Elevated plasma bile acids in hypergalactosaemic neonates: a diagnostic clue to portosystemic shunts

To determine whether plasma levels of total bile acids may provide a useful index for hypergalactosaemia due to porto-systemic shunts, these levels were determined in hypergalactosaemic neonates. Increased levels were found in all cases with portohepatic venous or portocaval shunts. The levels of both total bile acids and galactose were normalized when the shunts disappeared on the echograms. Both bile acids and galactose are almost completely absorbed by the liver via the first portal blood passage. Portosystemic shunts contribute to elevated levels of bile acids and galactose. Conclusion Increased plasma levels of total bile acids serve as a diagnostic clue to the presence of porto systemic shunts in neonates with hypergalactosaemia. Received: 22 November 1996 / Accepted: 14 April 1997     

The essential fatty acids omega-6 and omega-3: from their discovery to their use in therapy

In 1929 Burr and Burr discovered the essential fatty acids omega-6 and omega-3. Since then, researchers have shown a growing interest in unsaturated essential fatty acids as they form the framework for the organism's cell membranes, particularly the neurones in the brain, are involved in the energy-transformation process, regulate the information flows between cells. Polyunsaturated fatty acids are also precursors of 'hormonal' molecules, often with opposing effects, prostaglandins, prostacyclins, thromboxanes, leukotrienes, lipossines, resolvines, protectines that regulate immunity, platelet aggregation, inflammation, etc. They showed that raised levels of polyunsaturated fatty acids omega-3 in tissue correlate with a reduced incidence of degenerative cardiovascular disease, some mental illnesses such as depression, and neuro-degenerative diseases such as Alzheimer's. The balance between omega-3 and omega-6 acids allows the cell membranes to develop with exactly the right flexibility and fluidity, to carry messages between neurones, that is a determining factor in physical and mental well-being and has a profound influence on all the body's inflammatory responses. The results of a number of scientific studies suggest that omega-3 acids contribute to measuring and restricting inflammatory symptoms, whereas omega-6 acids (and saturated fats) give free range to inflammatory responses and amplify allergic reactions. Today in the Western countries, the ratio of omega-3 acids to omega-6 in the diet is weighted 1:10 in favour of omega-6 to up to 1:25 in some areas, while for proper functioning a 4:1 ratio of omega-6 acids to omega-3 acids is generally considered the optimum. In addition, the type of diet followed in the Western countries is very rich in saturated fats like butter and animal fats, but because of an excessive supply of these less noble fats, the cell membranes lose flexibility and this can affect the way they work. An appropriate supplement can be an efficient, effective and often necessary way to meet the body's needs, enhance its daily functions and promote health and longevity.     

Long-chain n-3 fatty acids in breast milk of Inuit women consuming traditional foods

The fatty acid composition of mature breast milk total lipids was analysed in milk from Inuit women living in a small Arctic community. Long-chain n-3 fatty acids were higher than in comparable samples from Vancouver residents. The data suggest that secretion of n-3 fatty acids in milk is influenced by the dietary intake of the lactating woman. The levels of these fatty acids in Inuit milk approximate those reported for the diet fat of adult Eskimo, and which have been associated with reduced plasma cholesterol levels and death from coronary heart disease.     

Uteroplacental insufficiency alters liver and skeletal muscle branched-chain amino acid metabolism in intrauterine growth-restricted fetal rats

Uteroplacental insufficiency causes intrauterine growth restriction (IUGR) and decreases plasma levels of the branched-chain amino acids in both humans and rats. Increased fetal oxidation of these amino acids may contribute to their decline in the IUGR fetus. The rate-limiting step of branched-chain amino acid oxidation is performed by the mitochondrial enzyme branched-chain alpha-keto acid dehydrogenase (BCKAD), which is regulated by a deactivating kinase. We therefore hypothesized that uteroplacental insufficiency increases BCKAD activity through altered mRNA and protein levels of BCKAD and/or the BCKAD kinase. In IUGR fetal liver, BCKAD activity was increased 3-fold, though no difference in hepatic BCKAD protein or mRNA levels were noted. Hepatic BCKAD kinase mRNA and protein levels were significantly decreased in association with the increase in BCKAD activity. In IUGR fetal skeletal muscle, BCKAD mRNA levels were significantly increased. IUGR skeletal muscle BCKAD protein levels as well as BCKAD kinase mRNA and protein levels were unchanged. We also quantified mRNA levels of two amino acid transporters: LAT1 (system L) and rBAT (cysteine and dibasic amino acids). Both hepatic and muscle LAT1 mRNA levels were significantly increased in the IUGR fetus. We conclude that uteroplacental insufficiency significantly increases hepatic BCKAD activity in association with significantly decreased mRNA and protein levels of the deactivating kinase. We speculate that these changes contribute to the decreased serum levels of branched-chain amino acids seen in the IUGR fetus and may be an adaptation to the deprived milieu associated with uteroplacental insufficiency.     

Short-chain fatty acid profile in the colon of newborn piglets using fecal water analysis

Short-chain fatty acid production and assimilation is unlikely to occur at significant levels in the newborn because the colon at birth is sterile, and only gradually acquires an anaerobic flora. This study profiled short-chain fatty acid levels in the colon lumen over the initial 21 days of life. Fecal samples were removed surgically from the cecum, right, and left colon from 36 York piglets, 0-21 days of life. Samples were subjected to in vitro dialysis and centrifugation methods to quantitate fecal water short-chain fatty acids, electrolytes, osmolality, and pH. A three-way analysis of variance examined piglet age, colon site of fecal samples, and method of fecal water analysis, for each variable. No differences were found between techniques of fecal water collection. Newborns showed production of short-chain fatty acids as early as the 1st day of life in limited amounts. Levels were stable between days 5 and 14, and then abruptly accumulated in the lumen. Acetate was predominant early, with propionate and butyrate responsible for late peaks. The production and assimilation of short-chain fatty acids was nearly complete proximal to the left colon. Age and colon site showed significant interactions for each fatty acid (p less than 0.001). The combined osmolar contributions of short-chain fatty acids and electrolytes accounted completely for the luminal osmolality after the 2nd wk of life. Previously there was an "osmolar gap" suggesting that lactose or its breakdown products were present in the lumen and were being removed by pathways other than through short-chain fatty acid production.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

Fetal and maternal amino acid concentrations during fasting in the ewe

The present investigation was undertaken to quantitate the whole-blood amino acid concentrations of the mother and fetus over an extended period of fasting using a chronic sheep preparation. Fetal amino acid levels generally increased or remained relatively high during the fast (e.g., alanine, glycine, valine, leucine). In contrast to the situation in the fetus, most amino acids (alanine, glycine, glutamine, serine, and threonine) decreased in concentration in the ewe with fasting. Alanine levels changed most dramatically in the ewe, decreasing to less than 50% of the fed state values within 72 h of fasting. Therefore, while the supply of gluconeogenic amino acids becomes limited within the maternal circulation during fasting, levels of these amino acids remain high or increase in the fetal arterial blood.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

小儿先天性胆管扩张症囊肿内胆汁酸盐的LC-MS/MS分析

目的 探讨小儿先天性胆管扩张症囊肿内胆汁中胆汁酸谱的特点及其意义.方法 通过建立人类已知的全部15种胆汁酸改良的液相色谱串联质谱定量检测方法 ,以我院2002年10月至2004年6月收治小儿先天性胆管扩张症(CBD)16例为研究对象,其中男11例,女5例,年龄11～134个月,平均(44.8±24.6)个月,术前B超和(或)CT、术中胆道造影确诊为先天性胆管扩张症.对16例先天性胆管扩张症囊肿内胆汁进行胆汁酸定量检测.结果 先天性胆管扩张症囊肿内总胆汁酸的浓度与对照组比较明显降低,组成胆汁酸谱的各种胆汁酸与对照组比较相应减少,未见致癌性胆酸浓度增高.儿童胆汁中甘氨熊脱氧胆酸含量较高,总胆汁酸的降低与肝纤维化程度呈正相关.结论 先天性胆管扩张症患儿存在胆汁酸代谢异常,可能与肝脏继发性损伤程度有关,异常的胆汁酸谱可能与胆结石的发生相关.     

0～4岁正常小儿尿氨基酸变化规律探讨

目的探讨0～4岁正常小儿尿氨基酸变化的生理规律。方法以HP1050反相高效液相色谱法测定113例0～4岁健康儿童的20种尿氨基酸值。并检测20例非重症婴儿CMV肝炎患儿的血、尿氨基酸值。结果0～4岁健康儿童尿氨基酸水平大部分呈现随年龄增加而下降规律,性别差异不明显。CMV婴肝患儿血浆氨基酸水平变化不大,而尿氨基酸呈现非特异性、普遍性增高。结论正常婴幼儿尿氨基酸水平随年龄增加而下降趋势,但性别差异不明显。儿童尿氨基酸谱可为临床某些疾病的鉴别诊断提供参考。     

Low erythrocyte docosahexaenoic acid in malnourished, often breast-fed, Pakistani infants: a matter of concern?

This article provides valuable information on the importance of long-chain polyunsaturated fatty acids (LCPUFAs) for the development of the central nervous system, including visual acuity. It was believed that breast milk contains a large range of LCPUFAs, including docosahexaenoic (DHA) and arachidonic acids which cannot be found in formula milks for term infants; but low RBC LCPUFAs have been observed in malnourished children receiving breast milk and weaning food low in fat. In these children, it has been postulated that the synthesis of LCPUFAs from their precursors is decreased making them more dependent on an adequate LCPUFA intake. Several studies were conducted to test this hypothesis. It was observed that although malnourished children in North Pakistan received breast milk, yet poor DHA status of these malnourished breastfed children is caused by a marginal DHA status of these mothers. Further studies are needed though to establish the relationship between maternal nutrition during lactation and children RBC DHA levels receiving breast milk.     

Free amino acid, protein, and fat contents of breast milk from Guatemalan mothers consuming a corn-based diet

Diet compositions and breast milk levels of free amino acids, protein, and lipid were compared in lactating American and Guatemalan women. The protein in the American diet derived principally from animal sources, whereas that in the Guatemalan diet was based most exclusively on cereal grains and legumes. Corn, in the form of cornmeal, was the predominant food. Because of these dietary differences, the Guatemalan women consumed significantly smaller amounts of protein than the American women. The concentrations of most of the free amino acids in aliquots of breast milk from the Guatemalan women were lower than those in samples from the American women. In particular, tryptophan and lysine levels were 23% and 29%, respectively, of the levels in the American breast milk samples. The Guatemalan samples contained less protein, and, when values from Guatemalan women who had nursed only for 6 mth or less were considered, the fat content was also less than in the American samples. The lipid content of the milk, however, did not differ between the two groups when the length of the lactation period was not controlled. The substantial cultural differences between the two groups in the frequency and duration of nursing probably prevented the identification of a clear dietary influence on milk lipid content. The correlations found between diet composition and either the amount of protein or the levels of free amino acids in breast milk suggest that, if a mother's diet is deficient in high-quality protein, the deficiency may be reflected in the composition of her milk, perhaps to the detriment of the nursing infant.