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Fatty acid (FA) composition of plasma phospholipids and phospholipids extracted from peripheral mononuclear white blood cells (MNC) was investigated in 11 allergic asthmatic children (age 8.9 +/- 4.6 years), in 10 age-matched non-allergic healthy controls and in 14 allergic and non-allergic children with an acute attack of asthma, who had received prednisolone medication for 2-4 days. In allergic asthmatics eicosapentaenoic acid (20:5n-3) was significantly elevated in both plasma and MNC. The relative amount of 20:5n-3 in MNC as well as in plasma correlated positively with increasing levels of total serum IgE (P less than 0.02). The pattern of the other FAs in plasma and of MNC phospholipids did not differ between allergic asthmatic and non-allergic control children. In children with an acute attack of asthma, who had been treated with glucocorticoids (2 mg prednisolone/kg body weight for 2-4 days), distinct changes of relative FA composition of phospholipids were restricted to plasma, where some very long chain FA (22:4n-6, 22:5n-6) were elevated. No significant changes in FA from MNC phospholipids could be observed after glucocorticoid treatment. These findings may indicate a possible role of 20:5n-3, the precursor of "group 3" eicosanoids, in allergic asthmatic children.

Total tocopherols and alpha-tocopherols were estimated in the plasma of children with severe malnutrition (14 marasmus; 11 marasmic kwashiorkor; five kwashiorkor) and related to the total plasma lipids and different plasma lipid classes. If the mere plasma concentrations were taken as an index of the vitamin E status, five children with marasmus, five children with marasmic kwashiorkor, and two children with kwashiorkor would have been regarded as deficient (less than 500 micrograms/dl). However, if total tocopherols and alpha-tocopherols were related to the total plasma lipids, all malnourished children--except one--showed values within the limits found in healthy American children. The study shows that low tocopherol/lipid ratios are not a constant feature in severely malnourished children.

The role of vitamin E in severe malnutrition is controversially discussed. In order to evaluate the vitamin E status of unselected African children the plasma tocopherol levels of 52 children from a village in the People's Republic of Congo were assessed by HPLC and related to the plasma lipid values. Although plasma concentrations of total tocopherols (mean 351.1 +/- 59.4 micrograms/dl) and alpha-tocopherol (mean 313.3 +/- 57.0 micrograms/dl) were found to be low, normal ratios of total tocopherols to total lipids (mean 0.99 +/- 0.17 mg/g) and alpha-tocopherol to total lipids (mean 0.89 +/- 0.09 mg/g) were estimated. It is concluded that plasma vitamin E levels should be related to plasma lipids if populations are monitored in which low lipid concentrations have to be expected. These data on Congolese children may be used for comparison if the vitamin E status in other agrogeographical areas of Africa or in clinically abnormal groups is investigated.

11 infants with persisting hypoglycemia due to hyperinsulinism (nesidioblastosis of the pancreas) were treated with somatostatin. Somatostatin administration in a relatively high dosage (initially 145 micrograms/m2 body surface as bolus followed by a continuous infusion of the same dose per hour) resulted in a suppression of the circulating insulin concentration leading to a less abrupt fall of the postprandial plasma glucose level. By somatostatin infusion we were able to keep two patients with intractable neonatal hypoglycemia in a normoglycemic state until subtotal pancreatectomy. Infants suffering from nesidioblastosis require 1.0-4.5 micrograms/kg/h somatostatin and a concomitant carbohydrate supply of 0.3-0.48 g/kg/h in order to maintain normoglycemia. An initial somatostatin bolus can be omitted. Somatostatin is very reliable in the treatment of neonatal hypoglycemia due to hyperinsulinism for a limited period of time until subtotal pancreatectomy is performed. In most cases of nesidioblastosis this operative measure seems to be inevitable for the control of hyperinsulinism.

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.

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)

Whereas today intensive-paediatric management of the acute, life-threatening situation in organically conditioned hypoglycaemias is quite successful, conservative long-term treatment of nesidioblastosis is still unsatisfactory with regard to the prevention of subsequent cerebral damage. In contrast, subtotal pancreatectomy as performed by us is a curative treatment. Chances of cure are of course greatest if the treatment is effected before the onset of secondary damage. This is demonstrated by means of the authors' own patient material which comprises 10 surgically treated children with nesidioblastosis. Three of four infants operated on during the first weeks of their lives became normoglycaemic after the operation and developed normally, whereas only two of the six remaining children operated on at a later stage in their lives showed a comparably good course of the disease. The criteria of the surgical indication, surgical mode of procedure, intraoperative and postoperative complications and results obtained by the authors, are presented.

The course of pyruvate dehydrogenase deficiency in an infant is described. During pregnancy fetal movements were reduced, and since birth severe neurologic involvement was noticed. Permanent metabolic acidosis due to lactic acidemia as well as hyperpyruvic acidemia and hyperalaninemia were present. Alanine accumulated in CSF and urine, urinary excretion of lactate and pyruvate was highly elevated. Pyruvate dehydrogenase activity in a liver biopsy was only 5% of that for normal controls, and pyruvate decarboxylation by cultured fibroblasts was equally decreased. Therapy required permanent administration of bicarbonate. The administration of thiamine had no effect. The infant died within three months. Recently prenatal diagnosis during a subsequent pregnancy of the mother revealed normal results when pyruvate degradation with cultured amniotic fluid cells was investigated, and a healthy child was born.

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.

The concentration of selenium was determined by instrumental neutron activation analysis in erythrocytes, platelets, and plasma of eight dietetically treated children with phenylketonuria (n=6) or maple-syrup-urine disease (n=2) with low selenium intake and for ten children with normal selenium intake. The normal selenium concentration in platelets was about 600 ng/g and about five times higher than in erythrocytes of the same children. A decreased selenium concentration in platelets was seen only when the corresponding concentrations in erythrocytes and plasma were very low. This suggests a special role of selenium in platelets.

The rubidium content of whole blood was estimated by instrumental neutron activation analysis. In 46 healthy children it amounts to {ie193-1} g/g dry weight. There was no difference between the values found for infants, toddlers, and school children. In 29 dietetically treated patients with phenylketonuria and maple-syrup-urine disease the values were significantly lower than in healthy children. During the first three months of diet therapy the rubidium levels remained in the lower range of the normal values, decreasing to about 60% of the mean of normal values later on. With increasing length of diet therapy these values tended to decrease. It remains questionable whether these decreased levels reflect only an induced biochemical phenomenon without biological importance, or whether they are the first signs of a deficiency syndrome.

The selenium content of serum, whole blood and hair was measured by neutron activation analysis in dietetically treated patients with phenylketonuria (PKU) and maple-syrup-urine disease (MSUD). Follow-up studies showed a decrease of the serum selenium content and the glutathione peroxidase activity of erythrocytes--a selenoenzyme--from normal values at the beginning of the diet to 20% (selenium) and 50% (gluthione peroxidase) of normal within 10--12 weeks of dietary treatment. In 36 patients the serum selenium content was lower at 6.7--28 X 10(-9) g/ml, independent of the age of the patients (0.5 to 10 years). The selenium content of whole blood was reduced: median = 98 X 10(-9) g/g dry weight; range 75 to 165 X 10(-9) g/g dry weitht (healthy children: median = 381 X 10(-9) g/g dry weight; range 245 to 588 X 10(-9) g/g dry weight). The selenium content of hair was markedly lower in the patients (median = 62 X 10(-9) g/g; range 13--140 X 10(-9) g/g) than in healthy children (median = 429; range 213 to 720 X 10(-9) g/g). The mean glutathione peroxidase activity of erythrocytes was reduced to 4.6 +/- 0.64 U37/g Hb, comparison to normal values (mean = 8.8 +/- 0.88 U37/g Hb).

Inulin and amino acid clearance studies were made in 6 boys with oculocerebrorenal syndrome (Lowe) aged 11 months to 5 years. Glomerular filtration rate ranged between 50 and 112 ml/min/1.73 m2. Clearance values of all amino acids were increased. Tubular reabsorption of cystine, ornithine, lysine and arginine was relatively more reduced than that of the other amino acids. In Lowe's syndrome the transport system of the basic amino acids and cystine appears to be more impaired than that of other amino acids.

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

Three children with primary hypomagnesemia are described. First symptoms of the disease were observed, when the children were 35, 19, and 20 days old, resp. The hypomagnesemia was accompanied by a severe hypocalcemia. Therapeutic trials with high doses of calcium given intravenously and vitamin D were without effect on the symptoms. The whole body retention and intestinal resorption of orally administered 28-Mg was greatly reduced in all three patients compared to healthy adults. Symptoms of tetany and seizures ceased immediately after intravenous application of magnesium. An oral Mg substitution with 42--85 mmol per day was necessary to maintain subnormal to normal serum magnesium levels. The patients are now 5, 4 3/12 and 1 5/12 years old, resp. Psychomotor development in all three children is normal. Height and weight are in the lower normal range around the 3rd percentile, while the oral Mg substitution sometimes caused frequent fluid stools. By family studies from these patients and from the literature an autosomal-recessive inheritance for primary hypomagnesemia is proposed.

The plasma aminogram in uremic children receiving conservative treatment or undergoing hemodialysis demonstrated a similar profile to that first described in adults and children. We were able to demonstrate that dialysis did not correct the abnormal plasma amino acid pattern and that free amino acid losses in the dialysate during hemodialysis could not be compensated for by children receiving an adequate protein intake. Oral supplementation with essential amino acids (EAA) or ketoanalogues (KAA) only partially corrected the amino acid abnormalities and biochemical improvement in protein metabolism was not observed, however, increasing the relative proportions of branch chain amino acids in the supplement might be associated with improvement in metabolism. These data are in contrast to previous studies on the use of EAA or KAA in adults and children in which patients received a reduced protein intake, whereas in the present study protein intake was not restricted. The raised plasma levels of methionine and 3-methyl-histidine were not associated with side effects.

The cornea and conjunctiva of rabbits are burned with 0.25 n NaOH for exactly 30 s. Then, the cornea and conjunctiva are cleaned with 0.9% NaCl. After sacrification the cornea is rinsed and the levels of the glucose and lactate are measured. The alterations of the metabolites for corneal nutrition are discussed.

Analytical methods and the results of the analysis of the lipids of commercially available canned supplementary foods for juniors are described. Lipids were extracted, separated into polar and non-polar fractions and the components (triglycerides, monoglycerides, free fatty acids, cholesterol ester, and phospholipids) were then identified and estimated, by comparison with reference substances and by gas chromatography. To determine the degree of unsaturation and positional distribution of the fatty acids, the triglycerides were hydrolyzed by pancreatic lipase to monoglycerides with the fatty acid in 2-position of glycerol, and the fatty acids and the monoglycerides were also separated by gas chromatography. Linoleic acid, 18.2 omega 6, was found as the predominant fatty acid in the 2-position of the triglycerides. The other fatty acids being distributed equally to all positions of glycerol with the exception of few minor fatty acids. Fat contributed to 31 to 48 percent of the total energy supply of the analyzed items; linoleic acid providing 0.4 to 2.4 percent of this energy assuming that one can of these products is consumed daily.

In 20 healthy infants and children, 5-20 months old, the Se intake was estimated by analysing food samples by instrumental neutron activation analysis. The intake was calculated by weighing the portions offered and actually consumed. The median Se content of the food amounted to 27 ng/g wet weight (gww) and median daily Se intake to 33.5 micrograms. The Se intake was not equally distributed over the day. About 50% of the daily Se intake was derived from the supper. The main Se sources (41%) for young children were cereal paps. Commercially available meals (30 ng/g) contained less Se than home-made ones (50 ng/g). In nine dietetically treated patients with phenylketonuria the median Se intake amounted only to 6.9 micrograms/day corresponding to a mean Se content of the diet of 7.9 ng/g. The main Se source in the diet was vegetables (36.3%) and 20% derived from their protein supplements. The Se intake of young children, healthy or dietetically treated, cannot be calculated accurately from tables but must be estimated by measuring the Se content of the local food because cereals and vegetables--the main Se sources--exhibit great regional variations.

The selenium content of human milk, cow's milk and cow's milk infant formula were estimated by instrumental neutron activation analysis. The highest values were found in 3 samples of human colostrum (524--865 x 10(-9) g/g dry weight). There was a significant decrease with increasing time post partum. Mature human milk exhibited a selenium content of 230 +/- 79 x 10(-9) g/g dry weight. The selenium content of 45 samples of cow's milk from the north-western area of Germany was 200 +/- 39 x 10(-9) g/g dry weight. While there was no significant difference between the values of mature human milk and of cow's milk, cow's milk infant formula exhibited significantly (P less than 0.01) lower values than human milk. The average selenium content of 107 samples of 10 different commercially available fluid and powdered cow's milk infant formulas (range: 18--171 x 10(-9) g/g dry weight) amounted to about only one third of that in mature human milk.

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.

High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and alpha-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.

Manganese was measured in blood of a patient with prolidase deficiency by neutron activation analysis. The manganese content of packed blood cells was 48.9 ng/g wet weight (controls 18.4 +/- 4.3 ng/g wet weight). It was proportionately increased in whole blood, whereas the serum manganese concentration was unchanged. The erythrocyte arginase activity was reduced to 46% of the mean control value. The reduced activities of two manganese-dependent enzymes, prolidase and arginase in erythrocytes in combination with an increased manganese content cannot be explained at the moment and leads to the speculation that manganese is inaccessible for enzyme activation.

A patient with a progressive neurological disorder beginning at the age of three years is described. Mental and visual disturbances were the first signs, soon followed by ataxia and myoclonic jerks. Fundoscopy revealed a decreased pigmentation of the retina. Ultramicroscopic investigations of muscle and skin disclosed the typical changes seen in the late infantile and juvenile forms of neuronal ceroid-lipofuscinosis. In contrast to the clinical and ultrastructural findings, the fatty acid pattern of the serum lecithin showed a significant increase of arachidonic acid and a corresponding decrease of linoleic acid which is characteristic of the so-called infantile form of neuronal ceroid-lipofuscinosis (Hagberg-Santavuori variant; polyunsaturated fatty acid lipidosis). The obvious heterogeneity of the clinical, histological and laboratory findings within the subgroups of neuronal ceroid-lipofuscinosis is briefly discussed.

Two neonates with maple syrup urine disease were treated by exchange transfusion. Within 15 h blood leucine and KICA concentrations were lowered from 2.6 mM to 1.1 mM using 570 to 620 ml blood per kg body weight. The other branched-chain amino acid/keto acid pairs fell to normal. During exchange transfusion the patient's nitrogen balance seems to be negative. Further exchange transfusion was useless. More importantly the patient should be forced into an anabolic state by high caloric supply or insulin plus glucose treatment. More KICA than leucine was eliminated, however, KICA blood levels remained slightly higher than that of leucine indicating different leucine/KICA equilibria in extravascular compartments than in blood. In a given time interval exchange transfusion was more effective than peritoneal dialysis, probably due to a lack of an additional (peritoneal) membrane. Renal excretion of branched-chain amino and keto acids was very inefficient. The allegedly most toxic metabolite, KICA, had the lowest renal clearance of the branched-chain keto acids.

In a male with a partial defect of ornithine transcarbamylase (OTC) we observed that maintenance of arginine supply was crucial for adequate metabolic control in conjunction with a low protein diet. The arginine supplement had to be given such that the concentrations of arginine and ornithine in plasma were above 50 mumol/l. It appears that arginine is needed not only as an essential amino acid for protein synthesis but also as a precursor of ornithine. In this patient the substitution thus aimed at increasing the intramitochondrial ornithine in order to reach a critical substrate concentration for the kinetically abnormal OTC.

The intestinal resorption of zinc using 65ZnCl2 was estimated in 3 patients with acrodermatitis enteropathica, 2 healthy controls, and 3 heterozygotes. After oral application of 65Zn the whole body activity was measured by a whole body counter for 34 days. The 65Zn resorption of the patients amounted to 16, 42 and 30% of the applied dose, whereas the resorption values of the heterozygotes and the controls were in the range of 58 and 77%. The elimination of 65Zn from the body amounted to about 0.7% of the applied dose with no difference between controls and patients with acrodermatitis enteropathica. Before therapy the serum-zinc levels of patients were markedly decreased. After oral application of high doses of zinc aspartate (2 times 400 mg/day) all clinical symptoms disappeared within a week. The results point at a causal connection between zinc and the pathogenesis of acrodermatitis enteropathica. Ultrastructural alterations of the Paneth cells of the intestines are also shown in this disease [12] as have also been seen in Paneth cells of zinc deficient rats [Beitr. Path. 145, 336 (1972)].

The diets of 5 patients with phenylketonuria of maple-syrup-urine disease were supplemented with yeast which was rich in selenium. For 120 days the patients received 45 micrograms Se/day to increase the Se content of their diets to 10--12 ng Se/Kjoule. Before supplementation the selenium content of serum (5--15 ng/ml) and whole blood (10--27 ng/ml), and the activity of the erythrocyte glutathione peroxidase (0.19--2.69 U37/g Hb), amounted to only 10--20% of normal. The serum selenium content reached normal values within 4 weeks of supplementation, followed by normalisation of the selenium content of whole blood within 4--8 weeks. Restoration of the activity of erythrocyte glutathione peroxidase took 9 to 15 weeks--the red cell life span. There was a significant positive correlation between the selenium content of the erythrocytes and the activity of erythrocyte glutathione peroxidase.

Three children with branched-chain ketoaciduria (maple syrup urine disease) were found to excrete an abnormal amino acid when they were on an artificial diet. This substance was identified as 4-amino-2-(S-cysteinyl)butyric acid with the use of column liquid chromatography, gas chromatography--mass spectrometry of various derivatives, and 360 MHz 1H-NMR spectroscopy. The same compound was detected in urine samples from subjects undergoing an oral loading test with L-proline. The chromatographic analysis of commercial proline from two sources indicated that one of the batches was contaminated (less than 1%) with L-azetidine-2-carboxylic acid (the homologue of proline with a four-membered ring). The latter compound is probably metabolized by the human via ring-opening and addition of a cysteine moiety. It is highly probable that the artificial diet given to the patients contained the impure proline and that the L-azetidine-2-carboxylic acid in the proline gave rise to the excretion of the 4-amino-2-(S-cysteinyl)butyric acid.

Serum zinc concentrations in subjects of different ages were estimated by neutron activation analysis to obtain reliable reference values for the diagnosis of primary or secondary zinc deficiency during childhood. In these healthy individuals a small age-dependent variation of the mean values was found. Serum zinc concentrations were lower in cord blood (mean value: 880 X 10(-9) g/ml) and in infant;s (mean value: 820 X 10(-9) g/ml) than in adults (mean value 1178 X 10(-9) g/ml). In comparison to these values, the serum zinc concentration in a patient with secondary zinc deficiency and 4 patients with acrodermatitis enteropathica was reduced to about 25% of normal.

In view of the controversial results regarding reversibility of urinary tract lesions due to Schistosoma haematobium infection, 103 patients in the People's Republic of Congo were followed up one year after treatment with Praziquantel (40 mg/kg body wt) by means of ultrasonography. The study group consisted mainly of children. Before therapy a total of 113 lesions were detected in the lower urinary tract. These consisted of bladder wall enlargements (N = 53), enlargements of the bladder wall in combination with singular (N = 16) or multiple polyps (N = 30), vesical calcifications (N = 10), and cases with cystitis cystica (N = 4). One year after therapy, a drastic reduction of ova output was observed, and only eight pathological abnormalities of the lower urinary tract were still detectable. Vesical calcifications persisted in four out of ten cases. Urinary tract obstructions of different degrees (N = 42) showed a comparably marked reversibility. It was concluded that, in children, the resolution of bladder lesions induced by urinary schistosomiasis resulted in reversibility of urinary tract obstructions one year after treatment with Praziquantel.

Serum-selenium concentrations were measured in 2 dietetically treated patients with maple-syrup-urine disease, in 11 dietetically treated patients with phenylketonuria, in 37 healthy children of different ages and in 183 healthy adults. The estimations were performed by instrumental neutron activation analysis. The values of the dietetically treated patients were much lower than those of healthy children of the same age group. Within 8 to 12 weeks the serum-selenium concentrations decreased from normal values before therapy to very low values under dieto-therapy. During infancy the serum-selenium concentrations of healthy individuals show an increase to the adult range of values.

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.

The fatty acid (FA) status in young children with cystic fibrosis (CF) was investigated. The FA composition of the plasma cholesterol esters (CE) and phospholipids (PL) and of the erythrocyte phosphatidylcholine (PC) and phosphatidylethanolamine (PE) was estimated in 11 patients with CF and pancreatic insufficiency (median age, 3.0 years; range, 3 months to 7 years) and in 10 age-matched controls. Linoleic acid values ranged widely but were not significantly reduced in the patients. However, arachidonic acid (20:4w6) and docosahexaenoic acid were decreased in all lipid classes. The ratio of dihomo-gamma-linoleic acid to arachidonic acid (20:3w6/20:4w6) was significantly increased in the patients, indicating an impairment of FA metabolism (delta 5-desaturation). Plasma retinol concentrations were normal and did not differ between the supplemented patients and controls. Plasma total tocopherols and alpha-tocopherol and their ratios to total lipids were significantly reduced in the CF patients, but all values were within the normal ranges for the pediatric age group, and no child met the criterion for vitamin E deficiency.