1.Regional homogeneity on resting state fMRI in patients with uremic restless legs syndrome
Jianxin GUO ; Dun DING ; Xueying MA ; Peng LI ; Ming ZHANG
Journal of Xi'an Jiaotong University(Medical Sciences) 2017;38(4):591-595
Objective To study neuromechanism for uremic restless syndrome (URLS),functional MRI (fMRI) analysis and were used to explor main activity.Methods Resting state functional magnetic resonance imaging (MRI) was performed on 29 patients with uremic restless legs syndrome and 25 healthy controls.The values of the regional homogeneity (ReHo) of the two groups were calculated and analyzed statistically.Result As compared with the control group,patients with uremic restless legs syndrome showed emotional and mental abnormality.Increased ReHo values in bilateral supplementary motor area,bilateral thalamus,left insular lobe,left hippocampus (P<0.05),and decreased ReHo values in anterior cingulate gyrus,bilateral posterior central gyrus,right amygdala were found in patients with uremic restless legs syndrome (P<0.05).The severity score of RLS was respectively positively correlated with dialysis duration (r =0.57,P =0.002),PTH level (r =0.419,P =0.033)BAI (r =0.528,P=0.006),and BDI (r =0.567,P =0.003).Conclusion Neuronal activity in related brain area in patients with uremic restless legs syndrome were found.This abnormality provides an objective diagnostic basis for the explanation of restless legs syndrome in maintenance hemodialysis patients.
2.Clinical features of acute megakaryocytic leukemia in children: analysis of 14 cases
Jianxin DUN ; Qun HU ; Aiguo LIU ; Ai ZHANG ; Yaqin WANG ; Wen YU ; Songmi WANG
Journal of Leukemia & Lymphoma 2022;31(10):599-602
Objective:To investigate the clinical features of acute megakaryocytic leukemia (AMKL) in children.Methods:The clinical data of 14 children with AMKL in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2012 to July 2021 were retrospectively analyzed, and the related literature was reviewed.Results:Among 14 children with AMKL, there were 5 males and 9 females, and the median age of onset was 19 months (0.1-109 months); 1 case was Down syndrome-related AMKL, and 13 cases were non-Down syndrome-related AMKL. Most of the children presented with fever, anemia or bleeding symptoms, and a few patients presented with joint pain as the primary symptom. Some children were accompanied by extramedullary infiltration such as hepatomegaly, splenomegaly or lymphadenovarix. Initial investigations of 14 children showed that the median white blood count, hemoglobin concentration and platelet count were 10.67×10 9/L [(6.56-83.62)×10 9/L], 84 g/L (55-121 g/L), 37×10 9/L [(8-1443) ×10 9/L], respectively, and the median proportion of naive cells in peripheral blood was 0.09 (0.00-0.79). Bone marrow smear showed that the primitive megakaryocytes were characterized by various size and irregular form, a few of which had cytoplasmic vacuoles, and the median proportion of bone marrow primitive megakaryocytes was 0.636 (0.332-0.976); the nuclei were round or irregular, with multiple nucleoli or hidden nucleoli. RAS staining was partially positive, and immunohistochemical assay showed that POX, AS-DNCE and α-NBE were negative. Detection of megakaryocyte-associated antigens by flow cytometry showed 12 children expressed CD41a or CD61, and 10 children expressed CD42b. Among 3 children who completed chemotherapy, 1 case of Down syndrome-related AMKL and 1 case of non-Down syndrome-related AMKL were event-free survival, and 1 case of non-Down syndrome-related AMKL died after bone marrow relapse. Conclusions:The clinical manifestations and biological characteristics of children with AMKL are complicated and the prognosis is poor. Some children can achieve disease-free survival through chemotherapy alone.