Objective To discuss MRI and pathological features of intracranial hemangioblastoma.Methods MRIdata of 26 cases of hemangioblastoma confirmed with operation and pathology were analyzed retrospectively.Results There were 22 cases with single lesion and multiple lesions in 4 eases.And a total of 31 lesions located in the posterior cranial fossa,including 14 lesions in right cerebellar hemispheres,9 lesions in left cerebellar hemispheres,2 lesions in cerebellar inferior vermis,4 lesions in medulla oblongata,and 2 lesions in fourth ventricle.Ninteen lesions showed cystic nodular type,1 lesion was cystic type,11 lesions showed parenchymal type or partial parenchyma type.Cystic lesions showed long T1 and long T2 signal,T1WI of solid area showed slightly long T1 and slightly long T2 signal,of which 12 lesions were slightly shorter T1 signal.Solid areas significantly enhanced,cystic area had no enhancement,but cystic wall of 3 cystic nodular lesions were slightly enhanced.DWI of solid areas showed low signal.MRS showed the peak of Cho obviously increased,and the peaks of Cr and NAA obviously decreased or nearly disappeared,and the high peak of lipids and lactate were observed.Conclusion The conventional and functional MRI of intracranial hemangioblastoma have some characteristics,MRI has a great value in the diagnosis and localization of intracranial hemangioblastoma.

Hypertension has a high global prevalence rate,which is the main preventable risk factor for cardiovascular disease.Home blood pressure monitoring plays an important role in diagnosis,adjustment of treatment strategies and long-term follow-up of hypertension.However,there are still enormous challenges for the popularization and standardized application of home blood pressure monitoring.Therefore,this review article describes indication,standardized implementation protocol,current status and influencing factors of implementation and management forms of home blood pressure monitoring.It can provide a reference for promoting the implementation of practical guidelines for home blood pressure monitoring and optimizing the application of home blood pressure monitoring.

Objective PINK1 and Parkin are directly invoveled in the regulation and maintenance of mitochondrial functional morphology. We aim to explore the effect of Wnt2 overexpression on PINK1^B9 Mutant Drosophila and its mechanism in this study. Methods The GAL4-UAS system was used to construct the normal control flies（W1118/ + ；MHC-GAL4/+）, PINK1^B9 transgenic Drosophila model flies（UAS-PINK1^B9 /y；MHC-GAL4 / +；Parkinson's disease model of Drosophila melanogaster), the Wnt2 overexpression flies（UAS-PINK1^B9 /y；MHC-GAL4 / Wnt2 OE） and the Wnt2 RNAi flies（UAS-PINK1^B9 /y；MHC-GAL4 /Wnt2）. On the 5th day, the abnormal wings phenotype rate and flying rate of flies were observed. The contents of Ndufs3 proteins were detected by Western blot. The mRNA expression levels of PGC-1α, Nrf1 and TFAM related to mitochondrial metabolism and synthesis were detected by real-time fluorescence quantitative PCR. The morphology of mitochondria was observed by electron microscopy. Complex I and Complex II function was detected by high-resolution mitochondrial respiratory system. Results Compared with the normal control flies, PINK1^B9 transgenic Drosophila model flies showed increased abnormal wings phenotype rate([1.87±0.06]% vs [68.79±0.70]%), decreased flying rate([ 97.51±0.52)% vs(3.95±0.53)%], and the differences were statistically significant(P<0.05); Compared with PINK1^B9 transgenic Drosophila model flies, the Wnt2 RNAi flies showed decreased abnormal wings phenotype rate[(10.14±1.72)%], increased flying rate([41.83±2.57]%)(P<0.05). Compared with the normal control flies, PINK1^B9 transgenic Drosophila model flies showed decreased expression levels of PGC-1α and Nrf1,Ndufs3 proteins, Complex I and Complex II(P<0.05);On the contrary, the Wnt2 RNAi flies showed increased trends compared with PINK1^B9 transgenic Drosophila model flies(P<0.05). Conclusion Overexpression of Wnt2 protects PINK1^B9 transgenic Drosophila models, which is related to the improvement of mitochondrial function.

Antineoplastic Combined Chemotherapy Protocols ; Bendamustine Hydrochloride/therapeutic use* ; Etoposide ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma/therapy* ; Melphalan ; Transplantation Conditioning ; Transplantation, Autologous

OBJECTIVETo establish a MDS mouse model with iron overload and to study the effect of iron overload on MDS.

METHODSThe exogenous mutant gene RUNX1-S291fs was inserted into the mice bone marrow mononuclear cell's genome in mice by retrovirus and transplanted into C57BL/6 mice irradiated by Co γ-ray. After 8 weeks,intraperitoneal injection of iron was performed to establish an MDS mouse model with iron overload. After 24 weeks of transplantation, the peripheral blood, bone marrow, femur, liver and spleen of mice were taken, then the morphological characteristics of peripheral blood and bone marrow cells were observed by Wright's staining; the liver, spleen and bone marrow were stained with Prussian blue to observe the iron deposition. The surface antigens of bone marrow cells were detected by flow cytometry. Bone marrow mononuclear cells and spleen tissue proteins were detected by Western blot to confirm the transfection of RUNX1-S291fs gene and expression of protein. The blood routine and transplanted cell chimeric rate of mice were monitored periodically.

RESULTSCompared with the empty plasmid control mice, levels of leukocyte and hemoglobin as well as platelet were decreased in RUNX1-S291fs mutant mice; the peripheral blood cells and bone marrow cells showed pathological hematopoiesis; the liver and spleen enlarged significantly; the tissue structure of femur, liver and spleen was abnormal; the expression of bone marrow cell surface antigens was abnormal. Bone marrow cells and spleen tissue expressed the RUNX1-S291fs protein. Compared with the controlled mice injected with normal saline, iron deposition occurred in the bone marrow, liver and spleen stained with Prussian blue in the mice injected with iron agent.

CONCLUSIONMice engineered to carry exogenous mutant gene RUNX1-S291fs and injected with iron showed pathologic features of MDS and iron overload, resulting in establishing MDS iron overloaded mouse model successfully, which lays a foundation for studying the effect of iron overload on MDS.

Animals ; Bone Marrow ; Disease Models, Animal ; Iron Overload ; Mice ; Mice, Inbred C57BL ; Spleen

OBJECTIVETo investigate the relationship of the mutational status of the ND4 gene and the clinical features of acute myelogenous leukemia (AML) patients with ND4 mutations.

METHODSUsing PCR combined with directly sequencing, we identified somatic mutations of ND4 in 121 primary AML patients to couple with their clinical features.

RESULTSThere were 58 male patients and 63 female patients (median age 49 years, 10-86 years). Eight of 121 patients (6.6%) with de novo AML were found harboring missense mutation of ND4 gene, including 3 patients with A131V (3/8, 37.5%), 2 patients with A404T (2/8, 25%), 1 patient with F149L (1/8, 12.5%), 1 patient with G242D (1/8, 12.5%) and 1 patient with Y409H (1/8, 12.5%), respectively. Patients with ND4 mutations were associated with good karyotype (P=0.049), regardless of gender, age, white blood cell, hemoglobin, platelet, blast cells of bone marrow or immunophenotype (P>0.05). There were no statistical significance in mutations of FLT3-ITD, NPM1, CEBPA, c-KIT and DNMT3A between patients with ND4 mutation and wild-type (wt) ND4 (P>0.05). The median overall survival of patients with ND4 mutations and wt ND4 were all not reached. The median relapse-free survival were not reached and 29(2-53) months, respectively (P>0.05). There was no significance in the ratio of CR and RR patients between wt ND4 and ND4 mutated groups (P>0.05).

CONCLUSIONIt was concluded that novel ND4 mutations could be found in de novo AML patients, especially in patients with good karyotype. Thus, ND4 mutations might play an important role in AML prognosis. However, whether the mitochondria dysfunction contribute to leukemogenesis needs to be further investigated.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Humans ; Leukemia, Myeloid, Acute ; drug therapy ; genetics ; Male ; Middle Aged ; Mutation ; NADH Dehydrogenase ; genetics ; Prognosis ; Young Adult