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Biomedical subjects

E Robberecht

Publications and source records attributed to E Robberecht.

At least 19 recordsLinked to original sources

Several mechanisms contribute to the abnormal fatty acid composition of serum phospholipids and cholesterol esters in cholestatic children with extrahepatic biliary atresia.

The fatty acid compositions of serum phospholipids and cholesterol esters and direct bilirubinemia were determined in 11 children with cholestasis due to extrahepatic biliary atresia. The levels of the different fatty acids in these lipid classes were compared with those of 22 appropriate controls and correlations with conjugated bilirubinemia were calculated. Significant differences were found in the levels of several fatty acids in these lipid classes, some of which were related to conjugated bilirubinemia. Relationships between fatty acids in phospholipids and cholesterol esters which exist in the control group were either absent or different in the patient group. The results found are compatible with the concept that malabsorption, overflow in blood of phospholipids, which are excreted in bile in healthy individuals, and liver disease per se contribute to the deviating fatty acid compositions. They suggest that administration in the diet may be required of preformed long chain polyunsaturated fatty acids in an easily absorbable form.

Biliary Atresia

Effect of administration of gamma-linolenic acid on the fatty acid composition of serum phospholipids and cholesteryl esters in patients with cystic fibrosis.

This study examined the effects of supplementing patients with cystic fibrosis daily for 4 weeks with 1,500 mg borage oil, containing 330 mg gamma-linolenic acid, on the fatty acid composition of serum phospholipids (PL) and cholesteryl esters (CE). Vital capacity and forced expiratory volume in 1 s were also measured before and after treatment. In serum PL the content of arachidonic acid (AA) increased and that of palmitic acid decreased significantly after borage oil administration. In serum CE dihomogamma-linolenic acid increased whereas docosahexaenoic acid decreased significantly. In this lipid class, AA and the sum of AA plus its precursor, dihomogamma-linolenic acid, increased significantly with the dose fed. A positive correlation was found between change in vital capacity and change in linoleic acid content of serum CE and AA content of serum PL.

Adolescent

Increase of long chain omega-3 fatty acids in the major serum lipid classes of patients with cystic fibrosis.

We studied the effects of supplementing patients with cystic fibrosis for 1 month with 3 g/day of a dietary supplement, containing 911 mg omega 3 fatty acids, on the fatty acid composition of serum cholesterol esters, free fatty acids, phospholipids and triglycerides. At the end of the supplementation period, the content of total omega 3 fatty acids was increased significantly in all lipid classes. The effect on individual omega 3 fatty acids depended on the fatty acid and lipid class studied, increased with the dose fed and decreased with the weight of the patient. One month after the end of the supplementation period, fatty acid levels had returned to pretreatment values. These results demonstrate that enrichment of serum lipid classes with omega 3 fatty acids is feasible in CF patients by dietary supplementation.

Adolescent

IgA isotyping of antigliadin antibodies. A possible clue for a less invasive diagnosis of coeliac disease.

An enzyme-linked immunosorbent assay was used to measure serum antigliadin antibodies (AGA) of IgG and IgA classes. The assay was modified to measure IgA1 and IgA2 subclasses with monoclonal anti-IgA subclass antibodies. Serum IgG- and IgA-AGA levels were elevated in patients with coeliac disease (CD) but an overlap was seen with control sera. IgA-AGA isotyping using monoclonal anti-human IgA1 and IgA2 antibodies increased the sensitivity and specificity of the assay to almost 100%. All patients with active untreated CD and none of the control groups had elevated IgA1-AGA and IgA2-AGA. In order to measure the relative distribution of IgA1-AGA versus IgA2-AGA an IgA1/IgA2 ratio was calculated. In patients with active untreated CD a ratio of 2.8 was found, declining to 2.2 during treatment. A gluten challenge increased the ratio to 3.4. These findings suggest that IgA1-AGA subclass measurements are a useful screening test before small bowel biopsies are performed. This method can also be used to assess the results of a gluten free diet.

Antibodies

Ultrasonic evaluation of gastric clearing in young infants.

Gastric emptying was determined using ultrasound in young infants. Gastric volume was calculated from anteroposterior, craniocaudal and laterolateral antral diameters. There was a linear decline of the logarithm of a gastric filling index over time in normal subjects. The mean clearing angle in normal subjects was 52 degrees (range 45-59). Examples of significantly different clearing rates could be demonstrated in a variety of clinical conditions. In several subjects gastric emptying rate was not exponential. Ultrasound seems very promising for the study of gastric emptying, especially in young children.

Evaluation Studies as Topic

Hepatitis B related childhood hepatocellular carcinoma. Childhood hepatic malignancies.

The case of hepatocellular carcinoma (HCC) with foci of hepatoblastoma in a 7-year-old boy, the son of a hepatitis B surface antigen (HBsAg) carrier mother, is described. Twelve other malignant liver tumors in children were tumors in children were also reviewed for HBsAg and hepatitis B core antigen (HBcAg). Both were negative in all (nine) hepatoblastomas. One of three HCC demonstrated positivity for HBsAg. These cases illustrate the importance of hepatitis B virus infection in early childhood and stress the need for careful screening in pregnant women, irrespective of ethnic backgrounds.

Carcinoma, Hepatocellular

Effect of supplementing medium chain triglycerides with linoleic acid-rich monoglycerides on severely disturbed serum lipid fatty acid patterns in patients with cystic fibrosis.

7 patients with cystic fibrosis, having serum lipids with fatty acid compositions (FACs) skewed in the direction of essential fatty acid deficiency, were given a mixture of medium chain triglycerides (MCTs) with linoleic acid-rich monoglycerides (LAMs) as food fat (about 1-1.25 g/kg body weight/day). Blood was sampled before and after a short period (4-11 days) of experimental fat feeding. The FACs of the serum triglycerides (TGs), cholesterol esters (CEs) and phospholipids (PLs) were determined before and after dietary treatment. Dietary treatment resulted in a significant increase of the previously reduced fraction of linoleic acid in all lipid classes. In TGs, the increase in the fraction of linoleic acid was accompanied by a reduction of the previously increased fraction of palmitoleic acid to normal values, by a reduction in the fraction of oleic acid to subnormal values and by an increase in the fraction of stearic acid which, however, remained normal. In PLs, the increase in the fraction of linoleic acid was accompanied by an increase in the fraction of dihomogammalinoleic acid which remained normal, however. In CEs, concomitant with the increase of the previously reduced linoleic acid fraction, there was a reduction of previously elevated palmitoleic and oleic fractions, the latter to a normal value. Thus, supplementing a MCT-containing diet with LAMs in cystic fibrosis patients results in a considerable amelioration of the previously disturbed FACs of the major serum lipid classes.

Adolescent