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Barbara Radomyska

Publications and source records attributed to Barbara Radomyska.

3 recordsLinked to original sources
PubMed2003

[Pregnancy and inborn errors of metabolism].

Increasing number of patients with inborn errors of metabolism (IEM) are now reaching adulthood and are in position to reproduce. Because of the rarity of individual disorders our knowledge of risks factors associated with pregnancy is limited. Obstetrics problems in IEM can be divided into two categories: pregnancy effects on maternal metabolic disorders and relation between mother and fetus related to who of them is affected. Detrimental effects upon the fetus may be directly caused by maternal disease, as it occurs in PKU, or indirectly by maternal supplementation with harmful substrate, as occurs in galactosemia. Less commonly, fetal inborn error of metabolism may affect the mother's health. Pregnancies in which the fetus had long-chain hydroxyacyl-CoA dehydrogenase deficiency have been complicated by life-threatening HELLP syndrome (haemolysis, elevated liver enzymes and low platelets) or AFL (acute fatty liver of pregnancy) during third trimester. The management of labor and the postpartum period (for women and newborns) has to be carefully planned to avoid significant metabolic decompensation.

Female↗
PubMedDate not supplied

[Vitamin E status in hypercholesterolemic children].

The prime role of oxidized low-density lipoprotein in the pathogenesis of atherosclerosis is almost universally accepted. Fat soluble antioxidant vitamin E associated with lipoproteins, appears to have antiatheroma properties. In the presented studies concentration of vitamin E and the relationship between tocopherol and lipids were studied in blood of hypercholesterolemic children. Level of vitamin E was determined by high-performance liquid chromatography (HPLC) method. Compared with normocholesterolemic children, hypercholesterolemic patients had a significantly lower red blood cell vitamin E content (2.55 +/- 0.19 micromol/l vs 3.15+/- 0.33 micromol/l; p<0.005) in spite of their higher plasma vitamin E concentration (27.9 +/- 8.3 micromol/l vs 21.01 +/- 3.6 micromol/l; pl;0.001). In the group of patient tocopherol-to-total cholesterol and tocopherol-to-lipids ratio was statistically lower compared to those in the control group. In hypecholesterolemic children vitamin E positively correlated with total cholesterol (r=0.43; p<0.02), LDL-C (low-density lipoprotein cholesterol) (r=0.42; p<0.02) and lipids (triglycerides + total cholesterol) (r=0.45; p<0.02). This study demonstrates that total plasma vitamin E concentration is not a suitable predictor of cell vitamin E status. Our results suggested that the tocopherol of erythrocytes and vitamin E to lipids ratio in plasma, could be more meaningful indicators to evaluate the vitamin E status in hypercholesterolemic children.

Adolescent↗