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Objective:To investigate the relationship between TP53 gene mutation and overall survival (OS) time of myelodysplastic syndrome (MDS).Methods:Databases, including PubMed, Cochrane Library and Embase were searched for relevant studies published up to 20 October, 2019. The corresponding hazard ratio ( HR) and their 95% confidence interval ( CI) for OS from multivariate Cox proportional hazard models were extracted. The combined HR with their 95% CI was calculated by using fixed or random effect models. Meta-analysis was performed by using Revman 5.3 software. Results:A total of 1 033 patients from 6 studies were enrolled. Meta-analysis results showed that OS time in TP53 gene mutation group was shorter than that in wild type group for patients with MDS ( HR = 2.16, 95% CI 1.52-3.07, P < 0.01). The prognostic risk for post-transplantation patients with MDS ( HR = 2.69, 95% CI 1.63-4.43, P < 0.01) was lower compared with that for patients treated by azacitidine( HR = 2.89, 95% CI 1.37-6.08, P = 0.005). Conclusion:TP53 gene mutation is a risk factor affecting OS of MDS patients.
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Objective:To investigate the relationship between TP53 gene mutation and overall survival (OS) time of myelodysplastic syndrome (MDS).Methods:Databases, including PubMed, Cochrane Library and Embase were searched for relevant studies published up to 20 October, 2019. The corresponding hazard ratio ( HR) and their 95% confidence interval ( CI) for OS from multivariate Cox proportional hazard models were extracted. The combined HR with their 95% CI was calculated by using fixed or random effect models. Meta-analysis was performed by using Revman 5.3 software. Results:A total of 1 033 patients from 6 studies were enrolled. Meta-analysis results showed that OS time in TP53 gene mutation group was shorter than that in wild type group for patients with MDS ( HR = 2.16, 95% CI 1.52-3.07, P < 0.01). The prognostic risk for post-transplantation patients with MDS ( HR = 2.69, 95% CI 1.63-4.43, P < 0.01) was lower compared with that for patients treated by azacitidine( HR = 2.89, 95% CI 1.37-6.08, P = 0.005). Conclusion:TP53 gene mutation is a risk factor affecting OS of MDS patients.
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Farnesoid X receptor (FXR) is a member of the nuclear receptor superfamily. It has extensive physiological functions in maintaining homeostasis of bile acids, lipids, and glucose. It also participates in the development of various tumors. Therefore, regulation of its transcriptional activity is accepted as an important strategy for treatment of many diseases, and various FXR agonists have been developed. In recent years, it has been found that inhibition of FXR transcriptional activity also shows beneficial effects on various diseases, therefore several FXR antagonists have been developed and their pharmacological activities have been tested in preclinical animal models. This article provides an update review on FXR antagonists and their pharmacological activities.
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Objective@#To investigate the clinical effects of Qinghuang powder, low-intensity chemotherapy and Qinghuang powder alternated with low-intensity chemotherapy in the treatment of elderly acute myeloid leukemia patients progressed from myelodysplastic syndromes (MDS-AML).@*Methods@#A total of 32 elderly patients with MDS-AML treated in Xiyuan Hospital, China Academy of Chinese Medical Sciences from January 2014 to December 2017 were retrospectively analyzed. Of them, 12 patients received Qinghuang powder (Qinghuang powder group), 6 patients received Qinghuang powder alternated with low-intensity chemotherapy (alternating group), and 14 patients received low-intensity chemotherapy (low-intensity chemotherapy group), based on the real world of patient's voluntary choice of treatment. The efficacy and adverse reactions in the 3 groups were observed.@*Results@#The overall response number of Qinghuang powder, alternating, and low-intensity chemotherapy groups were 2, 2 and 4 cases, respectively, there was no significant difference among the 3 groups (P > 0.05). The median overall survival time of 3 groups were 14, 12 and 8 months, respectively, there was no significant difference among the 3 groups (P > 0.05). Two cases in Qinghuang powder group presented with gastrointestinal reactions; 3 cases in alternating group with myelosuppression, 2 cases with liver function damage, and 2 cases with gastrointestinal reaction; 11 cases in low-intensity chemotherapy group presented with bone marrow suppression, 3 cases with liver function damage, 7 cases with gastrointestinal reactions, and 2 cases with myocardial enzyme changes. The incidences of myelosuppression, liver function damage and myocardial enzyme changes in Qinghuang powder group were lower than those in the alternating group and low-intensity chemotherapy group.@*Conclusion@#The efficacy of Qinghuang powder or Qinghuang powder alternated with low-intensity chemotherapy for the treatment of elderly MDS-AML is not worse than the low-intensity chemotherapy, furthermore Qinghuang powder or Qinghuang powder alternated with low-intensity chemotherapy has fewer adverse reactions and better tolerance.
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Objective To investigate the clinical effects of Qinghuang powder, low-intensity chemotherapy and Qinghuang powder alternated with low-intensity chemotherapy in the treatment of elderly acute myeloid leukemia patients progressed from myelodysplastic syndromes (MDS-AML). Methods A total of 32 elderly patients with MDS-AML treated in Xiyuan Hospital, China Academy of Chinese Medical Sciences from January 2014 to December 2017 were retrospectively analyzed. Of them, 12 patients received Qinghuang powder (Qinghuang powder group), 6 patients received Qinghuang powder alternated with low-intensity chemotherapy (alternating group), and 14 patients received low-intensity chemotherapy (low-intensity chemotherapy group), based on the real world of patient's voluntary choice of treatment. The efficacy and adverse reactions in the 3 groups were observed. Results The overall response number of Qinghuang powder, alternating, and low-intensity chemotherapy groups were 2, 2 and 4 cases, respectively, there was no significant difference among the 3 groups (P＞ 0.05). The median overall survival time of 3 groups were 14, 12 and 8 months, respectively, there was no significant difference among the 3 groups (P＞ 0.05). Two cases in Qinghuang powder group presented with gastrointestinal reactions; 3 cases in alternating group with myelosuppression, 2 cases with liver function damage, and 2 cases with gastrointestinal reaction; 11 cases in low-intensity chemotherapy group presented with bone marrow suppression, 3 cases with liver function damage, 7 cases with gastrointestinal reactions, and 2 cases with myocardial enzyme changes. The incidences of myelosuppression, liver function damage and myocardial enzyme changes in Qinghuang powder group were lower than those in the alternating group and low-intensity chemotherapy group. Conclusion The efficacy of Qinghuang powder or Qinghuang powder alternated with low-intensity chemotherapy for the treatment of elderly MDS-AML is not worse than the low-intensity chemotherapy, furthermore Qinghuang powder or Qinghuang powder alternated with low-intensity chemotherapy has fewer adverse reactions and better tolerance.
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Objective@#To analyze the gene mutations in the patients with myelodysplastic syndromes (MDS).@*Methods@#Forty-seven patients with MDS newly diagnosed in Xiyuan Hospital, China Academy of Chinese Medical Sciences from January 2016 to July 2017 were enrolled. NGS 127-gene panel was used to detect the gene mutations, and the relationship between the gene mutations and the clinicopathological features was also analyzed.@*Results@#Thirty-one (66.0 %) cases had gene mutations in 47 patients with MDS, and 23 gene mutations were detected with clinical significances. There were 7 mutant genes with a mutation frequency over 5 % in the population, including U2AF1 (23.4 %), SF3B1 (12.8 %), ASXL1 (10.6 %), TET2 (8.5 %), BCOR (8.5 %), TP53 (8.5 %) and DNMT3A (6.4 %) in turn. Among 31 patients with gene mutations, 16 (51.6 %) patients had ≥ 2 synergistic mutations, and 12 cases had synergistic mutations in different genetic functional groups, which was higher than that in same genetic functional groups (4 cases). There was a tendency of coexistence in IDH2-KRAS, IDH2-SRSF2, IDH2-STAG2, KRAS-SRSF2, KRAS-STAG2, RUNX1-PHF6, EZH2-ASXL1, EZH2-ZRSR2, and NPM1-NRAS (all P < 0.05). The variant allele frequency (VAF) of signaling pathway related genes including JAK2, KRAS, NRAS, SH2B3 was low in general and in a sub-clone status. JAK2 gene mutation was observed in 1 case with MDS-U. SH2B3 gene mutation was observed in a patient with very poor prognosis of karyotype. SETPB1 and EZH1 gene mutations were observed in two patients with high-risk revised international prognostic scoring system (IPSS-R).@*Conclusions@#The common mutated genes include U2AF1, SF3B1, ASXL1 and TET2. The genes in different genetic functional groups tend to synergistic mutations. Gene mutations can be used to predict the prognosis of diseases and become the target in the treatment of MDS.
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Objective To investigate the survival of oral arsenic-containing Qinghuang Powder (QHP) and low intensive chemotherapy (LIC) in the treatment of elderly patients with acute myeloid leukemia (AML).Methods Forty-two AML patients older than 60 years in Xiyuan Hospital from January 2015 to December 2017 were retrospectively analyzed.Of them,20 cases were treated with QHP (QHP group),22 cases were treated with LIC (LIC group).The survivals of the two groups were compared.Results There was no significant difference of median survival time (13 months vs.13.5 months,x2 =0.096,P =0.757),1-year survival rates (59.1％ vs.70.0 ％,x2 =0.543,P =0.461),2-year survival rates (13.6 ％ vs.15.0 ％,x2 =0.016,P ＞ 0.05),and 3-year survival rates (4.6 ％ vs.5.0 ％,x2 =0.005,P ＞ 0.05) between LIC and QHP groups.There was no significant difference of median survival time in age ≥75 year (12 months vs.12.5 months,x2 =1.317,P =0.251),performance status scores ＞ 2 (12 months vs.12 months,x2 =0.834,P =0.361),hematopoietic stem cell transplantation with combined disease index ＞ 2 (12 months vs.13 months,x2 =1.726,P =0.189),secondary AML (10 months vs.14 months,x2 =1.552,P =0.213),and poor cytogenetics (12 months vs.8 months,x2 =0.479,P =0.489) between LIC and QHP group.Conclusion The survival of elderly AML patients is considerable in patients treated with oral QHP and LIC,which suggests that oral QHP may be an equivalent alternative treatment since elderly AML (especially more than 75 years) patients refused to LIC therapy.
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Clonal progression to myelodysplastic syndromes (MDS) or acute myeloid leukemia (AML) remains a dreaded complication for a subset of patients with bone marrow failure (BMF). Recognizing risk factors for the development of MDS or AML would inform individualized treatment decisions and identify patients who may benefit from early or upfront hematopoietic stem cell transplantation. Now that next-generation DNA sequencing is available in the clinical laboratory, research has focused on the implications of germline and somatic mutations for diagnosing and monitoring patients with BMF. Most germline genetic BMF disorders are characterized by a high propensity to develop MDS or AML. Recent studies of somatic variants in marrow failure revealed a high frequency of clonal hematopoiesis with the acquisition of mutations in genes associated with MDS or AML. The evaluation and implications of germline and somatic mutations for the development of clonal disorders in patients with BMF and challenges of clinical genetic testing will be summarized in this paper based on the reports from the 58th American Society of Hematology (ASH) Annual Meeting.
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Objective To study the relationship between hearing loss and serum bilirubin of neonates with hypercholesterolemia .Methods The serum bilirubin level and auditory brainstem response thresholds of 70 hyper_cholesterolemia neonates of full term pregnancy and normal weight were tested ,and the correlations of serum biliru_bin ,and occurrence time of jaundice with hearing threshold were analyzed .ResuIts Among the 70 neonates ,there were 17 cases with normal ABR thresholds and 53 with abnormal ABR thresholds ,including 27 with middle and se_rious hearing loss .In the cases of serum bilirubin less than 342μmol/L ,the percentage of cases with middle and se_rious hearing loss and with occurrence time of jaundice less than 48 hours was higher than theses cases with occur_rence time of jaundice more than 48 hours (P<0 .05) .In the cases of serum bilirubin higher than 342μmol/L ,there was no obvious difference between the percentage of cases of middle and serious hearing loss with occurrence time of jaundice less than 48 hours and those with occurrence time of jaundice more than 48 hours .Among the 53 cases with abnormal hearing thresholds ,positive correlation was observed between serum bilirubin and ABR threshold (r=0 .041) ,whereas negative correlation was observed between occurrence time of jaundice and ABR threshold (r=-0 .291) .ConcIusion The higher probability of hearing loss for hypercholesterolemia neonates with earlier occur_rence of jaundice ,the more serious hearing loss with higher serum bilirubin .
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Objective To evaluate the diagnostic value of low-dose volume perfusion imaging with 320-detector CT in differentia-ting solitary pulmonary nodules (SPNs).Methods 130 patients with 130 SPNs (79 malignant tumors,1 6 active inflammations,1 6 benign nodules and 1 9 chronic inflammations)underwent contrast-enhanced dynamic volume perfusion CT prospectively.50ml of contrast medium with iodine concentration of 370 mg I/mL was injected at a flow rate of 5 mL/s,and the scan began at 0 second or 4 seconds after the bolus injection.20 or 21 intermittent low-dose volume acquisitions were made with 2 s intervals,and the total ac-quisition time was 38 s or 40 s.Post-procession was performed using a perfusion software on the CT (Body Perfusion,Dual-input single compartment mode).The perfusion parameters (PF,BF,PPI),T-DC and TPF were measured or calculated,and then as-sessed.Results PF and PPI of active inflammation nodules showed significant higher value than those of the other three kinds of nodules,and there were significant differences in PF and PPI between active inflammation nodules and other kinds of nodules.The value of BF in malignant nodules was higher than that in the other three kinds of nodules,however no statistic significant difference in PPI was found between malignant and chronic inflammation nodules.The value of TPF in malignant and active inflammation nod-ules was higher than that in benign and chronic nodules,and there were statistic significant differences between malignant and active nodules and chronic ones.Six types of T-DC were displayed on SPNs:malignant,benign and chronic nodules showed a variety of T-DC types,and active inflammatory nodules showed only one type-typeⅠ.Conclusion Low-dose volume perfusion technique with 320-detector row CT can provide abundant information about blood flow of SPNs,and it may be regarded as an important technique in the differential diagnosis of SPNs.
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<p><b>OBJECTIVE</b>To observe the effect of transnasal endoscopic choanal plasty for repairing congenital choanal atresia.</p><p><b>METHOD</b>A retrospective analysis of 15 patients with choanal atresia in Beijing Children's Hospital from January 2006 to September 2012 was made. All the 15 patients accepted electronic nasopharyngoscope examination and CT scanning of the paranasl sinus. The age was between 4 years old and 6 years old. Seven of them were male and 8 were female. Six cases were unilateral atresia, 9 were bilateral atresia. Ten cases (16 sides) were membranous atresia, 2 cases (4 sides) were mixed type atresia and 3 cases (4 sides) were bony atresia. All the patients accepted transnasal endoscopic choanal plasty. A tube was placed in order to support the enlarged nostrils. The nasal tube should be retained for 3 months and kept patency by routine care. Three months after operation, the tube could be removed. The patients were told to do physiological saline nasal irrigation and receive regular review by transnasal endoscopy.</p><p><b>RESULT</b>The patients were followed up for 1 year postoperatively by electronic nasopharyngoscope examination. There was no failure case in all the 15 patients. The total effective rate was 100%.</p><p><b>CONCLUSION</b>Transnasal endoscopic choanal plasty had better efficacy and safety in the treatment of congenital choanal atresia.</p>
Subject(s)
Child , Child, Preschool , Female , Humans , Male , Choanal Atresia , General Surgery , Endoscopy , Nasal Cavity , Nasal Lavage , Nasopharynx , Nose , Paranasal Sinuses , Postoperative Period , Retrospective Studies , Safety , Surgery, Plastic , Methods , Tomography, X-Ray ComputedABSTRACT
Objective:To establish the quality standard of berberine hydrochloride in Fuyanping capsules by HPLC. Methods:HPLC with Phenomenex C18(250?4.6mm,5?m)column was used.Acetonitrile-0.033mol/l potassium dihydrogen phosphate(30:70)was used as a mobile phase,flow rate was 1.0ml/min and detection wavelength was set at 346nm.Results: Berberine hydrochloride showed a good linear relationship at a range of 0.16-0.83?g,r=0.9999.The average recovery was 99.09%,and RSD was 0.20%.Conclusion:This method is simple,fast and accurate for the quality control of Fuyanping capsules.