小儿全身性癫痫与髓鞘碱性蛋白关系的研究

目的 通过检测脑脊液（ＣＳＦ）和血清髓鞘碱性蛋白（ＭＢＰ）的含量，了解小儿全身性癫痫是否引致髓鞘和血脑屏障（ＢＢＢ）的破坏，方法 采用简易ＭＢＰＥＬＩＳＡ法对４７ 小儿全身性癫痫病患儿脑脊液和血清标本作ＭＢＰ定量测定。结果 在一个月内有全身性癫痫发作的患儿其ＣＳＦ和血清ＭＢＰ含量均值明显高于正常对照组（Ｐ〈０．０１）。结论 小儿全身性癫痫发作可导致ＣＳＦ和血清ＭＢＰ升高，提示大脑器质性损伤和ＢＢＢ     

早搏与心功能的关系及影响因素的研究

为探讨小儿早搏对心功能的影响，采用多普勒超声心动图测定４０例早搏患儿的单个早搏射血分数（ＰＢＥＦ）、心脏指数（ＰＢＣＩ）；单个非早搏的射血分数（ＮＰＢＥＦ）、心脏指数（ＮＰＢＣＩ）及实际的射血分数（ＡＥＦ）、心脏指数（ＡＣＩ）。同时研究这些指标与心电图和心肌酶的关系。结果显示：（１）早搏患儿ＰＢＥＦ、ＰＢＣＩ均小于ＮＰＢＥＦ、ＮＰＢＣＩ；病程长者ＰＢＥＦ、ＡＥＦ及ＡＣＩ均下降；室性早搏者的ＡＣＩ减少；早搏＞１０次／分者的ＡＥＦ及ＡＣＩ下降；Ｒ－Ｒ′／Ｒ－Ｒ比值小者的ＰＢＥＦ、ＡＥＦ及ＰＢＣＩ亦小；早搏ＱＲＳ－Ｔ综合波长者的ＰＢＥＦ及ＰＢＣＩ减少。（２）肌酸磷酸激酶同功酶（ＣＫ－ＭＢ）升高者的ＰＢＥＦ、ＡＥＦ、ＰＢＣＩ、ＡＣＩ均值的下降比ＣＫ－ＭＢ不升高者显著。研究提示，早搏＞１０次／分、Ｒ－Ｒ′／Ｒ－Ｒ＜０．６、ＱＲＳ－Ｔ综合波＞０．４秒和ＣＫ－ＭＢ＞１６ＩＵ／Ｌ者的心功能多受到影响，应予以积极治疗。     

小儿隐孢子虫病的实验诊断研究

目的 探讨不同检测方法对小儿腹泻粪便中隐孢子虫卵囊（ＣＳＯ）的诊断效果。方法 对６４９例急性腹泻患儿的粪便采用７种方法检测ＣＳＯ：（１）番红－美兰染色法（ＳＭＢ）;（２）改良抗酸染色法（ＭＡＦ）;（３）蔗糖漂浮（ＳＳＦ）后ＳＭＢ染色法;（４）ＰＢＳ－乙醚（ＰＢＳ－Ｅ）离心沉淀后ＭＡＦ染色法;（５）聚蔗糖－泛影酸钠（ＦＳＤ）纯化后扫描电镜法（ＳＥＭ）;（６）ＦＳＤ或Ｐｅｒｃｏｌｌ纯化后免疫组化法（Ａ     

动态观察白血病患儿脑脊液β_2-微球蛋白变化的临床意义

早期发现、及时治疗中枢神经系统白血病是预防白血病复发、提高生存率的重要因素。本文通过连续观察白血病患儿脑脊液（ＣＳＦ）中β_2-微球蛋白（β_2-ＭＧ）的变化,发现在庇护所治疗（即大剂量甲氨噗吟治疗）时β_2-ＭＧ有所升高,而发生ＣＮＳＬ时显著升高（Ｐ＜０．０１）。在中枢浸润早期的ＣＳＦ常规检查可能阴性,但ＣＳＦ中β_2-ＭＧ已有所增高。本文结果显示：白血病患儿ＣＳＦ中β_2-ＭＧ的变化可作为ＣＮＳＬ的监测和早期诊断指标。     

心肌疾病抗ADP/ATP载体抗体与粒细胞-巨噬细胞集落刺激因子的临床研究

目的探讨病毒性心肌炎（ＶＭＣ）和扩张型心肌病（ＤＣＭ）的自身免疫发病机制。方法采用免疫印转和放射免疫技术检测了３０例ＶＭＣ、１４例ＤＣＭ患儿血浆中抗心肌线粒体ＡＤＰ／ＡＴＰ运载蛋白（ＡＮＴ）抗体和粒细胞－巨噬细胞集落刺激因子（ＧＭ－ＣＳＦ）。结果ＶＭＣ和ＤＣＭ患儿抗ＡＮＴ抗体和ＧＭ－ＣＳＦ的阳性率分别为７３％和５７％，而２５例正常健康儿分别为０和１２％（Ｐ均＜０．０１），且抗ＡＮＴ抗体与ＧＭ－ＣＳＦ的血浆水平呈正相关（ｒ＝０．４０９１，Ｐ＜０．０５）。结论小儿ＶＭＣ和ＤＣＭ的发病与心肌的自身免疫损伤有关，抗ＡＮＴ抗体和ＧＭ－ＣＳＦ均参与了这一过程。抗ＡＮＴ抗体可作为小儿ＶＭＣ和ＤＣＭ的一种特异性诊断指标。     

脑脊液纤维结合蛋白和铁蛋白在CNSL的临床意义

本文观察了急性白血病患者ＣＳＦ－ＦＮ和Ｆ含量，结果ＣＮＳＬ患者ＣＳＦ－ＦＮ和Ｆ含量全部升高，与对照组及无ＣＮＳＬ组比较有极显著性差异（Ｐ＜０．０１）。１０例ＣＮＳＬ患者治疗后８例缓解的ＣＳＦ－ＦＮ和Ｆ明显下降；一例虽缓解但下降不明显，半月后复发。另一例病情恶化，ＣＳＦ－ＦＮ和Ｆ反较治疗前升高。无ＣＮＳＬ组３０例患者９例ＣＳＦ－ＦＮ增高，８／２３例ＣＳＦ－Ｆ增高，其中二项同时增高的有７例，随访１５—８０天，有５例确诊为ＣＮＳＬ。本文提示ＣＮＳＬ确诊前ＣＮＳ已受到白血病细胞的浸润；ＣＳＦ－ＦＮ和Ｆ是诊断ＣＮＳＬ的敏感指标，有助于ＣＮＳＬ的早期诊断和疗效监测。     

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。     

肾病综合征患儿骨代谢研究

观察６６例不同病期肾病综合征（ＮＳ）患儿血清钙离子（ＩＣａ）、骨钙素（ＢＧＰ）、尿Ⅰ型胶原交联氨基末端肽（ＮＴＸ）、骨密度（ＢＭＣ）及腕骨Ｘ线变化。结果表明：①ＮＳ患儿存在骨代谢异常；②在反映骨代谢变化时，ＢＧＰ及ＮＴＸ较ＢＭＣ、Ｘ线摄片敏感，可作为肾性骨病早期改变的观察指标     

尿视黄醇结合蛋白与转铁蛋白对判断小儿重症肺炎时肾损害的价值

目的 探讨尿视黄醇结合蛋白（ＲＢＰ）和尿转铁蛋白（ＴＲＦ）水平在小儿重症肺炎肾功能损害的临床价值。方法 选择小儿童症肺炎６１例、轻症８４例、正常健康儿童６０例,用酶联免疫法检测尿ＲＢＰ及ＴＲＦ,同时测定其血尿素氮（ＢＵＮ）、肌酐（ＳＣｒ）。结果 重症肺炎组ＲＢＰ、ＴＲＦ明显高于对照组（Ｐ〈０．０１）,而ＢＵＮ、ＳＣｒ无显著变化。结论 ＲＢＰ、ＴＲＦ能敏感地反映肾功能,了解早期肾功能损害,用尿液检测     

小儿急性白血病转铁蛋白受体和铁代谢状况的研究

检测１９例ＡＬＬ和７例ＡＭＬ外周血白血病细胞ＴｆＲ位点数（ＴｆＲ）、血清铁（ＳＩ）、总铁结合力（ＴＩＢＣ）、血清铁蛋白（ＳＦ）、血清转铁蛋白（Ｔｆ）和细胞内铁蛋白（ＣＦ），发现急性白血病病例ＳＩ变化不大，ＳＦ、ＣＦ增加，Ｔｆ下降，ＴｆＲ与ＳＦ、ＣＦ呈正相关，与Ｔｆ呈负相关。     

癫痫发作后患儿血清和脑脊液中神经元特异性烯醇化酶S-100β蛋白髓鞘碱性蛋白的变化

目的　探讨癫痫发作是否引起脑损伤 ,生化标志物能否作为癫痫发作后脑细胞损伤早期诊断的指标。方法　应用电化学发光法和酶联免疫反应法测定癫痫发作后 2 4h内患儿血清和脑脊液 (CSF)中神经元特异性烯醇化酶 (NSE)、S 10 0 β蛋白 (S 10 0 β)、髓鞘碱性蛋白 (MBP)水平的含量。 结果　癫痫非反复发作组血清和CSF中NSE、S 10 0 β含量明显高于对照组 ,差异有非常显著性 (均 P <0 .0 1) ;癫痫反复发作组血清和CSF中NSE、S 10 0 β含量又明显高于非反复发作组 ,差异有显著性 (均 P <0 .0 5 ) ;持续状态组血清和CSF中NSE、S 10 0 β含量亦显著高于非反复发作组 ,差异有非常显著性 (均P <0 .0 1) ;但癫痫反复发作组与持续状态组比较 ,血清和CSF中NSE、S 10 0 β含量差异均无显著性意义 (均 P >0 .0 5 )。各组血清和CSF中MBP含量与对照组相比差异无显著性(均P >0 .0 5 )。结论　癫痫发作后 2 4h内血清和CSF中NSE和S 10 0 β已明显升高 ,两者均能作为癫痫发作后脑细胞损伤的早期指标 ;MBP不能作为癫痫发作后脑细胞损伤的早期诊断指标。     

Intrathecal production of neopterin in meningitis in childhood

A major activator of antigen presenting cells (APC) is gamma interferon a product of activated T-lymphocytes. CNS is not well studied and represents a unique system with respect to the immune reactions. Neopterin is an indirect marker of gamma interferon deliberation and may give some new information on the role of APC in CNS. Neopterin in serum and cerebrospinal fluid (CSF) was determined by specific RIA in children who were lumbar punctured to exclude meningitis. Neopterin was found in various concentrations in serum and CSF of all patients (n = 47). Bacterial meningitis (group 3) was diagnosed in 12 and aseptic meningitis in 18 children (group 2). CSF was drawn in 17 children with febrile convulsions (group 1). Elevated serum neopterin in childhood was only reported in children with an atypical PKU, while data on CSF neopterin were published only in a few cases of adults with CNS involvement. The results show that the APC is stimulated rapidly in childhood similar as in adults following severe viral or bacterial infections. Furthermore neopterin in CSF is not only explained by alteration of the blood-brain barrier but also it may reflect local intrathecal response with activation of accessory cells (APC) in the CNS itself. Between the stimulation of the cellular immune system indicated by increased levels of neopterin and the severity of the disease seems to be a positive correlation.     

CONCENTRATIONS OF CSF PROTEINS AS A MEASURE OF BLOOD BRAIN BARRIER FUNCTION AND SYNTHESIS OF IgG WITHIN THE CNS IN 'NORMAL' SUBJECTS FROM THE AGE OF 6 MONTHS TO 30 YEARS

ABSTRACT. Eeg-Olofsson, O., Link, H. and Wigertz, A. (Departments of Neurology and Pediatrics, University Hospital, Linköping, Sweden). Concentrations of CSF proteins as a measure of blood brain barrier function and synthesis of IgG within the CNS in 'normal' subjects from the age of 6 months to 30 years. Acta Paediatr Scand, 70:167, 1981. –Concentrations of proteins in cerebrospinal fluid (CSF) and serum have been studied in 105 'normal' subjects divided into age groups 0.5–5, 6–15, 16–20, and 21–30 years in order to obtain reference values for information concerning blood brain barrier damage and synthesis of immunoglobulins within the central nervous system (CNS). CSF/serum albumin ratio is superior to CSF total protein as a test of the blood brain barrier function. The IgG synthesis rate per 24 hours and IgG index [CSF/serum IgG ratio]: [CSF/serum albumin ratio] reflect the presence of IgG synthesis within the CNS. The variables reflecting the blood brain barrier function increased with age, the upper values of the CSF/serum albumin ratio being 4.0, 4.9, 6.7, and 6.5 for the mentioned age groups respectively. The upper values for the CSF IgG index reamined rather constant in relation to age at 0.62,0.68,0.64, and 0.63 respectively. An index value of less than 0.70 and an IgG synthesis of less than 10 mg/24 hours seem to be appropriate upper values as refereneces. Only about 1–2 ml of CSF and 0.5 ml serum are required for the analyses including cell counting and determinations of IgA and IgM index values as well as of glucose and lactate.     

Elevation of cerebrospinal fluid sialic acid concentration in children with central nervous system leukemia

We studied sialic acid in the cerebrospinal fluid (CSF) of 52 children with leukemia and 51 children with non-leukemic diseases. The CSF sialic acid concentration in the children with central nervous system (CNS) leukemia was significantly higher than that in the children with acute lymphoblastic leukemia without CNS involvement, acute non-lymphocytic leukemia without CNS involvement, non-hemopoietic diseases, non-suppurative meningitis, epilepsy, and other neurologic diseases. Serial determinations revealed a rapid decline in the CSF sialic acid concentrations in the patients with CNS leukemia who responded well to the therapy and who were free from relapse of CNS leukemia. The simultaneously determined CSF beta 2 microglobulin concentration did not show any significant changes. These results suggest that the CSF sialic acid may be a good indicator of CNS leukemia.     

Concentration of tumour markers CEA, AFP, alpha and beta subunits of hCG in cerebrospinal fluid in children with inflammatory diseases of the central nervous system

Carcinoembryonic antigen (CEA), alphafetoprotein (AFP) and alpha-beta subunits of human chorionic gonadotropin (hCG) were determined by radio-immunoassay in CSF of 83 children presenting some central nervous disease and compared to the corresponding values obtained in 88 children without neuroinfection. CEA and alpha hCG were absent in the CSF of children without neuroinfection. CEA and alpha hCG levels in CSF higher than 0.0 ng/ml were regarded as elevated. In patients with inflammatory process of CNS, CEA values were positive in 9% (maximum 8.0 ng/ml) and alpha hCG in 4% (maximum 5.0 ng/ml). AFP in CSF ranged from 15.0 to 49.0 ng/ml in children without neuroinfection, and from 0.0 to 100.0 ng/ml in patients with inflammatory diseases of the CNS. As a normal upper limit of AFP in CSF, 53.2 ng/ml is suggested; in 31% of the patients with inflammatory diseases of CNS the AFP level was elevated. The normal upper limit of beta hCG concentration in CSF was regarded as 0.4 ng/ml; in 12% of the patients with viral meningoencephalitis the beta hCG level in CSF was slightly elevated, it ranged from 0.5 to 3.0 ng/ml.     

Elevation of Cerebrospinal Fluid Sialic Acid Concentration in Children with Central Nervous System Leukemia

ABSTRACT. We studied sialic acid in the cerebrospinal fluid (CSF) of 52 children with leukemia and 51 children with non-leukemic diseases. The CSF sialic acid concentration in the children with central nervous system (CNS) leukemia was significantly higher than that in the children with acute lymphoblastic leukemia without CNS involvement, acute non-lymphocytic leukemia without CNS involvement, non-hemopoietic diseases, non-suppurative meningitis, epilepsy, and other neurologic diseases. Serial determinations revealed a rapid decline in the CSF sialic acid concentrations in the patients with CNS leukemia who responded well to the therapy and who were free from relapse of CNS leukemia. The simultaneously determined CSF β2 microglobulin concentration did not show any significant changes. These results suggest that the CSF sialic acid may be a good indicator of CNS leukemia.     

Central nervous system relapse surveillance by serial beta 2-microglobulin measurements in childhood acute lymphoblastic leukemia

Beta 2-microglobulin (beta 2m) is synthesized particularly in lymphocytes. Its value for early detection of central nervous system (CNS) involvement in acute lymphoblastic leukemia in children was tested by serial determinations. Before 9 overt CNS relapses, the mean increase of the cerebrospinal fluid (CSF) beta 2m concentration was 588 micrograms/l/month (range: -50 to +2020), which was significantly higher than the steady levels during maintenance treatment. Although the absolute value of CSF beta 2m was increased to 1 430 micrograms/l in the group with overt CNS relapse, individual variations in CSF beta 2m before a relapse were so great that no difference was seen between samples from CSF with or without lymphoblasts. The ratio between beta 2m in the CSF and in serum did not increase in serial samples prior to overt relapse, but the ratio was higher in patients with CNS relapse compared with a control group on maintenance therapy. In 9 children without CNS leukemia, the beta 2m concentration in CSF and serum decreased to a nadir 4 weeks after the start of induction treatment. The subsequent increase of CSF beta 2m was similar to the increase before a CNS relapse. Mean values of CSF beta 2m changes differed between groups of children with and without CNS leukemia early in the induction phase and during the maintenance treatment, but the wide range in individual values made serial beta 2m determinations unsuitable for detecting a CNS relapse.     

Central Nervous System Relapse Surveillance by Serial Beta2-Microglobulin Measurements in Childhood Acute Lymphoblastic Leukemia

ABSTRACT. Beta₂-microglobulin (β₂m) is synthesized particularly in lymphocytes. Its value for early detection of central nervous system (CNS) involvement in acute lymphoblastic leukemia in children was tested by serial determinations. Before 9 overt CNS relapses, the mean increase of the cerebrospinal fluid (CSF) β₂m concentration was 588 μg/l/month (range: -50 to +2020), which was significantly higher than the steady levels during maintenance treatment. Although the absolute value of CSF β₂m was increased to 1430 μg/l in the group with overt CNS relapse, individual variations in CSF β₂m before a relapse were so great that no difference was seen between samples from CSF with or without lymphoblasts. The ratio between β₂m in the CSF and in serum did not increase in serial samples prior to overt relapse, but the ratio was higher in patients with CNS relapse compared with a control group on maintenance therapy. In 9 children without CNS leukemia, the β₂m concentration in CSF and serum decreased to a nadir 4 weeks after the start of induction treatment. The subsequent increase of CSF β₂m was similar to the increase before a CNS relapse. Mean values of CSF β₂m changes differed between groups of children with and without CNS leukemia early in the induction phase and during the maintenance treatment, but the wide range in individual values made serial β₂m determinations unsuitable for detecting a CNS relapse.     

Creatine kinase BB and lactate in the cerebrospinal fluid of neonates and infants with perinatal injuries of the CNS

A study was made of the content of creatine kinase-BB (CK-BB) and lactate in cerebrospinal fluid (CSF) of 202 neonates and infants with perinatal CNS injuries. The relationship was found between the rise of the CK-BB content and the gravity of perinatal CNS injuries. The highest content of CK-BB in CSF was marked in neonates with cerebral disorders complicated by infectious and inflammatory diseases (pneumonia, sepsis). Within the first 5 days of life, the children of this group demonstrated the relationship between the content of CK-BB and lactate of CSF. The measurement of the content of CK-BB in CSF should be used for early diagnosis, assessment of the gravity and course of perinatal CNS injuries in neonates and in infants.     

大鼠缺氧缺血性脑损伤时血液和脑脊液中S-100、CK-BB、NSE、MBP水平变化的研究

目的　探讨大鼠缺氧缺血性脑损伤（ＨＩＢＤ） 时血液和脑脊液中Ｓ１００ 蛋白（Ｓ１００） 、磷酸肌酸激酶脑型同工酶（ＣＫＢＢ）、神经元特异性烯醇化酶（ＮＳＥ） 、髓鞘碱性蛋白（ＭＢＰ） 的水平变化。筛选其对ＨＩＢＤ 具有早期诊断价值的指标。方法　采用７ 日龄ＳＤ 大鼠缺氧缺血性脑损伤模型：乙醚吸入麻醉后,用４－０ 双线结扎右侧颈总动脉,恢复４ 小时后,在３７ ℃恒温低氧箱内,给予吸入８％ 氧气和９２ ％ 氮气混合气体２ 小时。用放射免疫法和琼脂糖凝胶电泳法对对照组、缺氧缺血后６、１２ 、２４ 、４８、７２ 、９６ 小时进行动态观测ＨＩＢＤ 后血液和脑脊液中Ｓ１００、ＣＫＢＢ、ＮＳＥ、ＭＢＰ水平的变化。结果　在ＨＩＢＤ后血液和脑脊液中Ｓ１００、ＣＫＢＢ、ＮＳＥ、ＭＢＰ水平都有升高,但各项指标升高的时间和幅度并不一致。与对照组比较,血液和脑脊液中Ｓ１００、ＣＫＢＢ、ＮＳＥ高峰出现较早,在２４ ～４８ 小时都有明显增加（Ｐ＜０－００１）,ＭＢＰ的水平在７２ 小时才有一定程度的增加（ Ｐ＜ ０－０５）,并且血液和脑脊液中Ｓ１００、ＣＫＢＢ、ＮＳＥ水平的变化具有较好相关性。结论　在ＨＩＢＤ时血液和脑脊液中Ｓ１００、ＣＫ