorylation was found, whereas at 25 degrees C a complete equilibrium of intra- and extramitochondrial [32P]Pi and [3H]ADP could be observed, excluding a functionally important compartmentation of these species in the intramitochondrial space under physiological conditions.