I. Lombeck

A metabolic disorder similar to Zellweger syndrome with hepatic acatalasia and absence of peroxisomes, altered content and redox state of cytochromes, and infantile cirrhosis with hemosiderosis.

A patient with a cerebro-hepato-renal syndrome was investigated. The visceral manifestations were those of the Zellweger syndrome (ZS); however, the child exhibited muscular hypertonia and survived into the 2nd year of life. Ultramicroscopically, hepatocytes were lacking peroxisomes, but, contrary to findings in one patient with ZS [2], contained smooth endoplasmic reticulum. No catalase was found by histochemistry or spectroscopy. Mitochondria showed normal succinate and glutamate respiration, and normal coupling of respiration to the phosphorylation potential. The cytochrome (cyt) content was diminished to one-third with an abnormally inversed redox patterns of the respiratory chain in the controlled state, cyt b being 5%, cyt c 23% reduced. The oxygen affinity of cyt a3 was normal. These findings exclude a defect in the nonheme iron protein region of the respiratory chain as described in ZS [2], but point to a functional abnormality of cyt b in our patient.

Plasma glutathione peroxidase after selenium supplementation in patients with reduced selenium state

The plasma glutathione peroxidase (GSHPx) activity was measured in normal adults and children and in patients with reduced selenium state because of dietary treatment of metabolic diseases (phenylketonuria or maple-syrup-urine disease) before and after selenium supplementation. Besides GSHPx (measured with t-butyl hydroperoxide, cumene hydroperoxide and hydrogen peroxide as acceptor substrates) the activity of glutathione S-transferase was estimated in plasma. Plasma GSHPx activity in healthy children was significantly lower than in healthy adults. In 11 dietetically treated patients with phenylketonuria or maple-syrup-urine disease the plasma GSHPx was reduced to about 17% of the values of healthy children of the same age. No glutathione S-transferase activity could be found in plasma of children in normal or reduced Se state.During administration of yeast rich in Se (200μg Se/d) for 90 days 2 healthy adults showed no significant change of plasma GSHPx activity. During Se supplementation (75–100μg Se/d) for 120–163 days 5 dietetically treated patients with PKU or MSUD exhibited a significant increase of plasma GSHPx activity within 2 days. The values reached a plateau after 1 to 3 weeks of supplementation and remained at this level within the following 4 to 5 months. Therefore, the activity of plasma glutathione peroxidase can be used as an indicator of short-term changes of selenium intake in selenium deficient individuals.

Acute selenium poisoning of a 2-year-old child.

After ingestion of an unknown amount of a gun blueing compound containing selenious acid (11 ml from the bottle fluid were missing, equivalent to 2.9 g Se) a 2-year-old girl suffered from continuous hyper-salivation, vomiting, diarrhoea, restlessness and muscle spasm. Blood pressure and pulse rate were increased. Symptomatic treatment was performed by parenteral fluid administration. The plasma Se concentration was increased to 20 times normal 5 h after ingestion. Erythrocyte Se exceeded plasma Se, 24 h after intoxication. Urinary Se excretion decreased parallel to the plasma Se concentration. Ten weeks later, the Se content of hair had risen to 10 times normal. The plasma glutathione peroxidase activity showed only a slight increase during the first 36 h, erythrocyte glutathione peroxidase, catalase and superoxide dismutase activities were not significantly altered. The child fully recovered.

Hair selenium content during infancy and childhood

Hair selenium content was estimated by instrumental neutron activation analysis in Libyan infants and children. There was no significant difference between the values of young infants from North and South Libya. The hair selenium content rapidly decreased from 1071 ±75 ng/g in newborns to 301±99 ng/g in 7–10 months old infants in North Libya and to 557±204 ng/g in South Libya. In North Libya the values of preschool children were 409±117 ng/g and those of school children 464±124 ng/g.

Platelet selenium in children with normal and low selenium intake

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.

Composition of foods and dishes commonly consumed in villages of the Gezira area of Sudan. III. Fatty Acids, Retinol, α‐, β‐ Carotene and Tocopherols

It is known that the fat content of ready‐to‐eat dishes of the people in the Gezira area of Sudan is low. In order to judge the adequacy intake of the essential fatty acids (FA), the FA composition of foods and dishes commonly consumed was analysed. Percentages of fatty acids are given for several types of sorghum, millet, sesame, fenugreek, different sorts of peas, as well as for processed foods like gruels, sauces, local thin‐leaf bread and vegetables. Because there is also a lack of information on the vitamin A and E contents of these food items and ready‐to‐eat meals in this area, retinol, a‐, s‐carotene and tocopherols were chemically estimated and their contents are given for most of the important foodstuffs.

The selenium content of infant food.

SummaryThe selenium content of food exhibits great regional differences. Food samples of infants and young children from the North Rhine-Westphalia State in the Fed. Rep. of Germany were analysed by instrumental neutron activation analysis and showed that the average selenium content of local vegetable and fruit is below 5 ng/g wet weight. Only samples of potatoes and bananas exhibit higher selenium contents. The bananas, probably imported from different areas of the world, show an extremely high variance with values between 4 and 164 ng Se/g. Commercially available ready-for-use meals for infants or young children have a low Se content (median 24 ng/g) which is comparable to cows milk. Besides eggs, cereals enriched with milk and some other cereal products contain more than 100 ng Se/g.ZusammenfassungDer Selengehalt der Nahrung weist große regionale Unterschiede auf. Nahrungsproben von Säuglings- und Kleinkinderkost aus Nordrhein-Westfalen wurden mit Hilfe der instrumentellen Neutronenaktivierungsanalyse untersucht und zeigten, daß der Selengehalt der hiesigen Gemüse und Früchte durchschnittlich etwa 5 ng/g beträgt. Nur Proben von Kartoffeln und Bananen weisen einen höheren Selengehalt auf. Bananen, importiert aus verschiedenen Gebieten der Erde, zeigen eine extrem hohe Schwankungsbreite mit Werten zwischen 4 und 164 ng Se/g. Fertiggerichte für Säuglinge und Kleinkinder haben einen der Kuhmilch vergleichbar niedrigen Selengehalt von durchschnittlich 24 ng/g. Außer Eiern enthalten nur Milchbreie und einige andere getreidehaltige Produkte mehr als 100 ng Se/g.

Clinical Studies on Polyunsaturated Fatty Acids and Antioxidants in African Children

Increased oxidative stress may play an important role in the pathophysiology of human malnutrition. However, deficiencies of antioxidative acting substances like tocopherols and selenium are not consistently found in African children suffering from various clinical forms of malnutrition. Comparative studies investigating clinically well defined groups of patients in different agrogeographical regions are necessary to elucidate the clinical significance of antioxidants in malnutrition.

Antioxidant status of insulin dependent diabetics.

Keywords: diabetes, child, selenium, glutathione peroxidase, vitamin A, vitamin E, antioxidants

Zinc status of Libyan children — a pilot study

SummaryHair zinc has been investigated in children of North and South Libya. The hair zinc content amounts to 213±36 μg/g in newborns. There is no difference between the values of newborns from North Libya and newborns from South Libya. The hair zinc values decrease during infancy. In toddlers and school-children the hair zinc content is 88±35 μg/g, 89±25 μg/g resp. These values are as low as those reported in American children with low height percentiles or nearly as low as those found in dwarfs with poor zinc status from Egypt and Iran.ZusammenfassungDer Haarzinkgehalt wurde in Haaren von Kindern aus Nord- und Südlibyen bestimmt. Der Zinkgehalt betrug 213±36 μg/g bei libyschen Neugeborenen. Es bestand kein Unterschied zwischen den Werten von Neugeborenen aus Nord- und Südlibyen. Der Zinkgehalt fällt während der Säuglingszeit ab. Bei Klein- und Schulkindern lag der Zinkgehalt bei 88±35 μg/g bzw. 89±25 μg/g. Diese Werte sind so niedrig wie die bei amerikanischen kleinwüchsigen Kindern oder fast so niedrig wie bei den ägyptischen und persischen Zwergen mit vermindertem Zincstatus.