Acid In Newborns Research Articles

Alterations in the metabolic status of amino acids in newborns of pre-eclampsia women.

Changes in amino acid (AA) levels have been observed in women with pre-eclampsia, but data on their newborns are scarce. This study aimed to evaluate 11 AAs in newborns of 378 PE and 18,666 non-PE pregnant women. We found that the levels of alanine, arginine, glycine, methionine, phenylalanine, valine, and leucine/isoleucine/hydroxyproline in newborns of PE women were significantly higher than those of non-PE women, whereas the level of tyrosine was lower. This different metabolic status deserves further study to determine whether this pattern persists or is likely to increase the risk of future diseases in these newborns.

Пероксисомні біогенні порушення в спектрі синдрому Цельвегера: діагностика, моніторинг і лікування відповідно до рекомендацій глобальної фундації пероксисомних захворювань

Стаття присвячена сучасним принципам діагностики та лікування пероксисомних біогенних порушень у спектрі синдрому Цельвегера відповідно до рекомендацій глобальної фундації пероксисомних захворювань 2016 р. Автори представили клінічні особливості захворювань даного спектра. Ураження нервової, кісткової, сечовидільної, ендокринної, сенсорної систем і зубної емалі є характерною ознакою захворювання. Окремо виділено власне клінічне спостереження, пов’язане з порушенням окислення жирних кислот із дуже довгими ланцюгами в дитини раннього віку. Розділ діагностики захворювань спектра синдрому Цельвегера заснований на світових стандартах і включає маркери β-окислення дуже довголанцюжкових жирних кислот, а також α-окислення пристанової і фітанової кислот, метаболізму піпеколінової кислоти й інших пероксисомних дисфункцій. Остаточний діагноз пероксисомних біогенних порушень у спектрі синдрому Цельвегера на сучасному етапі встановлюється методом генетичного тестування, за допомогою методів секвенування нового покоління для генів PEX. Тяжкість неврологічних розладів і прогредієнтний перебіг захворювання, а також можливість лікування Х-зчепленої адренолейкодистрофії відкрили перспективи скринінгу для Х-зчепленої адренолейкодистрофії (X-ALD), заснованого на комбінації рідинної хроматографії і тандемної мас-спектрометрії (LC-MS/MS), для виявлення підвищених рівнів VLCFA у новонароджених у плямах крові. Клінічний моніторинг із моменту встановлення діагнозу включає корекцію стартових порушень і виявлення симптомів захворювання, що з’являються пізніше.

Plasma lipidome profiling of newborns with antenatal exposure to Zika virus.

The 2015-2016 Zika virus (ZIKV) outbreak in Brazil was remarkably linked to the incidence of microcephaly and other deleterious clinical manifestations, including eye abnormalities, in newborns. It is known that ZIKV targets the placenta, triggering an inflammatory profile that may cause placental insufficiency. Transplacental lipid transport is delicately regulated during pregnancy and deficiency on the delivery of lipids such as arachidonic and docosahexaenoic acids may lead to deficits in both brain and retina during fetal development. Here, plasma lipidome profiles of ZIKV exposed microcephalic and normocephalic newborns were compared to non-infected controls. Our results reveal major alterations in circulating lipids from both ZIKV exposed newborns with and without microcephaly relative to controls. In newborns with microcephaly, the plasma concentrations of hydroxyoctadecadienoic acid (HODE), primarily as 13-HODE isomer, derived from linoleic acid were higher as compared to normocephalic ZIKV exposed newborns and controls. Total HODE concentrations were also positively associated with levels of other oxidized lipids and several circulating free fatty acids in newborns, indicating a possible plasma lipidome signature of microcephaly. Moreover, higher concentrations of lysophosphatidylcholine in ZIKV exposed normocephalic newborns relative to controls suggest a potential disruption of polyunsaturated fatty acids transport across the blood-brain barrier of fetuses. The latter data is particularly important given the neurocognitive and neurodevelopmental abnormalities observed in follow-up studies involving children with antenatal ZIKV exposure, but normocephalic at birth. Taken together, our data reveal that plasma lipidome alterations associated with antenatal exposure to ZIKV could contribute to identification and monitoring of the wide spectrum of clinical phenotypes at birth and further, during childhood.

The frequency of vitamin B12, iron, and folic acid deficiency in the neonatal period and infancy, and the relationship with maternal levels.

Aim:The most important function of vitamin B12 is to accomplish DNA synthesis, which is necessary for cell division and proliferation. Deficiency of vitamin B12 causes megaloblastic anemia, retardation of growth, and delay in neuromotor maturation. Newborns whose mothers have vitamin B12 deficiency are born with low vitamin B12 storages, and are at risk in terms of vitamin B12 deficiency symptoms during infancy. The aim of our study was to investigate the frequency of anemia and deficiency of vitamin B12, folic acid, and iron in pregnant women living in our region, in their newborn babies, and during the infancy period of these babies. Another aim of our study was to investigate the correlation between the levels of these vitamins in newborns and in their mothers.Material and Methods:In our study, 250 pregnant women at 38–42 gestational weeks, who were admitted for delivery to Gynecology and Obstetrics Clinic and their babies with a birth weight over 2500 g were included in the study.Results:We determined that 24.8% of the pregnant women had anemia, 28% had low ferritin levels, 90.4% had vitamin B 12 deficiency, and 22.4% had folic acid deficiency. Some 3.2% of the newborns had anemia, 2.8% had low ferritin levels, and 72.4% had vitamin B12 deficiency. Among the infants who presented for a follow-up visit at 6 months of age, 22.3% had anemia, 14.9% had low ferritin levels, 40.4% had vitamin B12 deficiency, and 1.06% had folic acid deficiency. In addition, we found that the levels of vitamin B12 and folic acid in newborns were related to the levels of vitamin B12 and folic acid in their mothers.Conclusion:Development of low vitamin B12 stores in newborns and the development of vitamin B12 deficiency during infancy, which may result in irreversible complications including neurologic complications, can be prevented by preventing vitamin B12 deficiency during pregnancy.

Position of folic acid in fortification of nutrition in neonatal period

Folic acid is an essential vitamin which has been known in recent 50 years. It plays an important role in period of neurogenesis. The substitution of folic acid is one of the important parts in the complex treatment of anaemia in premature newborns. It is also a component of artificial milk formulae or breast milk following mother’s intake. Fortification of foods with folic acid for population in the world is still discussed. To determine optimal dose of folic acid in premature newborns is difficult. Daily recommended doses of folic acid in infants under the six months were identified. The needs of folic acid in newborns vary. It depends upon the gestational age, body reserves at birth or maternal status of folates during gravidity. On the other hand there is a risk of accumulation of unmetabolised folic acid in circulation of newborns after mandatory folic acid fortification in some countries, which were reported in some studies. The safe upper limits of folic acid intake in premature newborns are not known. In this review article authors inform about the clear positive effect of folic acid in prenatal and neonatal period, but excessive doses of folic acid could present risk of accumulation and possible adverse effects. To follow up these notions further studies are required.

Elevated Bile Acids in Newborns with Biliary Atresia (BA)

Biliary Atresia (BA), a result from inflammatory destruction of the intrahepatic and extrahepatic bile ducts, is a severe hepatobiliary disorder unique to infancy. Early diagnosis and Kasai operation greatly improve the outcome of BA patients, which encourages the development of early screening methods. Using HPLC coupled tandem mass spectrometry, we detected primary bile acids content in dried blood spots obtained from 8 BA infants, 17 neonatal jaundice and 292 comparison infants at 3–4 days of life. Taurocholate (TC) was significantly elevated in biliary atresia infants (0.98±0.62 µmol/L) compared to neonatal jaundice (0.47±0.30 µmol/L) and comparison infants (0.43±0.40 µmol/L), with p = 0.0231 and p = 0.0016 respectively. The area under receiver operating characteristic (ROC) curve for TC to discriminate BA and comparison infants was 0.82 (95% confidence interval: 0.72–0.92). A cutoff of 0.63 µmol/L produced a sensitivity of 79.1% and specificity of 62.5%. The concentrations of total bile acids were also raised significantly in BA compared to comparison infants (6.62±3.89 µmol/L vs 3.81±3.06 µmol/L, p = 0.0162), with the area under ROC curve of 0.75 (95% confidence interval: 0.61–0.89). No significant difference was found between the bile acids of neonatal jaundice and that of comparison infants. The early increase of bile acids indicates the presentation of BA in the immediate newborn period and the possibility of TC as newborn screening marker.

Comparison of Blood-Sparing Efficacy of ε-Aminocaproic Acid and Tranexamic Acid in Newborns Undergoing Cardiac Surgery

ε-Aminocaproic acid (EACA) and tranexamic acid (TXA) are used for antifibrinolytic therapy in neonates undergoing cardiac surgery, although data directly comparing their blood-sparing efficacy are not yet available. We compared two consecutive cohorts of neonates for the effect of these two medications on perioperative blood loss and allogeneic transfusions. Data from the EACA group (n = 77) were collected over a 12-month period; data from the tranexamic acid group (n = 28) were collected over a 5-month period. Blood loss, rate of reoperation due to bleeding, and transfusion requirements were measured. There was no significant difference in blood loss at 6 hours (EACA 24 [17-30] mL/kg [median (interquartile range)] vs. TXA 20 [11-34] mL/kg, P = 0.491), at 12 hours (EACA 31 [22-38] mL/kg vs. TXA 27 [19-43] ml/kg, P = 0.496) or at 24 hours postoperatively (EACA 41 [31-47] mL/kg vs. TXA 39 [27-60] mL/kg; P = 0.625) or transfusion of blood products. ε-Aminocaproic acid and tranexamic acid are equally effective with respect to perioperative blood loss and transfusion requirements in newborns undergoing cardiac surgery.

Plasma Levels of Conjugated Bile Acids in Newborns After a Short Period of Parenteral Nutrition

Patients receiving parenteral nutrition (PN) frequently exhibit liver dysfunction. The authors previously reported that plant sterols of lipid emulsions added to the nutritional solution of newborns receiving PN accumulate in plasma and cell membranes and may contribute to the development of cholestasis. Conjugated bile acids (BA) have been shown to be useful markers of cholestasis. Plasma levels of several BA in newborns were quantified after administration of PN for less than 2 weeks. Plasma samples from 15 healthy control infants (CN), 22 patients who had received PN for 3-15 days (T1), and 9 patients scheduled to receive PN (T0) were analyzed. After a simple extraction procedure, plasma BA were analyzed by liquid chromatography-tandem mass spectrometry using a quantitative isotope dilution method. The concentrations of BA did not differ significantly between controls and patients before PN (CN vs T0), with the exception of glycocholic acid (GCA; 2.30 ± 2.60 µM vs 7.29 ± 5.39 µM, respectively). There was a significant difference in several BA between controls and patients after PN (2.30 ± 2.60 µM vs 7.61 ± 6.46 µM for GCA, respectively; 4.02 ± 3.49 µM vs 11.88 ± 11.05 µM for taurocholic acid [TCA], respectively; and 4.81 ± 3.49 µM vs 13.58 ± 12.22 µM for taurochenodeoxycholic + taurodeoxycholic + tauroursodeoxycholic acids [TCDCA+TDCA+TUDCA], respectively). In newborns receiving PN, a short period of PN is associated with an early increase of some conjugated BA. These results suggest that GCA, TCA, and TCDCA+TDCA+TUDCA levels could be used as early markers of PN-related cholestasis.

Dietary n−3 fatty acid restriction during gestation in rats: neuronal cell body and growth-cone fatty acids

Growth cones are membrane-rich structures found at the distal end of growing axons and are the predecessors of the synaptic membranes of nerve endings. This study examined whether n-3 fatty acid restriction during gestation in rats alters the composition of growth cone and neuronal cell body membrane fatty acids in newborns. Female rats were fed a standard control diet containing soy oil (8% of fatty acids as 18:3n-3 by wt) or a semisynthetic n-3 fatty acid-deficient diet with safflower oil (0.3% of fatty acids as 18:3n-3 by wt) throughout normal pregnancy. Experiments were conducted on postnatal day 2 to minimize the potential for contamination from synaptic membranes and glial cells. Dietary n-3 fatty acid restriction resulted in lower docosahexaenoic acid (DHA) concentrations and a corresponding higher docosapentaenoic acid concentration in neuronal growth cones, but had no effects on neuronal cell body fatty acid concentrations. These studies suggest that accretion of DHA in growth cones, but not neuronal cell bodies, is affected by n-3 fatty acid restriction during gestation. Differences in other fatty acids or components between the semisynthetic and the standard diet, however, could have been involved in the effects on growth-cone DHA content. The results also provide evidence to suggest that the addition of new membrane fatty acids to neurons during development occurs along the shaft of the axon or at the growth cone, rather than originating at the cell body.

Diet-related reference values for plasma amino acids in newborns measured by reversed-phase HPLC

We have measured by reversed-phase HPLC concentrations of amino acids in plasma in groups of 80 normal appropriate-weight term babies fed from birth either a casein formula (WhiteCap SMA, n = 26), a whey formula (Gold Cap SMA, n = 26), or breast milk (n = 28). They were studied from day 11 to week 15 postpartum. The trend was towards an increase in amino acid concentrations in plasma with age, more marked in formula-fed than in breast-fed infants. Reference values were derived for each group. Both formula-fed groups showed several differences from the breast-fed group. Detailed examination indicated that tyrosine, phenylalanine, and methionine concentrations were increased in the casein-fed group greater than 20% of the time, but only threonine was similarly increased in the whey-fed group. Other amino acids, different ones for each formula group, were increased less frequently. There were no consistent correlations with any aspect of infant growth. Appropriate reference values are important for interpreting amino acid concentrations in plasma from newborns and for evaluating the effects of any future dietary modifications to infant formulas. HPLC analysis provides a suitable highly sensitive method for undertaking such studies.

Pharmacoklnetic or clavulanic acid in newborns and children

Pharmacokinetic of clavulanic acid (C.A.) was studied in newborns and children in combination with amoxicillin (Augmentin) or Ticarcillin (Timentin). Augmentin was administered by 30 mn IV. infusions:100 mg amox/10 mg CA/kg/day before 3 months, 100-200 mg amox./10-20mg CA/kg/day after 3 months (4 infusions in children, 2 infusions in newborn period). Timentin was given identically : 250 mg TIC/10 mg CA/kg/day before 3 months, 300 mg TIC/20 mg CA/kg/day after 3 months. 8 newborns and 6 children were treated by Augmentin, 3 newborns and 10 children by Timentin. Serum levels were determined by microbiological method. Results are in the table I:These results suggest the usefulness of 4 infusions/day, to maintain inhibitor serum level of C.A. active against beta-lactamases.

Serum bile acids in newborns and children.

A specific and sensitive radioimmunoassay for cholic, chenodeoxycholic, and lithocholic acid conjugates and for sulfolithocholylglycine was used to measure serum bile acids (BA) in infants and children. Elevated cholic and chenodeoxycholic acid values were observed in the first year of life in fasting infants. Newborn babies presented high levels of primary BA not correlated with those of the mothers. In premature newborn babies who had not yet been fed, meal induced a considerable reduction in the primary BA levels in serum. In normally fed babies, meal induced a significant increase in the primary BA levels in serum. These data suggest a progressive maturity throughout the first year of life of the serum BA determinants, i.e., gallbladder emptying, intestinal motility and absorption, and hepatic uptake.

A rapid and sensitive method for the determination of short chain fatty acids in serum

A gas Chromatographie method is described for the rapid detection of elevated amounts of short chain fatty acids in small samples of serum. About 2 μl of acidified serum is brought directly onto the column. The temperature-programmed gas Chromatographie system permits a good and reproducible separation of the short chain fatty acids. The procedure takes about 45 min. Interference of other compounds normally occurring in serum could not be observed. Analysis of hydrolyzed urine (by the same gas Chromatographie procedure) may be applied to determine the excretion of short chain fatty acids during periods of remission. The small amount of serum needed and the speed of the method makes this method especially useful for the diagnosis of acidemias and acidurias, due to overproduction of volatile, short chain fatty acids in newborns.

Folate metabolism in newborns and during early infancy. I. Absorption of pteroylglutamic (folic) acid in newborns.

Folate Metabolism in Newborns and during Early Infancy: I. Absorption of Pteroylglutamic (Folic) Acid in Newborns