Objective:To investigate the relationship between mutated genes and clinical features in patients with essential thrombocythemia(ET).Methods:The clinical data of 69 patients with ET from October 2018 to March 2022 were retrospectively analyzed.According to driver mutation type,patients were divided into JAK2 group,CALR group and triple-negative group.The sex,age,cardiovascular risk factors,thrombosis,splenomegaly,routine blood test and coagulation status of patients in three groups were analyzed.Results:Among 69 ET patients,46 cases were associated with JAK2 mutation,14 cases with CALR mutation,8 cases with triple-negative mutation,and one with MPL gene mutation.There were no significant differences in age and sex among the three groups(P＞0.05).The highest thrombotic rate was 26.09％(12/46)in JAK2 group,then 12.5％(1/8)in triple-negative group,while no thrombotic events occurred in CALR group.The incidence of splenomegaly was the highest in JAK2 group(34.78％),while no splenomegaly occurred in triple-negative group.The white blood cell(WBC)count in JAK2 group was(9.00±4.86)× 109/L,which was significantly higher than(6.03±2.32)× 109/L in CALR group(P＜0.05).The hemoglobin(Hb)and hematocrit(HCT)in JAK2 group were(148.42±18.79)g/L and(0.44±0.06)％,respectively,which were both significantly higher than(131.00±15.17)g/L and(0.39±0.05)％in triple-negative group(P＜0.05).The platelet(PLT)in JAK2 group was(584.17±175.77)× 109/L,which was significantly lower than(703.07±225.60)× 109/L in CALR group(P＜0.05).The fibrinogen(Fg)in JAK2 and triple-negative group were(2.64±0.69)g/L and(3.05±0.77)g/L,respectively,which were both significantly higher than(2.24±0.47)g/L in CALR group(P＜0.05,P＜0.01).The activated partial thromboplastin time(APTT)in triple-negative group was(28.61±1.99)s,which was significantly decreased compared with(31.45±3.35)s in CALR group(P＜0.05).Conclusions:There are differences in blood cell count and coagulation status among ET patients with different driver gene mutations.Among ET patients,JAK2 mutation is most common.Compared with CALR group,the thrombotic rate,WBC and Fg significantly increase in JAK2 group,while PLT decrease.Compared with triple-negative group,the incidence of splenomegaly and HCT significantly increase.Compared with CALR group,Fg significantly increases but APTT decreases in triple-negative group.

ObjectiveMyelodysplastic syndromes （MDS） is a group of clonal hematopoietic stem cell disorders，and this study aims to investigate the expression of hypoxia-inducible factor-1α（HIF-1α） in the bone marrow cells of patients with MDS and its correlation with the clinical features of MDS，the therapeutic efficacy of arsenic-containing Chineseherbal compound，and the survival prognosis. MethodAccording to the inclusion and exclusion criteria，27 MDS patients treated with arsenic-containing Chinese herbal compound in the Department of Hematology，Xiyuan Hospital，China Academy of Chinese Medical Sciences from January 2022 to September 2022 were included，and their bone marrow samples were collected by myelotomy. HIF-1α expression level in bone marrow cells was detected by real-time polymerase chain reaction （PCR） to analyze its correlation with clinical features，and logistic and Cox regression was used to analyze the risk factors affecting the efficacy and prognostic survival of MDS patients. ResultThe HIF-1α mRNA expression level was lower in bone marrow cells of MDS patients than in healthy subjects. HIF-1α was positively correlated with the degree of myelodysplasia（r=0.384，P<0.05） and bone marrow granulocytic system%（G%）（r=0.560，P<0.01）. Logistic regression showed that HIF-1α was a risk factor for the prognosis in the follow-up of the efficacy of treatment（P<0.05）and Cox regression showed that HIF-1α was an independent factor affecting the survival prognosis of MDS patients ［odds ratio（OR）=398.968，95% confidence interval（CI）（1.281，116 858.743），P<0.05］. ConclusionThe level of HIF-1α expression in bone marrow cells of MDS patients was closely related to the degree of clinical myelodysplasia and G%，and HIF-1α was a risk factor for the efficacy for and survival prognosis of MDS patients.

Objective:To explore the influence of acupoint catgut embedding combined with Xuezhikang capsule on lipid metabolism and antioxidation effect in patients with statin intolerance and hyperlipidemia of spleen-kidney yang deficiency syndrome.Methods:Randomized controlled trial. A total of 82 patients with statin intolerance and hyperlipidemia who were treated in Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine , Shanghai Puto Area Central Hospital, and Lingyun community health Service center, Xuhui District from July 2020 to June 2022 were selected and randomly divided into two groups with 41 patients in each group by odd-even ball method. The two groups were given diet and exercise guidance, and the control group was given Xuezhikang capsule orally on this basis, and the observation group was given poly(l-lactide-co-glycolide) (PLGA) thread acupoint catgut embedding on the basis of the control group. Both groups were treated continuously for 3 months. The TCM syndromes were scored before and after treatment, and body weight (BW), waist circumference (WC) and body mass index (BMI) were recorded. The levels of TC, TG, HDL-C and LDL-C were detected by fully automatic biochemical analyzer. The levels of serum SOD, GSH-Px and total antioxidant capacity (TAOP) were measured by ELISA. The level of serum endothelin-1 (ET-1) was assessed by radioimmunoassay. Color Doppler ultrasound diagnostic instrument was used to detect the brachial artery flow-mediated dilation (FMD) and nitroglycerin-mediated dilation (NMD).Results:The scores of chilly sensation and cold limbs [(1.27±0.12) vs. (1.46±0.16), t=6.08], loose stool [(1.41±0.16) vs. (1.63±0.18), t=5.85], fatigue [(1.45±0.15) vs. (1.57±0.17), t=3.39], dizziness [(1.15±0.11) vs. (1.26±0.13), t=4.14] and loss of appetite [(1.21±0.13) vs. (1.39±0.15), t=5.81] in observation group after treatment were significantly lower than those in the control group ( P<0.01). BW [(68.03±6.57)kg vs. (71.55±6.76)kg, t=2.39], WC [(85.13±4.63)cm vs. (87.35±4.85)cm, t=2.12] and BMI [(27.35±2.84)kg/m 2vs. (29.18±3.05)kg/m 2, t=2.81]were lower than those in the control group ( P<0.05). The levels of serum TC [(3.15±0.13)mmol/L vs. (3.38±0.17)mmol/L, t=6.88], TG [(1.98±0.11)mmol/L vs. (2.21±0.15)mmol/L, t=7.92]and LDL-C [(2.46±0.26)mmol/L vs. (3.04±0.33)mmol/L, t=8.84]were significantly lower compared with those in control group ( P<0.01) while the level of HDL-C [(1.88±0.24)mmol/L vs. (1.74±0.21)mmol/L, t=2.81]was higher than that of the control group ( P<0.01). The levels of serum SOD [(57.82±5.84)μg/L vs. (55.06±5.61)μg/L, t=2.18], GSH-Px [(96.51±9.52)U/L vs. (92.26±9.25)U/L, t=2.30]and TAOP [(6.21±0.57)U/L vs. (5.94±0.54)U/L, t=2.20]were significantly higher compared to control group ( P<0.05). Serum ET-1 [(60.43±4.36) pg/L vs. (63.71±4.68) pg/L, t=3.28] level was significantly lower than that in control group ( P<0.01), while FMD [(12.48±1.02)% vs. (11.34±0.95)%, t=5.24] and NMD [(15.12±1.24)% vs. (14.44±1.18)%, t=2.54] were significantly higher than those in control group ( P<0.01). Conclusion:The combination of acupoint catgut embedding and Xuezhikang capsule can reduce the scores of TCM syndromes, lower the obesity and blood lipids, enhance the antioxidant function and improve the vascular endothelial function in patients with statin intolerance and hyperlipidemia.

Combining the knowledge of traditional Chinese medicine and modern medicine on glucolipid metabolism disorders， it is believed that the formation process of glycolipid metabolism disorders can be presented as five states "depression， phlegm-dampness， heat， blood stasis， and deficiency"， and the turbid pathogens run through the whole process. Accordingly， the method of "regulating states and removing turbidity" is proposed， which is specifically the method of resolving depression and turbidity， dispelling phlegm-dampness and turbidity， clearing heat and turbidity， dispelling blood stasis and turbidity， and replenishing deficiency and removing turbidity. Combined with the biological basis of glycolipid metabolism disorder， through the analysis of the clinical application of each method and the related mechanism of action， it is clarified that the method of regulating states and resolving turbidity can play a role in improving glycolipid metabolism disorder by regulating lipid metabolism disorder， insulin resistance， bile acid metabolism abnormality， intestinal bacterial flora， and its metabolite abnormality and other mechanisms of action.

Objective To investigate the clinical characteristics of survival in patients with high-risk myelodysplastic syndromes(HR-MDS)and provide a reference for the clinical prognosis of patients with HR-MDS.Methods General data,blood routine test,bone marrow smear with histopathology,cytogenetics,and other clinical data of 200 patients diagnosed with HR-MDS at Xiyuan Hospital of China Academy of Chinese Medical Sciences,during the period of January 2016-September 2022,were retrospectively analyzed.The included patients were categorized into the arsenic-containing herbal combination combined with demethylating agents(HMAs)treatment group and the arsenic-containing Chinese medicine compound combined with androgen treatment group.The influence of clinical indices on the survival characteristics of each group was analyzed.Results Comparison of the impact of clinical indicators on survival in 200 patients with HR-MDS who were treated with arsenic-containing herbal compounds in combination with HMAs or androgens showed that high-risk vs.very high-risk(P=0.018),hemoglobin(Hb)<80 g/L vs.Hb≥80 g/L(P=0.035),platelet(PLT)counts<50×109 L-1 vs.PLT counts≥50×109 L-1(P<0.001),and the difference in median progression-free survival(PFS)time between myelodysplastic syndromes converted to leukemia(MDS-AML)and non-MDS-AML(P=0.003)were statistically significant.Comparison of survival effects of clinical indicators in 68 patients with HR-MDS who were treated with arsenic-containing Chinese medicine compound combined with HMAs showed that the difference in median PFS between PLT count<50×109 L-1 and PLT count≥50×109 L-1(P<0.001)and the difference in median PFS between<5 and≥5 courses of chemotherapy(P=0.018)were statistically significant.Comparison of survival effects of clinical indicators in 132 patients with HR-MDS who were treated with arsenic-containing Chinese medicine compound combined with androgens showed that Hb<80 g/L and Hg≥80 g/L(P=0.028),PLT count<50×109 L-1 and PLT count≥50×109 L-1(P=0.002),and the mean differences in PFS between MDS-AML and non-MDS-AML(P=0.024)were statistically significant.Conclusion The clinical characteristics of long-surviving patients treated with arsenic-containing herbal combination in combination with HMAs included PLT counts≥50×109 L-1 and≥5 courses of chemotherapy.The clinical characteristics of long-surviving patients treated with arsenic-containing herbal combination in combination with androgens included Hg≥80 g/L,PLT count≥50×109 L-1,and non-MDS-AML.

Objective:To investigate the safety and therapeutic effect of split liver transplantation (SLT) in clinical application.Methods:This is a retrospective case-series study. The clinical data of 203 consecutive SLT, 79 living donor liver transplantation (LDLT) and 1 298 whole liver transplantation (WLT) performed at the Third Affiliated Hospital of Sun Yat-sen University from July 2014 to July 2023 were retrospectively analyzed. Two hundred and three SLT liver grafts were obtained from 109 donors. One hundred and twenty-seven grafts were generated by in vitro splitting and 76 grafts were generated by in vivo splitting. There were 90 adult recipients and 113 pediatric recipients. According to time, SLT patients were divided into two groups: the early SLT group (40 cases, from July 2014 to December 2017) and the mature SLT technology group (163 cases, from January 2018 to July 2023). The survival of each group was analyzed and the main factors affecting the survival rate of SLT were analyzed. The Kaplan-Meier method and Log-rank test were used for survival analysis.Results:The cumulative survival rates at 1-, 3-, and 5-year were 74.58%, 71.47%, and 71.47% in the early SLT group, and 88.03%, 87.23%, and 87.23% in the mature SLT group, respectively. Survival rates in the mature SLT group were significantly higher than those in the early SLT group ( χ2=5.560, P=0.018). The cumulative survival rates at 1-, 3- and 5-year were 93.41%, 93.41%, 89.95% in the LDLT group and 87.38%, 81.98%, 77.04% in the WLT group, respectively. There was no significant difference among the mature SLT group, the LDLT group and the WLT group ( χ2=4.016, P=0.134). Abdominal hemorrhage, infection, primary liver graft nonfunction,and portal vein thrombosis were the main causes of early postoperative death. Conclusion:SLT can achieve results comparable to those of WLT and LDLT in mature technology liver transplant centers, but it needs to go through a certain time learning curve.

Objective:To investigate the safety and therapeutic effect of split liver transplantation (SLT) in clinical application.Methods:This is a retrospective case-series study. The clinical data of 203 consecutive SLT, 79 living donor liver transplantation (LDLT) and 1 298 whole liver transplantation (WLT) performed at the Third Affiliated Hospital of Sun Yat-sen University from July 2014 to July 2023 were retrospectively analyzed. Two hundred and three SLT liver grafts were obtained from 109 donors. One hundred and twenty-seven grafts were generated by in vitro splitting and 76 grafts were generated by in vivo splitting. There were 90 adult recipients and 113 pediatric recipients. According to time, SLT patients were divided into two groups: the early SLT group (40 cases, from July 2014 to December 2017) and the mature SLT technology group (163 cases, from January 2018 to July 2023). The survival of each group was analyzed and the main factors affecting the survival rate of SLT were analyzed. The Kaplan-Meier method and Log-rank test were used for survival analysis.Results:The cumulative survival rates at 1-, 3-, and 5-year were 74.58%, 71.47%, and 71.47% in the early SLT group, and 88.03%, 87.23%, and 87.23% in the mature SLT group, respectively. Survival rates in the mature SLT group were significantly higher than those in the early SLT group ( χ2=5.560, P=0.018). The cumulative survival rates at 1-, 3- and 5-year were 93.41%, 93.41%, 89.95% in the LDLT group and 87.38%, 81.98%, 77.04% in the WLT group, respectively. There was no significant difference among the mature SLT group, the LDLT group and the WLT group ( χ2=4.016, P=0.134). Abdominal hemorrhage, infection, primary liver graft nonfunction,and portal vein thrombosis were the main causes of early postoperative death. Conclusion:SLT can achieve results comparable to those of WLT and LDLT in mature technology liver transplant centers, but it needs to go through a certain time learning curve.

Objective: To analyze the treatment and prognosis of patients with International Federation of Gynecology and Obstetrics (FIGO) 2018 stage Ⅲc cervical squamous cell carcinoma. Methods: A total of 488 patients at Zhejiang Cancer Hospital between May, 2013 to May, 2015 were enrolled. The clinical characteristics and prognosis were compared according to the treatment mode (surgery combined with postoperative chemoradiotherapy vs radical concurrent chemoradiotherapy). The median follow-up time was (96±12) months ( range time from 84 to 108 months). Results: (1) The data were divided into surgery combined with chemoradiotherapy group (surgery group) and concurrent chemoradiotherapy group (radiotherapy group), including 324 cases in the surgery group and 164 cases in the radiotherapy group. There were significant differences in Eastern Cooperation Oncology Group (ECOG) score, FIGO 2018 stage, large tumors (≥4 cm), total treatment time and total treatment cost between the two groups (all P<0.01). (2) Prognosis: ① for stage Ⅲc1 patients, there were 299 patients in the surgery group with 250 patients survived (83.6%). In the radiotherapy group, 74 patients survived (52.9%). The difference of survival rates between the two groups was statistically significant (P<0.001). For stage Ⅲc2 patients, there were 25 patients in surgery group with 12 patients survived (48.0%). In the radiotherapy group, there were 24 cases, 8 cases survived, the survival rate was 33.3%. There was no significant difference between the two groups (P=0.296). ② For patients with large tumors (≥4 cm) in the surgery group, there were 138 patients in the Ⅲc1 group with 112 patients survived (81.2%); in the radiotherapy group, there were 108 cases with 56 cases survived (51.9%). The difference between the two groups was statistically significant (P<0.001). Large tumors accounted for 46.2% (138/299) vs 77.1% (108/140) in the surgery group and radiotherapy group. The difference between the two groups was statistically significant (P<0.001). Further stratified analysis, a total of 46 patients with large tumors of FIGO 2009 stage Ⅱb in the radiotherapy group were extracted, and the survival rate was 67.4%, there was no significant difference compared with the surgery group (81.2%; P=0.052). ③ Of 126 patients with common iliac lymph node, 83 patients survived, with a survival rate of 65.9% (83/126). In the surgery group, 48 patients survived and 17 died, with a survival rate of 73.8%. In the radiotherapy group, 35 patients survived and 26 died, with a survival rate of 57.4%. There were no significant difference between the two groups (P=0.051). (3) Side effects: the incidence of lymphocysts and intestinal obstruction in the surgery group were higher than those in the radiotherapy group, and the incidence of ureteral obstruction and acute and chronic radiation enteritis were lower than those in the radiotherapy group, and there were statistically significant differences (all P<0.01). Conclusions: For stage Ⅲc1 patients who meet the conditions for surgery, surgery combined with postoperative adjuvant chemoradiotherapy and radical chemoradiotherapy are acceptable treatment methods regardless of pelvic lymph node metastasis (excluding common iliac lymph node metastasis), even if the maximum diameter of the tumor is ≥4 cm. For patients with common iliac lymph node metastasis and stage Ⅲc2, there is no significant difference in the survival rate between the two treatment methods. Based on the duration of treatment and economic considerations, concurrent chemoradiotherapy is recommended for the patients.
Female ; Humans ; Uterine Cervical Neoplasms/pathology* ; Neoplasm Staging ; Lymphatic Metastasis ; Lymph Node Excision ; Retrospective Studies ; Prognosis ; Chemoradiotherapy/methods* ; Carcinoma, Squamous Cell/pathology*

Adult ; Female ; Humans ; Granulosa Cell Tumor/pathology* ; Prognosis ; Ovarian Neoplasms/pathology*

Objective:To compare the efficacy of modified "double flaps" pyeloplasty and traditional dismembered pyeloplasty in the treatment of special types of hydronephrosis with small pelvis and long proximal ureteral stricture in children.Methods:The data of 39 children with special types of hydronephrosis treated in Seventh Medical Center, General Hospital of PLA from June 2018 to June 2019 were retrospectively analyzed. Among them, 33 were boys and 6 were girls. The median age of the patients was 12.0(4.5, 63.5) months. Nine of them had left hydronephrosis and four children had right hydronephrosis. These patients with small pelvis existed the characteristics that the anteroposterior diameter of pelvis was smaller than 2.5 cm and these patients existed the symptom of hematuria, flank pain or recurrent urinary tract infection with the imaging revealing ureteral obstruction. The length of proximal ureteral stenosis ranged from 2.0 to 4.0 cm. Among 39 cases, 19 cases were operated with modified "double flaps" pyeloplasty, which was the modified "double flaps" pyeloplasty group. 20 cases were operated with traditional Anderson-Hynes pyeloplasty, which was traditional pyeloplasty group. The technique of modified "double flaps" pyeloplasty mainly included that the renal pelvis was cut into double flaps, the inferior flap was anastomosed with the spatulated ureter and the superior was covered, so that the length and caliber of the ureter were partial extended. The median age of two groups were 12.0 (6.0, 44.0) months and 12.0 (4.8, 62.8) months respectively, the anterior and posterior diameter of renal pelvis were (2.8±0.8)cm and (2.6±0.6)cm respectively, and split renal function were (36.7±5.1)% and (36.0±6.8)% respectively. There were no statistically significant differences in above parameters between the two groups( P>0.05). The clinical efficacy of the two groups were compared by collecting and comparing the operation related data and postoperative follow-up data. Results:The operation of 39 children in this study was successfully completed without conversion to open surgery.The operation time of "double flap" pyeloplasty group and traditional pyeloplasty group were (142.6±9.6) min and (124.5±8.6) min respectively, and the intraoperative anastomosis time were (56.1±7.2) min and (47.6±4.8) min respectively. There were significant difference in operation time and intraoperative anastomosis time between the two groups( P<0.05). Thirty-nine children were followed up normally without loss. The mean follow-up time was (27.7±2.5) months after surgery. In the "double flaps" pyeloplasty group, 2 cases suffered with fever who were diagnosed as urinary tract infection and improved after antibiotic treatment. In the traditional pyeloplasty group, 2 cases suffered with fever who were diagnosed as urinary tract infection and improved after antibiotic treatment. Two children had flank pain during follow-up to more than one year and the examination revealed that the anteroposterior diameter of the renal pelvis gradually increased. So surgery were performed again and the two children recovered. There were no significant differences in complication rate (2/19 and 4/20) and short-term surgical success rate(19/19 and 18/20) between the two groups ( P>0.05). Conclusions:The operation time and anastomosis time of the modified "double flap" technique for treating hydronephrosis are longer than those of the traditional method. But in the treatment of special types of hydronephrosis with small renal pelvis or long proximal ureteral stricture, it may have application prospects in reducing complications.