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排序方式: 共有1545条查询结果,搜索用时 816 毫秒
41.
Gabriela E Jones Pia Ostergaard Anthony T Moore Fiona C Connell Denise Williams Oliver Quarrell Angela F Brady Isabel Spier Filiz Hazan Oana Moldovan Dagmar Wieczorek Barbara Mikat Florence Petit Christine Coubes Robert A Saul Glen Brice Kristiana Gordon Steve Jeffery Peter S Mortimer Pradeep C Vasudevan Sahar Mansour 《European journal of human genetics : EJHG》2014,22(7):881-887
Microcephaly with or without chorioretinopathy, lymphoedema, or mental retardation (MCLMR) (MIM No.152950) is a rare autosomal dominant condition for which a causative gene has recently been identified. Mutations in the kinesin family member 11 (KIF11) gene have now been described in 16 families worldwide. This is a review of the condition based on the clinical features of 37 individuals from 22 families. This report includes nine previously unreported families and additional information for some of those reported previously. The condition arose de novo in 8/20 families (40%). The parental results were not available for two probands. The mutations were varied and include missense, nonsense, frameshift, and splice site and are distributed evenly throughout the KIF11 gene. In our cohort, 86% had microcephaly, 78% had an ocular abnormality consistent with the diagnosis, 46% had lymphoedema, 73% had mild-moderate learning difficulties, 8% had epilepsy, and 8% had a cardiac anomaly. We identified three individuals with KIF11 mutations but no clinical features of MCLMR demonstrating reduced penetrance. The variable expression of the phenotype and the presence of mildly affected individuals indicates that the prevalence may be higher than expected, and we would therefore recommend a low threshold for genetic testing. 相似文献
42.
Non-alcoholic fatty liver disease(NAFLD) is an umbrella term to describe the entire spectrum of this common liver disease. In patients with NAFLD, especially those with non-alcoholic steatohepatitis(NASH), most often have one or more components of the metabolic syndrome, but this is not universal. Although most patients with NAFLD share many clinical features, only a subset of patients develops significant liver inflammation and progressive fibrosis. On the other hand, not all patients with NASH exhibit insulin resistance. NASH can be seen in patients who are lean and have no identifiable risk factors. Many clinical studies have tried numerous drugs and alternative medicine, however, investigators have failed to identify a safe and effective therapy for patients with NASH. As summarized, the heterogeneity of pathogenic pathways in individual patients with NASH may warrant the development of an individualized treatment according to the underlying pathogenic pathway. The differentiation of pathogenetic targets may require the development of diagnostic and prognostic biomarkers, and the identification of genetic susceptibilities. At present, evidence-based medicine provides only a few options including life-style modifications targeting weight loss, pioglitazone and vitamin E in non-diabetic patients with biopsy-proven NASH. 相似文献
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Introduction
Circulating microparticles support thrombin generation. The aim of this study is to determine the indirect microparticle activity and the parameters of thrombin generation in healthy infants.Materials and methods
A total of 85 infants who were brought to follow-up visits were taken into the study. Blood samples were collected. Thrombin generation parameters and indirect microparticle activity were measured.Results
The infants were divided into four groups according to the time of follow-up visits. Mean ages were 1.18 ± 0.19 months in Group 1, 6.15 ± 0.16 months in Group 2, 12.38 ± 0.46 months in Group 3 and 24.53 ± 0.39 months in Group 4, respectively. There was no statistical difference among the age-based groups with respect to the indirect microparticle activity. The lag time and the TTP levels in Group 1 were lower than that found in Group 2. The ETP and peak levels were higher in Group 1 than that of Group 2. The ETP and peak levels in Group 2 were found lower than those found in older children, but the TTP level was found relatively higher. Statistically correlations were found between indirect microparticle activity and all parameters of thrombin generation.Conclusions
The absence of a difference in terms of age-based microparticle levels may suggest that the features of microparticles in healthy children of this age group are similar. Age-dependent changes in thrombin generation parameters may suggest a regulation mechanism for the thrombin generation system over the first years of life. The results may provide mean values for indirect microparticle activity and thrombin generation in this healthy group. 相似文献46.
47.
Aygul Dogan ?elik Zerrin Yulugkural Cumhur Kilincer Mustafa Kemal Hamamcioglu Figen Kuloglu Filiz Akata 《Rheumatology international》2012,32(8):2547-2549
Two cases of brucellar spondylodiscitis of the lumbar area were presented. Although both cases showed typical radiological changes, serological tests could not detect Brucella agglutinating antibodies. One of the patients was bacteremic and Brucella spp. was identified from blood culture. In the second patient needle biopsy was required for definite diagnosis. Although small, serologic tests have a certain rate of false negative results in brucellosis. Thus, a negative serology should not exclude the diagnosis of brucellosis, as it is demonstrated in the current cases. 相似文献
48.
Jain P Javdan M Feger FK Chiu PY Sison C Damle RN Bhuiya TA Sen F Abruzzo LV Burger JA Rosenwald A Allen SL Kolitz JE Rai KR Chiorazzi N Sherry B 《Haematologica》2012,97(4):599-607
Background
The levels and clinical relevance of Th17 cells and other interleukin-17-producing cells have not been analyzed in chronic lymphocytic leukemia. The objective of this study was to quantify blood and tissue levels of Th17 and other interleukin-17-producing cells in patients with this disease and correlate blood levels with clinical outcome.Design and Methods
Intracellular interleukin-17A was assessed in blood and splenic mononuclear cells from patients with chronic lymphocytic leukemia and healthy subjects using flow cytometry. Interleukin-17A-producing cells were analyzed in formalin-fixed, paraffin-embedded spleen and lymph node sections using immunohistochemistry and immunofluorescence.Results
The absolute numbers of Th17 cells in peripheral blood mononuclear cells and the percentages of Th17 cells in spleen cell suspensions were higher in patients with chronic lymphocytic leukemia than in healthy subjects; in six out of eight paired chronic lymphocytic leukemia blood and spleen sample comparisons, Th17 cells were enriched in spleen suspensions. Circulating Th17 levels correlated with better prognostic markers and longer overall survival of the patients. Two “non-Th17” interleukin-17-expressing cells were identified in chronic lymphocytic leukemia spleens: proliferating cells of the granulocytic lineage and mature mast cells. Granulocytes and mast cells in normal spleens did not express interleukin-17. Conversely, both chronic lymphocytic leukemia and healthy lymph nodes contained similar numbers of interleukin-17+ mast cells as well as Th17 cells.Conclusions
Th17 cells are elevated in chronic lymphocytic leukemia patients with better prognostic markers and correlate with longer survival. Furthermore, non-Th17 interleukin-17A-expressing cells exist in chronic lymphocytic leukemia spleens as maturing granulocytes and mature mast cells, suggesting that the microenvironmental milieu in leukemic spleens promotes the recruitment and/or expansion of Th17 and other IL-17-expressing cells. The pathophysiology of Th17 and non-Th17-interleukin-producing cells in chronic lymphocytic leukemia and their distributions and roles in this disease merit further study. 相似文献49.
Dalar L Eryüksel E Koşar F Karasulu AL Urer N Sökücü SN Altın S 《Tüberküloz ve toraks》2012,60(2):167-171
Malign fibrous histiocytoma is one of the most observed soft tissue sarcomas seen in the adults. The most common metastasis region is the lung and metastasis. Mounier-Kuhn syndrome is characterized by the highly dilatation of the trachea and bronchi. We may encounter with the major airway obstruction in the endoluminal or extraluminal lung and mediastinal masses or those with both components together. In this article, we would like to highlight the occurrence of a rare seen clinical situation secondary to the giant mediastinal malign fibrous histiocytoma metastasis and the clinical difficulties experienced in resolving of the main airway obstruction caused by the mass. Since the lack of the similar studies conducted previously, we found the case worth presenting. 相似文献
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