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Biomedical subjects

B Koletzko

Publications and source records attributed to B Koletzko.

159 records · Page 9Linked to original sources
PubMed1986

Fatty acid composition of plasma lipids in Nigerian children with protein-energy malnutrition.

The fatty acid (FA) composition of the main plasma lipids was analysed in eight well-nourished, generally healthy Nigerian children aged 14.1 +/- 7.2 months and in 17 malnourished children (8 marasmus, 9 kwashiorkor) aged 14.6 +/- 3.8 months within the first 2 days of admission at the Dept. of Child Health, University of Benin. In comparison to the control group, the malnourished children showed a marked decrease of polyunsaturated FA with low linoleic acid, mainly in sterol esters (STE), and severely reduced linoleic acid metabolites, including arachidonic acid, in all lipid fractions. omega-3-FA were not altered except for a reduction of docosapentaenoic and docosahexaenoic acids in phospholipids. Clearly increased values were found for saturated FA in STE and for the non-essential monoenoic FA in all lipid classes. This pattern indicates the presence of essential fatty acid deficiency in the malnourished children. There was no significant difference between marasmus and kwashiorkor. Eight malnourished children were followed up in the early phase of recovery during hospital treatment 14.0 +/- 3.1 days after obtaining the first sample. Linoleic acid had increased again in STE, but its metabolites were as low or even lower than before. An impaired activity of delta-6-desaturase, the rate limiting enzyme of linoleic acid metabolism, in suggested by elevated substrate-product-ratios of this enzyme in untreated children with protein energy malnutrition and in the early phase of recovery, which may be due to low insulin levels, protein and zinc deficiency. The trientetraen ratio (20:3 omega 9/20:4 omega 6) thus is not a reliable indicator of essential FA status in protein-energy malnutrition.

Arachidonic Acid↗
PubMed1985

Fatty acid composition of plasma lipids in acrodermatitis enteropathica before and after zinc supplementation.

The fatty acid composition of different plasma lipid fractions has been estimated in a 6-month-old girl with acrodermatitis enteropathica before and after zinc supplementation. Linoleic acid and its metabolites were extremely reduced in triglycerides and sterol-esters. In contrast, n-3-fatty acids were increased in sterol-esters and phospholipids. Zinc supplementation led to quick clinical improvement, and linoleic and arachidonic acid increased rapidly in triglycerides and sterol-esters to the values of healthy infants. Fatty acids of phospholipids remained relatively stable. Our findings could be explained by impaired enteral absorption of linoleic acid. Further attention should be directed to the supply and metabolism of essential fatty acids in acrodermatitis enteropathica.

Acrodermatitis↗
PubMed1985

Effect of age and ambient temperature on n-pentane production in adult housefly, Musca domestica.

The objective of this study was to examine the relationship between lipid peroxidation and aging in the male housefly. Metabolic rate of flies is known to be higher and life span shorter at elevated ambient temperature. Evolution of n-pentane and level of thiobarbituric acid (TBA) reactive material were used as indicators of lipid peroxidation. n-Pentane accumulated by houseflies in vivo and by whole body homogenates of houseflies, in response to tert-butyl hydroperoxide (1 mM), increased with age. n-Pentane accumulation in vivo was markedly higher at higher ambient temperature. Furthermore, n-pentane generated by flies in vivo and by fly homogenates in vitro tended to be lower in flies raised at a lower ambient temperature. TBA-reactive material, elicited by tert-butyl hydroperoxide, was augmented in older flies, but no significant difference was found between flies aged at different ambient temperatures. Analysis of fatty acids in housefly homogenates indicated an age-associated increase in the ratio of polyunsaturated to saturated fatty acids.

Aging↗
PubMed1984

Congenital anomalies in patients with choanal atresia: CHARGE-association.

Six patients with both choanal atresia (ChA) and additional malformations are described and another 110 cases with this combination reviewed from the literature. Our study of these cases supports the existence of the CHARGE-association (Coloboma, Heart Disease, Atresia of choanae, Retarded mental development and growth, Genital hypoplasia, Ear anomalies and deafness). Our findings suggest the inclusion of orofacial clefts and oesophageal atresia among the main symptoms of this association. A certain degree of facial dysmorphism (low set, dysplastic ears, retrogenia, antimongoloid slant of palpebral fissures and anteverted nares) was observed in each of our cases. Infants with the bilateral type of ChA plus cardiac defects and those with ChA plus renal malformations have a high mortality rate. The aetiology of the association is not clear. The recurrence risk may be low.

Abnormalities, Multiple↗
PubMed1984

[Immunologic findings in juvenile Mediterranean kala-azar].

The course of immunological parameters in a 2 year old patient with mediterranean kala-azar is reported. Plasma-fibronectin was reduced to 33% of the normal value at the time of diagnosis. When the clinical symptoms of the disease were most severe, a marked activation of complement without demonstrable circulating immune complexes was observed. Therapy with sodium-stibogluconate lead to improvement of the child's condition, normalization of complement reaction and fibronectin levels, and detection of circulating immune complexes. The activity of the disease seems to be well indicated by the level of C-reactive protein and the complement cleavage product C3 d. Incubation of living Leishmania donovani with normal human serum leads to activation of complement in vitro.

Antibodies↗
PubMed1984

[Scintigraphic diagnosis of multiple pheochromocytomas in childhood with 131I-m-benzylguanidine].

Scintigraphy with 131I-m-BG was used in 4 children with a history of multiple pheochromocytomas in order to localize further catecholamine-producing tumors prior to surgery. We overlook 7 scintigraphies without any side effect. Phenoxybenzamine did not interfere with tracer uptake into tumors. Scintigraphic localization of even smallest extra-adrenal tumors was successful in all cases. In most cases we were dealing with benign pheochromocytomas, but also a ganglioneuroma and metastases of a malignant pheochromocytoma could be revealed. Scintigraphy is a reliable technique for tracing pheochromocytomas and catecholamine-producing ganglioneuromas. It seems to be superior to other non-invasive techniques, furthermore, invasive techniques bearing higher risks may be suspended.

3-Iodobenzylguanidine↗
PubMed1984

[Late manifestation of vitamin K deficiency in breast fed infants].

Two breast fed infants had late manifestations of Vitamin K deficiency. No underlying disease was found in the first case. The second patient was found to have alpha 1-Antitrypsin deficiency (Pi type ZZ). The latter patient initially responded well to a single dose of vitamin K administered orally (3 mg). However, three weeks later, he was admitted to our hospital with severe intracranial hemorrhage due to severe vitamin K deficiency. Vitamin K requirements in infants and clinical characteristics of vitamin K deficiency in infants older than 1 week are discussed.

Blood Coagulation Tests↗
PubMedDate not supplied

Deficiency in complex IV (cytochrome c oxidase) of the respiratory chain, presenting as a leukodystrophy in two siblings with Leigh syndrome.

Two siblings with Leigh syndrome presenting at the age of 6 months with clinical and radiological features suggestive of a leukodystrophy are reported. A deficiency in complex IV of the respiratory chain (cytochrome c oxidase) was demonstrated in muscle mitochondria of both patients. To our knowledge, this is the first familial case of Leigh syndrome due to cytochrome c oxidase deficiency, presenting clinically and radiologically with signs of a leukodystrophic process. We suggest that respiratory chain enzyme defects should be considered in the differential diagnosis of cases suggestive of a leukodystrophy.

Brain↗
PubMedDate not supplied

Comparison of fatty acid composition of plasma lipid fractions in well-nourished Nigerian and German infants and toddlers.

The dietary intake of essential fatty acids is reflected by the plasma lipid composition. Only scanty data is available on the pattern of plasma fatty acids in young children and the influence of different environments. We analyzed the fatty acid composition of plasma sterolesters, triglycerides, and phospholipids in well-nourished-appearing children from Benin City, Nigeria (n = 8; aged 14.1 +/- 7.2 months) and Düsseldorf, FRG (n = 17; aged 15.2 +/- 5.1 months). The Nigerian group tended to have bigger proportions of the essential omega-6-fatty acids, linoleic acid, and its metabolites, and by far higher values for long-chain omega-3-fatty acids, which are considered to be protective against atherogenesis. The saturated and nonessential monoenoic fatty acids tended to be lower in the Nigerian children, and the ratio of polyunsaturated to saturated fatty acids was higher. We conclude that the quality of dietary fat in the German children was worse and may imply an increased risk for development of atherosclerosis.

Arachidonic Acid↗
PubMedDate not supplied

Intestinal milk-bolus obstruction in formula-fed premature infants given high doses of calcium.

Supplementation of milk formulae with calcium salts is recommended for low birth weight infants. We observed a serious complication of calcium administration in too high a dosage. Three premature infants (birth weight 1,080-1,900 g and gestational age 28-35 weeks) developed an obstruction of the distal ileum during the second week of life, leading to multiple bowel perforations and development of septic peritonitis in two cases. All three patients required surgical removal of a bolus blocking the intestinal lumen. Analysis of the bolus material from one infant indicated that the formation of soaps from calcium and long-chain fatty acids had caused the obstruction. All three infants had been fed a formula for premature infants with the addition of a recently introduced commercial mineral supplement, resulting in a very high calcium concentration of the feed (40.6 mmol/L). Simulation of syringe feeding demonstrated that even higher calcium concentrations could occur in milk portions containing sedimented calcium compounds.

Animals↗