癫痫患儿细胞内外钙含量变化及其临床意义

应用密度离心法分离血细胞及火焰原子吸收光谱法同批测定２４例癫痫患儿血清、红细胞（ＲＢＣ）和单个核细胞（ＭＢＣ）内钙含量；应用钙离子选择性电极法检测２８例癫痫患儿脑脊液（ＣＳＦ）－Ｃａ２＋和全血Ｃａ２＋。结果表明：癫痫患儿发作后３天内ＭＢＣ－Ｃａ明显增高（Ｐ＜０．０５），ＣＳＦ－Ｃａ２＋明显降低（Ｐ＜０．０１），血清钙、ＲＢＣ－Ｃａ和全血Ｃａ２＋无显著变化。提示癫痫发作时可能存在钙内流。ＭＢＣ－Ｃａ增高于发作后３小时内最明显（Ｐ＜０．０５），与病因及每日发作次数无关，进一步提示ＭＢＣ－Ｃａ增高与癫痫发作有关。     

病毒性脑炎患儿脑脊液中一氧化氮和丙二醛变化及其临床意义

目的　探讨病毒性脑炎（ 病脑）患儿脑脊液中一氧化氮（ＮＯ）、一氧化氮合酶（ＮＯＳ）、丙二醛（ＭＤＡ） 和超氧化物歧化酶（ＳＯＤ）的变化特点。方法　应用比色法对病脑患儿急性期、恢复期脑脊液中ＮＯ,ＮＯＳ,ＭＤＡ 及ＳＯＤ 进行对比分析。结果　病脑患儿脑脊液中ＮＯ（５９ ．８±１５ ．０ μｍｏｌ／Ｌ）,ＮＯＳ（２．７３±０ ．５９ Ｕ／ｍｌ）,ＭＤＡ（１３．２ ±２ ．２０ ｎｍｏｌ／ｍｌ） 及ＳＯＤ（２８ ．９ ±１２．９ ＮＵ／ｍｌ） 水平明显高于对照组（Ｐ＜ ０．０１）;１４ 例病儿急性期ＮＯ,ＮＯＳ,ＳＯＤ含量明显高于恢复期（Ｐ＜ ０．０１） ,ＭＲＩ检查阳性组ＮＯ 水平高于阴性组（ Ｐ＜ ０ ．０５） 。结论　ＮＯ可能参与脑水肿的形成和髓鞘的破坏过程;合理应用甘露醇及抗氧化治疗应予重视。     

N-甲基-D-天门冬氨酸受体及其调控的钙内流在大鼠感染性脑损伤中的变化

为探讨Ｎ－甲基－Ｄ－天门冬氨酸（ＮＭＤＡ）受体及其调控的钙内流在感染性脑损伤发病机制中的作用，采用Ｆｕｒａ－２／Ａｍ荧光法检测大鼠大脑皮质突触体内游离钙浓度（［Ｃａ＋＋］ｉ）。结果表明，注射百日咳杆菌组的［Ｃａ＋＋］ｉ较对照组显著增加（Ｐ均＜０．０１），并与注菌时间呈正相关关系（４小时ｖｓ０．５小时和２４小时ｖｓ４小时，Ｐ＜０．０５和＜０．０１）。ＮＭＤＡ受体的非竞争性拮抗剂ＭＫ－８０１预处理组（ＭＢＰ）的［Ｃａ＋＋］ｉ、脑含水量和伊文思蓝含量均较注菌组显著减少（Ｆ值分别为７２．２５、１６．８９、１４．３６，Ｐ＜０．０１和＜０．０５），并以２４小时［Ｃａ＋＋］ｉ减少最为明显（Ｐ＜０．０１）。提示ＮＭＤＡ受体调控的神经元内钙超载在感染性脑损伤、尤其在迟发性脑损伤的发病机制中起着重要作用。     

CCHD的血液粘度与红细胞压积以及动脉血氧饱和度的关系

对２０例紫绀型先天性心脏病（ＣＣＨＤ）和１８例对照组检测血液流变学有关指标。观察组全血粘度（ＷＢＶ）和红细胞压积（ＨＣＴ）明显高于对照组，差异显著，Ｐ〈０．０１。观察组ＨＣＴ与ＷＢＶ有明显正相关（ｒ＝０．７３２）观察组动脉血氧饱和度（ＳＡＴ）０．８±０．０６，明显低于正常值，而ＳＡＴ与ＷＢＶ有明显负相关（ｒ＝－０．８９），本研究提示，ＳＡＴ和ＨＣＴ可做为ＣＣＨＤ患儿可能出现高粘综合征（ＨＶＳ），脑     

新生儿行为神经评价与高胆红素血症的关系

目的 探讨２０项新生儿行为神经测定（ＮＢＮＡ）与高胆红素血症的关系,了解高胆红素血症对新生儿行为神经的影响。方法 对６２例高胆红素血症患儿运用ＮＢＮＡ。结果 ①当血清胆红素水平≥２０５μｍｏｌ／Ｌ,对ＮＢＮＡ即有显著影响（Ｐ〈０．０１）,血清胆红素≥３４２ｕｍｏｌ／Ｌ,则与ＮＢＮＡ评分呈明显直线负相关关系（ｒ＝－０．８４１,Ｐ〈０．０１）;②溶血感染所致高胆红素血症ＮＢＮＡ评分均低于对照组,差异有     

急性下呼吸道感染患儿一氧化氮,循环内皮细胞改变及相关性研究

研究目的：急性下呼吸道感染（ＡＬＲＩ）时血管内皮细胞损伤机制及其临床意义。研究方法：ＡＬＲＩ患儿３６例，正常健康儿童３０例为对照组。测定一氧化氮（ＮＯ）水平及循环内皮细胞（ＣＥＣ）数量。研究结果：ＮＯ的稳定代射产物一循环亚硝酸／硝酸根离子（ＮＯ＾－２／ＮＯ＾－３）水平，对照组为２４．５±１４．１μｍｏｌ／Ｌ，ＡＬＲＩ组为４４．６±２２．６μｍｏｌ／Ｌ，差异显著（Ｐ＜０．０５）。ＣＥＣ数量对照组５．     

婴幼儿佝偻病血清诊断指标探讨

应用竞争蛋白结合法测定佝偻病血清２５（ＯＨ）Ｄ３浓度，同时测定了血清钙（Ｃａ）、磷（Ｐ）、碱性磷酸酶（ＡＬＰ）、尿羟脯氨酸与肌酐比值（ＴＨＰ／Ｃｒ）。结果显示：佝偻病患儿血清２５（ＯＨ）Ｄ３值降低，ＡＬＰ、尿ＴＨＰ／Ｃｒ值增高。相关分析显示，血清２５（ＯＨ）Ｄ３与Ｃａ、Ｐ无相关性（Ｐ＞００５）；与ＡＬＰ、ＴＨＰ／Ｃｒ呈负相关（Ｐ＜００１，Ｐ＜００５）。说明血清２５（ＯＨ）Ｄ３、ＡＬＰ、ＴＨＰ／Ｃｒ对佝偻病有诊断价值，血清Ｃａ、Ｐ对佝偻病诊断有参考价值。     

rhG-CSF对小儿化疗所致的白细胞缺乏症的临床观察

本文报告１９９４．２～１９９５．３我们用ｒｈＧ－ＣＳＦ（惠尔血．ＧＲＡＮ）对１９例儿童肿瘤进行了３０例次在化疗后出现粒细胞缺乏症时的临床观察。有自身前后疗程对照１３例（１９例次），前后疗程化疗方案完全相同。每例前１疗程单用化疗为对照疗程，后一疗程在化疗结束后，当ｗｂｃ＜２．０×１０９／Ｌ或ＡＮＣ＜０．５×１０９／Ｌ时使用惠尔血７５ｍｃｇ，每日１次，皮下注射。一般用３～５天，ｗｂｃ及ＡＮＣ即可达到临床要求标准（ｗｂｃ≥４．０×１０９／Ｌ，ＡＮＣ≥１．５×１０９／Ｌ）。试验疗程较对照疗程粒细胞恢复时间明显缩短（ｗｂｃ提前９．３天恢复），ＡＮＣ提前８天恢复。在６例患儿化疗后用惠尔血（１１例次）未设自身对照疗程，临床观察亦获得与上述相似的疗效。本组在３０例次中仅见２例次有发热和骨痛，停药后发热和骨痛消失，未见其它不良反应。观察结果显示，惠尔血对小儿肿瘤化疗所致的粒细胞缺乏症疗效肯定。我们采用在停化疗后ｗｂｃ＜２．０×１０９／Ｌ几或ＡＮＣ＜０．５×１０９／Ｌ时开始使用惠尔血７５ｍｃｇ，１／日，皮下注射。一般３天至５天即可达到预期效果。用药量小，毒副作用少，既能减轻患儿痛苦，又能减轻家庭经济负担．是一种适合国情的用药方     

G—CSF在白血病骨髓抑制期的应用

报告Ｇ－ＣＳＦ在小儿白血病骨髓抑制期的临床应用，共２３例（４３例次），并设对照组比较。在２３例急性白血病患儿，２０例为急性淋巴细胞白血病，３例为髓系白血病。结果：骨髓抑制期应用Ｇ－ＣＳＦ组与对照组比较，中性粒细胞绝对性（ＡＮＣ）恢复到ＡＮＣ〉１×１０＾９／Ｌ及血小板（ＰＬＴ）恢复到ＰＬＴ〉１００×１０＾９／Ｌ的时间明显缩短（Ｐ〈０．０５）及感染率明显降低（Ｐ〈０．０５），且副反应轻。此结果提示，Ｇ     

心力衰竭时内分泌改变与血液动力学关系的研究

对２０例健康儿童、２３例心衰病儿检查血浆肾素活性（ＰＲＡ）、血管紧张素Ⅱ（ＡＴⅡ）、醛固酮（ＡＬＤ）、心钠素（ＡＮＦ）。心衰病儿上述四项内分泌素显著高于健康儿童，其升高程度与心衰程度相一致，并分别分析他们与心脏指数（ＣＩ）、主动脉峰值流速（ＰＦＶＡ）、肺动脉峰值流速（ＰＦＶＰ）的关系。通过Ｂａｙｅ判别，Ｙ０＝－１５．９２００＋１２．１５３７ｌｏｇＡＴⅡ＋６．０９９４ｌｏｇＡＮＦ，Ｙ１＝－２７．０６８６＋１５．３５２２ｌｏｇＡＴⅡ＋８．３４６７ＡＮＦ，Ｙ０＞Ｙ１为正常；Ｙ１＞Ｙ０为心衰。     

Proton magnetic resonance spectroscopy: normal findings in the cerebellar hemisphere in childhood

BACKGROUND: The cerebellar hemispheres (CER) are different from the supratentorial white and gray matter embryologically, in cytoarchitecture, and probably in metabolic activity. Proton magnetic resonance spectroscopy ((1)H MRS) can provide a noninvasive biochemical analysis of this region. OBJECTIVE: To study, with (1)H MRS, metabolite concentrations in CER as a function of age and compare these metabolic data with those of parietoccipital white matter (PO WM) in healthy children. MATERIALS AND METHODS: Using single-voxel (1)H MRS, we studied 37 volunteers (3-18 years) with normal MRI scans of the brain. (1)H MRS was performed using the PRESS technique in CER and PO WM. The NAA/Cr, Cho/Cr, NAA/H(2)O, Cr/H(2)O, and Cho/H(2)O ratios were analyzed as a function of age. Metabolic data from these regions were compared. RESULTS: The NAA/Cr ratio tended to increase with age in CER. Mean NAA/Cr and Cho/Cr ratios were found to be lower in CER than in PO WM. Mean NAA/H(2)O, Cr/H(2)O, and Cho/H(2)O ratios in CER were higher than in the PO WM. CONCLUSION: Our data confirm the regional variations between CER and PO WM metabolite ratios, and demonstrate a tendency of age-dependent change of the NAA/Cr ratio in CER. The creatine concentration was significantly higher in the cerebellum than in the PO WM.     

核磁共振和核磁共振波谱结合粗大运动评定量表在儿童脑性瘫痪分度诊断的应用

目的 探讨头部核磁共振、核磁共振波谱以及粗大运动评定量表对儿童脑性瘫痪的分级诊断价值.方法 40例1.0～2.0周岁的健康儿童和42例1.1 ～2.0周岁的脑性瘫痪儿童为研究对象,对比正常儿童与脑性瘫痪患儿的MRI、MRS检测结果;根据核磁共振结果对脑性瘫痪进行分度,并用核磁共振波谱检测不同核磁共振分度的脑性瘫痪患儿脑内的NAA/Cr、CHO/Cr、LAC/Cr水平并分度;考察不同核磁共振和核磁共振波谱分度脑性瘫痪患儿的粗大运动评定量表得分情况.结果 核磁共振和核磁共振波谱均能有效区分脑性瘫痪患儿脑内的典型病变,脑内的NAA、Cr、Cho能被核磁共振波谱定量检测.不同核磁共振分度的患者脑内的NAA/Cr、CHO/Cr、LAC/Cr有所差异,重度患者与中度患者间的差异具有统计学意义(P＜0.05).脑性瘫痪的核磁共振波谱分度与核磁共振分度相符率不理想,但粗大运动评定量表评分表明各不同分度之间有差异(P＜0.05).结论 核磁共振和核磁共振波谱结合粗大运动评定量表可有效完善脑性瘫痪的分度诊断,这对不同类型儿童脑性瘫痪的临床治疗具有一定的指导意义.     

Cerebral metabolite differences in adolescents with low birth weight: assessment with in vivo proton MR spectroscopy

Background Children with very low birth weight (VLBW) have a significantly increased risk of later neurodevelopmental problems, while infants born small for gestational age (SGA) at term are also at some risk of developing neurological impairment.Objective To investigate possible brain metabolite differences in adolescents with VLBW, SGA at term and controls by proton in vivo magnetic resonance spectroscopy (MRS) at 1.5 T.Materials and methods MR spectra were acquired from volumes localized in the left frontal lobe, containing mainly white matter (54 subjects). Peak areas of N-acetyl aspartate (NAA), choline (Cho) and creatine (Cr) were determined, and the peak area ratio of NAA to Cr, total Cho to Cr, or NAA to Cho calculated. Probabilistic neural network (PNN) analysis was performed utilizing the chemical shift region containing resonances from NAA, Cho and Cr as inputs.Results No significant difference in the peak area ratios could be found using the Kruskal-Wallis test. By application of PNN, a correct classification of 52 of the 54 adolescents with a sensitivity and specificity exceeding 93% for all groups was achieved.Conclusion Small, yet systematic, differences in brain metabolite distribution among the groups were confirmed by PNN analysis.     

氢质子磁共振波谱在原发性癫癎中的应用研究

目的：应用氢质子磁共振波谱(proton magnetic resonance spectroscopy, 'H-MRS)检测癫癎患儿的脑组织生化代谢物,探讨'H-MRS在原发性癫癎中的临床应用价值及意义。方法：对33例原发性癫癎患儿（癫癎组,其中14例有热性惊厥史）和6例正常儿童（对照组）进行常规头颅MRI和颞叶海马区的'H-MRS检查。检测N-乙酰天门冬氨酸(NAA)、肌酸(Cr)、胆碱(Cho)和乳酸(Lac)的信号强度,计算并比较NAA/（Cho+Cr）和Lac/Cr的比值。结果：头颅MRI检查：除1例癫癎患儿表现为髓鞘发育不良外其余所有研究对象均无异常。'H-MRS检测：NAA/（Cho+Cr）比值在癫癎组为0.64±0.07,低于对照组(0.73±0.05)（P＜0.01）;同时伴有热性惊厥史的癫癎患儿为0.61±0.07,低于不伴有热性惊厥史的癫癎患儿（0.66±0.06）（P＜0.05）。癫癎组Lac/Cr比值与对照组差异无统计学意义。结论：'H-MRS作为一项无创的检查,能更敏感地发现癫癎患儿的早期脑损伤,为癫癎的诊断和评估预后提供客观依据。［中国当代儿科杂志,2010,12（6）：425-428］     

Neonatal hydrocortisone treatment related to 1H-MRS of the hippocampus and short-term memory at school age in preterm born children

Animal studies have shown that corticosteroids (dexamethasone) cause neuronal loss in the hippocampus and deficits in short term memory. Proton magnetic resonance spectroscopy can measure brain metabolites in vivo and give an indication of neuronal integrity. We investigated whether prolonged administration of hydrocortisone during the neonatal period for bronchopulmonary dysplasia (BPD) in preterm born children changes the metabolism in the hippocampus, measured at school age. Secondly, we investigated whether hippocampal metabolism and short-term memory and neurodevelopmental outcome are related. In this observational study 37 preterm born children (< or = 32 wk (range 25.0-33.0) and/or a birth weight < or = 1500 g) underwent proton spectroscopy of the hippocampus at school age. Eighteen children were treated with hydrocortisone for BPD (starting dose 5 mg/kg/d tapered over a minimum period of 22 d, median duration 28 d) and 19 never received corticosteroids during the perinatal period. N-acetyl aspartate/ Choline + Creatine/phosphocreatine (NAA/(Cho + Cr)) ratios were determined. A 15-word recall memory test and an IQ measurement were obtained on the same day. Hydrocortisone treated children were younger, lighter and sicker than their nonsteroid treated counterparts. Mean NAA/(Cho + Cr) ratios in the hippocampus were not significantly different in the hydrocortisone group compared with the non-steroid group. Performance on the 15-word memory test and IQ were similar in the two groups. There was no relation between NAA/(Cho + Cr) ratios and memory nor between NAA/(Cho + Cr) ratios and IQ. We conclude that hydrocortisone in the mentioned dose, administered in the neonatal period for BPD, does not appear to have any long-term effects on memory and/or hippocampal metabolism.     

Neuroimaging findings in infantile GM1 gangliosidosis

GM1 gangliosidosis is an autosomal recessive glycosphingolipid storage disease caused by defects in the enzyme beta-galactosidase. Three clinical forms (infantile-, juvenile-, and adult-onset) of the disease are recognized. Patients with infantile GM1 gangliosidosis present at birth or shortly thereafter with somatic and bony changes, followed by severe neurological deterioration ultimately leading to death within the first 2 years of life. We present the brain CT, MRI and MR spectroscopy (MRS) findings in a 17-month-old Turkish girl with infantile GM1 gangliosidosis. Neuroimaging findings in patients with infantile GM1 gangliosidosis have been reported only in a few cases. In this study, MRS of the thalamus was performed to study the metabolic changes in GM1 gangliosidosis. We showed a a decreased NAA/Cr ration and an increased Cho/Cr ratio. To our knowledge, this is the first report of magnetic resonance spectroscopy findings in type-1 GM1 gangliosidosis.     

中重度肥胖儿童脑代谢的质子磁共振波谱变化

目的探讨肥胖儿童额叶和海马感兴趣区的脑代谢特征。方法对象为2005年安徽省肥胖儿童夏令营小学生。分别对9例肥胖(肥胖组)及其配对的体质量健康儿童(健康对照组)进行测量(身高、体质量、腰围、臀围)和瑞文推理测验;双能X线吸收仪(DXA)测定全身体脂百分比;采用氢质子磁共振波谱(1H-MRS)检测额叶、海马部位脑代谢特点。结果1.肥胖组额叶部位N-乙酰天门冬氨酸(NAA)、额叶胆碱(Cho)、额叶肌酸(Cr)水平均显著低于健康对照组(126.88、80.38、53.13vs160.5、101.63、71.5)(Pa<0.05);2.肥胖组额叶NAA/Cr比高于健康对照组;海马部位NAA、Cr、NAA/Cr、Cho均较健康对照组高,但均无统计学差异(Pa>0.05)。结论中重度肥胖儿童额叶NAA减低表明额叶神经元数量减少,Cho减低提示中重度肥胖可能影响了儿童白质髓鞘化过程,造成白质髓鞘化过程慢于对照组;而Cr减低表明肥胖儿童额叶代谢低下。     

The use of short-echo-time 1H MRS for childhood cerebellar tumours prior to histopathological diagnosis

Background Proton magnetic resonance spectroscopy (MRS) measures concentrations of metabolites in vivo and provides a powerful method for identifying tumours. MRS has not entered routine clinical use partly due to the difficulty of analysing the spectra. Objective To create a straightforward method for interpreting short-echo-time MRS of childhood cerebellar tumours. Materials and methods Single-voxel MRS (1.5-T Siemens Symphony NUM4, TR/TE 1,500/30 ms) was performed at presentation in 30 children with cerebellar tumours. The MRS results were analysed for comparison with histological diagnosis. Peak heights for N-acetyl aspartate (NAA), creatine (Cr), choline (Cho) and myo-inositol (mIns) were determined and receiver operator characteristic curves used to select ratios that best discriminated between the tumour types. The method was implemented by a group of clinicians and scientists, blinded to the results. Results A total of 27 MRS studies met the quality control criteria. NAA/Cr >4.0 distinguished all but one of the astrocytomas from the other tumours. A combination of Cr/Cho <0.75 and mIns/NAA <2.1 separated all the medulloblastomas from the ependymomas. Conclusion Peak height ratios from short-echo-time MRS can accurately predict the histopathology of childhood cerebellar tumours.     

Magnetic resonance imaging in juvenile Canavan disease

We present a 2-year-old boy and a 6-year-old girl with mild Canavan disease (CD). Aspartoacylase activity in skin fibroblasts was deficient. Magnetic resonance imaging (MRI) of the brain did not show the prominent leucodystrophy previously reported in CD, but there was a hyperintense signal from the lentiform nuclei and the heads of the caudate nuclei on the T2-weighted MR images. This suggests a specific vulnerability of the corpus striatum in these patients. In the older patient, the white matter became affected at the age of 6 years. Proton magnetic resonance spectroscopy (¹H-MRS) of white matter revealed a normal concentration of N-acetyl-l-aspartate (NAA) and a markedly decreased concentration of choline containing compounds (Cho) in the boy but a normal ratio of NAA to Cho in the girl. We conclude that deficient NAA catabolism affects myelin metabolism. This may present as changes in the striatum and/or as a low concentration of Cho before leucodystrophy appears on MRI.     

孤独症谱系障碍儿童丘脑和小脑磁共振波谱特征和临床关系的前瞻性研究

目的 探究孤独症谱系障碍（autism spectrum disorder,ASD）儿童丘脑和小脑生化代谢物指标变化及与临床特征的关系。 方法 前瞻性采用磁共振波谱（magnetic resonance spectroscopy,MRS）单体素点分辨率波谱序列对50名2~6岁的ASD儿童双侧丘脑和小脑进行分析,以肌酸（creatine,Cr）为内标测定代谢物N-乙酰天冬氨酸（N-acetylaspartate,NAA）/Cr、胆碱（cholin,Cho）/Cr、肌醇（myoinositol,MI）/Cr、谷氨酸复合物（glutamine and glutamate complex,Glx）/Cr的相对值,比较各代谢物的差异和临床症状之间的关系。 结果 ASD儿童左侧丘脑NAA/Cr与Griffiths发育评估量表中文版中听力-语言和手眼协调评分呈正相关（P<0.05）;右侧小脑Cho/Cr与个人-社交、听力-语言、手眼协调呈正相关（P<0.05）;左侧丘脑和左侧小脑的NAA/Cr呈正相关,左侧丘脑和左侧小脑的Glx/Cr呈正相关（P<0.05）。ASD儿童丘脑和小脑部位左右两侧代谢物之间相比差异无统计意义（P>0.05）。 结论 ASD儿童小脑、丘脑存在代谢紊乱,且左侧小脑和左侧丘脑代谢物改变之间存在关联,部分指标与ASD临床症状相关。MRS可能揭示了ASD的病理基础并为其诊断、预后评估提供无创、定量检测方法。