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Biomedical subjects

J Fernandes

Publications and source records attributed to J Fernandes.

At least 145 records · Page 8Linked to original sources
PubMed1975

Plasma triglyceride clearing in obese children.

In 13 obese children plasma triglyceride concentrations were found to be significantly elevated, while plasma cholesterol concentrations were normal. In the hypertriglyceridemic obese children, the plasma fractional triglyceride removal, measured by the intravenous fat tolerance test, was significantly reduced. These abnormalities reverted to normal in 8 patients retested after weight loss. Plasma postheparin lipoprotein lipase activity was found to be increased and significantly related to the degree of obesity. As to carbohydrate metabolism, a decreased glucose tolerance and hyperinsulinemia were found. Hyperinsulinemia reverted to normal during dietary restriction, glucose intolerance did not.

Adolescent↗
PubMed1975

Utilization of fat emulsion during total parenteral nutrition in children.

Tolerance for Intralipid fat emulsion during total parenteral nutrition (PN) was studied in 6 children. The Intralipid dose was monitored by the daily determination of plasma Intralipid levels. Fat removal was investigated at the start of and during the PN period by the intravenous fat tolerance test (IVFTT) and by determining the plasma postheparin lipoprotein lipase (LPL) activity. When the plasma Intralipid levels exceeded a value of 100 mg/100 ml, hyper pre-beta lipoproteinaemia, hypertriglyceridaemia, hypercholesterolaemia and hyperphospholipidaemia appeared. During PN most patients showed marked increases of postheparin LPL. Return to normal values occurred after discontinuation of PN. Maximal LPL activities were found to correlate significantly with total daily caloric intake (r=0.95, 0.05 less than p less than 0.01). The Intralipid elimination constant hardly changed during PN, with the exception of patient 6, who showed a marked increase (from 7 to 22%). Conclusions of this study are as follows: First a high caloric intake during PN leads to a marked increase of postheparin LPL activity. Second, by monitoring plasma Intralipid levels at 100 mg/100 ml approximately, it is possible to adjust the Intralipid dose in order to prevent hyperlipaemia and to take maximal benefit from rising fat tolerance. Thirdly the IVFTT appeared to be of little value to estimate the child's fat elimination capacity.

Amino Acids↗
PubMed1975

Prenatal diagnosis of type II glycogenosis (Pompe's disease) using microchemical analyses.

1. In order to reduce the time interval between amniocentesis and prenatal diagnosis of Pompe's disease microchemical techniques were used for assay of acid alpha-1,4-glucosidase activities in cultured amniotic fluid cells. 2. Microtechniques used on homogenates of cultured amniotic fluid cells enabled the waiting period to be reduced to 2-3 weeks. 3. When dissected lyophilized groups of 200-300 cultured cells were analyzed, a prenatal diagnosis was possible at about 10 days after amniocentesis. 4. The acid alpha-1,4-glucosidase activity in the amniotic fluid supernatant is not informative in prenatal diagnosis of Pompe's disease. 5. Conditions of cell cultivation such as length of time in culture were found to influence markedly the acid alpha-1,4-glucosidase activity in cultured amniotic fluid cells. 6. For a reliable prenatal diagnosis of metabolic disorders primary cultures of control amniotic fluid cells should be used and the analytical results from the pregnancy at risk should be compared with primary cultures of control amniotic fluid cells and with those in cultured fibroblasts from heterozygous carriers, and an affected sibling from the particular family.

Amniocentesis↗