Studies on the Urinary Amino Acid Patterns in the Developing Monozygotic and Dizygotic Twins

Ten children aging from 3 to 9 years were fed on the same diet in the same circumstance and the urinary specimens were collected between meals in the morning, afternoon and evening. The amino acids in urinary specimens were analyzed by amino acid autoanalyzer and the patterns were determined. According to the results, the following conclusions were induced:

• 1) Individual differences were found in the urinary amino acid patterns of the children fed on the same diet in the same circumstance.
• 2) The particular individualities of the patterns did not generally change dependent on the diet.
• 3) However, some amino acids, threonine, glycine, alanine, lysine, histidine, tryptophan and tyrosine in the urinary specimens might slightly fluctuate with the change of diet.
• 4) The urinary amino acid pattern of children may be controlled more substantially by constitutional or genetic factors than by the environmental ones, including diet.

     

Acid Hematin Pigmentation of the Cornea in Stillborn Fetuses

During the postmortem histopathologic evaluation of eyes from stillborn fetuses we noted the presence of a prominent undescribed corneal pigment in 18 of 55 stillborn fetuses. The corneal pigment was frequently associated with documented meconium-stained amniotic fluid, and in no instance was a stained cornea coupled with recorded clear amniotic fluid. Pigmented corneas came from stillborn fetuses with a longer duration of intrauterine death than nonstained corneas. The pigment stained black with the Fontana-Masson stain, was birefringent, and treatment of tissue sections with 5% potassium permanganate and 5% oxalic acid as well as with saturated alcoholic picric acid solution removed the pigment indicating that it is acid hematin. The most likely cause of the acid hematin-stained corneas was tissue acidity created in utero with prolonged intrauterine death.     

游离氨基酸配方奶粉在牛奶蛋白过敏患儿中的应用

目的 观察游离氨基酸配方奶粉在牛奶过敏患儿中的应用效果及预后.方法 收集2009年10月- 2010年10月诊断为牛奶蛋白过敏并使用游离氨基酸配方奶粉治疗的31例患儿.应用年龄别身高Z值、年龄别体质量Z值、身高别体质量Z值评价患儿生长发育情况,根据皮肤病变范围、皮损严重程度、主观症状瘙痒和睡眠影响程度,应用特应性皮炎积分(SCORAD指数)评价患儿的湿疹严重程度,采用t检验、Fisher’s精确概率法等统计学方法分析患儿一般资料、治疗前后的Z评分、SCORAD指数、临床症状改善情况及各项实验室检查指标的差异.结果 31例牛奶蛋白过敏患儿年龄(3.46±2.22)个月,在性别、城乡分布、喂养方式上差异均无统计学意义(Pa＞0.05).治疗随访2个月,患儿湿疹有明显改善(SCORAD指数:21.70 ±20.24 vs 2.8 ±4.t=0.51,P=0.00);消化道症状呕吐、腹泻、血便有明显改善(P=0.00,0.02,0.00);低体质量患儿体质量增长显著(年龄别体质量Z值:-0.58±1.08 vs -0.08 ±0.89,P=0.048);治疗前后患儿外周血白细胞[(12.58±4.82)×109L-1 vs(10.36±2.22) ×109 L-1;t=2.32,P=0.024]、嗜酸性粒细胞总数[(1.62 ±2.05)×109 L-1 vs (0.42±0.50) ×109 L-1;t =3.14,P=0.003]明显降低.结论 使用游离氨基酸配方奶粉是治疗牛奶蛋白过敏的有效方法.     

隐睾患儿睾丸病理及血清性激素的改变

目的探讨隐睾患儿睾丸组织的病理改变及血清性激素改变。方法利用光镜和电镜对16例隐睾患儿睾丸组织超微结构改变进行观察。采用放射免疫法对18例隐睾患儿手术前后外周静脉血清睾酮(T)、雌二醇(E2)、卵泡刺激素(FSH)进行测定。结果镜下观察发现2岁内患儿睾丸组织仅有轻微改变,2岁后睾丸组织学变化随年龄增长渐加重。隐睾患儿术前组血清T明显低于正常,而E2、FSH均明显高于正常,睾丸固定术后3~6个月血清T较术前组明显升高,而血清E2、FSH低于术前组,差异均有显著性(P均<0.05)。结论2岁内隐睾患儿睾丸组织病理改变轻微,是手术的最佳时机。睾丸固定术后患儿血清性激素水平明显改善,有利于睾丸功能恢复,手术疗效可靠。     

TOTAL PARENTERAL NUTRITION IN INFANTS Blood Levels of Glucose, Lactate, Pyruvate, Free Fatty Acids, Glycerol, D-β-Hydroxybutyrate, Triglycerides, Free Amino Acids and Insulin

Abstract. Two regimens (A and B) for TPN were designed to meet the requirements of newborn infants for calories, amino acids, fatty acids, electrolytes, trace elements and vitamins. Both "A" and "B" included fat emulsion (Intralipid®). "A" contained fructose and glucose, "B" glucose only. "A" provided amino acids (Vamin®) in proportions similar to those of whole egg, "B" similar to those of human milk. All nutrients were given simultaneously into peripheral veins by constant infusion. Nineteen patients (11 newborns, 8 infants) were studied for 1–28 days. Twelve infants recovered, 7 died. In none could TPN be regarded as the cause of death. Treatment was complicated by sepsis in 5 infants. During the course of treatment, blood levels of substrates and insulin were measured before, during and 30 min after discontinuation of TPN. Highly raised concentrations of circulating substrates seen in 3 infants seemed to be related to a poor clinical condition rather than to the regimen used. Infants in good condition tolerated TPN well. Low levels of branch-chained amino acids and tendency to ketonemia, when infusion was stopped, suggested that minimal rather than optimal supply of energy and of amino acids in relation to energy was provided with both regimens. Low insulin levels associated with elevated blood levels of substrates suggested that insulin administration to selected cases might be indicated. Fructose (0.30 g/kg × hour^-1) given with regimen A increased blood lactate concentrations. Homocystinaemia appeared in 2 cases; disappearance after excess vitamin B₆ administration indicated increased B₆ requirement.     

Fasting Plasma Amino Acid Concentrations in PKU Children on Two Different Levels of Protein Intake

ABSTRACT. Two groups of children with phenylketonuria were treated with different amounts of protein. One group (RDA group) received protein as recommended by the U.S. Food and Nutrition Board, the other (FAO group) the amount recommended by the FAO/WHO 1973 ad hoc Expert Committee. Two of the children in the FAO group showed a decrease in length/growth velocity which raised the question of the adequacy of the protein content in the diet. Since deviations from normal plasma amino acids have been observed in protein deficient, energy-adequate diets, we examined these parameters in the PKU children. In the two children with retardation in length/growth, very high glycine values as well as very high alanine values were observed at the end of the study. These observations strengthened our suspicion that the FAO/WHO 1973 recommendations are marginal.     

新生儿脐血脂联素水平的变化及其与生长激素、胰岛素的关系

目的探讨新生儿脐血脂联素(APN)水平与生长激素、胰岛素之间的关系。方法采用放射免疫法检测我院正常足月新生儿78例脐血APN、生长激素和胰岛素水平,其中小于胎龄儿(SGA)13例,适于胎龄儿(AGA)53例,大于胎龄儿(LGA) 12例,并按体质量指数(BMI)=体质量(kg)/身长2(m2)评估新生儿营养状态。结果新生儿脐血APN水平与BMI呈明显正相关(r=0.367 P<0.05);SGA(19.95±4.71)mg/L,AGA(24.81±4.15)mg/L,LGA(26.57±4.29)mg/L,三者之间有显著差异(F=16.65 P<0.05);与胰岛素水平呈显著正相关(r=0.453 P<0.05);与生长激素无明显相关性(r=0.072 P >0.05)。结论新生儿体内APN可能来源于自身脂肪组织,在一定程度上反映了新生儿的营养状态,它可能和胰岛素共同调节新生儿的生长发育。     

注意缺陷多动障碍患儿血清铁蛋白和血铅的变化

目的 测定不同亚型的注意力缺陷多动障碍(ADHD)患儿血清铁蛋白(SF)、Hb、血铅水平.探讨其在ADHD发病中的变化.方法 采用<美国精神病学会精神障碍分类法>(第4版)(DSM-Ⅳ)标准,并结合Conner's量表和视听整和连续执行测试法进行诊断,选择2008年8-12月本院儿科门诊确诊为ADHD的患儿53例.其中注意力缺陷型20例,混合型18例,多动-冲动型15例,同期选择来本院行体检的健康儿童18例作为健康对照组.抽取清晨空腹静脉血进行Hb、SF及血铅水平测定,应用SPSS 11.0软件对其进行统计学分析.结果 1.ADHD患儿各亚组SF水平均低于健康对照组,差异均有统计学意义(Pa ＜0.05),其中注意力缺陷型亚组SF水平下降明显,均低于混合型和多动-冲动型亚组(Pa ＜0.05).2.ADHD患儿各亚组间血铅水平无显著性差异;各亚组与健康对照组比较,血铅水平差异亦均无统计学意义(Pa ＞0.05).3.ADHD患儿各亚组间Hb水平无显著性差异;各亚组与健康对照组比较,Hb水平差异亦均无统计学意义(Pa ＞0.05).结论 ADHD尤其是注意力缺陷型患儿的发病可能与低水平SF有关,在诊治此类型患儿时应常规检测,并及早纠正.     

单纯性肥胖儿童血浆卡尼汀、游离脂肪酸水平与胰岛素抵抗的相关性

目的了解单纯性肥胖儿童血浆卡尼汀(CT)、游离脂肪酸(FFA)水平与胰岛素抵抗相关参数的关系。方法选择56例确诊为单纯性肥胖的儿童作为研究对象(肥胖组),36例健康体检儿童为健康对照组。采用放射免疫法(RIA)测定二组血浆胰岛素,高效液相色谱法(HPLC)测定血浆CT水平,酶比色法测定血浆FFA及三酰甘油(TG)。计算体质量指数(BMI)及腰臀比(WHR),按稳态模式胰岛素(HOMA-IR)计算其胰岛素抵抗指数(InRI)和胰岛素敏感指数(InSI),应用SPSS13.0软件进行统计学分析。结果肥胖组血浆CT水平为(43.67±12.75)μmol/L,健康对照组为(58.31±21.25)μmol/L,二组比较差异有统计学意义(t=2.566P<0.05)。肥胖组血浆FFA水平为(785.32±284.91)μmol/L,健康对照组为(604.87±136.52)μmol/L,二组比较差异有统计学意义(t=2.143P<0.05)。肥胖组WHR、TG、胰岛素(Ins)水平、InRI与健康对照组比较差异均有统计学意义(Pa<0.05)。血浆FFA与BMI、WHR、TG均呈正相关(r=0.403,0.320,0.406Pa<0.05),与InSI呈负相关(r=-0.364P<0.01);血浆CT与空腹胰岛素(FINS)、InSI呈正相关(r=0.365,0.364Pa<0.01),与WHR呈负相关(r=-0.614P<0.01);肥胖组血浆CT水平与FFA水平呈负相关(r=-0.592P<0.01)。结论肥胖患儿存在明显的脂肪代谢紊乱,血浆FFA及CT水平的变化与胰岛素抵抗密切相关,这些变化可能参与肥胖及肥胖相关并发症的发生。