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Biomedical subjects

R C Sengers

Publications and source records attributed to R C Sengers.

At least 145 records · Page 8Linked to original sources
PubMed1976

Effects of parenteral nutrition with lipids on the human liver. An electron-microscopic study.

The ultrastructural findings in the Kupffer cells of a liver biopsy of a 5-week-old baby, who had received repeated i.v. administration of Intralipid (10%) in a dose of 20 ml/kg body weight/day for 12 days, are presented. Heterophagocytosis of free lipid droplets occurs by invagination of the Kupffer cell membrane. Large, solitary or conglomerated fat droplets surrounded by a membrane (lipophagosomes) are present within the hypertrophic and most active Kupffer cells. Small, round or elongated dark bodies (primary lysosomes) are linked with and/or seem to fuse with these lipophagosomes. In addition, smaller phagosomes almost completely surrounded by a dark small rim and a less electron-dense center probably represent (further stages of the lysosomal breakdown. The latter is also expressed by the condensation from the periphery towards the center of the original lipid droplets enclosed within the lysosomes. Angular inclusion bodies, build up of lipid remnants and a granular matrix of medium electron density, constitute far advanced stages of lysosomal digestion and represent the development or transformation of the phagocytosed lipids into lipogenous pigments. On the electron-microscopic level a distinction between lipofuscin granules and ceroid pigment can hardly be made at that stage. These pigment granules can be found in various amounts within the Kupffer cells and can stay there for a long time. This accumulation probably inhibits the clearance capacity of the cellular elements of the hepatic reticuloendothelial system. The hepatocytes and spaces of Disse seem not to be directly involved in the process of fat phagocytosis and breakdown as summarized above.

Ceroid↗
PubMed1976

Cardiomyopathy and short stature associated with mitochondrial and/or lipid storage myopathy of skeletal muscle.

The patient described in this report was an 11-year-old girl with a negligible heart murmur. The length was below 10th percentile. There appeared to be a cardiomyopathy with, at that moment, no signs of obstruction. There were no evident clinical symptoms of myopathy of skeletal muscle. However, electromyography was myopathic. Biochemical studies revealed no abnormalities. In muscle biopsy sections, the Sudan staining reactions revealed the presence of large amounts of sudanophilic droplets, predominantly in type I fibers. Electronmicroscopy demonstrated lipid excess and accumulations of enlarged, mostly rounded mitochondria in a subsarcolemmal location, with closely packed cristae. In many mitochondria a dense osmiophilic material was present in the spaces between the cristal membranes. The patient's parents were first cousins. One of her brothers who died at the age of 4 1/2 years presumably suffered from the same disease. The pattern of inheritance is most probably autosomal recessive.

Adenosine Triphosphatases↗
PubMed1975

Infantile Gaucher's disease: glucocerebrosidase deficiency in peripheral blood leukocytes and cultured fibroblasts.

A patient with infantile Gancher's disease is described. A marked decrease of the activity of beta-glucosidase at the lower pH 4.2 was found in peripheral blood leukocytes, whereas in cultured skin fibroblasts the activity at both pH'S 4.2 and 5.2 was decreased. Examining the leukocytes of the heterozygous parents the activities of beta-glucosidase at both pH's were within the values of controls, but the ratio's of activity of pH 5.2 to that at pH 4.2 were increased lying between the ratio's from the patient and controls.

Cells, Cultured↗
PubMedDate not supplied

Association between the change in nutritional status in response to tube feeding and the occurrence of infections in children with a solid tumor.

In 32 children with a solid tumor, the association between the change in weight for height, in response to 4 weeks of tube feeding during the intensive phase of treatment, and the occurrence of leukopenia, leukopenic infections, and nonleukopenic infections in a period thereafter (4-10 weeks) was studied. Factors possibly influencing the change in weight for height during the first 4 weeks of tube feeding were also assessed. A statistically significant negative correlation (rho = -0.59; p < .001) was found between the change in z-score of weight for height in response to the first 4 weeks of tube feeding, and the occurrence of nonleukopenic infections between 4 and 10 weeks. A reduced occurrence of nonleukopenic infections resulted in a significant reduction of the number of days of infection-related hospital admission (rho = .45; p = .009), which, besides providing advantages for the patient, also had economical benefits. The change in weight for height in response to tube feeding was mainly influenced by the incidence of therapy-induced vomiting (r = -.45; p = .02) and by the amount of energy provided by tube feeding (r = .47; p = .007). Based on these findings, it is recommended that naso-gastric tube feeding be used in children with a solid tumor during the early intensive phase of treatment, and that one should aim for a considerable increase in weight for height during the first 4 weeks of administration, since this has been shown to reduce the number of nonleukopenic infections in a subsequent period. The increase in weight for height may be improved by providing an optimal antiemetic protocol, which will increase energy uptake, and an energy-enriched formula, which will increase energy intake.

Adolescent↗