I . Mn and Cu intake and retention in twenty full-term infants and six preterm infants were studied on the basis of 72 h balances. The age of the infants was 2-16 weeks and the gestational age of the preterm infants (triplets) 34 and 36 weeks. Three nutrition schemes were pursued : breast-fed, formula-fed with unsupplemented adapted formula and formula-fed with trace element supplementation.2. The mean M n concentration of all breast-milk samples (n 2339) was 6.2 pg/l. The two formulas had similar Mn concentrations (77 and 99 pg/I) but had different Fe, Cu (121 and 619 ,ug/l), Zn and I contents. The mean Cu concentration in mother's milk was 833 yg/l.3 . The following mean daily Mn intakes and retentions (pg/kg) respectively were measured: breast-fed fullterm 1.06 (SD 0.43) and 0.43 (SD 0.65), formula-fed full-term 14.2 (SD 3.1) and 2.8 (SD 4%), formula-fed preterm 15.0 (SD 2.2) and 0.06 (SD 5.87). The results for Cu were 114.5 (SD 22.3) and 88.0 (SD 46.5) pg/kg in breast-fed, 19-8 (SD 4.2) and 4.6 (-11.5-9.6) in the unsupplemented formula-fed and 106.4 (SD 18.9) and 55.5 (SD 20.3) in the supplemented formula full-term infant group. No significant influence of the trace element contents of the formulas on the relative retention of Mn or Cu was found.4. Young preterm infants, and to some degree young full-term infants, often had negative Mn balances caused by a high faccal excretion. The formulas with a Mn concentration below 100 pg/l gave a sufficient supply of Mn. Preterm infants fed on the unsupplemented formula had a marginal Cu supply and their first balances were negative (-3.8 (SD 1.8) pglkg).5. In accordance with the estimated safe and adequate daily dietary intakes (recommended dietary allowances), formula-fed infants receive much more Mn than breast-fed infants and their absolute retention is higher.6. Cu from breast-milk had a significantly better biological availability than that from cow's milk formula. If retentions similar to those in breast-fed infants are intended, we conclude, therefore, that cow's milk formula should be fortified with Cu up to a level of at least 600 pg/l.The biological relevance of Mn was noted in poultry and rats showing defective calcification of the otoliths (resulting in ataxia) and the skeleton and other defects based on a depressed mucopolysaccharide synthesis (Hurley, 1985). Furthermore, Mn-deficient rats have second-generation offspring which show an abnormal glucose tolerance test, possibly caused by decreased insulin output (Hurley, 1985). Mn deficiency in man has been observed only once in a patient under parenteral nutrition (Doisy, 1974). He suffered from a prolonged prothrombin clotting time. In newborns and infants no symptoms of deficiency have yet been identified. The vitamin K-dependent coagulopathy in breast-fed infants, however, could be hypothetically connected to Mn deficiency by low Mn intake in these children. At this age it should be relatively easy to diagnose a deficiency, as body-weightrelated increase of tissues and the need for nutrients are highe...
Urinary Hypoxanthine, Xanthine and Uric Acid Excretion in Newborn Infants with Perinatal Complications. Acta Paediatr Scand, 66: 713, 1977.-The concentration of hypoxanthine, xanthine and uric acid in the first 24-h urine of 105 newborn infants was measured densitometrically by thin-layer chromatography. 45 of them had moderate or severe perinatal complications. Among these newborns, 26 infants with perinatal complications (58 %) and 4 infants without perinatal complications (7 %) had an elevated urinary excretion rate of hypoxanthine. Urinary xanthine was not increased, uric acid was slightly higher in the group of infants with perinatal complications. It seems that a crucial mark is involved, if the rate of hypoxanthine exceeds 15% of the total urinary oxypurine excretion, or, if related to urinary creatinine, more than 0.075 pmol hypoxanthine/pmol creatinine. Apparently, with hypoxic newborns increased values of urinary hypoxanthine excretion can be used to quantify the lack of oxygen retrospectively.
Serum creatinine, uric acid, and hypoxanthine and xanthine concentrations were determined in 17 mother-infant pairs at the time of delivery. Creatinine and uric acid levels were nearly similar, but hypoxanthine and xanthine were more than twice as high in the blood of the infants than in the blood of their mothers. In the same newborns the urinary excretion of creatinine, uric acid, hypoxanthine and xanthine, uracil, cAMP, and cGMP was measured on the first and fourth day of life. Creatinine, uracil, and cAMP increased, hypoxanthine and xanthine, and cGMP decreased, whereas the output of uric acid was nearly the same on both days. Correlations of the excreted substances to each other were calculated.
Iron balance studies were performed in 17 full term male infants from their 3rd until their 17th week of life. The balance studies were made in the infant's home and comprised 5 periods with an interval of 3-4 weeks, each consisting of three 24-hour collections. Ten infants were breast-fed, 3 received an adapted infant formula (P1, iron content 1.1 mg/l) and 4 were given the same formula enriched with iron, copper and zinc (P2, iron content 10.35 mg/l). From the 3rd to the 17th week of life the breast-fed infants got a mean iron intake of 0.2 mg/kg body weight X 3 days and they retained 0.09 mg/kg b.w. X 3 days. The P1 group received 0.48 and 0.47 mg/kg b.w. X 3 days and retained -0.01 and -0.5 mg iron/kg b.w. X 3 days, while the P2 group had an intake from 5.04 to 6.38 mg b.w. X 3 days and retained between 1.13 and 3.66 mg iron/kg b.w. X 3 days. Comparing the 3 groups it can be concluded that the P1 group retained definitely less iron than the breast-fed group, whereas the P2 group retained 12 to 40 times more iron than the breast-fed babies.
Iron balance studies were performed in 16 term infants from their 3rd until their 17th week of life. The balance studies were performed at home and comprised five periods with an interval of 3 to 4 weeks, each consisting of three 24-hour collections of milk and stool samples. Seven infants were fed an adapted infant formula supplemented with bovine lactoferrin (100 mg/100 ml) and nine received the same formula without lactoferrin. The lactoferrin supplemented group received 169 micrograms iron/kg b.w. x day and retained 63 micrograms/kg b.w. x day. The mean iron intake of infants fed with the adapted formula without supplementation of lactoferrin was 118 micrograms/kg b.w. x day. The retention of iron was 43 micrograms/kg b.w. x day. Mean percentage retention of iron in the supplemented group was 36%, in the non-supplemented group 28%.
This is apparently the first report on connatal hemihypertrophy with malignant pheochromocytoma. The coincidence of hemihypertrophy with other diseases, particularly neuroectodermal dysplasias on the one hand and the frequent association of neuroectodermal dysplasias with pheochromocytoma on the other, are emphasized. Furthermore, basically known particularities of this case as malignancy of the tumor, the unusual size of the tumor in children, and the normal catecholamine levels in serum as well as the normal excretion of vanillylmandelic acid are discussed.
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