Plasma lipid concentrations in 42 treated phenylketonuric children.
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Biomedical subjects
Publications and source records attributed to C Agostoni.
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Twenty-two Italian HIV-infected patients developed leishmaniasis, clinically manifested as visceral (13 cases), cutaneous (2 cases) and disseminated disease (7 cases). Twenty were males and two females (mean age: 32.8 years) with a mean CD4+ cell count of 46.8/microliter at diagnosis; risk factors were intravenous drug use (17 patients) and sexual behaviour (two bisexual, two homosexual, one heterosexual). All but one patient lived or travelled in hypoendemic Italian regions and other Mediterranean countries. Apart from the two patients with cutaneous leishmaniasis, the clinico-pathological and biological spectrum of the infection was often atypical, especially in patients with disseminated disease. The diagnosis was routinely made by direct recovery of parasites in biological specimens, mainly in bone marrow aspirate, whereas serology was negative or borderline in most of the patients. Among 17 in vitro isolates, Leishmania infantum was the only species involved with previously undescribed isoenzyme patterns in two cases. Treatment with antimonials and other drugs showed only temporary clinical improvement in some patients. Relapses were the rule. Leishmaniasis confirms itself as an opportunistic infection in HIV-positive persons. Secondary chemoprophylaxis should be considered in cases of relapsing disease.
In order to explain processes underlying the transfer of fatty acids from the maternal compartment into human milk, the lipid content and the fatty acid composition of maternal plasma and milk have been analyzed in breastfeeding mothers at 1 day and 3 months of lactation. The rise in milk lipids occurring during the study period was concomitant with a fall in plasma total fat content, mainly due to the decrease of triglycerides. Significant correlations between plasma and milk fatty acids at the two time points were observed only for linoleic (LA, 18:2 n-6) and (alpha;-linolenic acid (alpha LNA, 18:3 n-3), while for arachidonic (AA, 20:4 n-6) and docosahexaenoic acid (DHA, 22:6 n-3) correlations were found only at one day and 3 months, respectively. These data suggest that levels of the n-6 and n-3 18C polyunsaturated fatty acids in milk are closely dependent on their concentrations in maternal plasma, in turn related with the dietary intake, while the accumulation of AA and DHA in milk is the result of a sequence of transfer and metabolic processes.
The clinical efficacies of 50 mg/kg.day miocamycin and 60 mg/kg.day amoxycillin were studied in 23 patients aged 3-11.5 years with presumed bacterial infection of the lower respiratory tract (bronchopneumonia and acute bronchitis). During the therapy, which continued for 10 days, non-specific immune function, represented by natural killer cell activity, was monitored by measurement of the rate of lysis induced on target K-562 51Cr-labelled tumour cells. The results confirmed the therapeutical efficacy of miocamycin and amoxycillin in the oral therapy of bronchopneumonia and acute bronchitis in paediatric patients. The natural killer cell activity of patients treated with miocamycin was increased on days 7 and 10 of therapy compared with baseline. This finding did not occur in patients treated with amoxycillin.
Carnitine has a fundamental biological role as a long-chain fatty acid carrier across the mitochondrial membrane and in ketone body formation. Although the body normally synthesizes carnitine, in certain circumstances such as total parenteral nutrition and haemodialysis a dietary supplement may be needed to maintain adequate levels. Several considerations suggest that carnitine is a truly essential nutrient in infancy and in other situations where the energy requirement is particularly high, e.g. pregnancy and breast feeding. There are, for example, congenital deficit syndromes due to enzymatic inadequacies. There is also the possible role of carnitine in serious metabolic disorders such as organic acidaemias and, above all, it has multiple physiological functions in major metabolic pathways which are essential for development and growth.
The most important functional components of dietary lipids (triglycerides, cholesterol esters and phospholipids) are fundamental to normal growth and development of infants. Fatty acids are classified according to their chain length and their degree of saturation. Each class of fatty acid is involved in specific metabolic reactions: short-chain fatty acids act as local growth factors in the colon; medium- and saturated long-chain fatty acids are a good source of energy; polysaturated long-chain fatty acids are involved in metabolic regulation; and very long-chain fatty acids are important structural components of membranes. The development of the central nervous system depends on the amount and the quality of the lipid supply in the last months of prenatal and the first months of postnatal life. Placental cord blood during foetal life and breast milk provide fatty acids in the correct amounts and ratios. Fats in breast milk provide half the infant's calorific needs and maternal dietary habits can influence the lipid composition of milk. The preparation of blends of fats for formulas is under investigation in order to improve the lipid quality and to make formulas more similar to breast milk. A major goal of current research is to define the nutritional and metabolic relationships between fatty acid groups and between fats and the other dietary sources of calories in order to improve the composition of infant formulas, in particular for premature babies.
The efficacy and safety of gamma-linolenic acid in the treatment of atopic dermatitis was evaluated. The children (mean age, 11.4 months) with atopic dermatitis (mean duration, 8.56 months) were openly treated with 3 g/day gamma-linolenic acid, for 28 days. Clinical evaluations were carried out every 7 days, and parents were asked to keep a diary, recording details of symptoms of eczema every day. Blood chemistry and immunological tests were done before and after treatment. None of the children showed complete recovery of symptoms. A gradual improvement in erythema, excoriations and lichenification was seen; significant differences were shown for itching (P < 0.01), and the use of antihistamines (P < 0.01). A significant rise in the percentage of circulating CD8 was found. No side-effects were recorded. Dietetic and pharmacological approaches are the basis of the treatment of atopic dermatitis and gamma-linolenic acid appears to be a safe and efficient additional therapy for infants and young children.
Adolescence is an intense anabolic period. The requirement for all nutrients is increased, but particularly that for dietary calcium. A balanced intake of the macronutrients (protein, fats and carbohydrates) is recommended to prevent the chronic degenerative disorders of adulthood. The temporal pattern of the calorie intake also deserves attention since it may affect homeostatic regulation. Adolescents often show disorders of dietary behaviour predisposing them to both obesity and anorexia. Dietary intervention in this age-group should promote the regular consumption of breakfast, a balanced intake of animal and vegetable foods and an increased calcium supply to maximize bone density. Dairy products and vegetables (mainly enriched cereals) constitute the basis of a good diet for adolescents, to supply their needs for growth and for subsequent good health.
Infant formulae are the only alternatives to breast milk for infants who are unable to continue breastfeeding through the first year of life. They aim to provide formula-fed infants with the same structural and functional benefits observed in breastfed infants. To achieve this, bioactive nutrients have been added to infant formulae in recent years: long-chain polyunsaturated fatty acids for neurodevelopment; probiotics and prebiotics for local gastrointestinal defence; and nucleotides for promoting the immune response. Changes in protein quantity and quality allow infant formulae to achieve a balance between providing the correct plasma amino acid profile and reducing the protein intake, which could prevent obesity in later life. Hydrolysed proteins may help prevent atopic disorders. Many short-term trials have been published but long-term follow-up data are needed in infants who have been fed the newer infant formulae, to fully understand the role of bioactive nutrients.
Lactoferrin (LF) is a mammalian iron-binding glycoprotein with antiviral effects. This preliminary study evaluated 6 months' LF (3 g/day, orally) treatment in 22 human immunodeficiency virus type 1 (HIV-1) vertically infected children. Plasma viral load and CD4+ cell counts were assessed every 3 months; before, during and after LF administration. No significant changes were observed during the pre-treatment period. By 6 months, mean (+/- SD) plasma viral load (log10) declined from 4.54 (+/- 0.65) to 4.28 (+/- 0.60); median percentage CD4+ cell count increased from 21.5% to 24.5%. Two months after treatment discontinuation, mean plasma viral load did not differ significantly from baseline or month 6 levels, but the percentage CD4+ cell count remained significantly higher than the baseline value. LF plus antiretroviral (ARV) therapy was more effective at increasing CD4+ cell count than LF alone. None of the patients showed any new HIV-1-related symptoms at follow-up. LF might be a useful addition to ARV therapy, but further large-scale studies are required.
Seventeen children with vertically acquired hepatitis C virus (HCV) infection were followed from birth for a mean of 104 months. Alanine aminotransferase (ALT) levels were increased significantly at 3 and 6 months of age but were stable thereafter. HCV polymerase chain reaction was positive at 3 months in 16 patients and at 12 months in one patient. Viral load remained stable during follow-up at a mean value of 5.4 +/- 0.4 log10. Mild chronic hepatitis was the most common histopathological feature on liver biopsy, occurring in six of the seven children biopsied at a mean age of 4.0 +/- 2.4 years. Genotype did not seem to be related to the type of liver involvement. The results of this study suggest that vertically acquired HCV infection has a benign course in children, despite the presence of viraemia and persistent alterations in ALT levels.
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Fatty acids (FA) constitute the main component of phospholipids, triglycerides and cholesterol esters. FA are acidic, monocarboxylic linear chains of variable length: short-chain FA (2-4 carbon atoms), medium-chain FA (6-12 carbon atoms), long-chain FA (14-18 carbon atoms), very long-chain FA (derived from parental 18-carbon molecules). They can be further subdivided into saturated (no double bond), monounsaturated (one double bond) and polyunsaturated (two or more double bonds). They are all involved in energetic, metabolic and structural activities. Short-chain FA act as growth factors; medium chain FA are readily available as energy source; saturated long-chain FA constitute a source of energy but may be implicated in the development of the atherosclerotic process; unsaturated long-chain FA include oleic acid and the essential fatty acids (linoleate and linolenate), and are all implicated in fundamental metabolic processes; very-long chain FA are the most characteristic molecules in biologic membranes. From recent works it is clearly established that the physiological role of FA depends on the chain length, and that the very-long chain molecules could determine the quality of human development. A functional classification of FA today must be based not only on the rate of unsaturation, but also (and most importantly) on the chain length.
Fatty acids (FA) are involved in both physiologic and pathologic biochemical pathways. Saturated FA (mainly 12-C and 14-C) are implicated in the development of hypercholesterolemia and early atherogenesis. On the contrary, monounsaturated and polyunsaturated FA (mainly 18:2 n-6 and n-3 very long-chain FA) have been claimed to have hypocholesterolemic and protective effects. In particular, oleic acid (18:1 n-9) seems to lower only the atherogenic fraction of circulating cholesterol (the low-density lipoproteins), leaving immodified the level of the protective fraction (the high-density lipoproteins). Polyunsaturated very long-chain FA have been recently studied for the prevention and therapy of atopic and cutaneous disorders, with conflicting results. In this respect, the 20:4 n-6 acts as proinflammatory agent, while the n-3 very long-chain FA could act as modulators of the immuno-allergic and inflammatory reactions. However, attention must be paid to the high peroxidative potential of the more unsaturated FA. Very recently, VLCFA have been considered for the therapy of some inherited disorders of metabolism. Monounsaturated very long-chain FA have been used in the treatment of x-linked adrenoleukodystrophy. A dietetic deficiency of polyunsaturated very long-chain FA has been observed in treated phenylketonuric children. Some inborn errors of metabolism could represent an useful model for better understanding the biochemistry of FA.
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