Biomedical subjects
Publications and source records attributed to G Hübner.
Report on a sporadic case with congenital slowly progressive neuromuscular disease. Light microscopic, ultrastructural and histochemical changes of muscle biopsy reveal the characteristics of central core disease with "structured cores" in type II-fibers.
The effect of body weight on the deiodination of L-di-iodotyrosine and L-thyroxin in the liver and kidney was studied in vitro in male Wistar rats of same age, but differing in weight (light and heavy, respectively), which were fed a standard diet. In the livers of the heavy animals, the deiodination of L-di-iodotyrosine and L-thyroxin was significantly lower than in those of the light animals. As to the kidneys, no such difference was observed. From a comparison with literature data it is concluded that the reduction in deiodination in the livers of the heavy animals might be due to a reduction in thyroid hormone metabolism.
The sera from light and heavy-weight rats varying in age and belonging to different groups on, respectively, pellets or diets containing 3% or 50% of fat were analysed for pyruvate, lactate, aceto-acetate, beta-hydroxybutyrate, free fatty acids, triglycerides and total cholesterol. There were no significant differences in these parameters between light and heavy-weight animals of the same diet group. The total cholesterol content increased with age in the rats which were fed pellets. These findings are compared with previous results of the authors and discussed in the light of recent literature.
An intracerebral metastatic teratoma in a 10-year-old girl is described. This is only the third case that has been reported in literature. Autopsy revealed a cystic tumour measuring 4 cm in diameter in the region of the optic chiasm. Microscopic examination showed that it contained all 3 germinal layers. It was classified in accordance with the criteria of Collins and Pugh as a malignant teratoma intermediate A (M.T.I.A.). Metastases had developed upstream and downstream of the CSF flow in the rostral segment of the pons, the medulla oblongata and the cerebellum as well as in the region of the posterior commissure above the lamina quadrigemina and the pineal body. In addition, there were also leptomeningeal metastases in the region of the left Sylvian furrow. The different opinions of various authors regarding the malignant potency of intracranial teratomas are raised and discussed.
In hyperthyroxinemic and hypothyreotic rats the lipoprotein level in serum was investigated using agarosegel-electrophoresis and changes in serum level of cholesterol, triglycerides, free fatty acids and glycerol were determined. In hyperthyroxinemic animals the beta-lipoproteins were found in the same level as the control animals, while the prae-beta-fraction was significantly elevated and the alpha-lipoproteins lowered. The cholesterol was significantly reduced, the triglycerides and the glycerol significantly increased. The free fatty acids were slightly elevated. In hypothyreotic animals the beta-and prae-beta-fraction of lipoproteins was significantly elevated. The alpha-lipoproteins were found diminished. Cholesterol and triglyceride values were also significantly increased. The levels of free fatty acids and glycerol did not differ in both groups of animals.
Report of a 20 months old child showing a mild form of glycogenosis type II (POMPE, 1932) with preferential involvement of skeletal muscle. First muscle biopsy reveals, on light microscopic examination, only a mild vacuolar myopathy. By PAS-staining pathologic glycogen storage can be shown. Glycogenosis type II is proved by ultrastructural and biochemical studies of muscle tissue obtained by a second biopsy. Consequently in all cases of a floppy infant syndrome with myopathic features it is necessary to obtain tissue for biochemical and ultrastructural analysis and to carry out these techniques if by light microscopic examination a vacuolar myopathy with increase of glycogen is found.
Tumour cells in Kaposi's sarcoma with visceral involvement were studied by fine structural analysis. In the cytoplasm of these cells rod-shaped tubulated bodies resembling Weibel-Palade granules could be demonstrated. This finding and the occurrence of a basement membrane around the tumour cells characterize Kaposi cells as endothelial cells of blood vessels which may originate from undifferentiated vasoformative cells of the adventitia.
Within the broad range of activities in pharmaceutical research, the technological development and analytical evaluation of a new drug formulation represent only one step. The pharmaceutical development of three oral formulations of a combination product containing N1-(4,5-dimethyl-2-oxazolyl)-sulfanilamide (sulfamoxole) and 2,4-diamino-5-(3,4,5-trimethoxy-benzyl)-pyrimidine (trimethoprim) in a 5:1 ratio (investigational drug CN 3123; Nevin; Supristol), which was based on the physico-chemical characteristics of the 2 active substances, is presented. The various chemical and physical tests conducted with the drug formulations are described. The pharmaceutical or in-vitro availabilities (dissolution rate) of the active ingredients were determined by way of release-rate profiles, both of the active ingredients alone and in their final formulations. Also presented are the results of plasma level determinations following oral administration of film-coated tablets and a suspension. Finally, preliminary results of extensive stability tests with the three drug formulations are discussed.
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The pyruvate and lactate levels in blood of patients with thyroid diseases were investigated and also the lactate/pyruvate ratio. The levels of pyruvate were significantly elevated in hyperthyreotic patients in comparison with the euthyreotic control group; in hypothyreotic patients the levels were lowered. Only in hypothyreotic patients the lactate level was slightly increased. In all the three groups the lactate/pyruvate ratio showed highly significant differences, in hyperthyreosis decreased, in hypothyreosis increased in comparison with normal subjects. In patients suffering from hyperthyreosis as well as in cases with euthyreotic goitre we found similar pyruvate levels and lactate/pyruvate ratios as in the hyperthyreotic cases. It seems likely to be an inexpected effect of the treatment with pure triiodothyronine or triiodothyronine/thyroxine in combination.
A report is given on a case of proliferative myositis in a 75 year old woman. By fine structural analysis it can be shown, that the characteristic giant cells in proliferative myositis are mesenchymal cells with an intensive protein metabolism. They can be compared to fibroblasts; for a myogenic origin of these cells we found noevidence. Furthermore, various stages in the development and function of the proliferating cells were observed, by which the course of the disease can be expalined.
The unusual status of the cerebellar astrocytoma is based upon clinical and histological evidence. "Bergstrand Tumour" is proposed as a new name for it. A new concept of its origin is put forward, namely that it is derived from the "tanycytes" (Horstmann) of the periventricular structures.
The capacity for recovery of the normothermic left ventricular myocardium from a regional complete ischemia (RCI) was investigated using changes in the myocardial metabolic status (ATP, ADP, AMP, creatine phosphate (CrP), free creatine, glycogen, glucose, lactate) and alterations of the morphology as parameters. In dogs, an area of the anterior wall of the left ventricular myocardium was temporarily deprived completely of its blood supply by 5--7 overlapping ligatures extending into the heart cavity. The metabolites of the adenylic acid-CrP system returned to normal tissue levels after 30 and 60 min of RCI within 14 and 35 days of recovery, respectively; restoration averaged 82% after 100 min, 74% after 140 min, and 38% after 180 min of RCI after 5 weeks of recovery. At the same time glycogen amounted to 163% after 100 min, 114% min, and 65% after 180 min of RCI. The biochemical data correlated well with the structural changes in the affected myocardium, especially with the amount of de- and regenerating heart muscle cells. These obviously were functionally defect and were not comparable with normal structured and functioning heart muscle cells.
In rats hypothyroidized with methylthiouracil (MTU), methimazol (MMI), or radiothyroidectomy, the extent of deiodination for L-diiodotyrosine (L-DIT) and L-thyroxine (L-T4) was investigated in homogenate supernatants of liver and kidney. Deiodination in liver and kidney for DIT is twice as high as for T4, but the kidney allows only 25% of the liver deiodination activity both for DIT and T4. In the livers of all hypothyroid animals, iodide splitting both from DIT and T4 is highly significantly reduced by one-half compared with controls. In the kidney of all hypothyroid animals, the DIT deiodination is highly significantly lowered in comparison with controls; the T4 deiodination is significantly reduced only in animals treated with MTU and MMI, and is not significantly enhanced in radiothyroidectomized rats. Thus, there is no difference between MTU and MMI in the extent of deiodination for DIT and T4 in the homogenate supernatants of rat liver or kidney.
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In serum of hyperthyroxinemic and hypothyreotic rats the activities of MDH and LDH were investigated. In both groups the level of MDH was significantly elevated in comparison with control animals. The activity of LDH did not show any significant alterations.
The L-DIT and L-T4 deiodinating activity in supernatants from liver or kidney homogenates of normal rats or rats loaded with T4, T3 or TSH, was investigated. Deiodination of L-DIT occurs in liver supernatants twice as much as in liver, referring to mg of protein of g of tissue, while the deiodination of L-T4 seems rather equal. The over all rate of deiodination, however, reaches in liver for L-DIT the threefold, for L-T4 the fivefold value, as compared with kidney. Short periods of loading do not alter deiodination of L-DIT in liver or kidney at all; but lead to significant elevation of L-T4-deiodination in liver tissue. Higher doses or longer periods of loading cause a significant rise of L-DIT-deiodination in liver and kidney, while the L-T4-deiodination in liver is significantly decreased and in kidney significantly elevated.