Early plasma exchange for treating ricin toxicity in children after castor bean ingestion.

Plasma exchange (PE) for the treatment of ricin toxicity has not been previously reported. Here we describe the use of PE to treat children who experienced ricin toxicity after ingesting castor beans. Seven children (median age: 8.1 years) who consumed castor beans (median: 5 beans) were treated with PE. All had bradycardia and sinus arrhythmia, and most had experienced episodes of vomiting and/or diarrhea. PE settings were blood flow, 50-80 mL/min; PE rate, 600-800 mL/h; volume of exchange, 1440-1950 mL. Median time from ingestion to PE was 73 h. All clinical symptoms disappeared and vital signs rapidly returned to normal after PE; no severe organ dysfunction occurred. All children were discharged and recovered uneventfully. Concentrations of all serum biochemical parameters significantly decreased immediately after PE. Some, but not all, of these parameters were also significantly decreased at 48 and 72 h after PE compared with before PE. Our findings suggest that PE can be an effective early intervention in the treatment of ricin toxicity due to castor bean ingestion.

[The Relationship between ASXL2 and ZBTB7A Gene Mutations and Prognosis in Patients with Acute Myeloid Leukemia].

OBJECTIVE: To investigate the relationship between acute myeloid leukemia (AML) patients ASXL2, ZBTB7A gene mutations and the prognosis. METHODS: 42 AML Patients treated in our hospital from January 2014 to January 2016 were selected and ASXL2 and ZBTB7A genes of their bone marrow samples were sequenced, the genetic characteristics and prognosis of core-binding factor-AML(CBF-AML) patients with ASXL2 and ZBTB7A mutations were analyzed. RESULTS: ASXL2 (33.3%) and ZBTB7A (9.5%) mutations were found in t (8; 21) AML patients. Compared with wild-type, patients with ASXL2 mutations showed significantly higher white blood cell count at diagnosis ï¼»(9.49±1.85)×109/L vs (8.3±1.14)×109/L，P=0.03ï¼½ and lower frequency of sex chromosome deletions (21.43% vs 71.43%, P=0.02), respectively. ASXL2 mutation showed mutually exclusive with ASXL1 mutation (P=0.035). The proportion of chromatin modifier gene ATRX and BCOR mutations was higher in patients with ASXL2 mutation (P=0.032, P=0.005).ASXL2 and ZBTB7A mutations showed no significant effect to overall survival or event-free survival rate in patients with AML. CONCLUSION: ASXL2 and ZBTB7A mutations are frequently found in t (8; 21) AML patients. The mutation of ASXL2 and ZBTB7A genes shows no significant effect on the prognosis of AML patients.

[Relation of Circulating Follicular Helper T Cell Changes with B Cell Dysfunction in MDS Patients].

OBJECTIVE: To explore the relation of circulating follicular helper T cell (c Tfh) changes with B cell dysfunction in MDS patients. METHODS: 20 patients diagnosed as MDS from Auguct 2015 to October 2017 were enrolled in MDS group, and 20 healthy valuntears matching in age and sex were enrolled in healthy control (HC) group. The perepheral blood in 2 groups were collected, the mononuclear cells (PBMC) from which were isolated by densily gradient contrifugation, at the same time, the serum left in isolation process was reserved for further study. The flow cytometry was used to detect the ratio of cTfh such as CD4+CXCR5+ T cells and its subset CD4+CXCR5+ICOS+ T cells, CD4+CXCR5+PD-1+ T cells in PBMC, as well as the ratio of plasmablast CD19+CD20-CD38+ B cells. The ELISA was used to detect the concentration of IgA, IgM and IgG. The differences in ratio of cTfh cells and plasmablast B cells, as well as the concentration of IgA, IgM and IgG between MDS and HC groups were compared, at the same time, the correlation of cTfh cell ratio with the plasmablast B cell ratio and the concentration of IgA, IgM and IgG in MDS patient was analyzed. RESULTS: The ratio of CD4+CXCR5+T, CD4+CXCR5+ICOS+T cells and CD19+CD20-CD38+B cells and the concentration of IgA, IgM and IgG decreased in MDS patients, while the ratio of CD4+CXCR5+PD-1+T cells increased in MDS patients. The ratio of CD4+CXCR5+T cells, CD4+CXCR5+ICOS+T cells positively correlated with the ratio of CD19+CD20-CD38+B cells, as well as with the concentration of IgA, IgM and IgG in MDS patients. However, the ratio of CD4+CXCR5+PD-1+T cells negatively correlated with the ratio of CD19+CD20-CD38+B cells, as well as with the concentration of IgA, IgM and IgG. CONCLUSION: The ratio of circulating Tfh cells and their subsets showed significant changes, that correlate with B cell dysfunction in MDS patients.

Knockdown of Peripheral Myelin Protein 22 Inhibits the Progression of Chronic Myeloid Leukemia.

We aimed to explore the underlying mechanism of peripheral myelin protein 22 (PMP22) in the development of chronic myeloid leukemia (CML). The level of PMP22 expression in CD34(+) cells isolated from CML patients' bone marrow samples (BMMCs) and peripheral blood samples (PBMCs) was determined by RT-PCR. In addition, PMP22-siRNA and scrambled control siRNA were transfected into human CML cell line K562 with Lipofectamine 2000 reagent. Cell viability and apoptosis were, respectively, determined by MTT assay and flow cytometry. Besides, the level of caspase 3 and Bcl-xL was then detected using Western blot. The level of PMP22 expression in CML patients' CD34(+) cells isolated from both PBMCs and BMMCs was significantly higher than the control group. PMP22 expression in K562 cells was successfully knocked down by siRNA. MTT analysis showed that knockdown of PMP22 inhibited the proliferation of CML cells. Flow cytometry showed that knockdown of PMP22 promoted the apoptosis of CML cells. Besides, Bcl-xL expression markedly decreased, while the expression of caspase 3 in CML cells significantly increased after knockdown of PMP22 expression. Our findings indicate that high expression of PMP22 may promote cell proliferation and inhibit cell apoptosis via upregulation of Bcl-xL or inhibition of caspase 3 activation, and thus may contribute to the development of CML. PMP22 may serve as a novel therapeutic target for the treatment of CML.

[Presurgical nasoalveolar molding in infants with cleft lip and palate: analysis of 29 cases].

PURPOSE: The objective of this study was to treat the cleft lip and alveolus, nasal deformity with presurgical nasoalveolar molding (PNAM), to elucidate the problems and treatment methods, which may be helpful for the use of PNAM in clinic. METHODS: Twenty nine infants with cleft lip and palate (CLP) were treated with PNAM in our center. There were 19 unilateral and 10 bilateral CLP patients. The initial visit time was 3 to 150 days after birth. Treatment time ranged from 2.5 to 3 months. The appliance was modified at 2-week interval. RESULTS: According to the evaluation standards, 17 infants were treated successfully with the closure of cleft lip and alveolar processes, reposition of the deformed nasal cartilages, and increased length of columella. The lip and nasal deformities of 9 infants were corrected partly, which were helpful for surgery. There were 3 infants giving up PNAM. CONCLUSIONS: There were five important facts for the successful treatment, including initial visit time, impression of the intraoral cleft defect, modification of the plate and the nasal stent, and use of nasal splints. Orthodontics and plastic surgeons should have the same views for PNAM in infants, which will advance the treatment level for cleft lip and palate.