Effects of oral L-carnitine supplementation in low-birth-weight premature infants maintained on human milk.

Effects of oral L-carnitine supplementation on fat and protein metabolism have been studied in 20 low-birth-weight premature infants (mean weight at birth 1.519 g, range 1,200-1,880 g) fed with pooled pasteurized human milk. Throughout 7 consecutive days, started at various postnatal ages (range 10-33 days) infants were fed exclusively with milk containing 300 nmol/ml L-carnitine as added supplement. The amount of extra carnitine intake ranged from 42.6 to 72.0 mumol/kg/day. Until day 5 of supplementation there was a continuous increase in the daily urinary excretion of total carnitine, which levelled off thereafter, corresponding approximately to 50% of the extra L-carnitine intake, indicating that a part of the supplement was retained by the body. Total, free and esterified carnitine levels were significantly elevated in the plasma at the end of the study period. The increased levels of acylcarnitines in plasma and urine indicate that the carnitine supplement was taken up by tissues and entered the intermediary metabolism. Plasma triglyceride level was decreased, whereas 3-hydroxybutyrate level was increased at the end of supplementation, indicating an enhanced fat utilization. Plasma and urine analysis also revealed an altered nitrogen handling. There was a marked decrease in plasma urea level as well as a significant fall in the urea and total N excretion, with a trend of decrease in excretion of 3-methylhistidine, suggesting a reduced amino acid and protein catabolism during L-carnitine supplementation.

Oral L-carnitine supplementation in low-birth-weight newborns: a study on neonates requiring combined parenteral and enteral nutrition.

Effect of L-carnitine supplementation on plasma ketone body (KB) and triglyceride (TG) concentrations was studied in ten premature infants requiring combined enteral and parenteral nutrition. At the second week of life (9 to 14 days of age) the infants were randomly divided into two groups. Five of them (plasma carnitine value, 33.77 +/- 2.48 mumol/l; mean +/- SEM) received oral L-carnitine supplementation (60 mumol/kg daily) added to pasteurized pooled human milk for seven consecutive days; additional five (plasma carnitine value, 36.70 +/- 5.19 mumol/l) served as controls. Composition of the daily diet was nearly constant in the study period. On the seventh day, prior to an Intralipid infusion, plasma carnitine and ketone body levels were significantly increased in the supplemented group as compared to controls or to previous values of the same group. In response to lipid infusion the fat load induced ketone body production was significantly higher in the supplemented group as compared to controls, whereas the triglycerides reached higher levels in the control group. It is suggested that L-carnitine supplementation in low-weight newborns promotes ketone body formation from endogenous stores as well as from exogenous fat supply, and thus may enhance triglyceride utilization.

Uptake of beta-hydroxybutyrate, acetoacetate and glucose by the forearm of the newborn infant.

Forearm muscle metabolism was studied in twelve appropriate for gestational age premature infants suffering from respiratory distress. Arterial blood was obtained by puncture of the radial artery as clinically indicated for measuring oxygen tension, and venous blood samples were taken from the same arm's deep brachial vein. This arrangement allowed to study the arterial-deep venous differences of beta-hydroxybutyrate, acetoacetate and glucose in simultaneously taken blood samples. Net muscular uptakes of beta-hydroxybutyrate and glucose were observed, however, in four studies a virtual net production of acetoacetate was found. The arterial-deep venous concentration differences of both ketone bodies correlated positively with their arterial concentration within the 20 to 120 nanomol/ml range. Such a correlation was not observed in glucose utilization. It is concluded that forearm muscles in the neonate take up ketone bodies and this is in part concentration regulated.

Absence of responses in energy metabolism and respiratory quotient to carnitine infusion in premature infants.

Plasma levels of total, free and acylcarnitine, as well as oxygen consumption and respiratory quotient were determined in premature infants maintained at neutral temperature. The effects on these parameters of intravenous infusion of 24 mg/kg/day carnitine were studied. Total, free and acylcarnitine increased and the acyl/free carnitine ratio decreased significantly during the four-hour study period. Resting heat production and respiratory quotient remained practically unchanged throughout the study period, indicating that in the face of carnitine sufficiency exogenous carnitine did not influence whole body heat production and substrate utilization pattern in premature infants. Further examinations in carnitine depleted infants will be required to clarify the regulatory role of carnitine in neonatal fatty acid metabolism and non-shivering thermogenesis.

Perinatal asphyxia and jaundice in newborn infants.

The serum bilirubin concentration was studied in 114 full term and 199 preterm babies suffering from either perinatal asphyxia or idiopathic indirect hyperbilirubinaemia, in order to establish the effect of asphyxia on the serum bilirubin level. Infants with any other disease causing non-physiologic jaundice were excluded. It was found that perinatal asphyxia per se does not exaggerate hyperbilirubinaemia either in full term or in preterm babies. Weight loss correlated significantly with the peak bilirubin concentration in all groups of patients. This would suggest the possible role of feeding and hydration in the genesis of hyperbilirubinaemia.

[Results and problems in the treatment of childhood leukaemia (author's transl)]. / Ergebnisse und Probleme bei der Behandlung der kindlichen Leukämien in Ungarn.

391 children received complex chemotherapy according to uniform treatment schedules, proposed by the Hungarian Study Group for Childhood Leukaemia, which was established in 1971. Survival among the patients showed an increasing tendency: more than 50% of patients with ALL are stille alive 3 years after the beginning of treatment. One patient is in complete remission 9 3/4 years after the establishment of the diagnosis. Two types of maintenance therapy were investigated among the patients entered for this study in 1974. "Pulses" with Vincristine-Prednisolone every second month were found to be more optimal than monthly "pulses".