Blood levels of total carnitine and lipid utilization with and without carnitine supplementation in newborn infants

Postnatal changes of the plasma carnitine level were compared in orally and parenterally fed newborn infants. As expected, in contrast to the increasing plasma level of carnitine in infants fed with human milk, a gradual and significant fall was observed during parenteral feeding. Next, the effect of carnitine supplementation on the elimination rate of an Intralipid load was tested. The increased disappearance rate of triglycerides associated with the exogenously administered carnitine suggested an increased lipid utilization during the carnitine supplemented period of parenteral nutrition.     

L-carnitine supplementation of a soybean-based formula in early infancy: plasma and urine levels of carnitine and acylcarnitines

The absence of carnitine in the diet of normal infants results in marked reduction of plasma carnitine levels. In order to evaluate the effects of L-carnitine supplementation of soybean formula, plasma and urine levels of free carnitine and acylcarnitine were compared in infants receiving carnitine-free soybean protein-based formula and the same formula supplemented with 50 and 250 nmol/ml L-carnitine. In infants receiving soybean formula with 50 nmol/ml L-carnitine, the plasma levels of free carnitine were not significantly different from those in infants receiving formula with 250 nmol/ml L-carnitine; however, urine levels of free carnitine were significantly increased when the infants received formula with 250 nmol/ml L-carnitine. In normal full-term infants, supplementation of soybean formula with 50 nmol/ml L-carnitine was sufficient to maintain normal plasma levels that were comparable to breast-fed infants.     

Plasma carnitine levels in preterm infants with respiratory distress syndrome

BACKGROUND: Antenatal carnitine administration has been shown to induce fetal lung maturity by increasing pulmonary surfactant in animal and human studies. In this study, the aim was to investigate the status of carnitine in maternal and neonatal plasma of preterm infants with respiratory distress syndrome (RDS) in the first hours of life. METHODS: Maternal plasma carnitine levels were determined before delivery and neonatal plasma carnitine levels were determined within 2 h of birth in preterm infants (< 34 weeks gestational age) who developed RDS in the first 6 h of life and in the control group. RESULTS: The mean neonatal plasma free carnitine level was significantly lower in preterm infants with RDS than in the control group (28.3 +/- 8.8 micromol/L and 36.9 +/- 18.4 micromol/L, respectively; P < 0.05) while the mean maternal plasma-free carnitine levels were similar in both groups. CONCLUSIONS: Low neonatal plasma carnitine levels in preterm infants with RDS may be due to decreased maternal-fetal transfer of carnitine or to increased consumption of carnitine in fetal lung tissue for surfactant synthesis. This could be a contributing factor in the pathogenesis of respiratory distress syndrome in preterm infants.     

Serum total and free carnitine levels in children with asthma

Background  Serum carnitine is decreased in recurrent pulmonary infections. We aimed to evaluate serum carnitine levels in asthmatic children. Methods  Study group consisted of children with stable asthma and those with acute asthma attacks, while control group included healthy children. Attack severity was determined by the pulmonary score system. Total and free carnitine levels were studied in one blood sample from the control group and stable asthmatics and in two samples from children with acute asthma exacerbation during and after the attack. Results  All the 40 patients in the study group had moderate asthma including 30 with acute attack (13 mild and 17 moderate) and 10 with stable asthma. Carnitine levels were significantly lower in acute attack asthmatics than in the stable asthmatics and controls, while there was no significant difference between the latter two groups. Carnitine levels were not different between asthmatics with mild and moderate attack, and were similar during and after an acute attack. Conclusions  Serum carnitine levels decrease in children with moderate asthma during exacerbation of asthma and shortly thereafter. Further studies are needed to evaluate the effect of carnitine treatment on serum carnitine level.     

Serum-free carnitine levels in children with heart failure.

The serum-free carnitine (SFC) levels of 91 children with heart failure (HF) and of a control group consisting of 30 healthy children were measured. Twenty-four of 91 children with HF were administered oral L-carnitine. The mean SFC level of children with HF (20.16 +/- 0.30 nmol/l) was significantly lower than that of the control group (38.98 +/- 0.79 nmol/ml) (p < 0.01). Mean SFC levels of 24 patients, after L-carnitine administration, increased significantly (p < 0.01). Patients administered L-carnitine displayed a marked difference in time taken for clinical improvement compared with those not given oral L-carnitine.     

缺铁性贫血患儿血清胃泌素水平变化及“铁维生素C叶酸速溶冲剂”的疗效观察

目的：探讨缺铁性贫血(IDA)儿童胃泌素水平的变化,观察“铁、维生素C、叶酸速溶冲剂”对IDA的治疗作用。方法：34例IDA患儿每天口服1包“铁、维生素C、叶酸速溶冲剂”(含元素铁10 mg),共12周,治疗前后检测Hb,ZPP,SF及血清胃泌素并与对照组作比较。结果：IDA患儿胃泌素水平明显高于对照值(P<0.01),治疗后Hb,SF均显著升高(P<0.01),胃泌素水平较治疗前明显下降(P<0.01),34例患儿治疗后精神食欲明显改善,无1例中途停药,所有患儿均未出现胃肠道副作用。结论：IDA患儿胃泌素水平增高可能与铁缺乏引起的胃粘膜萎缩有关,“铁、维生素C、叶酸速溶冲剂”治疗IDA具有依从性好、疗效确切、无副作用、服用方便等特点。     

Glycosylated haemoglobin levels in children with protein-energy malnutrition.

Fasting blood glucose, serum protein and glycosylated haemoglobin level (HbA1c) were determined in 50 children (aged 1-5 years) suffering from protein-energy malnutrition and in 25 healthy and nutritionally normal children of the same age group. It was observed that HbA1c correlated well with the blood glucose values of the children. It was also observed that they had significantly higher values of HbA1c than the controls, indicating the existence in them of glucose intolerance. Long-term monitoring of the glycaemic status is therefore suggested as a means of assessing any relationship between glycosylated haemoglobin and impaired pancreatic function in such patients.     

Serum free carnitine levels in children with recurrent pulmonary infections

The serum free carnitine levels of 33 children with recurrent pulmonary infection and 30 healthy children were measured and found to be 26.12 ± 0.98 nmol/mL and that of the control group 38.98 ± 0.79 nmol/mL on the average. The mean free carnitine level was statistically determined to be significantly lower when compared with that of the control group (P < 0.01). The results indicate that oral L-carnitine therapy is recommended for pediatric patients with recurrent pulmonary infection.     

Effect of carnitine supplementation on cardiac function in hemodialyzed children

Thirteen carnitine-deficient children (mean age, 16.1 ±2.56 years) on a three-times-weekly hemodialysis program for at least 1 year, and 11 healthy age matched children were involved in the study. All the patients had stable blood pressure and hemoglobin (Hb) levels with a maintenance dose of erythropoetin and none were digitalized. The total carnitine (TC) and free carnitine (FC) plasma levels were sampled prior to hemodialysis (HD) before and after 3 months of carnitine supplementation. A free carnitine (FC) to acylcarnitine (AC) ratio less than 4 was defined as carnitine deficiency. Intravenous L-carnitine was injected at a dose of 20–4.0 mg/kg three times weekly at the end of each dialysis session for a 3-month period. Echocardiographic examination was performed the day following HD, before and after carnitine treatment. Systolic and diastolic functions of the left ventricle, including the ejection fraction, were measured. Almost all the parameters were significantly different in controls and hemodiaiyzed patients. In carnitine-deficient hemodiaiyzed patients. 3 months of L-carnitine supplementation resulted in a significant increase in blood carnitine levels and the FC/AC ratio, but this was not associated with any significant improvement of cardiac function. Furthermore no significant changes were observed in plasma triglycerides, total cholesterol or other lipoprotein parameters before or after carnitine supplementation. Although there was a moderate increase in mean hematocrit (Hct) and Hb levels, these also did not reach statistically significant levels. These results suggest that the 3 months of carnitine supplementation is not sufficient to ameliorate cardiac function or increase Hb levels in children.     

Plasma somatomedin and growth hormone values in children with protein-calorie malnutrition.

This study was undertaken to define the interrelationships of somatomedin, growth hormone, and an inhibitor of SM in protein-calorie malnutrition. Twenty-seven patients, eight to 60 months of age, were studied. Twenty-one well-nourished children acted as controls. SM was significantly depressed at Days 2 and 8 (p less than 0.01) but was not different from controls at Day 29 and 50. In ten out of 27 Day 2 samples and six out of 27 Day 8 samples an inhibitor was identified. When SM values were compared to simultaneous hGH levels, there was an inverse relationship. The low SM levels in the face of markedly elevated hGH levels suggests a functional block in the synthesis and/or release of SM.     

Alpha 2 HS-glycoprotein levels in children with protein-energy malnutrition and infections

Serum alpha 2 HS-glycoprotein levels were studied, using the single-radial immunodiffusion method, in 39 severely malnourished children (22 kwashiorkor, 17 marasmus) of whom 15 (8 kwashiorkor, 7 marasmus) were infected and 24 (14 kwashiorkor, 10 marasmus) had no signs of infection. Thirty-two well-nourished children, 16 of whom were infected, served as controls. The uninfected controls had higher serum alpha 2 HS-glycoprotein levels than the uninfected kwashiorkor (82.0 +/- 14.7; 57.0 +/- 15.8; p less than 0.001) and marasmic children (82.0 +/- 14.7; 45.0 +/- 13.9; p less than 0.001). Infection was associated with a significant decrease in the mean serum alpha 2 HS-glycoprotein levels in the well-nourished (82.0 +/- 14.7; 57.0 +/- 7.7; p less than 0.001) and kwashiorkor (57.0 +/- 15.8; 34.0 +/- 20.4; p less than 0.02) children, while no such decrease was observed in the marasmic children. While the mean serum glycoprotein level in the infected controls was higher than that in the infected kwashiorkor children (57.0 +/- 7.7; 34.0 +/- 20.4; p less than 0.02), it was comparable to that in the infected marasmic children (57.0 +/- 7.7; 50.0 +/- 11.6; p greater than 0.05). Furthermore, the mean alpha 2 HS-glycoprotein level in the infected malnourished children was higher in marasmus than in kwashiorkor (50.0 +/- 11.6; 34.0 +/- 20.4; p greater than 0.05); this difference was, however, not statistically significant.(ABSTRACT TRUNCATED AT 250 WORDS)     

Effect of chronic valproic acid treatment on plasma and urine carnitine levels in children: decreased urinary excretion

Plasma levels and urinary carnitine excretion rates were determined in children treated with valproic acid (n = 11) and in age and sex matched controls (n = 11). Urine was collected throughout two consecutive 24 h periods in both groups, and blood samples were taken on the first day of collection after an overnight fast. The plasma level of total and free carnitine was significantly lower in the treated group (24.3 +/- 2.2 vs 34.9 +/- 2.4 and 16.8 +/- 1.8 vs 26.5 +/- 2.1 nanomol/ml; values are means +/- SEM), while there was no significant alteration in the acylcarnitine fraction. In the treated group of children a significant reduction was found in the plasma beta-hydroxybutyrate level indicating a limited fatty acid utilization (23.2 +/- 2.5 vs 81.9 +/- 7.8 nanomol/ml). Urinary total and free carnitine decreased from 286.4 +/- 57.8 to 120.8 +/- 18.2 and from 154.3 +/- 33.6 to 21.2 +/- 5.8 mumol/day, respectively; the acyled fraction was not significantly reduced. In one child, urinary carnitine excretion was followed during the first ten days of treatment. After the 2nd day a decrease of the total and free fraction was observed, confirming previous data obtained during chronic VPA treatment. It has been concluded that the decreased plasma carnitine associated with chronic VPA treatment is not a result of an increased excretion rate, but more likely the consequence of a relatively insufficient endogenous carnitine synthesis. The decreased plasma BOB level probably due to limited fatty acid utilization might also be a metabolic consequence of depressed carnitine concentration.     

Leucocyte ascorbic acid levels in Nigerian children with protein-energy malnutrition

Leucocyte ascorbic acid (LAA) levels were estimated in 26 Nigerian infants and preschool children with protein energy malnutrition (PEM) in order to ascertain their ascorbic acid status. The children included eight with kwashiorkor, 12 with marasmus and six with marasmic kwashiorkor. The mean (SD) LAA level of 13.7 (8.2) micrograms/10(8) leucocytes in the malnourished children did not vary significantly from the mean level of 14.4 (8.8) micrograms/10(8) leucocytes found in 26 age-matched controls, and the values were unrelated to the presence or absence of megaloblastic change in the 14 bone marrows examined. It was concluded that ascorbic acid deficiency was not prevalent amongst children with PEM in Lagos, and was therefore not usually contributory to the anaemia, megaloblastic changes or other features of the syndrome seen in Nigerian children in Lagos.     

Serum carnitine levels in children with iron-deficiency anemia with or without pica

Carnitine is ingested through animal-derived foods as well as synthesized in vivo. It plays an important role in the energy metabolism of many tissues. Iron acts as a co-factor for the synthesis of carnitine. However, the importance of iron deficiency as a cause of secondary carnitine deficiency is not well established. The aim of this study was to investigate the serum levels of carnitine in children with iron-deficiency anemia compared to those of healthy children and to determine if serum carnitine levels in with or without pica differ. The mean serum carnitine concentration in the iron-deficiency group was significantly lower than that in healthy children (12.44+/- 5.09 and 32.48 +/- 7.92 micromol/L, respectively, p < .001). In the iron-deficient group, serum carnitine levels, ferritin levels, and other hematological parameters were lowest in patients with pica (p < .001). Pearson correlation test indicated a positive correlation between serum carnitine and ferritin levels in iron-deficient patients. Based on the evidence about the effect of low iron on carnitine stores in animal studies, the authors propose that low serum carnitine levels in these children may be secondary to iron-deficiency anemia. However, further large-scale studies are needed to establish the frequency of carnitine deficiency in children with iron-deficiency anemia.     

Preliminary results on blood carnitine levels in children with hepatic pathology

A deficiency of exogenous and endogenous carnitine is present in those pathologies in which the most important clinical sign is represented by weakness and steatosis. We have studied the serum levels of carnitine in 14 children with hepatic disease (8 with acute HAV hepatitis, 2 with acute HBV hepatitis, 2 with toxic hepatitis, 2 with chronic hepatitis). In patients with acute, infective and toxic hepatitis we have found levels of carnitine (25.71 +/- 2.14 nM/ml) below normal (50.87 +/- 1.46 nM/ml). In 5 cases we have performed two blood tests, at admittance to the hospital and at the end of the illness. The variability in carnitine levels in these two blood exams shows a clear correlation with clinical improvement, decrease in aminotransferase and increase in serum carnitine. In chronic hepatitis we have found normal levels of carnitine. Probably it is correlated with the absence of steatosis seen at hepatic biopsy.     

长期肠外营养新生儿血浆肉碱水平分析

目的 了解长期全肠外营养的足月新生儿血浆肉碱浓度变化和相关的代谢改变.方法 需全肠外营养支持的新生儿12例(男4例,女8例),采用同一肠外营养方案,于肠外营养支持第1天和最后1天测定血浆肉碱、肝功能和血脂水平.结果 最后1天与第1天比较血浆肉碱的浓度明显下降(29.3μmol/L对32.2 μmol/L,P=0.021);肝功能中AIJT、AKP显著上升(ALT:22.8 IU/L对12.3 IU/L,P:0.020:AKP:191.5 μmol/L对125.5μmol/L,P=0.043);血甘油三酯明显上升(1.17 mmol/L对0.65mmol/L,P=0.035).结论 长期接受不含肉碱的全肠外营养将影响血浆肉碱的浓度,导致血脂和肝功能变化.     

Plasma alpha-tocopherol (vitamin E) levels and tocopherol-lipid ratio among children with protein-energy malnutrition (PEM)

The fat-soluble vitamin E (alpha-tocopherol) status of 47 malnourished children was assessed and compared with that of a control group of ten age-matched normal children. Plasma levels of alpha-tocopherol, total lipid and the ratio of alpha-tocopherol to total lipid were determined. The plasma vitamin E level was low, with a value of less than 11.61 mumol/l (500 micrograms/dl) in 40 (85%) of the malnourished children while the remaining seven (15%) children had values that ranged between 11.61 and 17.20 mumol/l (500-741 micrograms/dl). On the other hand, the tocopherol/total lipid ratio was less than 0.8 mg/g of total lipid in only seven of the malnourished children. The remaining 40 (85%) subjects had values that ranged between 0.8 and 1.96 mg/g of total lipid, whereas in the control group, both the plasma tocopherol levels and the tocopherol/lipid ratio were greater than 11.61 mumol/l (500 micrograms/dl) and 0.8 mg/g of plasma total lipid respectively.     

Free carnitine levels in children with steroid–sensitive nephrotic syndrome

BACKGROUND: Carnitine transports long-chain fatty acids across the inner mitochondrial membrane. Carnitine metabolism is disturbed in some renal diseases, such as chronic renal failure. Previous studies have shown that children had normal serum free carnitine (FC) and total carnitine levels in idiopathic nephrotic syndrome, IgA nephropathy, non-IgA nephropathy and focal segmental glomerulosclerosis. The aim of the present study was to determine FC concentrations in plasma and urine during acute and remission periods of steroid-sensitive nephrotic syndrome (SSNS) and its association with hyperlipidemia. METHODS: Plasma and urinary FC concentrations were assayed in 15 children with SSNS in acute and in 16 children in remission period. Six of them were followed-up longitudinally in both periods. RESULTS: Plasma FC concentrations were significantly higher in the acute period of the disease than in the remission period and of the controls. The patients had lower FC levels in the remission period as compared to the controls. Urinary FC concentration was decreased in acute disease period when compared to the remission period and the controls. The FC concentrations in plasma and urine did not correlate with each other. Plasma FC exhibited significant positive correlation with low-density lipoprotein cholesterol, total cholesterol and trygliceride, but negative correlation with high-density lipoprotein cholesterol. CONCLUSIONS: The present study showed disturbed FC concentration in SSNS. Increased plasma and decreased urinary FC levels in acute disease might be associated with its altered renal handling or some extrarenal factors such as hyperlipidemia.