Filiberto Donzelli

Plasma and Urine Carnitine Levels During Development

Summary: Plasma and urine free carnitine concentration during human development was evaluated.A positive correlation was found between plasma level and body weight in premature neonates weighing between 1.15 and 1.80 kg.In full terms newborns, the free carnitine concentration (mean ± S.E., 31.2 ± 2.5. nmoles/ml) is similar to that of premature babies with 33 to 36 wk of gestational age (37.5 ± 3.1 nmoles/ml) but significantly lower than that of premature babies aged 30 to 33 wk of gestation (43.0 ± 5.6 nmoles/ml).In the first 2 wk of life, free carnitine level showed a good correlation with age in the full-term newborns.Adult levels are reached by the end of the first 6 months. No sex related difference was observed in any of the different groups during growth.Urinary excretion of free carnitine per day is significantly lower in infants 0 to 3 years old (mean ± S.E., 15.5 ± 1.8 μmoles/24 hr) and in children 3 to 10 years old (115.3 ± 11.4μmoles/24 hr) than in subjects ranging in age from 22 to 70 years (216.9 ± 20.6 μmoles/24 hr).We found no difference between day- and nighttime urinary excretion in newborns and infants.Speculation: The present observations suggest that fetal free carnitine storage mainly occurs during the 30th to 33rd wk of life. Because the fatty acids are the main energy fuel in the postnatal period and their /%oxidation is carnitine dependent, an exogenous source of this substance seems useful to maintain plasma levels similar to those found in the fetus.The lower free carnitine excretion observed in our children group compared to adult may be related to a smaller muscle mass, different kidney excretion, or reduced endogenous synthesis.

Effect of Carnitine on Lipid Metabolism in the Newborn

The effect of carnitine administration on neonatal lipid metabolism was studied during endovenous loading with Intralipid (1 g/kg body weight over a 4-hour period). During a 6-hour period the plasma level of triglycerides, glycerol, free fatty acids (FFA), beta-hydroxybutyrate (beta-OHB), and acetoacetate were monitored in a group of newborns infused with carnitine and compared with a control group infused only with Intralipid. Carnitine administration caused an increased plasma concentration of ketone bodies, probably consequent to an increased rate of FFA mitochondrial beta-oxidation. An increased plasma level of glycerol and FFA was also observed, whereas the triglyceride plasma levels were not different between the two groups. Carnitine administration in the neonatal period seems to act by increasing ketogenesis and lipolysis.

Diagnostic accuracy of pH monitoring in gastro-oesophageal reflux.

One hundred and eleven children admitted with suspected gastro-oesophageal reflux were studied, with 24 hour oesophageal pH monitoring as the first line of investigation. Barium swallow examination, or oesophagoscopy, or both, were carried out only in children with abnormal pH, who subsequently had a trial of 1-12 months medical treatment. All patients were followed up for eight months to two years. A final diagnosis of gastro-oesophageal reflux was made in 41 patients, in all of whom the pH study was abnormal (100% sensitivity). The final diagnosis was different in 70 patients; 66 of these had a normal pH (94% specificity). All children with gastro-oesophageal reflux were treated with drugs. All those with a percentage reflux time of more than 27 and more than 20 episodes of reflux lasting more than 5 minutes failed to improve and needed operation. We conclude that monitoring of the oesophageal pH should be the first line of investigation in patients with gastro-oesophageal reflux and should be used together with clinical data and other investigations, to identify those children who will need operation.

Effect of carnitine on lipid metabolism in the neonate. II. Carnitine addition to lipid infusion during prolonged total parenteral nutrition

The effect of carnitine administration on lipid metabolism and carnitine and acylcarnitine plasma values of newborn infants, given total parenteral nutrition for the first 7 days of life, was studied during a 4-hour infusion of Intralipid. An increase in plasma concentrations of total carnitine, free carnitine, and short-chain and long-chain acylcarnitine was found, but no significant change in triglycerides, free fatty acids, glycerol, or beta-hydroxybutyrate plasma values was noted, as compared with values obtained without carnitine administration. Moreover, the low free carnitine and short-chain and long-chain acylcarnitine plasma levels found in newborn infants after 7 days of total parenteral nutrition did not seem to impair the utilization of infused lipids. The results support the concept that the relation between the carnitine pool and lipid metabolism can be influenced by intravenous glucose infusion. Low carnitine plasma concentrations do not necessarily signify a depletion of body carnitine, and sufficient tissue carnitine concentrations can probably maintain good lipid utilization for an extended period.

Carnitine and the Premature

After birth, the main energy fuel for the newborn is constituted by fat. Carnitine is necessary for the beta-oxidation of long chain fatty acids at the mitochondrial level, and seems also to have a role in the metabolism of the branched-chain amino acids, in ammonia detoxification, and in urea production. Colostrum is particularly rich in carnitine whereas semi-elemental formulae and soy-based formulae contain little or no carnitine. Since the newborn has a low capacity for carnitine biosynthesis, it seems useful to administer L-carnitine to infants on total parenteral nutrition, soy-based or semi-elemental formulae.

Effect of Phototherapy on Hepatic Excretory Function in Newborns as Measured by Bromsulphalein Clearance

BSP clearance from the plasma was studied during phototherapy in a group of jaundice newborns. This therapy did not have any influence on the plasma dye disappearance c curve. The obtained results are consistent with the hypothesis that this therapy causes the photodegradation of bilirubin, and that the increased excretion of unconjugated bilirubin is not mediated by a generalized enhancement of the hepatic output.

A STUDY ON THE EFFICIENCY OF COMMUNITY TO HOSPITAL REFERRAL SYSTEM IN THE CATCHMENT AREA OF DODOMA HOSPITAL, TANZANIA

Unnecessary or delayed hospital admissions are prevented by an efficient referral system.Objectives of the study: Community Health Services (CHS) coverage, accessibility, efficiency, and interaction with the hospital.Method: from March to June 1991 four Dodoma hospital paediatricians, using two structured questionnaires, studied 1,154 consecutive case of children (< 5 yrs) examined at the Outpatients Dept and 796 admitted to the Paediatric Ward.Preliminary results: 1) Outpatients Dept: 84,5% of the children bypassed the CHS. Of them, 73% could have been treated in the CHS without needing to go directly to hospital. 2) Paediatric Ward: 66% of the children bypassed the CHS; 48% of these unreferred patients were admitted to the hospital with delay as compared to only 17% of the previously referred children. This delayed admission was followed by a high mortality rate within 48 hours (12,5%).Conclusion: there is a need to study the factors which influence the flow of patients from the Community to the CHS and ultimately to the Hospital.

127: ALPHA-1-ANTITRYPSIN (A1AT) FECAL CONCENTRATION IN INFANTS WITH ATOPIC DERMATITIS AND IN HEALTHY INFANTS WITH ATOPIC PARENTS

In food allergy the intestinal permeability from the lumen to the gut wall increases, probably due to mucosal damage. No data are available concerning the intestinal permeability from gut wall to lumen (IPGWL). We studied 24 infants (mean age 6 mos; range 4-18) with untreated atopic dermatitis related to food allergy (Rast and/or Prick test positive); 39 healthy infants with at least one atopic parent (mean 1,6 mos;, range 1-4); 25 healthy infants as controls (mean 5,5 mos; range 1-20). As a marker of IPGWL we studied the excretion of endogenous A1AT in random lyophilised fece samples determined by a nephelometric method. Infants with atopic dermatitis showed significantly higher fecal excretion of A1AT than the controls (1,43 ± 0,74 S.D. mg/g dry weight versus 0,74 ± 0,23 S.D. - p<.001). Also in healthy infants with atopic parent(s) the A1AT excretion was significantly increased (1,32±0,88 - p<.001). These babies were followed every three months for the first year of life: 10 of them subsequently became affected with atopic dermatitis. We conclude that in infants with untreated atopic dermatitis there is an increase in IPGWL which seems to begin before the dermatitis develops and could predispose infants to such disease.

ADAPTED AND PRETERM FORMULA: EFFECTS ON GROWTH AND AMINOACIDS METABOLISM IN LOW-BIRTH WEIGHT INFANTS

Two groups of premature infants were fed with isocaloric formulae. One group, 11 babies (b), mean gestational age 32 weeks(w), mean birth weight 1600 gm (g), received a preterm formula. This is an attempt by dietary industries to provide a food closer to the nutritional needs of premature neonates (mean protein intake 3.36 gm/kg/day). The other group (10b, 32w, 1706g) was nourished with an adapted formula, which is recommended for full-term neonates by the ESPGAN (2.88 gm/kg/die). No statistically significant differences were found in the anthropometric parameters between the two groups of infants during this study. Moreover, the time taken to reach the weight of 2100 gm was the same: 30.5 days for the adapted formula fed group and 30.9 days in those fed with the preterm formula. The total length and head circumference at the end of the fourth week were similar in the two groups of infants. The preterm formula fed group presented a higher total blood protein during the second week (5.35 gm/dl versus 5.11-p<0.05 and a higher BUN during the third week (0.13 gm/dl versus 0.08-p<0.01). Serum essential aminoacids/total aminoacids ratio is constantly higher in the infants fed with the richer proteic preterm formula. Our data show no definitive advantages for anthropometric or biochemical parameters regarding the use of the formula studied for the preterm infant in comparison to the adapted formula for the full term infant.

Eating Difficulties in Infancy: An Indication of Psychosomatic Pathology

An infant manages to survive and grow thanks to the care guaranteed him by his environment, represented during the first few months of life principally by the mother who manages to provide for infant’s needs, making use of that capacity of being able to relate —which is a fundamental characteristic of the “Primary Ma-ternal Preoccupation”1. At this point, the father exists only in that he provides affectionate support to the mother during her withdrawal from external interests, and in her investment in her world represented by the new unit as formed by her and her baby. For the infant, his father begins to exist later, when in the symbiotic phase, a distancing from the mother occurs. Then, the infant is able to recognize his father, even if only as an extension of his mother. The definitive recognition of the father is possible after the mother has been accepted as an external object by the aby: at that point, the baby can accept the triangular situation2. Maternal care is, initially, physical care, directed towards the baby’s body and his/her needs. It is really thanks to her support and investment that the process of body-mind integration is possible, which represents the nucleus of the baby’s basic mental organisation3.