Objective:To analyze the clinical characteristics of adult patients with phytosterolemia presenting with thrombocytopenia as the initial manifestation.Methods:A retrospective analysis was conducted on eight adult patients with phytosterolemia who visited Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, from December 2020 to December 2023.Results:① The participants consisted of 2 (25%) male and 6 (75%) female patients, with a median age at diagnosis of 55 years (range: 29-66 years). The median duration from the discovery of thrombocytopenia to diagnosis was 10 years (range: 0.2-50 years). ② Compared with the normal control group (30 healthy adult volunteers) and the immune thrombocytopenia (ITP) control group (20 patients with ITP), patients with phytosterolemia exhibited significantly higher mean platelet volume and large platelet ratio. Peripheral blood smears revealed that the mean platelet diameter and the proportion of large platelets (diameter> 4 μm) were significantly higher in patients with phytosterolemia than those in the normal and ITP control groups ( P<0.01). ③ After a low-plant-sterol diet and ezetimibe treatment, five patients demonstrated decreased serum sitosterol and campesterol levels, increased hemoglobin concentration and platelet counts, and reduced platelet volume. Conclusion:Adult-onset phytosterolemia presenting with thrombocytopenia as the initial manifestation is prone to misdiagnosis. The presence of hemolytic anemia, splenomegaly, increased large platelets and schistocytes on peripheral blood smears, and xanthomas are crucial diagnostic indicators. Restricting dietary plant sterol intake and using ezetimibe to inhibit sterol absorption effectively lowers serum plant sterol levels and improves hematological abnormalities.

Objective:To analyze the clinical characteristics of adult patients with phytosterolemia presenting with thrombocytopenia as the initial manifestation.Methods:A retrospective analysis was conducted on eight adult patients with phytosterolemia who visited Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, from December 2020 to December 2023.Results:① The participants consisted of 2 (25%) male and 6 (75%) female patients, with a median age at diagnosis of 55 years (range: 29-66 years). The median duration from the discovery of thrombocytopenia to diagnosis was 10 years (range: 0.2-50 years). ② Compared with the normal control group (30 healthy adult volunteers) and the immune thrombocytopenia (ITP) control group (20 patients with ITP), patients with phytosterolemia exhibited significantly higher mean platelet volume and large platelet ratio. Peripheral blood smears revealed that the mean platelet diameter and the proportion of large platelets (diameter> 4 μm) were significantly higher in patients with phytosterolemia than those in the normal and ITP control groups ( P<0.01). ③ After a low-plant-sterol diet and ezetimibe treatment, five patients demonstrated decreased serum sitosterol and campesterol levels, increased hemoglobin concentration and platelet counts, and reduced platelet volume. Conclusion:Adult-onset phytosterolemia presenting with thrombocytopenia as the initial manifestation is prone to misdiagnosis. The presence of hemolytic anemia, splenomegaly, increased large platelets and schistocytes on peripheral blood smears, and xanthomas are crucial diagnostic indicators. Restricting dietary plant sterol intake and using ezetimibe to inhibit sterol absorption effectively lowers serum plant sterol levels and improves hematological abnormalities.

Objective:To explore the effectiveness of online and offline blended teaching in caregivers of individuals with high-risk pressure injury in primary elderly care institutions.Methods:From December 2020 to December 2021, convenience sampling was used to select 100 caregivers of individuals with high-risk stress injuries from 15 elderly care institutions in cooperation with the Linhai Second People's Hospital as participants. The participants were randomly divided into a control group ( n=50) and an experimental group ( n=50) using the random number method. The control group was treated with usual face-to-face training, and the experimental group was treated with online and offline blended teaching, with an intervention period of three months. The two groups' caring behavior, pressure injury knowledge, and caregiver burden were compared before and after the intervention. Results:After the intervention, the scores of the Pressure Ulcer Caring Behaviors Questionnaire and Caregivers' Knowledge about Pressure Ulcer Questionnaire in the experimental group were higher than those in the control group ( P<0.05). The score of the Zarit Caregiver Burden Interview in the experimental group was lower than that in the control group ( P<0.05) . Conclusions:Online and offline blended teaching is beneficial for improving the knowledge and behavior of caregivers of individuals with high-risk pressure injury in primary elderly care institutions, reducing caregiver burden, and is worth promoting and applying in primary elderly care institutions.

Objective:To explore the effectiveness of online and offline blended teaching in caregivers of individuals with high-risk pressure injury in primary elderly care institutions.Methods:From December 2020 to December 2021, convenience sampling was used to select 100 caregivers of individuals with high-risk stress injuries from 15 elderly care institutions in cooperation with the Linhai Second People's Hospital as participants. The participants were randomly divided into a control group ( n=50) and an experimental group ( n=50) using the random number method. The control group was treated with usual face-to-face training, and the experimental group was treated with online and offline blended teaching, with an intervention period of three months. The two groups' caring behavior, pressure injury knowledge, and caregiver burden were compared before and after the intervention. Results:After the intervention, the scores of the Pressure Ulcer Caring Behaviors Questionnaire and Caregivers' Knowledge about Pressure Ulcer Questionnaire in the experimental group were higher than those in the control group ( P<0.05). The score of the Zarit Caregiver Burden Interview in the experimental group was lower than that in the control group ( P<0.05) . Conclusions:Online and offline blended teaching is beneficial for improving the knowledge and behavior of caregivers of individuals with high-risk pressure injury in primary elderly care institutions, reducing caregiver burden, and is worth promoting and applying in primary elderly care institutions.

Objective To study the effect of bilateral arm training on cortical reorganization in cerebral infarction patients using blood oxygen level-dependent functional MRI (BOLD-fMRI).Methods Fourteen cerebral infarction subjects with moderate to severe upper limb impairment participated in this randomized and single-blinded training study.Subjects in the bilateral arm training group (n =7) practiced bilateral symmetrical or opposite activities,while the control group (n =7) performed conventional,mainly unilateral,arm training.Those in both groups received fMRI scans before and after the training with passive elbow movement as the task in the imaging.Results Both groups had significant improvements in their average scores on the Fugl-Meyer upper extremity assessment and in their modified Barthel index scores after the treatment,and there were no significant differences between the two groups.Brain activation had increased in both groups after the treatment,especially in the bilateral training group.After the treatment,the first motor area (M 1),the first somatosensory area and the supplementary motor area on both sides were activated in the bilateral training group.Moreover,for patients in the bilateral training group with injuries of moderate severity,the M1 of the opposite side tended to be activated,while for those more severely injured the same side had a tendency to be activated.Conclusion Convalescing subcortical infarction patients with moderate to severe upper limb impairment benefit from bilateral arm training in terms of motor activity and performing activities in daily life.It may be related to the normalization of inhibition between the hemispheres of the bilateral cerebral cortex and the reconnection of ipsilateral corticospinal pathways.

OBJECTIVETo investigate the expression of vascular endothelial growth factor (VEGF) of human multiple myeloma (MM) cell line RPMI8226 regulated by brain-derived neurotrophic factor (BDNF), and preliminarily approach the close relationship between BDNF and angiogenesis of MM.

METHODSThe recombinant eukaryotic BDNF siRNA expression vector was designed and constructed. The empty vector pGenesil-1, and the recombinant plasmid, pGenesil-shRNA-BDNF were transfected into RPMI8226 cells using Lipofectamine™ 2000 (groups P0 and P1, respectively). BDNF mRNA and protein level in RPMI8226 cells were detected by RT-PCR and Western blotting, respectively; the cellular proliferation activity was determined by MTT assay, while the cell apoptosis was measured by flow cytometry; the variation of VEGF mRNA level in RPMI8226 cells via transfection was determined by RT-PCR, the secretion of VEGF was detected by ELISA.

RESULTS(1)The recombinant eukaryotic BDNF siRNA expression vectors were successfully constructed. BDNF mRNA expression and protein level in P1 group were significantly inhibited compared to those in non-transfected group (Pn) and P0 groups (P<0.05); (2)MTT tests demonstrated that the cellular proliferation activities were obviously decreased in Pn (0.42 ± 0.06) vs P0 (0.56 ± 0.06) and P1 (0.50 ± 0.04) groups (P<0.05); (3)The early cell apoptosis rates were statistically increased in P1 [(53.84 ± 9.95)%] vs Pn [(5.23 ± 2.46)%] and P0 [(9.10 ± 3.46)%] groups (P<0.01); (4)The silence of endogenous BDNF significantly decreased the expression of VEGF in RPMI8226 cells:the relative expression level of VEGF121, VEGF145 and VEGF165 in P1 group were (0.62 ± 0.07), (0.47 ± 0.09) and (0.57 ± 0.02) folds compared to Pn group (P<0.05); (5)ELISA demonstrated that secretion of VEGF in P1 group were (0.36 ± 0.05) and (0.44 ± 0.06) folds compared to Pn and P0 group, respectively (P<0.05).

CONCLUSIONBDNF gene silence can obviously increase apoptosis of RPMI8226 cells, inhibit their proliferation and decrease the expression of VEGF. BDNF might mediate the expression of VEGF in MM cells, which may be involved in MM angiogenesis.

Apoptosis ; Brain-Derived Neurotrophic Factor ; Cell Line, Tumor ; Gene Silencing ; Genetic Vectors ; Humans ; Multiple Myeloma ; Neovascularization, Pathologic ; RNA, Messenger ; RNA, Small Interfering ; Transfection ; Vascular Endothelial Growth Factor A

In this study, we administered a modified schedule of weekly intravenous Bortezomib at 1.6 mg/m(2) with dexamethasone (BD) and compared it to the standard 1.3 mg/m(2) twice-weekly BD regimen in Chinese patients with newly diagnosed multiple myeloma (MM). We assessed the difference in efficacy, safety profile and survival between the once-weekly and twice-weekly cohorts (13 vs. 24 patients). The over response rate was similar with both arms of the study, being 77% in the once-weekly schedule and 74.9% in the twice-weekly schedule (P=0.690). The median overall survival was not reached in either schedule. Also, the median progression-free survival and duration of response of the once-weekly schedule did not significantly differ from those of the twice-weekly schedule (8 months vs.10 months, P=0.545 and 6 months vs.7 months, P=0.467 respectively). Peripheral sensory neuropathy and grade 3/4 hematologic toxic effects were more frequently reported in the twice-weekly schedule than the once-weekly schedule, but there was no statistically significant difference. This preliminary experience in Chinese patients with newly diagnosed MM indicated that once-weekly infusion of Bortezomib plus dexamethasone may improve safety without affecting outcome.
Adult ; Aged ; Boronic Acids ; administration & dosage ; Bortezomib ; Dexamethasone ; administration & dosage ; Female ; Humans ; Male ; Middle Aged ; Multiple Myeloma ; drug therapy ; Pyrazines ; administration & dosage

In this study, we administered a modified schedule of weekly intravenous Bortezomib at 1.6 mg/m(2) with dexamethasone (BD) and compared it to the standard 1.3 mg/m(2) twice-weekly BD regimen in Chinese patients with newly diagnosed multiple myeloma (MM). We assessed the difference in efficacy, safety profile and survival between the once-weekly and twice-weekly cohorts (13 vs. 24 patients). The over response rate was similar with both arms of the study, being 77% in the once-weekly schedule and 74.9% in the twice-weekly schedule (P=0.690). The median overall survival was not reached in either schedule. Also, the median progression-free survival and duration of response of the once-weekly schedule did not significantly differ from those of the twice-weekly schedule (8 months vs.10 months, P=0.545 and 6 months vs.7 months, P=0.467 respectively). Peripheral sensory neuropathy and grade 3/4 hematologic toxic effects were more frequently reported in the twice-weekly schedule than the once-weekly schedule, but there was no statistically significant difference. This preliminary experience in Chinese patients with newly diagnosed MM indicated that once-weekly infusion of Bortezomib plus dexamethasone may improve safety without affecting outcome.