Objective:To investigate the safety and effectiveness of cytoreductive nephrectomy and tumor thrombectomy in patients with metastatic renal cancer with renal vein or inferior vena cava tumor thrombus.Methods:From February 2015 to May 2019, 56 cases of metastatic renal cell carcinoma with venous tumor thrombus were analyzed retrospectively, including 44 male (78.6%) and 12 female (21.4%)cases, and the average age was (59.2±10.7)(22-82). The clinical presentations covered local symptoms in 26 cases (46.4%), systemic symptoms in 8 cases (14.3%), both local symptoms and systemic symptoms in 12 cases (21.4%), and asymptomatic in 10 cases (17.9%). Among them, renal tumors were located in 35 cases (62.5%) on the right and 21 cases (37.5%) on the left. The average tumor diameter was (10.1±3.8)(1.5-21.1) cm. Forty-five cases (80.4%) scored 2 points and 11 cases (19.6%) scored 3 points by the American Society of anesthesiologists(ASA). Preoperative hemoglobin was (118.2±23.1)(72-178) g/L, and albumin was (37.9±5.6)(23-50) g/L, total protein was (67.7±6.7)(43-81) g/L, serum creatinine was (111.3±119.6)(32-958) μmol/L. There were 16 cases of Mayo 0 (28.6%), 14 cases of Mayo Ⅰ(25.0%), 17 cases of Mayo Ⅱ(30.4%), 4 cases of Mayo Ⅲ(7.1%), and 5 cases of Mayo Ⅳ(8.9%). Fourteen cases (25.0%) were in the stage of cN 0 and 42 cases (75.0%) in the stage of cN 1. Five cases (8.9%) had simple bone metastasis, 16 cases (28.6%) had simple lung metastasis, 2 cases (3.6%) had simple adrenal metastasis, 6 cases (10.7%) had simple liver metastasis, and 27 cases (48.2%) had 2 or more multiple system metastasis. According to the location of the organ system, 91 metastatic lesions were found in 56 patients. Among them, 37 cases (40.7%) had lung metastasis, 18 cases (19.8%) had liver metastasis, 21 cases (23.1%) had bone metastasis and 15 cases (16.5%) had adrenal metastasis. All 56 patients belonged to IMDC prognosis score model medium risk group. The surgical treatment of Mayo grade 0 tumor thrombus was the same as that of routine radical nephrectomy. The tumor thrombus of Mayo grade Ⅰ was removed after IVC was partially blocked by Satinsky′s forceps. The Mayo Ⅱ tumor thrombus was removed, after blocking the distal vena cava, the contralateral renal vein and the proximal vena cava. Mayo grade Ⅲ tumor thrombus needed pringer's method to block the first porta hepatis. For grade Ⅳ tumor thrombus the diaphragm could be cut directly, or the thrombus could be removed by cardiopulmonary bypass. Laparoscopic surgery was performed in 22 cases (39.3%) and open surgery in 30 cases (53.6%). Six cases (10.7%) underwent IVC wall resection because of tumor invasion. 32 cases (57.1%) underwent ipsilateral adrenalectomy because of tumor invasion or adrenal metastasis, and 23 cases (41.1%) underwent ipsilateral lymphadenectomy. In this study, there were 11 cases of solitary metastasis, 8 cases of which were operated on and 3 cases of which were not operated on. Forty-three patients were treated with sunitinib after palliative nephrectomy, 9 patients were treated with pazopanib, 3 patients were treated with acitinib, and 1 patient was treated with sorafenib. Results:The operations were successfully completed in 56 patients. Four cases (7.1%) changed from laparoscopic surgery to open surgery. The operation time was (326.8±114.9)(108-589) min. Intraoperative hemorrhage was (1 435.2±1 513.4)(20-6 000) ml, intraoperative red blood cells transfusion was (1 456.7±832.8)(400-3 600) ml in 30 cases, and intraoperative plasma transfusion was (700.0±473.6)(200-1 800) ml in 15 cases. The postoperative hospital stay was (10.6±4.6)(5-26) days. The serum creatinine one week after operation was (109.5±98.7) (47-772) μmol/L. There were 46 cases (82.1%) of renal clear cell carcinoma, 7 cases (12.5%) of papillary renal cell carcinoma and 3 cases (5.4%) of unclassified renal cell carcinoma. One case was WHO/ISUP 2016 nuclear grade 1 (1.8%), 20 cases(36.4%) was grade 2, 18 cases(32.7%)was grade 3, and 16 cases(29.1%)was grade 4. Early postoperative complications occurred in 22 cases (39.3%). Among them, 1 case of Clavien gradeⅠ was wound infection. There were 16 cases with Clavien gradeⅡ, including 5 cases who received blood transfusion due to anemia, 3 cases with chylous fistula, 4 cases with postoperative pulmonary infection, 2 cases with postoperative lower extremity venous thrombosis, 1 case with atrial fibrillation and 1 case with epididymitis. Clavien gradeⅢ a was found in 1 case with pneumothorax. Clavien gradeⅣ was found in 2 cases, including 1 case of acute cerebral infarction and 1 case of renal insufficiency.There were 2 cases with Clavien gradeⅤ with perioperative death. Among the 56 patients, 5 lost the follow-up, 2 died during the perioperative period, and the other 49 patients were followed up for 1-39 months, with a median follow-up of 14 months. The mean survival time was (25.6±2.5) months, and the median survival time was 25 months.Conclusions:It was relatively safe and effective to perform cytoreductive nephrectomy and tumor thrombectomy in patients with metastatic renal cancer with tumor thrombus. For the patients with clinical symptoms, IMDC prognosis score model medium risk group, and strong desire for surgery, the combination of cytoreductive nephrectomy with tumor thrombectomy and postoperative targeted medical therapy was recommended.

Objective:To explore the value of preoperative aspartate transaminase(AST) and aspartate transaminase/alanine transaminase ratio(AST/ALT)for predicting the prognosis in patients with non-metastatic renal cell carcinoma with tumor thrombus.Methods:A retrospective analysis was made of the patients with renal cell carcinoma with tumor thrombus in our institution from February 2015 to December 2018. This study included 80 patients, 56 males and 24 females with mean age of 58 years (range 15-83 years). There were 17 in Mayo level 0, 24 Mayo level I, 22 in Mayo level II, 12 in Mayo level III and 5 in Mayo level IV. All the patients received radical nephrectomy or palliative nephrectomy with tumor thrombectomy. The continuous variable of AST/ALT was collected by ROC curve. The maximum value of Youden index was taken as the critical value, and the continuous variables were adjusted to binary variables. Cancer-specific survival (CSS) was calculated according to the Kaplan-Meier analysis and compared by the log-rank test. Cox multivariate regression analysis was used to analyze the independent factors of the prognosis of patients with non-metastatic renal cancer and tumor thrombus.Results:There were 70 cases of clear cell carcinoma, 10 cases of non-clear cell carcinoma, 30 cases of low Fuhrman grade (grade 1&2), and 50 cases of high Fuhrman grade (grade 3&4). AST was 19U/L (8-226 U/L) and AST/ALT was 1.3(0.4-3.3). There was a significant difference in AST between different lymphovascular invasion groups ( P=0.04), but there was no significant difference in sex, age, Mayo classification, pathological type, Fuhrman grade and lymph node metastasis. The difference of AST / ALT between age groups was significant ( P=0.025). The average follow-up time was 14.7 months (0-44 months). During the follow-up, 11 (13.8%) patients died of tumor. Univariate analysis showed that Fuhrman grade ( P=0.007), lymph node metastasis ( P=0.019), hemoglobin ( P=0.001), alkaline phosphatase (ALP, P=0.001), AST ( P=0.004) and AST / ALT ( P=0.038) were risk factors for CSS. In terms of prognosis, considering the potential correlation between AST level and AST/ALT ratio, after excluding AST, multivariate Cox regression analysis showed that high nuclear grade ( HR=3.049, 95% CI 1.292-7.196, P=0.011), high ALP ( HR=1.018, 95% CI 1.007-1.029, P=0.001) and high AST/ALT ratio ( HR=4.094, 95% CI 1.064-15.759, P=0.04) were associated with poor CSS. After excluding AST/ALT ratio, multivariate Cox regression analysis showed that high nuclear grade ( HR=5.836, 95% CI 1.867-18.240, P=0.002) and high AST ( HR=1.040, 95% CI 1.017-1.062, P<0.001) were associated with poor CSS. Conclusions:In patients with non-metastatic renal cell carcinoma with tumor thrombus, high AST/ALT ratio and AST levels indicate poor pathological types and poor prognosis.

Bartter syndrome (BS, OMIM #601678) is a rare inherited salt-losing tubulopathy characterized by hypokalemia metabolic alkalosis with secondary renin-angiotensin-aldosterone system activation. As reported, BS type 1 is generally presented prenatal and neonatal period, and symptoms usually appear before and after birth or in infancy, accompanied by severe salt loss, whilst kidney function remains mostly normal. In this study, we report a case of BS type 1 with childhood onset and proteinuria and renal impairment. The child was born preterm due to hyperamniotic fluid, but there were no apparent symptoms after birth until the age of 3 when the child began to present with polydipsia, polyuria and increased nocturnal uria. At the age of 5, she had elevated serum creatinine level and proteinuria. After admission, she was diagnosed with chronic tubulointerstitial disease and stage 2 chronic kidney disease(CKD). According to the chloride clearance test, the abnormal function of medullary thick ascending limb Henle′s loop, was confirmed and BS type 1 was diagnosed by gene sequencing. After active management of complications, kidney function of the child improved. In the long-term follow-up, the urinary protein amount of the child still increased, eGFR slowly decreased, and the child was currently in the CKD2 stage. Children with prenatal BS may not present typical clinical manifestations immediately after birth until the onset of relevant clinical symptoms in childhood. BS type 1 patients may have renal impairment, which needs to be identified in time. Clinical differentiation diagnosis between BS and Gitelman syndrome can be made by chloride clearance tests. Early diagnosis and treatment are critical to improve prognosis.

Squamosa promoter binding protein-like (SPL) family is a group of important transcription factors involved in the regulation of plant growth and development and the response to environmental stress, but there are few studies in perennial fruit trees such as citrus. In this study, Ziyang Xiangcheng (Citrus junos Sib.ex Tanaka), an important rootstock of Citrus, was used as the material for analysis. Based on plantTFDB transcription factor database and sweet orange genome database, 15 SPL family members were genome-widely identified and cloned from Ziyang Xiangcheng, and named CjSPL1-CjSPL15. Sequence analysis showed that the open reading frame (ORF) length of CjSPLs ranged from 393 bp to 2 865 bp, encoding 130-954 amino acids. Phylogenetic tree divided 15 CjSPLs into 9 subfamilies. Gene structure and conserved domain analysis predicted 20 different conserved motifs and SBP basic domains. Analysis of cis-acting promoter elements predicted 20 different promoter elements, including those related to plant growth and development, abiotic stress and secondary metabolites. The expression patterns of CjSPLs under drought, salt and low temperature stresses were analyzed by real-time fluorescence quantitative PCR (qRT-PCR), and many CjSPLs were significantly up-regulated after stress treatment. This study provides a reference for further study on the function of SPL family transcription factors in citrus and other fruit trees.
Phylogeny ; Transcription Factors/metabolism* ; Gene Expression Regulation, Plant ; Plant Proteins/metabolism* ; Multigene Family ; Stress, Physiological