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        Abnormal frequency of the memory B cell subsets and plasmablasts in patients with congenital severe hemophilia A: correlation with “Inhibitor” formation

        Omid Reza Zekavat,Yasaman Movahednezhad,Amin Shahsavani,Sezaneh Haghpanah,Negin Shokrgozar,Hossein Golmoghaddam,Mehdi Kalani,Mohammad Reza Bordbar,Nargess Arandi 대한혈액학회 2024 Blood Research Vol.59 No.2

        Background Development of antibodies against infused Factor VIII (FVIII) or "inhibitors" represents a major challenge following FVIII replacement therapy in patients with hemophilia A (HA). Recent studies have shown that certain cellular compartments of the immune system contribute to the production of such antibodies. Herein, we determined the frequency of class-switched CD19+ IgD−CD27+/non-class-switched CD19+ IgD+CD27+ memory B cell subsets and CD19+ CD27hiCD38hi plasmablasts in patients with severe HA and their association with the development of inhibitors in these patients. Methods This cross-sectional case–control study enrolled 32 patients with severe HA, including 8 with and 24 without inhibitors, and 24 healthy individuals. The frequencies of the memory B cell subsets and plasmablasts were determined using flow cytometry. Results The frequency of CD19+ IgD+CD27+ non-class-switched memory B cells was significantly lower in patients with HA (including both patients with and without inhibitors) than in healthy controls. The percentages of both CD19+ IgD−CD27+ class-switched and CD19+ IgD+CD27+ non-class-switched memory B cells did not differ significantly between patients with and without inhibitors. HA patients with inhibitors had significantly higher proportions of CD19+ CD27hiCD38hi plasmablasts than the control group as well as the inhibitor (-) ones. No significant correlation was observed between the inhibitor levels with the percentages of memory B cell subsets and plasmablasts. Conclusion This study is the first to demonstrate a dysregulated proportion of CD19+ IgD+CD27+ non-class-switched memory B cells and CD19+ CD27hiCD38hi plasmablasts in patients with severe HA. Therefore, strategies targeting memory B-cell/plasmablast differentiation may have promising outcomes in the management of inhibitor formation in patients with severe HA.

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        Association between Transfusion-Related Iron Overload and Liver Fibrosis in Survivors of Pediatric Leukemia: A Cross-Sectional Study

        Mahsa Sobhani,Naser Honar,Mohammad Reza Fattahi,Sezaneh Haghpanah,Nader Shakibazad,Mohammad Reza Bordbar 대한소아소화기영양학회 2024 Pediatric gastroenterology, hepatology & nutrition Vol.27 No.4

        Purpose: Patients who receive frequent blood transfusions are at an elevated risk of developing hepatic fibrosis due to iron overload in the liver. In this study, we evaluated the effectiveness of transient elastography (TE) (FibroScan®) for assessing liver fibrosis in patients with pediatric cancer. Methods: We enrolled 106 consecutive cases of acute leukemia in individuals under 21 years of age. The participants were followed for 2 years. Based on their serum ferritin (SF) levels, the patients were divided into two groups: group 1 (SF≥300 ng/mL) and group 2 (SF<300 ng/mL). A liver FibroScan® was performed, and a p-value of less than 0.05 was considered statistically significant. Results: Among the various parameters in the liver function test (LFT), alkaline phosphatase was significantly higher in a subgroup of patients aged 5–8 years in group 2 compared to those in group 1. The indices of liver fibrosis determined by TE, including the FibroScan score, controlled attenuation parameter score, steatosis percentage, and meta-analysis of histological data in viral hepatitis score, as well as indirect serum markers of liver fibrosis such as the aminotransferase (AST)/alanine aminotransferase (ALT) ratio, Fibrosis 4 score, and AST to platelet ratio index, did not differ significantly between the two groups. The association between the TE results and LFT parameters was only significant for ALT. Conclusion: Transfusion-associated iron overload does not have a significant correlation with severe liver fibrosis. FibroScan® is not a sensitive tool for detecting early stages of fibrosis in survivors of pediatric leukemia.

      • Relationship between Exposure to Pesticides and Occurrence of Acute Leukemia in Iran

        Maryam, Zakerinia,Sajad, Amirghofran,Maral, Namdari,Zahra, Lesan,Sima, Pooralimohamad,Zeinab, Attabac,Zahra, Mehravar,Fariba, Ebrahimi,Sezaneh, Haghpanah,Davood, Mehrabani Asian Pacific Journal of Cancer Prevention 2015 Asian Pacific journal of cancer prevention Vol.16 No.1

        Background: One of the causes of acute leukemia can be exposure to certain chemicals such as pesticides. This study determined the relationship between exposure to pesticides and the occurrence of acute leukemia in Fars province, south of Iran. Materials and Methods: Between April 2011 and April 2013 in a case-control study conducted in Nemazee Hospital in Shiraz, Southern Iran; 314 subjects diagnosed with acute leukemia (94 pediatric cases and 220 adults) were enrolled to determine any correlation between exposure to pesticides and the occurrence. Controls (n=314) were matched by sex and age. Results: There was a history of exposure to pesticides among 85% of pediatric cases and 69% of their controls and 83% of adult cases and 75% of their controls while 87.5% of pediatric cases and 90% of adult cases reported exposure to intermediate and high doses of pesticides and among the controls, the exposure to low doses of pesticides was 70.5% and 65%, respectively. Exposure to indoor pesticides was seen among most of cases and controls. Being a farmer was at a significantly more increased risk of developing acute leukemia in comparison to other jobs, especially for their children. Conclusions: Exposure to pesticides was shown to be one of the most important causes of acute leukemia. It seems that there is a need to educate the people on public health importance of exposure to pesticides especially during school time to reduce the risk of malignancies during childhood.

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