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Biomedical subjects

E Granot

Publications and source records attributed to E Granot.

89 records · Page 5Linked to original sources

Core modification of human low-density lipoprotein by artificial triacylglycerol emulsion.

To determine whether an apolipoprotein-free artificial triacylglycerol emulsion can substitute for VLDL in studying cholesterol ester-triacylglycerol exchange processes between triacylglycerol-rich lipoproteins and cholesterol ester-rich lipoproteins, we used Intralipid to modify human plasma LDL. Intralipid was incubated with LDL in the presence of lipoprotein-poor plasma (d greater than 1.21 g/ml) at 37 degrees C. Intralipid served as an acceptor for cholesterol ester and as a donor of triacylglycerol, modifying the low-density lipoproteins so that triacylglycerol became the major core lipid in the particle - the contribution of cholesterol ester to LDL mass decreased from 38% to 18%, while that of triacylglycerol increased from 4.9% to 26%. On lipolysis most added LDL triacylglycerol (59-72%) was hydrolyzed, resulting in a smaller particle than the "native' LDL particle with net loss of cholesterol ester. Incubation of LDL with the original Intralipid emulsion resulted in modified LDL with a high relative weight of phospholipid (27.7%). On removal of excess phospholipid from Intralipid and incubation of the resultant "washed' Intralipid with LDL, the relative weight of phospholipid in modified LDL decreased to 20%, which was similar to that observed after incubation of LDL with VLDL. We demonstrate that artificial triacylglycerol emulsion can indeed substitute for VLDL in neutral lipid exchange processes, and further confirm that transfer of core cholesterol ester and triacylglycerol occurs independently of the apolipoproteins present in triacylglycerol-rich lipoproteins and LDL.

Cholesterol↗

Identification of urinary 3-ethoxy-4-hydroxybenzoic and 3-ethoxy-4-hydroxymandelic acids after dietary intake of ethyl vanillin.

It has been discovered recently that several patients undergoing urinary organic acid profiling excrete high concentrations of 3-ethoxy-4-hydroxybenzoic acid and traces of 3-ethoxy-4-hydroxymandelic acid. These are believed to be the consequences of feeding the patients with synthetic diets flavoured with 3-ethoxy-4-hydroxybenzaldehyde, a vanilla-like artificial flavouring added to improve patient acceptance and taste. The syntheses, mass spectra and gas chromatographic behaviour of these and related compounds are presented.

Adult↗

Incorporation of [14C]glucose into alpha-1,4 bonds of glycogen by leukocytes and fibroblasts of patients with type III glycogen storage disease.

In two patients assay of alpha-1,6-amyloglucosidase activity by incorporation of [14C]glucose into glycogen revealed normal activity in leukocytes, erythrocytes, and fibroblasts, whereas no activity was detected in liver and muscle. No activity in any tissue was found when enzyme activity was assayed by following the release of glucose from a phosphorylase limit dextrin. Labeling of glycogen by incubation with crude tissue homogenates according to the protocol used for the [14C]glucose method and subsequent degradation of the outer portion of the polysaccharide molecule with beta-amylase showed that with tissues from normal controls more than 90% of the label of the glycogen was retained in the limit dextrin. When fibroblasts or leukocytes of the patients served as enzyme source up to 80% of the label was released after incubation with beta-amylase or phosphorylase a. Addition of Tris to the assay inhibited enzyme activity in fibroblast homogenates of the patients and of controls to the same extent and had no effect on the distribution of the label between supernatant and limit dextrin after beta-amylolysis of the labeled glycogen. A pH curve performed with fibroblast preparations from the patients and a normal control did not reveal differences in the effect of changes in pH on [14C]glucose incorporation. We propose that incorporation of [14C]glucose into glycogen by the enzyme present in the patients' cells was into alpha-1,4 linkages in glycogen.

Cells, Cultured↗

Recurrent Reye-like syndrome: possible association with Krebs cycle abnormality.

During a 7-year period, a 15-year-old boy experienced recurrent attacks that clinically and pathologically resembled Reye's syndrome. The attacks were precipitated by prolonged exercise, fasting or infections. An extensive investigation failed to identify a toxic cause or specific metabolic disorder. Low 14CO2 production after in vitro incubation of the patient's liver with 14C-labeled palmitate, citrate and glutamine suggested a defect in the Krebs cycle or the oxidative phosphorylation pathway. This error may be responsible for the recurrent Reye-like syndrome attacks.

Adolescent↗

Giardiasis in childhood: poor clinical and histological correlations.

Ten pediatric patients investigated for chronic diarrhea, chronic weight loss, or failure to thrive were found on intestinal biopsy and/or in a duodenal aspirate to have Giardia lamblia. Serum immunoglobulin levels were normal or elevated in all patients. Three children had increased excretion of fecal fat and three other children had low D-xylose absorption. Jejunal biopsy specimens showed two severe, three moderate, and two mild morphological abnormalities, and three were normal. Except for lactase deficiency, disaccharidase activities correlated poorly with the severity of mucosal damage on biopsy. Steatorrhea was seen only with the more normal biopsies. Immunofluorescent staining of the biopsies for IgG, IgM, IgA, and secretory piece revealed no immune defects. Thus, there was no single malabsorption defect associated with giardiasis, and the specific defects did not necessarily correlate with morphological changes.

Child↗

"Pseudoascites" as a presenting physical sign of celiac disease.

Two children initially referred for evaluation of ascites were diagnosed as suffering from celiac disease. Positive physical signs of ascites were present in the absence of free fluid in the peritioneal cavity. Physical findings of ascites were caused solely by dilated small intestinal loops filled with copious fluid. "Pseudoascites" should be recognized as a physical sign and possible mode of presentation of celiac disease.

Ascites↗

Strongyloidiasis in infancy. Case report and review.

A 15-month-old child with extreme failure to thrive due to intractable diarrhea since the age of 20 days was found to have live Strongyloides larvae in aspirated duodenal juice. Larvae were seen embedded in crypts of jejunal mucosa obtained by peroral biopsy. Following treatment with thiabendazole, a dramatic recovery ensued. The pathophysiology and clinical spectrum of strongyloidiasis is reviewed and the importance of its recognition stressed.

Biopsy↗

Duplication of the gallbladder associated with childhood obstructive biliary disease and biliary cirrhosis.

A 4-yr-old girl presented with recurrent attacks of abdominal pain, vomiting, fever, and jaundice, progressing to biliary cirrhosis. The diagnosis of duplication of the gallbladder was established by transhepatic cholangiography. Clinical recovery and histologic improvement followed removal of the gallbladders. In every case of obstructive biliary disease or biliary cirrhosis in childhood, no effort should be spared in searching for a malformation of the biliary system.

Child, Preschool↗

Plasma triglyceride determines structure-composition in low and high density lipoproteins.

Because the association of hypertriglyceridemia and premature atherosclerosis is not due to the direct effects of the triglyceride molecule itself, we studied the effects of increased plasma triglyceride-rich lipoproteins on the composition and structure of low density lipoprotein (LDL) and high density lipoprotein (HDL). We found profound changes in the core and surface domains of both lipoproteins with increasing triglyceridemia. Core cholesterol esters were progressively depleted and replaced by triglyceride molecules. Highly significant negative correlations were found between cholesterol ester/protein ratios (r = -0.64 for LDL and -0.58 for HDL (p less than 0.001); positive correlations were found for triglyceride/protein ratios (r = 0.62 for LDL and 0.58 for HDL) and for triglyceride/cholesterol ester ratios (r = 0.70 for LDL and 0.83 for HDL) when these variables were assayed as a function of plasma triglyceride concentrations. With severe hypertriglyceridemia, triglyceride/cholesterol ester ratios of more than 1.0 were consistently observed (normal, less than 0.02). This leads to an underestimation of LDL and HDL levels when cholesterol alone is measured. At the surface, LDL and HDL were depleted of phospholipid and free cholesterol, with a relative enrichment of protein. These changes can be explained on the basis of high levels of plasma triglyceride-rich lipoproteins serving as acceptors for cholesterol esters and other constituents from LDL and HDL. Concomitantly, triglycerides are transferred to LDL and HDL. These transfer processes are likely to be mediated by the activity of lipid transfer proteins present in human plasma.

Blood Proteins↗

Functional gastrointestinal obstruction in a child with chronic granulomatous disease.

A child with chronic granulomatous disease developed an antral-pyloric obstruction, followed a month later by a postbulbar duodenal obstruction. At both areas, there was no evidence of an anatomical lesion, and some improvement in the passage of barium was observed following glucagon and metoclopramide administration. Presumably, symptoms have resulted from a functional disturbance of gastrointestinal motility.

Child, Preschool↗