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51.
52.
C. BIGORGNE A. LE TOURNEAU B. MESSING B. RIO V. GIRAUD T. MOLINA J. AUDOUIN & J. DIEBOLD 《British journal of haematology》1996,95(2):258-262
Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months. The most striking change within the bone marrow was the presence of many pigment-laden histiocytes which had the typical morphology of sea-blue histiocytes seen in the so-called idiopathic sea-blue histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis. 相似文献
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54.
Charlotte W Ockeloen Marjolein H Willemsen Sonja de Munnik Bregje WM van Bon Nicole de Leeuw Aad Verrips Sarina G Kant Elizabeth A Jones Han G Brunner Rosa LE van Loon Eric EJ Smeets Mieke M van Haelst Gijs van Haaften Ann Nordgren Helena Malmgren Giedre Grigelioniene Sascha Vermeer Pedro Louro Lina Ramos Thomas JJ Maal Celeste C van Heumen Helger G Yntema Carine EL Carels Tjitske Kleefstra 《European journal of human genetics : EJHG》2015,23(9):1270-1185
Loss-of-function variants in ANKRD11 were identified as the cause of KBG syndrome, an autosomal dominant syndrome with specific dental, neurobehavioural, craniofacial and skeletal anomalies. We present the largest cohort of KBG syndrome cases confirmed by ANKRD11 variants reported so far, consisting of 20 patients from 13 families. Sixteen patients were molecularly diagnosed by Sanger sequencing of ANKRD11, one familial case and three sporadic patients were diagnosed through whole-exome sequencing and one patient was identified through genomewide array analysis. All patients were evaluated by a clinical geneticist. Detailed orofacial phenotyping, including orthodontic evaluation, intra-oral photographs and orthopantomograms, was performed in 10 patients and revealed besides the hallmark feature of macrodontia of central upper incisors, several additional dental anomalies as oligodontia, talon cusps and macrodontia of other teeth. Three-dimensional (3D) stereophotogrammetry was performed in 14 patients and 3D analysis of patients compared with controls showed consistent facial dysmorphisms comprising a bulbous nasal tip, upturned nose with a broad base and a round or triangular face. Many patients exhibited neurobehavioural problems, such as autism spectrum disorder or hyperactivity. One-third of patients presented with (conductive) hearing loss. Congenital heart defects, velopharyngeal insufficiency and hip anomalies were less frequent. On the basis of our observations, we recommend cardiac assessment in children and regular hearing tests in all individuals with a molecular diagnosis of KBG syndrome. As ANKRD11 is a relatively common gene in which sequence variants have been identified in individuals with neurodevelopmental disorders, it seems an important contributor to the aetiology of both sporadic and familial cases. 相似文献
55.
In vivo treatment with recombinant IL-12 protects C57BL/6J mice against secondary alveolar echinococcosis 总被引:1,自引:0,他引:1
ISABELLE EMERY CLAUDE LECLERC KAPPRASEUTH SENGPHOMMACHANH DOMINIQUE ANGÈLE VUITTON & MARTINE LIANCE 《Parasite immunology》1998,20(2):81-91
Using an experimental model of hepatic Echinococcus multilocularis infection in C57BL/6J mice, intraperitoneal administration of 0.8 μg of recombinant IL-12 to mice with an established infection was shown to reduce the parasite burden as soon as two weeks after the end of treatment. At that time, in vitro Echinococcus multilocularis -induced spleen T cell proliferative responses as well as IFN-γ and IL-5 production were higher in IL-12 treated mice than in untreated mice. Administration of 0.8 μg of IL-12 at the time of infection was shown to be without effect on the parasite establishment. However, this treatment greatly inhibited the subsequent metacestode development. Indeed, ten weeks after infection, it induced a complete healing in 37.5% of mice. At that time, the development of metastases was inhibited in 68.75% of IL-12-treated mice. This reduction of parasite burden was mainly associated with a strong proliferation of spleen cells to E. multilocularis antigen and with a high IFN-γ production. Altogether, our results show that IL-12 is of crucial importance in inhibiting the larval growth after the metacestode establishment in the liver and suggest that this cytokine could be of potential value in the treatment of human alveolar echinococcosis . 相似文献
56.
目的:建立了β-司他夫定原料有关物质的RP-HPLC测定方法和水解破坏制备系统适用性试验溶液方法。方法:采用Agilent 1100型高效液相色谱仪,使用SUPELCOSIL LC-18-DB(4.6 mm×250 mm,5μm)色谱柱,以0.01 mol·L-1醋酸铵溶液-乙腈(96.5∶3.5)和0.01 mol·L-1醋酸铵溶液-乙腈(75∶25)为流动相,梯度洗脱,检测波长254 nm,柱温25℃。结果:β-司他夫定和4种已知杂质及其他未知杂质均能达到有效分离;经水解破坏产生的α-司他夫定与β-司他夫定的分离度均达2.8;β-司他夫定、胸腺嘧啶、β-胸苷与5-O’-苯甲酰-司他夫定线性范围分别为0.51~26μg·m L-1(r=1.000)、0.13~27μg·m L-1(r=1.000)、0.50~25μg·m L-1(r=1.000)、1.7~6.3μg·m L-1(r=1.000),已知杂质胸腺嘧啶、β-胸苷与5-O’-苯甲酰-司他夫定的平均加样回收率(n=9)分别为102.8%(RSD=1.5%)、100.6%(RSD=0.9%)、101.9%(RSD=2.1%);β-司他夫定与3种已知杂质的最小检出量均在2.5 ng以下;经水解破坏制备的系统适用性溶液的重复性良好;供试品溶液在4℃下的30 h内基本稳定。结论:本方法灵敏、准确、可靠,专属性强,可用于β-司他夫定原料的有关物质测定。 相似文献
57.
SG Lindquist M Duno M Batbayli A Puschmann H Braendgaard S Mardosiene K Svenstrup LH Pinborg K Vestergaard LE Hjermind J Stokholm BB Andersen P Johannsen JE Nielsen 《Clinical genetics》2013,83(3):279-283
Recently, a hexanucleotide (GGGGCC) repeat expansion in the first intron of C9ORF72 was reported as the cause of chromosome 9p21‐linked frontotemporal dementia‐amyotrophic lateral sclerosis (FTD‐ALS). We here report the prevalence of the expansion in a hospital‐based cohort and associated clinical features indicating a wider clinical spectrum of C9ORF72 disease than previously described. We studied 280 patients previously screened for mutations in genes involved in early onset autosomal dominant inherited dementia disorders. A repeat‐primed polymerase chain reaction amplification assay was used to identify pathogenic GGGGCC expansions. As a potential modifier, confirmed cases were further investigated for abnormal CAG expansions in ATXN2. A pathogenic GGGGCC expansion was identified in a total of 14 probands. Three of these presented with atypical clinical features and were previously diagnosed with clinical olivopontocerebellar degeneration (OPCD), atypical Parkinsonian syndrome (APS) and a corticobasal syndrome (CBS). Further, the pathogenic expansion was identified in six FTD patients, four patients with FTD‐ALS and one ALS patient. All confirmed cases had normal ATXN2 repeat sizes. Our study widens the clinical spectrum of C9ORF72related disease and confirms the hexanucleotide expansion as a prevalent cause of FTD‐ALS disorders. There was no indication of a modifying effect of the ATXN2 gene. 相似文献
58.
目的 探讨胃泌素对胃癌细胞SGC7901 Reg Ⅰ(Reg Ⅰ)基因转录因子的效应.方法 应用巢式PCR技术从胃癌细胞SGC7901基因组DNA扩增Reg Ⅰ基因启动子1414bp片段,将该片段插入pMD19-T载体,序列分析鉴定.应用随机引物法以地高辛分别标记1414bp及其HindⅢ酶切800bp和614bp片段,经灵敏度检测后,作为探针.应用Genomatix MatInspector在线分析软件分析Reg Ⅰ基因启动子1414bp片段的转录因子结合位点.分别以10-7 mol/L和10-8mol/L胃泌素G-17处理胃癌细胞SGC7901 48h,提取核蛋白.应用DNA-蛋白质印迹法(Southern blotting),分别以地高辛标记的1414bp、800bp和614bp片段为探针检测胃泌素对胃癌细胞SGC7901 Reg Ⅰ基因转录因子的效应.结果 1414bp探针可检测到20条蛋白主带.胃泌素孵育后,带型没有变化,但是一些条带的灰度值改变,带9、12、13、14、15和16的灰度值明显降低(P<0.05);不同浓度胃泌素处理组之间上述6个条带的灰度值差异不明显(P>0.05).614bp探针可检测到灰度值变化的6条主带中的带9、12和13,胃泌素处理后,此3条主带的灰度值明显降低(P<0.05).800bp探针可检测到灰度值变化的6条主带中的带9、12和14,胃泌素处理后,仅带14的灰度值明显降低(P<0.05).614bp和800bp探针均未检出带15和带16.结论 胃癌细胞SGC7901Reg Ⅰ基因表达由多个转录因子协同调控.降低几个转录因子的结合活性可能是胃泌素上调胃癌细胞SGC7901Reg Ⅰ基因表达的途径之一. 相似文献
59.
目的:探讨乙型肝炎病毒(HBV)对肝内TGF-β1蛋白表达及Smads信号通路的作用,为制定慢性乙肝肝纤维化临床治疗策略提供理论依据。方法:(1)运用免疫组化PV-6000法检测对照组和慢性乙肝组肝组织中TGF-β1、HBsAg和HBcAg的表达,并采用荧光定量PCR法测定慢性乙肝患者血清HBV DNA含量。(2)应用体外细胞培养技术培养HBV刺激的人肝星状细胞系LX-2细胞,Western blotting方法测定其细胞内TGF-β1、Smad3和Smad7的蛋白表达。结果:(1)慢性乙肝组肝组织内TGF-β1的表达高于对照组(P<0.01);肝内TGF-β1表达水平与血清HBV DNA含量呈正相关(P<0.01),且HBcAg阳性肝组织水平较高(P<0.01)。(2)体外细胞学实验中,HBV刺激组LX-2细胞内TGF-β1和Smad3蛋白含量高于对照组和HBV+抗-TGF-β1组(P<0.01);Smad7蛋白表达差异无统计学意义(P>0.05)。结论:(1)TGF-β1在慢性乙肝患者肝组织中的表达与血清HBV DNA含量及肝内HBcAg的表达有关。(2)在TGF-β1/Smads信号通路中,HBV致纤维化作用机制以Smad3的正性调控为主,Smad7的作用不明显。 相似文献
60.
目的探讨尿微量白蛋白(MA)、尿α1-微球蛋白(α1-MG)、尿β2-微球蛋白(β2-MG)及血清β2-微球蛋白(β2-MG)在糖尿病肾病早期诊断中的意义。方法收集67例2型糖尿病患者和82名正常健康人的血清及尿液,分别利用化学发光法检测血清β2-MG、放射免疫法测定尿β2-MG及散射速率比浊法测定尿MA和尿α1-MG水平。结果与正常对照组相比,糖尿病组4组特定蛋白水平明显升高,结果有显著性差异(P均<0.05);此4组蛋白在正常对照组测得的结果无一例阳性,但在糖尿病组的阳性率分别为32.8%、47.8%、35.8%、34.3%;实验还发现随着病程的延长,糖尿病组4组蛋白的阳性率也相应升高(P<0.05)。结论测定尿液中MA、α1-MG、β2-MG及血清β2-MG水平对糖尿病肾病的早期诊断具有一定意义,能够帮助患者及早发现病情,减少疾病迁延。 相似文献