Objective:To detect the expression of Silent Information Regulator 1 (SIRT1), phosphorylated P53 protein (P53 protein), and Survivin in pancreatic cancer tissues, and to analyze their relationship with the prognosis of pancreatic cancer.Methods:A retrospective analysis was conducted on the clinical data of 102 patients with pancreatic cancer admitted to the Affiliated Hospital of Yan'an University from January 2020 to January 2023. The expression levels of SIRT1, P53, and Survivin in pancreatic cancer tissues and adjacent non-cancerous tissues were determined. Patients were followed up for 12-18 months and were divided into a survival group ( n = 59 and a death group ( n = 43) based on their survival status. The relationship between the expression of SIRT1, P53, and Survivin and the patients' pathological characteristics and prognosis was analyzed. Results:The positive expression rates of SIRT1, P53, and Survivin in pancreatic cancer tissues were 66.67% (68/102), 71.57% (73/102), and 73.53% (75/102), respectively, all of which were significantly higher than the rates in adjacent non-cancerous tissues, which were 26.47% (27/102), 29.41% (30/102), and 31.37% (32/102) ( χ2 = 33.11, 36.25, 36.34, all P < 0.001). Among patients with tumor stages Ⅱ-Ⅲ, low differentiation, and lymphatic metastasis, the positive rates of SIRT1 [82.61% (38/46), 76.06% (54/71), 81.82% (27/33)], P53 [84.78% (39/46), 80.28% (57/71), 87.88% (29/33)], and Survivin [86.96% (40/46), 81.69% (57/71), 87.88% (29/33)] were significantly higher than those in patients with tumor stage Ⅰ, moderate to high differentiation, and without lymphatic metastasis [SIRT1: 53.57% (30/56), 45.16% (14/31), 59.42% (41/69); P53: 60.71% (34/56), 51.61% (16/31), 63.77% (44/96); Survivin: 62.50% (35/56), 54.84% (17/31), 66.67% (46/96), χ2 SIRT1 = 9.58, 9.26, 5.04; χ2 P53 = 7.19, 8.71, 6.37; χ2 Survivin = 11.36, 7.99, 5.16, all P < 0.05]. The survival rates of patients with positive expression of SIRT1, P53, and Survivin in cancer tissues were 47.06% (32/68), 49.32% (36/73), and 49.33% (37/75), respectively, all of which were lower than the survival rates of patients with negative expression of SIRT1, P53, and Survivin, which were 79.40% (27/34), 79.31% (23/29), and 81.48% (22/27) (Log Rank χ2 = 8.79, 10.98, 12.65, all P < 0.05). In the survival group, the proportions of patients with tumor stages Ⅱ-Ⅲ, low differentiation, lymphatic metastasis, and positive expression of SIRT1, P53, and Survivin were 45.65% (21/46), 49.30% (35/71), 39.39% (13/33), 47.06% (32/68), 49.32% (36/73), and 49.33% (37/75), all of which were lower than the corresponding rates in patients with tumor stage Ⅰ, moderate to high differentiation, no lymphatic metastasis, and negative expression of SIRT1, P53, and Survivin, which were 67.86% (38/56), 77.42% (24/31), 66.67% (46/69), 79.41% (27/34), 79.31% (23/29), and 81.48% (22/27) ( χ2 = 5.10, 6.99, 6.80, 9.73, 7.65, 8.41, all P < 0.05). Tumor stage Ⅱ-Ⅲ ( OR = 2.413), low differentiation ( OR = 3.527), lymphatic metastasis ( OR = 3.077), positive SIRT1 expression ( OR = 4.339), positive P53 expression ( OR = 3.940), and positive Survivin expression ( OR = 4.519) were all identified as risk factors for poor prognosis in patients with pancreatic cancer (all P < 0.05). Conclusions:The expression of SIRT1, P53, and Survivin in the cancer tissues of patients with pancreatic cancer is closely associated with tumor stage, differentiation, and lymphatic metastasis. Additionally, the expression levels of SIRT1, P53, and Survivin are all significant factors influencing patient prognosis. Monitoring their expression can aid in the clinical prediction of patient outcomes.

Stereotactic radiotherapy is widely favored because of its high treatment precision and less fractionations.ZND-A is a new domestic gamma-ray cone-beam focused stereotactic radiotherapy system.Herein the technical characteristics of ZND-A system are described in detail from the aspects of the treatment frame,gamma-ray module,collimator module,six-dimensional treatment couch module and image-guided system module,and the main parameters are compared with the mainstream gamma knife equipments at home and abroad.With reference to Response Evaluation Criteria in Solid Tumors(RECIST 1.1),the initial efficacy of the patients treated by the ZND-A system is analyzed to evaluate the advantages and disadvantages of the ZND-A system for providing a reference for the hospital clinical use of this type of gamma knife.

This study aims to investigate the effects of Pulsatilla powder(PP)on colonic intestinal flora and tryptophan metabolism in piglets with diarrhea.Twenty-four weaned piglets were ran-domly divided into the normal control group,model group,self-healing group,and PP group.The model of piglet diarrhea was established by the compound factor of external evil+internal injury+epi-demic virus,and treated with PP.During the modeling period,the body weight,mental status and fecal patterns of piglets were monitored daily.After the experiment,the colon tissues were collect-ed for histopathological observation,and the inflammatory factors IL-1β and IL-6 in the colon tis-sue was detected by ELISA,16S rRNA was used to analyze colonic intestinal flora and RT-qPCR was used to determine the expression of genes related to tryptophan metabolism in the colon.The results showed that,compared with the model group,PP significantly improved the pathological status of the colon in piglets with diarrhea,with elevated body mass and a significant increase in goblet cells(P＜0.05);and significantly reduced the levels of IL-1β and IL-6 in the colon of diar-rhea piglets(P＜0.05).The results of intestinal flora showed that PP increased the abundance of Firmicutes(P＜0.01),Spirochaeota(P＜0.01)and Lactobacillus(P＜0.05),reduced the abun-dance of Bacteroidota(P＜0.05)、Prevotella_NK3B31_group(P＜0.05)and Clostridium_sensu_stricto 1(P＜0.01).Meanwhile,PP significantly reduced the expression level of TPH1 mRNA(P＜0.05),increased the expression levels of AhR(P＜0.05)and IL-22 mRNA in the co-lon of diarrhea piglets.These results indicates that PP may alleviate diarrhea in piglets by regula-ting colonic intestinal flora and genes related to intestinal tryptophan metabolism.This study pro-vides data support for the subsequent investigation of piglet diarrhea prevention and treatment.

Objective:To assess the preliminary efficacy and safety of modified Société Internationale d′Oncologie Pédiatrique Epithelial Liver Tumor Study Group (SIOPEL)-4 protocol for pediatric hepatoblastoma (HB) with lung metastases.Methods:This prospective cohort study enrolled 27 newly diagnosed pediatric HB with lung metastases who received the modified SIOPEL-4 protocol at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine, and Shanghai Children′s Hospital between January 2020 to December 2023. Clinical characteristics, lung response rates to induction chemotherapy, treatment outcomes, prognostic factors and sever chemotherapy toxicities at different stages were analyzed. Survival analysis was performed by Kaplan-Meier method. Univariate prognostic analysis was conducted by Log-Rank test.Results:Of the 27 patients, there were 17 males and 10 females, with the age of 21 (15, 33) months. During the follow-up of 31 (12, 45) months for 17 continuous complete remission patients, 4 cases disease progression (2 cases death) and 6 cases relapse were observed. The 2-year event free survival (EFS) and overall survival (OS) rate was (58±11)% and (89±7)%, respectively. All the 27 patients had response to block 1-3 induction chemotherapy (cisplatin+doxorubicin), with 14 cases (52%) achieving complete response and 13 cases (48%) achieving partial response of lung metastatic lesions, the 2-year EFS rate was (81±12)% and (34±14)%, respectively ( χ 2=6.76, P=0.009), the 2-year OS rate was 100% and (79±13)%, respectively ( χ2=2.12, P=0.145). Patients with caudate lobe tumors or ≥10 pulmonary metastatic nodules had significantly lower EFS rates ( χ2=5.36, 7.84, P=0.021, 0.005, respectively). The incidence of grade 3/4 neutropenia after block 1-3 induction chemotherapy, CD (carboplatin+doxorubicin), and VI (vincristine+irinotecan) consolidation chemotherapy was 90% (73/81), 75% (58/77), and 31% (11/35), respectively. The incidence of grade 3/4 thrombocytopenia was 77% (62/81), 69% (53/77), and 14% (5/35), respectively. The incidence of grade 3/4 infections was 64% (52/81), 25% (19/77), and 20% (7/35), respectively. The differences between the groups were statistically significant ( χ2=43.51, 42.69, 33.00, all P<0.001). Two patients (10%) of the 20 evaluable patients for ototoxicity occurred grade 3 and higher hearing impairment, with 1 patient requiring a hearing aid. Conclusions:The modified SIOPEL-4 regimen shows good preliminary efficacy and safety in treating pediatric HB with lung metastases. The prognosis for patients with residual lesions in the lungs after induction chemotherapy needs to be improved. Attention should be given to the ototoxicity induced by high-dose cisplatin chemotherapy.

Stereotactic radiotherapy is widely favored because of its high treatment precision and less fractionations.ZND-A is a new domestic gamma-ray cone-beam focused stereotactic radiotherapy system.Herein the technical characteristics of ZND-A system are described in detail from the aspects of the treatment frame,gamma-ray module,collimator module,six-dimensional treatment couch module and image-guided system module,and the main parameters are compared with the mainstream gamma knife equipments at home and abroad.With reference to Response Evaluation Criteria in Solid Tumors(RECIST 1.1),the initial efficacy of the patients treated by the ZND-A system is analyzed to evaluate the advantages and disadvantages of the ZND-A system for providing a reference for the hospital clinical use of this type of gamma knife.

This study aims to investigate the effects of Pulsatilla powder(PP)on colonic intestinal flora and tryptophan metabolism in piglets with diarrhea.Twenty-four weaned piglets were ran-domly divided into the normal control group,model group,self-healing group,and PP group.The model of piglet diarrhea was established by the compound factor of external evil+internal injury+epi-demic virus,and treated with PP.During the modeling period,the body weight,mental status and fecal patterns of piglets were monitored daily.After the experiment,the colon tissues were collect-ed for histopathological observation,and the inflammatory factors IL-1β and IL-6 in the colon tis-sue was detected by ELISA,16S rRNA was used to analyze colonic intestinal flora and RT-qPCR was used to determine the expression of genes related to tryptophan metabolism in the colon.The results showed that,compared with the model group,PP significantly improved the pathological status of the colon in piglets with diarrhea,with elevated body mass and a significant increase in goblet cells(P＜0.05);and significantly reduced the levels of IL-1β and IL-6 in the colon of diar-rhea piglets(P＜0.05).The results of intestinal flora showed that PP increased the abundance of Firmicutes(P＜0.01),Spirochaeota(P＜0.01)and Lactobacillus(P＜0.05),reduced the abun-dance of Bacteroidota(P＜0.05)、Prevotella_NK3B31_group(P＜0.05)and Clostridium_sensu_stricto 1(P＜0.01).Meanwhile,PP significantly reduced the expression level of TPH1 mRNA(P＜0.05),increased the expression levels of AhR(P＜0.05)and IL-22 mRNA in the co-lon of diarrhea piglets.These results indicates that PP may alleviate diarrhea in piglets by regula-ting colonic intestinal flora and genes related to intestinal tryptophan metabolism.This study pro-vides data support for the subsequent investigation of piglet diarrhea prevention and treatment.

Objective:To detect the expression of Silent Information Regulator 1 (SIRT1), phosphorylated P53 protein (P53 protein), and Survivin in pancreatic cancer tissues, and to analyze their relationship with the prognosis of pancreatic cancer.Methods:A retrospective analysis was conducted on the clinical data of 102 patients with pancreatic cancer admitted to the Affiliated Hospital of Yan'an University from January 2020 to January 2023. The expression levels of SIRT1, P53, and Survivin in pancreatic cancer tissues and adjacent non-cancerous tissues were determined. Patients were followed up for 12-18 months and were divided into a survival group ( n = 59 and a death group ( n = 43) based on their survival status. The relationship between the expression of SIRT1, P53, and Survivin and the patients' pathological characteristics and prognosis was analyzed. Results:The positive expression rates of SIRT1, P53, and Survivin in pancreatic cancer tissues were 66.67% (68/102), 71.57% (73/102), and 73.53% (75/102), respectively, all of which were significantly higher than the rates in adjacent non-cancerous tissues, which were 26.47% (27/102), 29.41% (30/102), and 31.37% (32/102) ( χ2 = 33.11, 36.25, 36.34, all P < 0.001). Among patients with tumor stages Ⅱ-Ⅲ, low differentiation, and lymphatic metastasis, the positive rates of SIRT1 [82.61% (38/46), 76.06% (54/71), 81.82% (27/33)], P53 [84.78% (39/46), 80.28% (57/71), 87.88% (29/33)], and Survivin [86.96% (40/46), 81.69% (57/71), 87.88% (29/33)] were significantly higher than those in patients with tumor stage Ⅰ, moderate to high differentiation, and without lymphatic metastasis [SIRT1: 53.57% (30/56), 45.16% (14/31), 59.42% (41/69); P53: 60.71% (34/56), 51.61% (16/31), 63.77% (44/96); Survivin: 62.50% (35/56), 54.84% (17/31), 66.67% (46/96), χ2 SIRT1 = 9.58, 9.26, 5.04; χ2 P53 = 7.19, 8.71, 6.37; χ2 Survivin = 11.36, 7.99, 5.16, all P < 0.05]. The survival rates of patients with positive expression of SIRT1, P53, and Survivin in cancer tissues were 47.06% (32/68), 49.32% (36/73), and 49.33% (37/75), respectively, all of which were lower than the survival rates of patients with negative expression of SIRT1, P53, and Survivin, which were 79.40% (27/34), 79.31% (23/29), and 81.48% (22/27) (Log Rank χ2 = 8.79, 10.98, 12.65, all P < 0.05). In the survival group, the proportions of patients with tumor stages Ⅱ-Ⅲ, low differentiation, lymphatic metastasis, and positive expression of SIRT1, P53, and Survivin were 45.65% (21/46), 49.30% (35/71), 39.39% (13/33), 47.06% (32/68), 49.32% (36/73), and 49.33% (37/75), all of which were lower than the corresponding rates in patients with tumor stage Ⅰ, moderate to high differentiation, no lymphatic metastasis, and negative expression of SIRT1, P53, and Survivin, which were 67.86% (38/56), 77.42% (24/31), 66.67% (46/69), 79.41% (27/34), 79.31% (23/29), and 81.48% (22/27) ( χ2 = 5.10, 6.99, 6.80, 9.73, 7.65, 8.41, all P < 0.05). Tumor stage Ⅱ-Ⅲ ( OR = 2.413), low differentiation ( OR = 3.527), lymphatic metastasis ( OR = 3.077), positive SIRT1 expression ( OR = 4.339), positive P53 expression ( OR = 3.940), and positive Survivin expression ( OR = 4.519) were all identified as risk factors for poor prognosis in patients with pancreatic cancer (all P < 0.05). Conclusions:The expression of SIRT1, P53, and Survivin in the cancer tissues of patients with pancreatic cancer is closely associated with tumor stage, differentiation, and lymphatic metastasis. Additionally, the expression levels of SIRT1, P53, and Survivin are all significant factors influencing patient prognosis. Monitoring their expression can aid in the clinical prediction of patient outcomes.

Objective:To assess the preliminary efficacy and safety of modified Société Internationale d′Oncologie Pédiatrique Epithelial Liver Tumor Study Group (SIOPEL)-4 protocol for pediatric hepatoblastoma (HB) with lung metastases.Methods:This prospective cohort study enrolled 27 newly diagnosed pediatric HB with lung metastases who received the modified SIOPEL-4 protocol at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine, and Shanghai Children′s Hospital between January 2020 to December 2023. Clinical characteristics, lung response rates to induction chemotherapy, treatment outcomes, prognostic factors and sever chemotherapy toxicities at different stages were analyzed. Survival analysis was performed by Kaplan-Meier method. Univariate prognostic analysis was conducted by Log-Rank test.Results:Of the 27 patients, there were 17 males and 10 females, with the age of 21 (15, 33) months. During the follow-up of 31 (12, 45) months for 17 continuous complete remission patients, 4 cases disease progression (2 cases death) and 6 cases relapse were observed. The 2-year event free survival (EFS) and overall survival (OS) rate was (58±11)% and (89±7)%, respectively. All the 27 patients had response to block 1-3 induction chemotherapy (cisplatin+doxorubicin), with 14 cases (52%) achieving complete response and 13 cases (48%) achieving partial response of lung metastatic lesions, the 2-year EFS rate was (81±12)% and (34±14)%, respectively ( χ 2=6.76, P=0.009), the 2-year OS rate was 100% and (79±13)%, respectively ( χ2=2.12, P=0.145). Patients with caudate lobe tumors or ≥10 pulmonary metastatic nodules had significantly lower EFS rates ( χ2=5.36, 7.84, P=0.021, 0.005, respectively). The incidence of grade 3/4 neutropenia after block 1-3 induction chemotherapy, CD (carboplatin+doxorubicin), and VI (vincristine+irinotecan) consolidation chemotherapy was 90% (73/81), 75% (58/77), and 31% (11/35), respectively. The incidence of grade 3/4 thrombocytopenia was 77% (62/81), 69% (53/77), and 14% (5/35), respectively. The incidence of grade 3/4 infections was 64% (52/81), 25% (19/77), and 20% (7/35), respectively. The differences between the groups were statistically significant ( χ2=43.51, 42.69, 33.00, all P<0.001). Two patients (10%) of the 20 evaluable patients for ototoxicity occurred grade 3 and higher hearing impairment, with 1 patient requiring a hearing aid. Conclusions:The modified SIOPEL-4 regimen shows good preliminary efficacy and safety in treating pediatric HB with lung metastases. The prognosis for patients with residual lesions in the lungs after induction chemotherapy needs to be improved. Attention should be given to the ototoxicity induced by high-dose cisplatin chemotherapy.

Objective:To summarize the genotype and clinical characteristics of chylomicron retention disease (CMRD) caused by secretion associated Ras related GTPase 1B (SAR1B) gene variations.Methods:Clinical data and genetic testing results of 2 children with CMRD treated at Children′s Hospital of Fudan University and Jiangxi Provincial Children′s Hospital from May 2022 to July 2023 were summarized. To provide an overview of the clinical and genetic characteristics of CMRD caused by SAR1B gene variations, all of the literature was searched and reviewed from China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, China VIP database, China Biology Medicine disc and PubMed database (up to January 2024) with "chylomicron retention disease" "Anderson disease" or "Anderson syndrome" as the search terms. All relevant literatures were reviewed to summarize the clinical and genetic features of CMRD caused by SAR1B gene variations.Results:One 11-year-old boy and one 4-month-old girl with CMRD. Both patients had lipid malabsorption, failure to thrive, decreased cholesterol, elevated transaminase and creatine kinase, and Vitamin E deficiency, with homozygous variations (c.224A>G) and compound heterozygous variations (c.224A>G and c.554G>T) in SAR1B gene, respectively. Case 1 was followed up for over a month, and he still occasionally experienced lower limb muscle pain. Case 2 was followed up for more than a year, and her had caught up to normal levels. Both patients had no other significant discomfort. Literature search retrieved 0 Chinese literature and 22 English literatures. In addition to the 2 cases reported in this study, a total of 51 patients were identified as CMRD caused by SAR1B gene variations. Twenty-one types of SAR1B variants 10 missense, 4 nonsense, 3 frameshift, 1 in-frame deletion, 1 splice, 1 gross deletion, and 1 gross insertion-deletion were found among the 51 CMRD cases. Among all the patients, 49 cases had lipid malabsorption (43 cases had diarrhea or fatty diarrhea, 17 cases had vomiting, and 12 cases had abdominal distension), 45 cases had lipid soluble Vitamin deficiency (43 cases had Vitamin E deficiency, 10 cases had Vitamin A deficiency, 9 case had Vitamin D deficiency, and 5 cases had Vitamin K deficiency), 35 cases had failure to thrive, 32 cases had liver involvement (32 cases had elevated transaminases, 5 cases had fatty liver, and 3 cases had hepatomegaly), 29 cases had white small intestinal mucosa under endoscopy, and 17 cases had elevated creatine kinase, 14 cases had neuropathy, 5 cases had ocular lesions, 2 cases had acanthocytosis, 1 case had decreased cardiac ejection fraction, and 1 case was symptom-free.Conclusions:Early infancy failure to thrive and lipid malabsorption are common issues for CMRD patients. The laboratory tests are characterized by hypocholesterolemia with or without fat-soluble Vitamin deficiency, elevated liver enzymes and (or) creatine kinase. Currently, missense variations are frequent among the primarily homozygous SAR1B genotypes that have been described.