Biomedical subjects
B Koletzko
Publications and source records attributed to B Koletzko.
[Supply, metabolism and biological effects of trans-isomeric fatty acids in infants].
Consumption of trans-fatty acids increased markedly during this century due to the widespread use of partially hydrogenated fats. A sensitive analytical method was developed which enables the precise determination of 7 trans-isomers in small sample volumes. With this method we documented the materno-fetal transfer of trans-fatty acids across the human placenta. The content in human milk depends on maternal diet and is lower in Germany than in the Sudan. The distribution in fore- and hind-milk, in milk fat fractions and within the triglyceride molecule was determined. The content of trans-fatty acids is lower in commercial and home-made infant formulae than in human milk, although there is a certain batch to batch variation in formulae. Infants absorb dietary trans-isomers and incorporate them into endogenous lipids, subcutaneous tissue and cell membranes. Trans-fatty acids in plasma lipids are significantly higher in infants fed human milk than in those fed formulae. African children have a lower exposure than Germans. The relative contribution of trans-octadecenoic acid is lower in plasma sterol esters than in triglycerides and phospholipids, pointing to a negative selectivity of plasmatic cholesterol esterification with this fatty acid. Thus, a high supply of trans-octadecenoic acid might have negative effects on the cholesterol levels. In premature infants we found an inverse correlation between trans-fatty acid exposure and birthweight, thus interference with intrauterine growth appears possible. A possible causative factor could be impaired biosynthesis of long-chain polyunsaturated fatty acids by trans-isomers, for which we found strong indications. The results of our investigations the question whether the consumption of trans-fatty acids in pregnant and lactating women and in infants is nutritionally safe.
Fatty acid composition of mature human milk in Nigeria.
The fatty acid composition of mature human milk from 10 rural Nigerian women was analyzed by high-resolution capillary gas-liquid chromatography and compared to previously determined results on mature human milk from 15 German mothers. Human milk of the Nigerian group contains significantly higher proportions of saturated fatty acids (median 54.07 vs. 42.76% wt/wt). The difference is primarily caused by high values for lauric (C12:0, 8.34%) and myristic acids (C14:0, 9.57%), but not of medium chain fatty acids (C8:0, C10:0), presumably due to increased de novo fatty acid synthesis in the African women consuming a high carbohydrate and low-fat diet. Markedly lower values of oleic and total cis-monounsaturated (22.82 vs. 37.98%) as well as trans-isomeric fatty acids (1.20 vs. 4.40%) in Nigerian milk appear to result from low dietary intakes of animal and partially hydrogenated fats, respectively. Although percentage contribution of linoleic acid (18:2n-6) is similar, arachidonic acid (C20:4n-6) and total n-6 long-chain polyunsaturates with 20 and 22 carbons (n-6 LCP) are higher in the African samples. N-6 LCP secretion with human milk lipids is not correlated to the precursor linoleic acid and seems not to depend on maternal dietary intake of preformed dietary LCP with animal fats. N-3 LCP are very high in milk of the Nigerian women who obtain a large portion of dietary lipids from sea fish, but even then docosaehexanoic (C22:6n-3) and not eicosapentaenoic (C20: 5n-3) is the predominant n-3 LCP in milk.(ABSTRACT TRUNCATED AT 250 WORDS)
Arachidonic acid and early human growth: is there a relation?
Growth failure is a classical sign of essential fatty acid deficiency. We investigated whether birth weight correlates with the postnatal essential fatty acid status in a group of 29 premature infants. A significant and positive correlation between body weight and plasma triglyceride content of arachidonic acid (20:4n-6) (r = 0.47, p = 0.01) and total omega-6 long-chain polyunsaturated fatty acids (r = 0.49, p less than 0.01) was found. In contrast, there was no positive relation to linoleic acid (18:2n-6) or any omega-3 fatty acid but a significant inverse correlation to alpha-linolenic acid (18:3n-3). We propose that during early life arachidonic acid may have a growth-promoting effect which could be related to its role as an eicosanoid precursor or to its structural function in membrane lipids.
Nutritional and dietetic aspects of food allergy and food intolerance in childhood.
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[Clinical findings in newborn infants of mothers with kidney transplants].
Renal transplant patients who become pregnant require continued immunosuppression. Little is known on the risk of infants born under these conditions. We observed 6 neonates of renal transplant patients, of whom 4 were premature and 3 small for gestational age (SGA). There were no congenital malformations. Transient thrombopenia in 1 preterm baby probably resulted from maternal immunosuppression. Further 232 cases were published since 1980. Among the total of 238 patients, including our observations, rates for prematurity (49%) and SGA-infants (29%) were high, 6% had congenital malformations. Maternal immunosuppression with Cyclosporin A, as compared to Azathioprine, seems to carry a higher risk of prematurity (66 vs. 43%) and SGA-babies (56 vs. 19%). On the contrary, Azathioprine might be more muta- and teratogenic. At present, no data are available on the long-term outcome of these children.
Antibiotic therapy for improvement of metabolic control in methylmalonic aciduria.
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Cis- and trans-isomeric fatty acids in plasma lipids of newborn infants and their mothers.
The proportionate fatty acid composition of plasma lipids was determined by high-resolution gas-liquid chromatography in 30 pairs of mothers and their term infants at the time of birth. Trans-fatty acids were found at similar percentage levels in maternal and infantile plasma, evidence for the first time of their placental permeability. In umbilical plasma, linoleic and alpha-linolenic acids contributed markedly smaller portions to total fatty acids, in contrast to clearly higher proportions of their long-chain polyunsaturated metabolites (LCP). Significantly larger percentage values in cord than in maternal plasma were found for those LCP that are structural components of brain lipids, which may reflect a discriminating placental transport mechanism for certain physiologically important LCP.
Effects of dietary long-chain polyunsaturated fatty acids on the essential fatty acid status of premature infants.
The effect of different diets on the percentage content of long-chain polyunsaturated fatty acids (LCP; metabolites of linoleic and alpha-linolenic acids) in plasma lipids was studied in 29 premature infants on days 4 and 21 of life. Eleven infants were fed human milk which supplies LCP (1.7% of the fatty acids), 10 a commercially available milk formula without LCP, and 8 a new formula enriched with LCP of the omega-6 and the omega-3 series (0.5% LCP). LCP values in plasma lipids remained stable during the observation period in infants fed human milk. In contrast, LCP decreased markedly in plasma lipids of infants fed the conventional formula. Since the precursor fatty acids linoleic and alpha-linolenic acids were high in their diet and plasma, this finding indicates that premature infants have a limited capacity for LCP biosynthesis and may require their dietary supplementation. Infants fed the LCP enriched formula had significantly higher LCP proportions in plasma lipids than infants given the conventional formula, but less than infants fed human milk. Our results demonstrate that small concentrations of dietary LCP have marked effects on plasma lipid composition, particularly on phospholipids, suggesting that dietary LCP are preferentially channelled into structural lipids. We conclude that the essential fatty acid status of formula-fed premature infants can be improved by a supplementation of omega-6- and omega-3-LCP.
Intestinal milk-bolus obstruction in premature infants.
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Resting energy expenditure is increased in infants and children with extrahepatic biliary atresia.
To determine if liver dysfunction in children affects energy and macronutrient homeostasis, we performed 13 metabolic studies in 11 patients (age, 17.8 +/- 5.9 months [mean +/- SEM]) with extrahepatic biliary atresia (EHBA). Nutritional balance, indirect calorimetry, anthropometry, and biochemical liver function tests were utilised. Sixty-four percent of the energy losses were in the form of stool fat. Energy expenditure (68 kcal/kg/d) was 29% higher than normal (P less than 0.0025). Only one third of the metabolisable energy intake (37 kcal/kg/d) was stored in the body for new tissue synthesis. In spite of the bountiful protein intake for age, the increased protein oxidation (2g/kg/d) resulted in a virtually zero mean nitrogen balance. In addition, four patients oxidised endogenous protein as well. The respiratory quotient was 0.96, and did not change significantly between pre- and post-meal measurements, suggesting a predominant utilisation of carbohydrate for energy metabolism. Net lipid oxidation was severely diminished. We found that the higher the serum aspartate aminotransferase level (previously named SGOT), the lower the net fat oxidation, and the higher the conversion of glucose to fat. These data suggest that markedly increased energy expenditure contributes to the malnutrition of patients with EHBA. We characterised for the first time how severe liver disease in infants and children affects carbohydrate, fat, and protein metabolism, thus inducing protein-energy malnutrition.
Fat content and fatty acid composition of infant formulas.
Fat content and fatty acid composition of 25 commercial infant formulas sold in the Federal Republic of Germany and of 3 home-made milk formulas were analysed, using gravimetry of extracted lipids and high-resolution capillary gas-chromatography. Results were compared with the composition of human milk. Fat contents of all commercial formulas were similar to human milk values and met current recommendations, but 2 home-made preparations were at the upper and lower limits of the recommended range. Milk formulas tended to contain higher percentages of saturated and lower ones of cis-monounsaturated and trans-isomeric fatty acids than human milk. Linoleic acid (C18:2n-6) content was similar to human milk in most products but deviated clearly from recommended values in 2 home-made mixtures. Alpha-linolenic acid (C18:3n-3) values were often low in formulas, resulting a high n-6/n-3-ratios. In contrast to human milk, all formulas contained only minor amounts of the physiologically important long-chain polyunsaturated fatty acids with 20 and 22 carbon atoms. Some seasonal variation in the content of palmitic (C16:0), oleic (C18:1n-9), linoleic and trans-fatty acids was found when five arbitrarily selected adapted formulas were analysed repeatedly over one year. The composition of a home-made formula made from fresh cow's milk was markedly different in winter and in summer, when percentages of saturated and trans-fatty acids were higher and of linoleic acid were lower. We conclude that the composition of most commercial formulas is better suited to meet the lipid requirements of young infants than the home-made preparations investigated.(ABSTRACT TRUNCATED AT 250 WORDS)
Acrodermatitis enteropathica with normal serum zinc levels: diagnostic value of small bowel biopsy and essential fatty acid determination.
We report a patient with acrodermatitis enteropathica and a normal serum zinc level in whom the diagnosis was confirmed by plasma phospholipid fatty acid and a small bowel biopsy response to oral zinc therapy. Acrodermatitis enteropathica is a rare autosomal recessive condition of zinc deficiency characterised by chronic diarrhoea associated with failure to thrive, periorificial dermatitis and alopecia, susceptibility to infections and behavioural changes. Diagnosis is usually established by reduced serum zinc levels (classical acrodermatitis enteropathica). Paneth cell abnormalities on electron microscope of a small bowel biopsy can be supportive. A few cases with the typical picture of acrodermatitis enteropathica without hypozincaemia (variant acrodermatitis enteropathica) have been described. The diagnosis of variant acrodermatitis enteropathica to date has been based on an entirely empiric, but nonetheless convincing clinical response to oral zinc therapy. Laboratory aids to diagnosis have been lacking.
Prevalence of coeliac disease in diabetic children and adolescents. A multicentre study.
Screening for coeliac disease (CD) with serum antigliadin antibodies (AGA) was performed in 1032 diabetic children and adolescents. In 8 children CD had been diagnosed before study entry. Of the remaining 1024 children, 33 had an elevated AGA titre in the first serum sample. On follow-up an elevated AGA titre was confirmed in only 17 of 31 patients. Nine of the repeatedly positive patients underwent jejunal biopsy, and CD was diagnosed in two asymptomatic patients; both were positive for IgG- and IgA-AGA. Among 10 AGA-positive patients in whom biopsies could not be performed, only 1 showed IgA-AGA and thus carried a high risk for CD. From our results we estimate a prevalence of CD in Swiss and German diabetic children between 1.1% and 1.3%. False-positive AGA titres occurred significantly more often in patients with diabetes duration of less than 1 year. AGA testing reached a specificity of 99% if performed at least 1 year after the onset of diabetes. Children suffering from both diabetes and CD showed a diabetes manifestation at a significantly younger age than non-coeliac patients, whereas CD tended to be diagnosed at a remarkably late age.
Human alpha-linolenic acid deficiency.
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Fatty acid composition of mature human milk in Germany.
An improved gas-chromatographic method with high resolution, sensitivity, and precision was used for analyzing the fatty acid composition of human milk lipids. In 24-h collections of mature hindmilk of 15 German women, 42 different fatty acids could be separated and quantified. Among the saturated fatty acids (median sum 42.76%, wt/wt), six odd-chain fatty acids accounted for 1.16%. Cis monounsaturated acids represented 37.98%. Trans fatty acids, which may have untoward effects on the recipient infant, contributed 4.40% with seven isomers. The polyunsaturated fatty acid (PUFA) fraction (13.82%) included 10 long-chain PUFAs (LCPs; 1.66%). The content of the physiologically important LCPs in milk lipids did not correlate with their parent fatty acids (ie, linoleic and alpha-linolenic acids) but there was a significant correlation between the sum of omega-3 and omega-6 LCPs suggesting interindividual differences in the capacity for secretion of milk-lipid LCPs between mothers.
Long chain polyunsaturated fatty acids in infant formulae.
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Ataxia-deafness-retardation syndrome in three sisters.
Three sisters aged 16, 12 and 8 years from a consanguineous family presented with a progressive spinocerebellar ataxia combined with moderate mental retardation, progressive sensorineural hearing loss and signs of both upper and lower motor neuron disease. The patients represent the only known cases of the ataxia-deafness-retardation syndrome (McKusick #20885) except for the three brothers in the original description by Berman et al (1973). In the family described here transmission of the disease appears to be linked with occurrence of red hair colour.