The Kasabach-Merritt syndrome: severe bleeding disorder caused by celiac arteriography--reversal by heparin treatment.

Studies are presented on a 62-yr-old woman with extreme hepatomegaly due to a giant hemangioma with alterations in the clotting system indicating a consumption coagulopathy. There was a fall of hemoglobin, fibrinogen, antithrombin III, and platelet number after arteriography of the truncus celiacus. Furthermore, there was sustained bleeding in the patient's right thigh caused by puncture of the arteria femoralis. Continuous administration of iv heparin corrected the clotting disorder including a rise in platelets from 95,000/microliters to 148,000/microliters, permitting surgical removement of the hematoma. Celiacography is a useful tool for the diagnosis of hepatic hemangiomata, as well as ultrasound, computed tomography, and magnetic resonance imaging. Being an invasive technique, it requires testing for possible consumption coagulopathy if used in patients with hemangiomatosis. Its application should be restricted to cases in which exact diagnosis cannot be established by other means.

[Correlation of roentgen picture and lung function testing within the scope of silicosis evaluation]. / Korrelation von Röntgenbild und Lungenfunktionsprüfung im Rahmen der Begutachtung der Silikose.

The chest roentgenograms codified according to the 1980 ILO classification and the lung function tests of 100 persons with silicosis have been compared with each other. The results show that, with a higher degree of dissemination and a growing size of nodes, there is a statistically significant tendency to increasing gravity of pathological results of resistance and the arterial blood gas analysis. A correlation between the degree of dissemination and the size of nodes to other lung function parameters (vital capacity, intrathoracic gas volume) cannot be finally established. Furthermore, neither coding as category B nor the employment of the symbols em (emphysema) and tb (tuberculosis) allow conclusions to be drawn about the results of lung function tests. The poor correlation is caused on one hand by the insufficient description of the morphological parameters important for function, due to the restricted classification. On the other hand there is little correlation between chest roentgenogram and lung function, as lung function in anthracosis is essentially influenced by obstructive bronchitis, which has only a loose connection with silicosis.--Lung function must be the central point in the process of forming an opinion. Chest roentgenograms are important for clarifying the causes of pathological changes and allow a simple observation of progress.

Evaluation by electron microscopy of the integrity of iodinated plasmalipoproteins.

Iodination of proteins and lipoproteins is a widely used "in vitro" labelling procedure in metabolic, autoradiographic and various other studies. However, all available iodination techniques have involved the possible damage to the proteins by self-irradiation, oxidizing agents, the alkaline milieu or by the introduction of iodine into the molecular structure itself. To evaluate the integrity of iodinated lipoprotiens, we observed the electron microscopic appearance of normal and iodinated rabbit very low density lipoproteins (VLDL) by negative staining with phosphotungstic acid. Iodination up to a molar iodine/protein ratio of 2.89 did not results in any change of shape, size or aggregating tendency of the particles. No stacks or disk-like particles like those of various hyperlipoproteinemic states were found. We conclude that electron microscopy is a valuable tool in assessing the morphological appearance of lipoprotein iodination, but it should be complemented by other techniques.

Comparative studies of the electroencephalogram and the cerebral oxidative metabolism in patients with liver cirrhosis.

In two groups of patients with liver cirrhosis and normal EEG (Group A) and with pathological EEG (Group B) it was possible to demonstrate a correlation between the severity grade of the EEG changes, the livertypical deviations of serum chemistry and alterations in cerebral oxidative metabolism. The metabolism of the brain showed a reduced oxygen consumption and carbon dioxide output in the patients with pathological EEG changes. All patients showed a raised glucose uptake, an increased lactate release, a raised ammonia uptake and glutamine output. These findings in patients with liver cirrhosis indicate a disturbance of the oxidative energy metabolism of the brain with secondary intensification of glycolysis. Pathological changes in the EEG only appear if the oxygen consumption of the brain is limited (as in the patients of Group B). These EEG changes have a poor prognosis in respect to life expectancy. With consideration of the data from animal experiments and the reported results of cerebral blood flow and oxydative metabolism in patients with liver cirrhosis it might be assumed that liver insufficiency with elevated serum ammonia results in a deranged oxydative cerebral metabolism which might explain hepatic encephalopathy.