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Biomedical subjects

W Mlekusch

Publications and source records attributed to W Mlekusch.

7 recordsLinked to original sources

Multiple deficiency of mucopolysaccharide sulfatases in mucosulfatidosis.

Fibroblasts of four patients affected with mucosulfatidosis (multiple sulfatase deficiency, Austin variant of metachromatic leukodystrophy) were assayed for activities of the five sulfatases known to degrade mucopolysaccharides. These were iduronide 2-sulfate sulfatase, sulfamidase, N-acetyl-galactosamine 6-sulfate sulfatase, arylsulfatase B (N-acetylgalactosamine 4-sulfate sulfatase), and N-acetylglucosamine 6-sulfate sulfatase. The activities of these five sulfatases were severely depressed, thus confirming the known deficiency of arylsulfatase B and the absence of the Hunter and Sanfilippo III A corrective factors that have iduronide 2-sulfate sulfatase and sulfamidase activity, respectively. Together with earlier reports of the deficiencies of arylsulfatases A and C, cholesteryl sulfatase, and dehydroepiandrosterone sulfatae, mucosulfatidosis is now characterized by the deficiency of nine different sulfatases.

Acetylglucosamine

Steatorrhea, a simple and rapid method of diagnosis.

A simple and rapid method for the diagnosis of steatorrhea is described. The method is based on the assessment of the difference of specific gravity and lipophilic property between a normal stool and that in steatorrhea, using a benzene-NaBr system. The stool samples are dried, pulverized, and then investigated. Steatorrhea is defined either as a fat or more in a 24-hour sample. Stool samples of 71 infants and children were examined. Among these were 23 healthy subjects, 10 with upper respiratory tract infections, and 6 with pneumonia. A further 31 children had celiac disease, 9 of them were on a gluten-free diet, and one child had cystic fibrosis. The procedure described renders single stool samples suitable for the assessment of steatorrhea. As a screening test the method proved to be rapid and accurate in the diagnosis of steatorrhea.

Adolescent

Time function of corticosteroid levels in the blood plasma of rats under the influence of 222Rn inhalation.

The time function of cortocosteroid level in plasma of rats under two different 222Rn concentrations was investigated. Both curves show a maximum after 8h. Whereas the higher activity of 222Rn produces a second maximum after 5 days, the lower activity reaches its second maximum not before 9 days. From both time functions, a two-step mechanism in the intracellular control can be concluded.

Adrenal Cortex Hormones

The effect of hunger on free fatty acid and corticosterone plasma levels in rats.

The time function of free fatty acids and of corticosterone in plasma in fed and fasted rats was investigated. Also a study of the influence of hypoxia on the concentration of plasma free fatty acids was carried out. Whereas fasting does not seem to stimulate lipolysis markedly before 12 h, 2 h of hypoxia elevate the free fatty acid level to its maximum. In the fasted condition, all values are significantly higher than corticosterone in the fed animal.

Animals