Objective:To discuss the distinctive sonographic feature and the biological behavior of renal angiomyolipoma(RAML),and to provide the reference for the clinicians to make the accurate diagnosis of RAML.Methods:The clinical data of one patient with invasive classical RAML combined with pseudaneurysm formation were collected.The sonographic appearances were analyzed in conjunction with the pathological characteristics to clarify the biological behavior of RAML,and the relevant literatures were reviewed.Results:The patient,a 60-year-old female,visited the local hospital due to discomfort in the lumbar area,and received CT examination,and the CT examination results revealed a left renal mass,so the patient came to our hospital.The specialist clinical examinations and laboratory investigations were unremarkable.The ultrasound results indicated an enlarged left kidney with a cystic and solid mass at the upper pole,which featured pseudaneurysm formation(originating from the interlobar arteries);the enhanced CT image results suggested a high probability of upper pole renal carcinoma combined with aneurysmal formation within the tumor,alongside invasion into the left adrenal gland.The patient underwent laparoscopic radical left nephrectomy,and the postoperative pathology confirmed the diagnosis of invasive classical RAML.Conclusion:The classical RAML can exhibit the invasive biological behavior.The pseudaneurysm formation is a special sonographic manifestation of RAML,which can be challenging to differentiate from the other renal tumors.

Pancreatic metastases originating in colon cancer are very rare clinically, and there are few reports on their imaging manifestations. In this paper, it improves the diagnosis of the disease by reporting a case of pancreatic head metastases and focusing on the appearance of contrast-enhanced ultrasound.

Objective To search,evaluate and summarize the relevant evidence of management on xerostomia and thirst of maintenance hemodialysis(MHD)patients,and provide the evidence for clinical medical staff to relieve the symptoms of xerostomia and thirst in MHD patients.Methods We search guideline websites such as the BMJ Best Practice,UpToDate,NICE,GIN,NGC,CMA Infobase,Medlive,and databases such as Cochrane Library,JBI,PubMed,Embase,CINAHL,CNKI,Wanfang,VIP,SinoMed related to interventions on xerostomia and thirst of MHD patients.The literature types include guidelines,expert consensuses,clinical decisions,best practice,evidence summaries,systematic reviews,randomized controlled trials.The search period is from January 1,2013 to September 30,2023.Results A total 12 pieces of the literature were included,including 3 systematic reviews and 9 randomized controlled trials.A total of 19 pieces of evidence were summarized in 6 aspects,including evaluation,basic interventions,oral local interventions,auricular acupressure,dialysis scheme,Chinese herbal medicine combined with acupoint therapy and Chinese herbal medicine therapy.Conclusion Blood purification medical staff should comprehensively consider MHD patients and specific clinical situations,and make interventions to relieve the symptoms of xerostomia and thirst of MHD patients,relieve their physical and mental pain and improve their quality of life.

Objective:To investigate the influencing factors of delayed methotrexate (MTX) elimination after high-dose methotrexate (HD-MTX) treatment in children with acute lymphoblastic leukemia (ALL) and the effects of delayed MTX elimination and HD-MTX reduction on the prognosis of children with ALL.Methods:The clinical data of 242 children with ALL diagnosed and treated in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2015 to June 2020 in accordance with the Chinese Children's Cancer Group study ALL 2015 (CCCG-ALL 2015) were retrospectively analyzed. Low risk and intermediate/high risk children respectively received 3 g/m 2 and 5 g/m 2 HD-MTX for 4 times, and the serum MTX concentration was monitored. The serum MTX concentration > 1 μmol/L at 44 h of administration was considered as the delayed elimination, which was divided into mild (> 1 μmol/L and ≤ 5 μmol/L), moderate (> 5 μmol/L and ≤ 10 μmol/L) and severe (> 10 μmol/L) delayed elimination. Univariate and multivariate logistic regression analysis were used to analyze the influencing factors of delayed MTX elimination, and univariate Cox proportional hazards model was used to analyze the related factors of ALL relapse. Results:The 242 children with ALL completed 962 times of HD-MTX chemotherapy. The median serum MTX concentration [ M ( Q1, Q3)] at 44 h of administration was 0.45 μmol/L (0.33 μmol/L, 0.72 μmol/L). The total incidence of delayed MTX elimination was 17.7% (170/962). The incidence of mild, moderate and severe delayed elimination was 13.8% (133/962), 2.6% (25/962) and 1.2% (12/962), respectively. Logistic regression analysis showed that age ≥ 7 years old ( OR = 1.68, 95% CI 1.17-2.41, P = 0.005), MTX dose >3 g/m 2 at each course ( OR = 2.14, 95% CI 1.52-3.03, P < 0.001) and the first course of HD-MTX chemotherapy ( OR = 2.05, 95% CI 1.43-2.93, P < 0.001) were independent risk factors for delayed MTX elimination. The median follow-up time was 50 months (34 months, 68 months), 12.8% (31/242) of the children relapsed, and the median relapse time was 30 months (30 months, 39 months). Univariate Cox regression analysis showed that there were no significant differences in the relapse rates among children with different gender, immunophenotype, risk, the number of delayed MTX elimination, and the completion of HD-MTX chemotherapy (the ratio of MTX average dose to initial planned dose) (all P > 0.05). Conclusions:The independent risk factors of delayed elimination of MTX in children with ALL are age ≥ 7 years old, MTX dose > 3 g/m 2 at each course and the first course of HD-MTX chemotherapy. Delayed elimination of MTX and reduction of HD-MTX have no significant effect on ALL relapse.

Pancreatic metastasis of small cell lung cancer is very rare in clinic. The purpose of this article is to improve the knowledge of clinical and radiologists about this disease by reporting one case.

Objective:To study the effect of Sanjie Xiaoliu Recipe on transplanted tumors in breast cancer mice through in vivo experiments, in order to explore the efficacy and mechanism of Sanjie Xiaoliu Recipe on breast cancer patients. Methods:45 BaL B/c female mice were selected to establish the transplanted tumor model of breast cancer. All the transplanted tumor models of breast cancer mice were randomly divided into five groups: the control group (intragastric administration of normal saline), the low, medium and high dose group of Sanjie Xiaoliu Recipe (intragastric administration of different doses of Sanjie Xiaoliu Decoction), and the paclitaxel group (intraperitoneal injection of paclitaxel). After 24 days of continuous administration, the diet and activities of mice were observed; the body weight, weight and volume changes of transplanted tumor mice were recorded, and the tumor inhibition rate was calculated. Western blot was used to detect the expression of epithelial mesenchymal transformation related proteins (E-cadherin, N-cadherin, Vimentin) in the transplanted tumor tissues of mice in each group.Results:(1) The food intake and activity status of mice treated with Sanjie Xiaoliu Recipe were less affected by the transplanted tumor of breast cancer. (2) The volume and weight of transplanted tumor in the treat groups were smaller than those in the control group (all P<0.01), and the volume of transplanted tumor in the middle dose group of Sanjie Xiaoliu Recipe was smaller than that in the low dose group ( P<0.05). The tumor inhibition rates among the treatment groups were: Sanjie Xiaoliu Recipe medium dose group 52.4%, paclitaxel group 40.3%, Sanjie Xiaoliu Recipe low dose group 39.5%, Sanjie Xiaoliu Recipe high dose group 34.1%. (3) The results of Western blot showed that the expression level of E-cadherin in the transplanted tumor tissue of the treat groups was higher than that in the control group, and the expression levels of N-cadherin and Vimentin were lower than that in the control group, with statistically significant difference (all P<0.05). Conclusions:The Sanjie Xiaoliu Recipe can improve the weakness and reduced consumption of breast cancer mice, which can inhibit the tumor mass growth in mice to a certain extent. Its mechanism may be that Sanjie Xiaoliu Recipe can inhibit the epithelial mesenchymal transformation of breast cancer and the invasion and metastasis of breast cancer.

Objective To investigate the risk factors for intraoperative hypotension (IOH) in patients undergoing double plasma molecular adsorption system (DPMAS) artificial liver support therapy. Methods Clinical data were collected from 181 patients (670 cases in total) who underwent DPMAS artificial liver support therapy in Liver Disease Center of The First Affiliated Hospital of University of Science and Technology of China from October 1, 2017 to December 31, 2020, and according to the presence or absence of IOH during DPMAS therapy, they were divided into IOH group with 70 patients and non-IOH group with 111 patients.Clinical indicators were compared between the two groups and their association with IOH was analyzed; prognosis was analyzed at 12 and 24 weeks.The independent samples t -test was used for comparison of normally distributed continuous data between groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between groups; the chi-square test was used for comparison of categorical data between groups.Univariate and multivariate Logistic regression analyses were used to investigate the risk factors for IOH.The Kaplan-Meier method was used to plot receiver operating characteristic (ROC) curves, and the Z test was used for comparison of the area under the ROC curve (AUC) of independent risk factors. Results The univariate Logistic regression analysis showed that female individuals, individuals aged ≥50 years, and individuals with normal or low body mass index (BMI) tended to have a higher risk of IOH (all P < 0.05), and the multivariate analysis showed that normal or low BMI (odds ratio [ OR ]=3.290, 95% confidence interval [ CI ]: 1.523-7.108, P =0.002) and female sex ( OR =5.146, 95% CI : 2.316-11.432, P < 0.001) were independent risk factor for IOH in patients undergoing DPMAS artificial liver support therapy.The ROC curve analysis of female sex+BMI ≤24 kg/m 2 showed that it had an AUC of 0.639 in predicting IOH ( P =0.002).The patients experiencing IOH had a 12-week survival rate of 55.77%(29/52) and a 24-week survival rate of 50%(26/52), and there were significant differences between the two groups in 12-and 24-week survival rates (12-week: 76.53% vs 55.77%, χ 2 =6.887, P =0.009;24-week: 74.49% vs 50.00%, χ 2 =9.080, P =0.003). Conclusion The risk of hypotension was higher in female patients and that with normal or low BMI during DPMAS artificial liver therapy.Patients with IOH had poor survival prognosis at 24 weeks after DPMAS therapy.

Objective:To determine classification and clinical features of morphea.Methods:A retrospective analysis was conducted on epidemiological information about clinical manifestations of and laboratory data from 180 patients with morphea, who visited Zhongshan Hospital, Fudan University from January 2010 to July 2021. Two-independent-sample t test was used to compare the age at onset between genders, and chi-square test to analyze differences in clinical characteristics between different genders and subtypes. Results:Among the 180 patients, 123 were females and 57 were males, with a male-to-female ratio of 1∶2.16. The age at onset of morphea was 28.69 ± 17.97 years for female patients, and 29.90 ± 20.67 years for male patients. Among them, linear morphea was the most common type in this study (68 cases, 37.78%), followed by plaque morphea (63 cases, 35.00%), mixed morphea (28 cases, 15.56%) and deep morphea (21 cases, 11.67%). The disease occurred in all age groups, but the age at onset significantly varied among different clinical subtypes ( F = 5.95, P < 0.001). No significant difference was observed in the age at onset or proportion of clinical subtypes between genders ( F = 0.15, P = 0.696; χ2 =2.88, P = 0.410). Atrophoderma of Pasini and Pierini (APP) was very common (62 cases, 34.44%) in the 180 patients, which mainly manifested as plaques or linear lesions, and 26 out of 45 patients with plaque APP and 11 out of 17 with linear APP were both accompanied by other subtypes of morphea. Among the 75 patients tested for autoantibody profiles, 34 (45.33%) presented with positive results. More diverse types of autoantibodies were found in female patients compared with male patients, and antinuclear antibodies, anti-SSA and anti-SSB antibodies were the most common types. There were various types of comorbidities in female patients, but lichen sclerosus et atrophicus and vitiligo were the most common comorbidities in both genders. Conclusion:High incidence and frequent co-occurrence with other subtypes of APP may be the characteristics of Chinese patients with morphea, and it is recommended to classify morphea into plaque, linear, deep and mixed subtypes.

OBJECTIVE: To report on a case with severe hemophilia A (HA) due to a large duplication of F8 gene. METHODS: Inversion detection, Sanger sequencing, and multiplex ligation-dependent probe amplification (MLPA) were used to detect the mutation in the proband and his mother. RESULTS: The patient, a 7-year-old boy, was diagnosed with severe HA at 8 months. No inhibitor was developed over 150 exposure days. Intronic inversion detection and Sanger sequencing have failed to identify pathogenic variants, while MLPA revealed a large duplication [Ex 1_22 dup (2 copies)] in the proband, for which his mother was a carrier [Ex 1_22 dup (3 copies)]. Large duplications of the F8 gene have so far been found in 24 HA patients, all of whom had a severe phenotype, only one had a history of inhibitors. CONCLUSION Large duplications of F8 gene are associated with severe HA. The diagnostic rate for HA may be increased by MLPA.
Child ; Factor VIII/genetics* ; Gene Duplication ; Hemophilia A/genetics* ; Humans ; Introns ; Male ; Mutation ; Phenotype

【Objective】 To investigate the main causes of blood donor deferral in domestic blood center. 【Methods】 The causes of donor deferral were classified into 12 categories as previous medical history, drug use, alcohol consumption, menstrual period, underweight, abnormal blood pressure, abnormal body temperature, abnormal hemoglobin (Hb), lipemic blood, positive hepatitis B surface antigen (HBsAg), elevated alanine aminotransferase (ALT) and others according to the comparison indicators of Asia-Pacific Blood Network (APBN) and the national standard Blood Donor Health Examination Requirements. The relevant data of the top 3 causes of donor deferral, voluntarily reported by the members of Practice Comparison Working Group of China’s Mainland Blood Collection and Supply Institutions from 2014 to 2019, were collected and a histogram was generated. 【Results】 The median donor deferral rate of 20 domestic blood centers from 2014 to 2019 was 12.14%, with the lowest at 0.18% and highest at 32.32%, respectively. The top three causes for donor deferral were elevated ALT, abnormal Hb and abnormal blood pressure in year 2014, 2015, 2018 and 2019; elevated ALT, lipemic blood and abnormal blood pressure in 2016; elevated ALT, abnormal Hb, and lipemic blood in 2017. 【Conclusion】 The main causes of donor deferral were elevated ALT, abnormal Hb, abnormal blood pressure and lipemic blood.