Objective:To utilize the left ventricular myocardial work（LVMW）technique for early identification of myocardial damage in patients with fabry disease（FD）.Methods:In an observational cross-sectional study，35 patients with FD who visited the Second Hospital of Hebei Medical University from February 2023 to April 2024 were included. They were categorized into two groups based on left ventricular mass index（LVMI）：the non-left ventricular hypertrophy group（LVH-FD group，14 cases）and the left ventricular hypertrophy group（LVH+FD group，21 cases）. Further gender-stratified analysis was performed on parameters related to left ventricular global longitudinal strain（LVGLS）and LVMW. Additionally，for the FD group with normal LVGLS，a comparison of their LVMW-related parameters was made with a control group. A control group consisting of 28 healthy subjects from the same period was selected for comparison. The LVGLS，global work index（LVGWI），global constructive work（LVGCW），global wasted work（LVGWW），and global work efficiency（LVGWE）of the three groups were analyzed using two-dimensional speckle tracking and non-invasive myocardial work techniques，and intergroup comparisons of these parameters were performed. Pearson's linear correlation was applied to analyse the correlation between LVGWI，LVGWE and LVMI.Results:① According to the LVMI grouping results，compared with the control group，the LVH-FD group showed decreased LVGWI（ P<0.05），while the LVH+FD group exhibited reductions in LVGLS，LVGWI，and LVGWE（all P<0.05）. Compared to the LVH-FD group，the LVH+FD group demonstrated lower LVGLS，LVGWI，and LVGWE（all P<0.05）. ② Gender subgroup analysis revealed that in the female subgroup，no statistically significant differences were observed in LVGLS，LVGWI，and LVGWE between the female control group and the female LVH-FD group（all P>0.05）. However，the female LVH+ FD group showed decreased LVGLS，LVGWI，and LVGWE compared to both the female control group（all P<0.05）and the female LVH-FD group（all P<0.05）. In the male subgroup，compared to the male control group，the male LVH-FD group had reduced LVGWI（ P<0.05）but no significant differences in LVGLS or LVGWE（both P>0.05）. The male LVH+ FD group displayed lower LVGLS，LVGWI，and LVGWE compared to both the male control group（all P<0.05）and the male LVH- FD group（all P<0.05）. ③Compared with the control group，the LVGLS-normal FD group had a reduced LVGWI（ P<0.05），while there were no statistically significant differences in LVGLS，LVGWE，LVGCW，and LVGWW between these two groups（all P>0.05）. ④LVGWI and LVGWE were negatively correlated with LVMI（ r=-0.617，-0.707；both P<0.001）. Conclusions:LVMW can detect early cardiac dysfunction in patients with FD and is of guiding value for their clinical management.

Objective:To utilize the left ventricular myocardial work（LVMW）technique for early identification of myocardial damage in patients with fabry disease（FD）.Methods:In an observational cross-sectional study，35 patients with FD who visited the Second Hospital of Hebei Medical University from February 2023 to April 2024 were included. They were categorized into two groups based on left ventricular mass index（LVMI）：the non-left ventricular hypertrophy group（LVH-FD group，14 cases）and the left ventricular hypertrophy group（LVH+FD group，21 cases）. Further gender-stratified analysis was performed on parameters related to left ventricular global longitudinal strain（LVGLS）and LVMW. Additionally，for the FD group with normal LVGLS，a comparison of their LVMW-related parameters was made with a control group. A control group consisting of 28 healthy subjects from the same period was selected for comparison. The LVGLS，global work index（LVGWI），global constructive work（LVGCW），global wasted work（LVGWW），and global work efficiency（LVGWE）of the three groups were analyzed using two-dimensional speckle tracking and non-invasive myocardial work techniques，and intergroup comparisons of these parameters were performed. Pearson's linear correlation was applied to analyse the correlation between LVGWI，LVGWE and LVMI.Results:① According to the LVMI grouping results，compared with the control group，the LVH-FD group showed decreased LVGWI（ P<0.05），while the LVH+FD group exhibited reductions in LVGLS，LVGWI，and LVGWE（all P<0.05）. Compared to the LVH-FD group，the LVH+FD group demonstrated lower LVGLS，LVGWI，and LVGWE（all P<0.05）. ② Gender subgroup analysis revealed that in the female subgroup，no statistically significant differences were observed in LVGLS，LVGWI，and LVGWE between the female control group and the female LVH-FD group（all P>0.05）. However，the female LVH+ FD group showed decreased LVGLS，LVGWI，and LVGWE compared to both the female control group（all P<0.05）and the female LVH-FD group（all P<0.05）. In the male subgroup，compared to the male control group，the male LVH-FD group had reduced LVGWI（ P<0.05）but no significant differences in LVGLS or LVGWE（both P>0.05）. The male LVH+ FD group displayed lower LVGLS，LVGWI，and LVGWE compared to both the male control group（all P<0.05）and the male LVH- FD group（all P<0.05）. ③Compared with the control group，the LVGLS-normal FD group had a reduced LVGWI（ P<0.05），while there were no statistically significant differences in LVGLS，LVGWE，LVGCW，and LVGWW between these two groups（all P>0.05）. ④LVGWI and LVGWE were negatively correlated with LVMI（ r=-0.617，-0.707；both P<0.001）. Conclusions:LVMW can detect early cardiac dysfunction in patients with FD and is of guiding value for their clinical management.

Acute intermittent porphyria(AIP) is a rare inherited metabolic disease that can cause severe and fatal acute attacks. This article shares the treatment and management of a severe AIP patient. It is proposed that (1) avoiding incentives is essential; (2) emotional problems easily overlooked should be paid attention; (3) long-term follow-up and patient education can improve the prognosis. The patient underwent renal biopsy during the remission period. We found a red-brown-yellow-white refractive index crystal under a polarized light microscope that had not been reported in the previous literature, which was speculated to be a porphyrin crystal.

Objective:To investigate the effect of rapamycin (Rapa) on apoptosis of acute myeloid leukemia THP-1 cells induced by idarubicin (IDA) and its molecular mechanism.Methods:The THP-1 cells were treated with 10, 20, 40 and 80 nmol/L Rapa for 1 h, and the cells without Rapa treatment were set up. Western blot was used to detect the conversion of autophagy marker LC3 protein in THP-1 cells (the ratio of LC3Ⅱ/LC3Ⅰ), flow cytometry was used to detect the apoptotic rate, and the pretreatment concentration of Rapa was determined. THP-1 cells were treated with different concentrations of IDA for 24 h, the cell proliferation inhibition rate of IDA for THP-1 cells was detected by CCK-8 method, and the half maximal inhibitory concentration ( IC50) was calculated. THP-1 cells with or without Rapa treatment were treated by IDA with the concentration of lower than IC50 for 24 h, CCK-8 method was used to detect cell proliferation inhibition rate, flow cytometry was used to detect cell apoptosis, real-time fluorescent quantitative polymerase chain reaction was used to detect the expression changes of autophagy-related genes Beclin-1, LC3 and p62, and Western blot was used to detect the conversion of autophagy marker LC3 protein. Results:The ratio of LC3Ⅱ/LC3Ⅰ in THP-1 cells treated by 20 nmol/L Rapa was higher than that in the untreated cells ( P=0.002 4). The apoptotic rate in THP-1 cells treated by 80 nmol/L Rapa was higher than that in the untreated cells ( P=0.007 3). According to the results of Western blot and flow cytometry, 20 nmol/L Rapa was selected as the pretreatment concentration. The IC50 of IDA for THP-1 cells treated with IDA for 24 h was 59.874 nmol/L. After treated with 50 nmol/L IDA for 24 h, the proliferation inhibitory [(69.67±5.03)% vs. (41.67±3.51)%] and apoptotic rates [(74.35±4.83)% vs. (41.25±5.24)%] in THP-1 cells pretreated by Rapa were higher than those in the unpretreated cells (both P<0.05); the Beclin-1 and LC3 mRNA expression levels and the ratio of LC3Ⅱ/LC3Ⅰ in THP-1 cells pretreated by Rapa were higher than those in the unpretreated cells, and the expression of p62 mRNA was lower than that in the unpretreated cells (all P<0.05). Conclusion:Rapa can enhance the apoptosis of THP-1 cells induced by a relative low dose of IDA, which may be achieved through inducing excessive autophagy in THP-1 cells.

Objective: To explore the clinical and laboratory features of chronic myeloid leukemia(CML) patients with blast phase as initial presentation. Methods: The clinical manifestation, laboratory bindings and treatment of 3 CML patients with blast phase as the initial presentation from January 2015 to November 2016 in Luoyang Central Hospital Affiliated to Zhengzhou University were analyzed. The first symptoms of 3 patients were similar to acute leukemia. Results: The patients were presented with splenomegaly, obvious rise of white blood cell count in peripheral blood, increasing number of initial cells in the bone marrow, Philadelphia chromosome-positive (Ph⁺), fluorescence in situ hybridization of bcr-abl1 with fusion signals in segmented granulocytes, fusion gene with p210 positive and without the typical chronic phase of CML. Conclusions Due to the increased number of marrow blasts and the similarity of clinical manifestations to acute leukemia in CML patients with blast phase as the initial presentation, it is easy to misdiagnose as Ph⁺ acute leukemia in the initial phase. While some differences in genetics, clinical manifestations and genomics between them may help in differential diagnosis.

This study was aimed to make clear the origin and clinical applications of the Tibetan medicine "Bangga".Based on the systematical consultation of the Tibetan medical literature documents and the Chinese version,such as The King's Medicine,The Four Medical Tantras,Jing Zhu Materia Medica,the herbal textual research was made on the name,based source,origin and harvesting season,function,indication and etc.of Tibetan medicine "Bangga".The results showed that Tibetan medicine "Bangga" comes from the whole dried plant of Aconitum naviculare Stapf or A.tanguticum (Maxim) Stapf of Ranunculaceae plants.It was concluded that the research on the origin of Tibetan medicine "Bangga" can provide a basis for the application and promotion of quality standards of "Bangga".

Objective To investigate the role of neuroglobin ( NGB) in oxygen-glucose deprivation and reoxygenation ( OGD/R ) induced mitochondrial depolarization and reactive oxygen species ( ROS ) production in a human neuroblastoma cell line (SH-SY5Y).Methods SH-SY5Y cells were transfected with lentivirus to establish a stable cell line of NGB knockdown ( KD).After treated with OGD/R, cells were collected at different time points to analyze NGB mRNA and protein levels.Furthermore, cells were stained with JC-1 and DCFH-DA to evaluate mitochondrial depolarization and ROS production by inverted fluorescence microscope.Also, to determine the neurotoxicity , we measured the lactate dehydrogenase ( LDH) level in the cell culture medium.Results After the treatment of OGD/R, the NGB mRNA and protein started to elevate and peak at 4 h and 8 h (2.04 ±0.35 fold,1.69 ±0.18 fold).Compared with the vector group , NGB KD group had much more mitochondrial depolarization [ JC-1 red/green ( 1.10 ±0.10 ) vs (1.46 ±0.11),P<0.05] and ROS production [DCFH-DA fluorescence (36.30 ±5.32) vs (16.26 ± 2.97),P<0.05].Furthermore, NGB KD groups had a higher level of LDH release [(63.42 ±6.14)%vs (49.65 ±5.09 )%, P <0.05 ].Conclusions NGB plays an important role in the homeostasis of mitochondria.Knockdown of NGB results in increased mitochondrial depolarization , ROS production and neurotoxicity under hypoxia circumstances.

Castleman Disease ; Heart Neoplasms ; Humans ; Myxoma ; Retroperitoneal Space

Acute Disease ; Dermatitis ; Humans ; Hydrazines ; Hypersensitivity ; Nervous System Diseases ; Poisoning

Objective To observe the changes in fibrinolytic factors in rats with pulmonary thromboembolism (PTE) after recombinant prourokinase ( rPro-UK) treatment and its significance .Methods PTE was induced in male Sprague-Dawley (SD) rats by injecting heated 125iodine-labeled fibrinogen(Fib) autologous thromboemboli into external jugular veins.Twenty-eight rats were randomly assigned into following groups (7 rats each):①healthy control group;②PTE 5 d group,the rats in which were sacrificed at 5 d after the PTE model was made; ③ PTE3d receiving rPro-UK thrombolytic treament groups including multibolus treatment sub group ( rPro-UK was given in 1 mg/kg on the post-PTE third day followed by 2 consecutive days of a lower dose 0.25 mg/kg and rats were sacrificed 2 h after the last injection at the same time as PTE5d group) and single bolus treatment sub group ( rPro-UK was given in 1 mg/kg on the post-PTE third day followed by 2 consecutive days of 0.5 ml saline and rats were sacrificad at the same time as the former group ).The rats were quickly sacrificad at the fixed time through carotid bleeding and plasma samples were reserved for analysis of uroki -nase-type plasminogen activator (u-PA), urokinase-type plasminogen activator receptor (u-PAR), fibrinogen (Fib) andα2-antiplasmin (α2-AP) .Results ①Plasma concentrations of u-PA and u-PAR were increased were significantly in rPro-UK multibolus treatment sub group than in PTE 5 d group(Pu-PA <0.05,Pu-PAR <0.01)and rPro-UK single bolus treatment sub group(Pu-PA <0.01,Pu-PAR <0.05),correlated with the thrombolysis rate in rPro-UK multibolus treatment sub group (ru-PA =0.766,P<0.05;ru-PAR=0.785,P<0.05).② No difference of plasma Fib and α2-AP was seen between Pro-UK treatment groups and PTE 5 d group(P >0.05).Conclusion ① Plasma levels of endogenous u-PA and u-PAR are increased at different time points after PTE and are further enhanced after Pro-UK treatment, which promotes endogenous fibrinolysis and thrombus lysis .This is probably related to increased synthesis and secretion of endothelial cells which may be a key thrombolytic mechanism of Pro-UK.②Absence of systemic activation of the fibrinolytic system in Pro-UK multibo-lus treatment sub group means that the regimen is feasible and Pro-UK is fibrin specific .