反复呼吸道感染患儿外周血淋巴细胞凋亡及其与免疫功能的相关性探讨

目的探讨反复呼吸道感染（ＲＲＩ）患儿外周血成熟淋巴细胞的免疫功能与细胞凋亡的相关性。方法测定３０例ＲＲＩ患儿的Ｔ细胞亚群、免疫球蛋白及细胞凋亡,并测定２８例正常儿童作为对照。结果ＲＲＩ患儿ＣＤ４＋、ＣＤ４＋／ＣＤ８＋明显低于对照组（Ｐ＜００１）,ＩｇＡ、ＩｇＭ、ＩｇＧ亦低于对照组（Ｐ＜００５）,外周血淋巴细胞凋亡极显著高于对照组（Ｐ＜００１）。结论ＲＲＩ患儿的整体免疫功能降低,其原因是由于淋巴细胞凋亡过度所致。     

婴幼儿哮喘与T辅助细胞亚群功能失衡研究

目的探讨Ｔ辅助细胞（Ｔｈ）亚群功能失衡在婴幼儿哮喘发病中的作用及其影响因素。方法酶联免疫吸附试验方法，对２０例哮喘患儿和１５例健康对照者外周血单个核细胞（ＰＢＭＣ）分别经植物血凝素（ＰＨＡ）和脂多糖（ＬＰＳ）刺激后，培养上清液中各细胞因子含量进行测定。结果经ＰＨＡ刺激后哮喘组Ｔｈ产生ＩＦＮγ、ＩＬ２水平明显低于正常对照组（ｔ′＝４．１５，４．０７；Ｐ均＜０．０１），而ＩＬ４、ＩＬ６、ＩＬ１０水平则显著升高（ｔ′＝４．７３，５．９１，３１８，Ｐ均＜０．０１）。经ＬＰＳ刺激后，哮喘组单核巨噬细胞产生ＩＬ１０水平明显高于正常对照组（ｔ′＝５．６０，Ｐ＜０．０１）而ＩＬ１２水平则降低（ｔ′＝３．３４，Ｐ＜０．０１）。相关分析发现ＩＦＮγ与血清ＩｇＥ水平呈高度负相关（ｒ＝－０．６６４，Ｐ＜０．０１），ＩＬ４、ＩＬ１０与ＩｇＥ呈高度正相关（ｒ＝０．７７６，０７４０；Ｐ＜０．０１）。结论哮喘患儿生成Ｔｈ１类细胞因子不足，Ｔｈ２类因子增多；单核巨噬细胞产生ＩＬ１０增多，ＩＬ１２减少，导致Ｔｈ１／Ｔｈ２功能失衡     

罗钙全对肾病综合征患儿钙、磷及25-羟维生素D_3和甲状旁腺素水平的影响

目的探讨罗钙全（骨化三醇）短期口服冲击治疗对小儿肾病综合征（ＮＳ）的疗效。方法对１７例长期糖皮质激素（ＧＣ）治疗的ＮＳ患儿血钙、磷、碱性磷酸酶（ＡＬＰ）、骨碱性磷酸酶（ＢＡＬＰ）、２５－羟维生素Ｄ３［２５－（ＯＨ）Ｄ３］、甲状旁腺素（ＰＴＨ）水平进行了测定,并观察了罗钙全短期口服冲击治疗对上述指标的影响。结果ＮＳ患儿血钙偏低（Ｐ＜００１）,ＡＬＰ、ＢＡＬＰ、ＰＴＨ升高（Ｐ＜００１）,２５－（ＯＨ）Ｄ３下降（Ｐ＜００５）;经罗钙全治疗后血钙磷上升（Ｐ＜００１）,ＡＬＰ、ＢＡＬＰ下降（Ｐ＜００１）,ＰＴＨ水平降低（Ｐ＜００１）,２５－（ＯＨ）Ｄ３无明显变化（Ｐ＞００５）。结论罗钙全短期口服冲击治疗可有效地改善ＮＳ患儿钙磷代谢紊乱,抑制ＰＴＨ的异常过度分泌。     

支气管哮喘患儿T淋巴细胞及细胞因子的变化

为观察儿童支气管哮喘时Ｔ淋巴细胞亚群分布以及Ｔ细胞活化相关因子及受体的表达状况，应用放射免疫分析等技术，对３４例发作期、２５例缓解期支气管哮喘患儿和１５例正常对照的外周血Ｔ淋巴细胞亚群、血浆及淋巴细胞膜表面白细胞介素－２受体（ＩＬ－２Ｒ）和相关细胞因子等水平进行系统检测。结果：（１）发作期患儿外周血Ｔ细胞亚群ＣＤ３，ＣＤ４及ＣＤ４／ＣＤ８值与缓解期患儿及正常对照比较差异无显著意义，但发作期ＣＤ８高于正常对照（Ｐ＜０．０１）和缓解组（Ｐ＜０．０１）；（２）发作期患儿血浆可溶性ＩＬ－２Ｒ、（ｓＩＬ－２Ｒ）、ＩｇＥ水平明显高于缓解期患儿和正常对照（Ｐ＜０．０１）；（３）免疫电镜观察显示，发作期患儿淋巴细胞膜表面ＩＬ－２Ｒ表达高于正常对照（Ｐ＜０．０５）。提示：（１）哮喘患儿外周Ｔ淋巴细胞亚群的分布发生了变化，哮喘发作期Ｔ淋巴细胞处于激活状态；（２）血浆ｓＩＬ－２Ｒ、ＩｇＥ水平与哮喘病情变化密切相关，可作为临床哮喘病情监测的指标。     

抗病毒iRNA对婴幼儿病毒性肺炎患儿外周血NK细胞活性的影响

本文应用抗ＡＤＶ－ＲＳＶ－ｉＲＮＡ治疗婴幼儿病毒性肺炎２６例，治疗后患儿外周血ＮＫ细胞活性比治疗前明显提高，与对照组比较差异有非常显著（Ｐ〈０．０１），提示抗病毒ｉＲＮＡ可通过增强患儿外周血ＮＫ细胞活性，达到治疗病毒性肺炎的目的。     

抗病毒iRNA对婴幼儿病毒性肺炎患儿外周血NK细胞活性的影响

本文应用抗ＡＤＶ－ＲＳＶ－ｉＲＮＡ治疗婴幼儿病毒性肺炎２６例，治疗后患儿外周血ＮＫ细胞活性比治疗前明显提高，与对照组比较差异有非常显著意义（Ｐ＜０．０１）。提示抗病毒ｉＲＮＡ可通过增强患儿外周血ＮＫ细胞活性，达到治疗病毒性肺炎的目的。     

小儿支气管哮喘时NO自由基与氧自由基的作用初探

采用镉还原柱层析和比色法测定了１７例哮喘患儿血浆亚硝酸／硝酸根离子（ＮＯ－２／ＮＯ－３）水平，另用生物学活性法测定了肿瘤坏死因子（ＴＮＦ）及丙二醛（ＭＤＡ）和超氧化物歧化酶（ＳＯＤ）水平。结果：小儿哮喘发作期血浆ＮＯ－２／ＮＯ－３、ＭＤＡ及ＴＮＦ水平明显升高（Ｐ分别＜０．０５或００１）；在缓解期ＮＯ－２／ＮＯ－３和ＭＤＡ水平恢复正常（Ｐ＞０．０５），ＴＮＦ水平虽有明显降低，但仍高于对照组（Ｐ＜０．０１）；而ＳＯＤ水平在急性发作期明显降低（Ｐ＜０．０１），缓解期恢复正常（Ｐ＞０．０５）。提示：哮喘发作时不仅有氧自由基增多，而且还有一氧化氮自由基增加，它们相互作用，共同损伤气道上皮等肺组织，加重炎症反应，导致气道高反应性而引起和（或）加重哮喘发病     

新生儿非感染性疾病免疫功能的研究

为探讨新生儿非感染性疾病免疫状态,本文测定了４５例新生儿非感染性疾病患儿多项免疫指标。结果显示：新生上患儿血ＩＬ－２水平、ＮＫ细胞活性明显低于对照组及其它患儿（Ｐ〈０．０１）,ＳＩＬ－２Ｒ水平及Ｔｓ抑制率则明显增高（Ｐ〈０．０１）,所有患儿血清Ｉｇ水平均无明显变化（Ｐ〉０．０５）。ＩＬ－２水平与ＮＫ细胞活性及Ｔｓ抑制率与ＳＩＬ－２Ｒ水平间均呈正相关（Ｐ〈０．０１）,ＩＬ－２水平与ＳＩＬ－２Ｒ水平、     

围产期窒息和缺氧缺血性脑病红细胞免疫粘附功能及机体免疫状态的研究

目的探讨围产期窒息及缺氧缺血性脑病（ＨＩＥ）时机体免疫状态,特别是红细胞免疫状态的变化与ＨＩＥ发病、病理变化及病情演变的关系。方法应用红细胞酵母菌花环试验、间接免疫荧光流式细胞仪检测法、单向免疫扩散法对围产期窒息及ＨＩＥ患儿红细胞免疫粘附（ＲＣＩＡ）功能,血Ｔ细胞亚群、血清免疫球蛋白及补体进行检测。结果窒息及ＨＩＥ组红细胞Ｃ３ｂ受体花环（Ｅ－Ｃ３ｂＲＲ）较对照组明显降低（Ｐ＜００５和＜００１）,且随着病情加重降低更为明显。在病情恢复期Ｅ－Ｃ３ｂＲＲ较急性期明显增高（Ｐ＜００５）,但仍低于正常组（Ｐ＜００５）。与正常组比较,窒息及ＨＩＥ组ＣＤ３＋、ＣＤ４＋明显降低（Ｐ＜００５）,ＣＤ８＋、ＣＤ４＋／ＣＤ８＋无显著变化。ＨＩＥ组血清ＩｇＧ、ＩｇＡ、ＩｇＭ及补体Ｃ３水平明显降低,且Ｅ－Ｃ３ｂＲＲ与ＣＤ８＋呈负相关,与ＣＤ４＋／ＣＤ８＋及ＩｇＭ呈正相关。结论围产期窒息及ＨＩＥ时红细胞及白细胞免疫功能均低下,且两者之间存在显著的相关性,ＲＣＩＡ功能的变化参与了ＨＩＥ的病理生理过程,可间接反映ＨＩＥ时脑损伤的程度及病情演变过程。     

CD44在神经母细胞瘤预后评价中的意义

目的：探讨ＣＤ４４在神经母细胞瘤（ＮＢ）中的预后价值。方法：对３０例ＮＢ进行ＣＤ４４免疫组化研究，同时与其他预后因素，如年龄、分期、病理分型、核分裂核碎裂指数（ＭＫＩ）、ＮＳＥ、Ｓ－１００蛋白等作对比分析。结果：①Ⅰ、Ⅱ、Ⅳ－Ｓ期二年生存率７６．７％，Ⅲ、Ⅳ期二年生存率３３．３％，（Ｐ＜０．０５）。②病理分型，生存率随着级别升高而降低，Ｐ＜０．０５。③ＭＫＩ测定结果与生存率无显著性差异，Ｐ＞０．０５。④２０例Ｓ－１００蛋白染色呈阳性，二年生存率（６０．０％）高于Ｓ－１００蛋白阴性组（２０．０％），Ｐ＜０．０５。⑤１４例ＣＤ４４染色阳性，其中≤１岁，Ⅰ、Ⅱ、Ⅳ－Ｓ期，Ｓ－１００蛋白阳性，高分化型，低ＭＫＩＮＢ中ＣＤ４４阳性率显著增高。ＣＤ４４阳性ＮＢ生存率（６８．８％）高于ＣＤ４４阴性者（２１．４％），统计学处理差异有显著性意义（Ｐ＜０．０５）。结论：ＣＤ４４是ＮＢ的一个新的肿瘤标记物，ＣＤ４４阳性是ＮＢ更为可靠的预后良好的指标。     

Sickle cell anemia and hematopoietic cell transplantation: When is a pound of cure worth more than an ounce of prevention?

Abstract:  Hematopoietic cell transplantation (HCT) is curative therapy for sickle cell anemia (SCA). However, its widespread use is constrained by donor availability and by concerns about its short-term and long-term toxicities. Current efforts to identify suitable candidates for HCT, to decrease the toxicity of HCT, and to broaden its availability are discussed.     

DEVELOPMENT OF THE HUMAN FETAL TESTIS

We describe the histological features of the fetal testis, utilizing 68 fetuses ranging in gestational age from 10 to 41 weeks. During fetal life, the tunica albuginea progressively increases in thickness, and between 29 and 32 weeks it develops two layers. Beyond 25 to 28 weeks, septa are invariably present. Tubules begin as straight structures and become maximally coiled by 30 weeks. Tubular diameter reaches its maximum by 16 weeks and remains constant throughout the rest of gestation. Germ cell and Sertoli cell numbers per tubular diameter have a wide range, but the median number for each cell type remains constant after 13 to 16 weeks. Leydig cells are most numerous between 17 and 19 weeks and decline thereafter. They are infrequent but still present at term. Interstitial lipochrome pigment accumulates during the latter half of gestation and may represent breakdown products from Leydig cell degeneration.     

Multifocal metachronous giant cell tumor in a 15-year-old boy

We report a case of multifocal metachronous giant cell tumor (GCT) that involved the fibula, tibia, and sacrum of a 15-year-old boy. Multifocal GCT of bone presenting in children is an exceedingly rare phenomenon; however, there is evidence that multifocal GCT presents, on average, at a younger age than solitary GCT. Pediatric radiologists should be aware of this when encountering a single lesion with characteristic radiographic features of GCT and when encountering multiple lytic skeletal lesions.     

DOR-1, A novel CD10+ stromal cell line derived from progressive Langerhans cell histiocytosis of bone

BACKGROUND: Langerhans cell histiocytosis (LCH) is granulomatous proliferative disorder characterized by the presence of activated Langerhans cells admixed with macrophages, lymphocytes, and eosinophils. In an effort to obtain an LCH ex vivo model, we succeeded in establishing the DOR-1 cell line from an LCH lesion of bone in a 3-year-old girl. PROCEDURE: The DOR-1 cell line was established from a CD1a immunoreactive LCH lesion of bone maintained in long-term cell culture. The phenotypic characteristics were assessed by immuno-cytochemistry and fluorescence activated cell sorter (FACS) analysis. Cytogenetic analysis was performed by RHG-banding that was supplemented by fluorescence in situ hybridization (FISH). RESULTS: The DOR-1 cells grew in vitro as a poorly differentiated mesenchymal-like cells with a doubling time between 72 and 96 hr. The cells exhibited pleomorphism and consistent immuno-reactivity for CD10 (50%), CD13 (55%), CD68 (65%), and CD117 (70%) while CD1a, Langerin and HLA-DR were not detected. By RHG-banding, several aberrant chromosomes were detected including the t (9; 17) (p23; p13) translocation and a pair of long dicentric marker chromosomes indicating clonal abnormality. Functionally, exposure to 33 nM 12-O-tetradecanoyl phorbol mirystate-13-acetate (TPA) induced DOR-1 cell differentiation with appearance of cytoplasmic extensions. CONCLUSIONS: The DOR-1 cell line exhibits distinct immuno-cytochemical features and carries the t (9; 17) (p23; p13) translocation suggesting involvement of stromal-like cell lineage in LCH initiation and progression.     

肺干细胞移植在急性呼吸窘迫综合征中的应用

近年来对肺干细胞的研究已取得了进展,肺干细胞属成体干细胞,来源广泛,无伦理争议,成为近几年的关注热点.本文探讨肺干细胞的生物学特征、表面标志,迁移、归巢以及治疗急性呼吸窘迫综合征的临床应用.     

蛋白激酶Cα对T细胞生物学功能影响的研究

目的探讨蛋白激酶Cα(PKCα)对T细胞增殖、凋亡、分化、细胞因子的生成、诱导性调节性T细胞(iTreg)生成的影响。方法分离野生型(PKCα+/+组)或PKCα基因敲除(PKCα-/-组)小鼠T细胞,体外培养,在T细胞表面受体(TCR)信号刺激下,利用3H胸腺嘧啶掺入法、CSFE/Annexin V染色结合流式细胞技术检测T细胞增殖及凋亡情况;收集细胞培养上清,用ELISA方法检测细胞因子IL-2、IL-4、IFN-γ和IL-17的生成。分离PKCα+/+组或PKCα-/-组小鼠CD4+T细胞,在TCR信号刺激下,按Th17细胞、iTreg分化条件进行体外诱导,用流式细胞技术检测细胞分化结果。结果与PKCα+/+组相比,PKCα缺失时,在TCR信号刺激下,T细胞的增殖降低,IL-2生成增多,IL-4和IL-17生成减少,Th17细胞分化减少,iTreg生成增加(均P结论 PKCα具有促炎作用。     

Monoclonal Antibody MT1: A Marker for Langerhans Cell Histiocytosis

Langerhans cells and their pathologic counterparts can be identified in paraffin sections using immunohistochemical staining for S-100 protein. This procedure is useful in confirming a diagnosis of Langerhans cell histiocytosis (LCH). However, many other cell types are also positive for S-100 protein. Positive staining for CD1 (Leu 6) supports a diagnosis of LCH, but requires frozen tissue. A panel of antibodies would be desirable in confirming a diagnosis of LCH, particularly if these antibodies could be used on paraffin-embedded material. We studied the pattern of staining for commercially available monoclonal antibodies MT1, MT2, MB2, and LN1, which were originally marketed as lymphocyte markers, using paraffin-embedded tissue sections of cases of LCH. In all 20 cases pathologic Langerhans cells stained positively with MT1 only. Various other S-100 protein-positive lesions were also examined with MT1 and were consistently negative for MT1. Other cutaneous histiocytic and mast cell lesions were positive with MT1, but S-100 protein negative. Our results demonstrate that the monoclonal antibody MT1 serves as an additional marker for LCH and, together with S-100 protein, would make up a diagnostic panel of antibodies for LCH to be used on routine paraffin-embedded sections.