DNA Mutational Analysis ; Diagnosis, Differential ; Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; genetics ; therapy ; Mutation ; genetics ; Perforin ; Pore Forming Cytotoxic Proteins ; genetics ; Qa-SNARE Proteins ; genetics

Objective To meet an emergency of film development by automatic-manual X-ray film development machine when malfunction or power failure occurs.Methods The field dark room was made along with development and fixing baths.The film was developed artificially.Results Satisfactory films for limbs and chest were developed.Conclusion The equipment is easy to load,spread and operate.It can be used not only as assistant field film development equipment,but also as training equipment for field hygiene unit that isn't allotted automatic-manual X-ray film development machine.

In this study, norcanthridin (NCTD)-encapsulated liposomes were modified with a novel murine anti-human CD19 monoclonal antibody 2E8 (2E8-NCTD-liposomes) and the targeting efficiency and specific cytotoxicity of 2E8-NCTD-liposomes to CD19(+) leukemia cells were evaluated. BALB/c mice were injected with 2E8 hybridoma cells to obtain 2E8 monoclonal antibody (mAb). NCTD-liposomes were prepared by using film dispersion method. 2E8 mAbs were linked to NCTD-liposomes using post-incorporation technology. Flow cytometry showed that the targeting efficiency of purified 2E8 mAbs on CD19(+) Nalm-6 cells was 99.93%. The purified 2E8 mAbs were conjugated with NCTD-liposomes to prepare 2E8-NCTD-liposomes whose targeting efficiency on CD19(+) Nalm-6 was also 95.82%. The average size of 2E8-NCTD-liposomes was 118.32 nm in diameter. HPLC showed that the encapsulation efficiency of NCTD was 46.51%. When the molar ratio of 2E8/Mal-PEG(2000)-DSPE reached 1:50, we obtained the liposomes with 9 2E8 molecules per liposome. The targeting efficiency of 2E8-NCTD-liposomes on CD19(+) leukemia cells was significantly higher than that on CD19-leukemia cells. Similarly, the targeting efficiency of the immunoliposomes was also higher than that of the NCTD-liposomes on CD19(+) leukemia cells. Those results were consistent with those observed by laser scanning confocal microscopy. 3-(4, 5-Dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay demonstrated that 2E8-NCTD-liposomes specifically killed Nalm-6 cells in a dose- and time-dependent manner. The viability of Nalm-6 cells treated by 2E8-NCTD-liposomes was significantly lower than that of Molt-3 cells and it was also significantly lower than that of Nalm-6 cells treated with the same concentration of NCTD-liposomes or free NCTD. We are led to concluded that 2E8 antigen can serve as a specific targeting molecule of B lineage hematopoietic malignancies for liposome targeting, and 2E8-NCTD-liposomes can be used as a new and effective means for the treatment of B lineage hematopoietic malignancies.

Adolescent ; Antibodies, Monoclonal ; therapeutic use ; Antigens, Neoplasm ; immunology ; Cancer Vaccines ; immunology ; Child ; Child, Preschool ; Humans ; Immunotherapy ; methods ; Infant ; Killer Cells, Natural ; immunology ; Lymphocyte Transfusion ; Neoplasms ; immunology ; therapy ; Pediatrics ; Stem Cell Transplantation

Objective To observe the influencing of Yiqi Huayu Jiedu Prescription on the growth of HepG2 nude mice transplantation tumor and the expression of related factors of vascular mimicry. Methods Models of transplanted tumors, which were made by HepG2 cells in nude mice, were randomly divided into 7 groups, Yiqi Huayu Jiedu Prescription group, Astragali Radix group, Curcumae Rhizoma group, Paridis Rhizoma group, Gecko group, cis-platinum group, and model group. Except for the model group, the rest groups were given relevant medicine for intervention. 21 days later. HIF-1α, MMP-2, MMP-9, and E-cad were detected by immunohistochemistry, and Twist1 and Bcl-2 were detected by fluorescence quantitative PCR. Results Compared with the model group, tumor volume in the rest groups decreased (P<0.05), and the effect in the Yiqi Huayu Jiedu Prescription group was more obvious than the Astragali Radix group, Paridis Rhizoma group and Gecko group (P<0.05);The expression of vasculogenic mimicry structure was rare in each group, and the model group and cis-platinum group were the most obvious;Except for the Astragali Radix group, the expressions of HIF-1α, MMP-2, and MMP-9 showed statistical significance compared with model group (P<0.05);The expression of E-cad in the Yiqi Huayu Jiedu Prescription group and Astragali Radix group showed statistical significance (P<0.05);The expression of Bcl-2 in the Yiqi Huayu Jiedu prescription group, Paridis Rhizoma group, and Gecko group decreased significantly compared with the model group (P<0.05);The expression of Bcl-2 in the Yiqi Huayu Jiedu prescription group was much better than the other groups (P<0.05);The expression of Twist1 showed statistical significance in the Yiqi Huayu Jiedu Prescription group, Curcumae Rhizoma group, Paridis Rhizoma group, and cis-platinum group (P<0.05). Conclusion Yiqi Huayu Jiedu Prescription can reduce expression of HIF-1α, Twist1, Bcl-2, MMP-2, and MMP-9, and increase expression of E-cad, thereby inhibiting the formation of vascular mimicry.

hematological malignant tumors with CD19 high expression.

In this study,norcanthridin(NCTD)-encapsulated liposomes were modified with a novel murine anti-human CD19 monoclonal antibody 2E8(2E8-NCTD-liposomes)and the targeting efficiency and specific cytotoxicity of 2E8-NCTD-liposomes to CD19+leukemia cells were evaluated.BALB/c mice were injected with 2E8 hybridoma cells to obtain 2E8 monoclonal antibody(mAb).NCTD-liposomes were prepared by using film dispersion method.2E8 mAbs were linked to NCTD-liposomes using post-incorporation technology.Flow cytometry showed that the targeting efficiency of purified 2E8 mAbs on CDI9+Nalm-6 cells was 99.93%.The purified 2E8 mAbs were conjugated with NCTD-liposomes to prepare 2E8-NCTD-liposomes whose targeting efficiency on CD19+Nalm-6 was also 95.82%.The average size of 2E8-NCTD-liposomes was 118.32 nm in diameter.HPLC showed that the encapsulation efficiency of NCTD was 46.51%.When the molar ratio of 2E8/MaI-PEG2000-DSPE reached 1:50,we obtained the liposomes with 9 2E8 molecules per liposome.The targeting efficiency of 2E8-NCTD-liposomes on CD19+leukemia cells was significantly higher than that on CD19-leukemia cells.Similarly,the targeting efficiency of the immunoliposomes was also higher than that of the NCTD-liposomes on CD 19+leukemia cells.Those results were consistent with those observed by laser scanning confocal microscopy.3-(4,5-Dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide(MTT)assay demonstrated that 2E8-NCTD-liposomes specifically killed Nalm-6 cells in a dose-and time-dependent manner.The viability of Nalm-6 cells treated by 2E8-NCTD-liposomes was significantly lower than that of Molt-3cells and it was also significantly lower than that of Nalm-6 cells treated with the same concentration of NCTD-liposomes or free NCTD.We are led to concluded that 2E8 antigen can serve as a specific targeting molecule of B lineage hematopoietic malignancies for liposome targeting,and 2E8-NCTD-liposomes can be used as a new and effective means for the treatment of B lineage hematopoietic malignancies.

Hemophagocytic lymphohistiocytosis (HLH) in different ethnicities has been described in the literature, but few cases in patients of Chinese descent have been reported. Here, we describe the case of a Chinese neonate presenting with HLH carrying novel, compound heterozygous mutations of the UNC13D gene, including [c.2295_2298delGCAG, p.Glu765Aspfs*27] in exon 23, c.-250C>T, c.1+30G>A, c.279C>T, c.888G>C, c.18+36A>G, c.20-48T>C, c.1977C>T, c.2296C>T, c.24-46C>T, c.26-9_26-8insC, c.2599A>G, c.28+48C>T and c.3198A>G, some of which have not been reported in the literature. Cytokine profile analyses were performed in this patient, and the results were consistent with our previous findings in HLH patients. Cytokine profile monitoring may be helpful in differentiating among various clinical phases of HLH.
Asian Continental Ancestry Group/genetics ; Cytokines/blood ; Heterozygote ; Humans ; Infant, Newborn ; Lymphohistiocytosis, Hemophagocytic/drug therapy/*genetics ; Male ; Membrane Proteins/*genetics ; *Mutation

Objective:To summarize our experience and outcomes of surgical repair of type Ⅱ right-sided aortic arch(RAA) with Kommerell's diverticulum(KD).Methods:From May 2010 to August 2020, a total of 13 patients with type Ⅱ RAA and KD underwent surgery at our center. Mean age was(50.46±10.31) years, 10 were male, and 3 were female. All patients had an aneurysmal KD and aberrant left subclavian artery(ALSA). Preoperative comorbidities included type B aortic dissection in 1 case, aortic arch pseudoaneurysm in 2 cases, and type B intramural hematoma in 2 cases, respectively. Eight(61.5%) patients underwent stented elephant trunk procedures under moderate hypothermic circulatory arrest combined with selective antegrade cerebral perfusion via median sternotomy, and all of them had ALSA reconstruction. Five(38.5%) patients underwent distal arch and descending thoracic aortic replacement through a right posterolateral thoracotomy, the ALSA was reconstructed or ligated in 1 each, and ALSA embolization was performed before surgery in the other 3 cases.Results:No operation deaths occurred. Recurrent laryngeal nerve injury occurred in 2 cases. There was 1(7.69%) in-hospital death. Follow-up was complete in 100 % at mean(5.28±3.84) years. No late death occurred. A persistent anastomotic leak of the proximal arch was detected in a patient who underwent stented elephant trunk procedure, but no aortic dilatation or tracheal and esophageal compression was observed during follow-up. Meanwhile, aortic events, limb ischemia, or symptoms of tracheal and esophageal compression were not observed in the remaining 11 patients.Conclusion:Surgical repair of type Ⅱ RAA with KD can achieve favorable early and midterm outcomes. Surgical strategies should be chosen based on the anatomy of the aorta and whether it is combined with compression symptoms.

Objective To summarize our experience and the early and midterm outcomes of stented elephant trunk procedure for right-sided aortic arch (RAA) with Kommerell's diverticulum (KD). Methods From April 2013 to July 2020, patients with RAA and KD who underwent stented elephant trunk procedure at our center were collected. Surgery was performed under moderate hypothermic circulatory arrest combined with selective antegrade cerebral perfusion via median sternotomy. Results A total of 8 patients were included, including 7 males and 1 female with a mean age of 51.88±9.61 years. All patients had an aneurysmal KD and aberrant left subclavian artery. Preoperative comorbidities included acute Stanford type B aortic dissection in 1 patient, aortic arch pseudoaneurysm in 1 patient, acute type B intramural hematoma in 2 patients, and coronary artery disease in 1 patient. Concomitant procedures included reconstruction of the left subclavian artery in all patients and coronary artery bypass grafting in 1 patient. The mean time of operation, cardiopulmonary bypass, aortic cross-clamping, and selective cerebral perfusion was 6.25±1.16 h, 157.75±40.07 min, 77.75±33.10 min, and 28.50±5.55 min, respectively. No intraoperative death occurred. There was 1 in-hospital death. Follow-up was completed in all patients with a mean period of 3.58±2.08 years. No late death occurred. A persistent anastomotic leak of the proximal arch was detected in 1 patient, but reintervention was not performed because neither aortic dilatation nor symptoms of tracheal and esophageal compression were observed during the follow-up. The remaining 6 patients showed positive aortic remodeling with complete thrombosis of the aneurysmal KD, and neither aortic event nor tracheal and esophageal compression occurred. Conclusion Stented elephant trunk procedure is a safe and feasible technique for selected patients with RAA and KD, which can achieve favorable early and midterm outcomes.