Objective:To investigate the effect of different surgical timing on the surgical treatment of renal angiomyolipoma(RAML)with rupture and hemorrhage.Methods:The demographic data and peri-operative data of 31 patients with rupture and hemorrhage of RAML admitted to our medical center from June 2013 to February 2023 were collected.The surgery within 7 days after hemorrhage was defined as a short-term surgery group,the surgery between 7 days and 6 months after hemorrhage was defined as a me-dium-term surgery group,and the surgery beyond 6 months after hemorrhage was defined as a long-term surgery group.The perioperative related indicators among the three groups were compared.Results:This study collected 31 patients who underwent surgical treatment for RAML rupture and hemorrhage,of whom 13 were males and 18 were females,with an average age of(46.2±11.3)years.The short-term surgery group included 7 patients,the medium-term surgery group included 12 patients and the long-term surgery group included 12 patients.In terms of tumor diameter,the patients in the long-term surgery group were significantly lower than those in the recent surgery group[(6.6±2.4)cmvs.(10.0±3.0)cm,P=0.039].In terms of operation time,the long-term surgery group was significantly shorter than the mid-term surgery group[(157.5±56.8)min vs.(254.8±80.1)min,P=0.006],and there was no sig-nificant difference between other groups.In terms of estimated blood loss during surgery,the long-term surgery group was significantly lower than the mid-term surgery group[35(10,100)mL vs.650(300,1 200)mL,P＜0.001],and there was no significant difference between other groups.In terms of intraoperative blood transfusion,the long-term surgery group was significantly lower than the mid-term surgery group[0(0,0)mL vs.200(0,700)mL,P=0.014],and there was no significant difference between other groups.In terms of postoperative hospitalization days,the long-term surgery group was sig-nificantly lower than the mid-term surgery group[5(4,7)d vs.7(6,10)d,P=0.011],and there was no significant difference between other groups.Conclusion:We believe that for patients with RAML rupture and hemorrhage,reoperation for more than 6 months is a relatively safe time range,with minimal intraoperative bleeding.Therefore,it is more recommended to undergo surgical treatment after the hema-toma is systematized through conservative treatment.

Objective:To investigate the postoperative renal function and oncologic outcomes of cystic renal cell carcinoma with partial nephrectomy,and to compared the single-center data on surgical out-comes with the Surveillance,Epidemiology,and End Results(SEER)database.Methods:This was a retrospective study that included the patients with cystic renal cell carcinoma who underwent partial ne-phrectomy in the Department of Urology,Peking University Third Hospital(PUTH)from 2010 to 2023.The clinical data and depicting baseline characteristics were collected.Renal dynamic imaging and the Chinese Coefficients for Chronic Kidney Disease Epidemiology Collaboration(C-CKD-EPI)formulae were used to calculate the estimated glomerular filtration rate(eGFR).The renal function curves over time were then plotted,and the patients were followed-up to record their survival status.Cases of cystic renal cell carcinoma in the SEER database between 2000 and 2020 were included,propensity score matching(PSM)was performed to balance the differences between SEER cohort and PUTH cohort,and the cancer-specific survival(CSS)curves for both groups were plotted and statistical differences were calcu-lated by the Kaplan-Meier method.Results:A total of 38 and 385 patients were included in the PUTH cohort and SEER cohort,respectively,and 31 and 72 patients were screened in each cohort after PSM.Of the baseline characteristics,only tumor size(P=0.042)was found to differ statistically between the two groups.There was no statistically significant difference between the two cohorts in terms of CSS after PSM(P=0.556).The median follow-up time in the SEER cohort was 112.5(65,152)months and a 10-year survival rate of 97.2％,while the PUTH cohort had a median follow-up of 57.0(20,1 172)months and a 10-year survival rate of 100.0％.There was no statistically significant difference between eGFR determined by preoperative renal dynamic imaging and the results of the C-CKD-EPI formulae based on creatinine estimation(P=0.073).There was a statistically significant difference in eGFR among the preoperative,short-term postoperative,and long-term postoperative(P＜0.001),which was characterized by the presence of a decline in renal function in the short-term postoperative period and the recovery of renal function in the long-term period.Conclusion:Partial nephrectomy for cystic renal cell carcinoma is safe and feasible with favorable renal function and oncologic outcomes.

Objective:To investigate the clinicopathological characteristics and prognosis of pT 3a stage non-clear cell renal cell carcinoma (nccRCC). Methods:The clinical data of 438 patients with pT 3a stage renal cell carcinoma treated by surgery at Peking University Third Hospital from March 2013 to March 2023 were retrospectively analyzed. Among them, there were 58 cases in the nccRCC group and 380 cases in the clear cell RCC (ccRCC) group. There were statistically significant differences in age, American Society of Anesthesiologists (ASA) classification, and comorbidities between the two groups (all P<0.05). Therefore, propensity score matching was used to adjust the baseline data of the two groups. After matching, there were 58 cases in the nccRCC group and 232 cases in the ccRCC group. There were no statistically significant differences in gender (male/female: 34/24 cases and 165/67 cases), age (53.3±16.8 years and 56.6±11.6 years), ASA classification (1/2/3/4: 19/34/5/0 cases and 60/163/8/1 cases), comorbidities (present/absent: 16/42 cases and 76/156 cases), tumor maximum diameter [6.7 (5.3, 8.4) cm and 5.8 (4.6, 7.8) cm], and nephron sparing surgery(yes/no: 4/54 cases and 15/217 cases) (all P > 0.05). The overall survival (OS) and progression-free survival (PFS) of two groups were compared, the Kaplan-Meier method was employed to plot survival curves. Cox proportional hazards regression model was used to analyze the relationship between different pT 3a characteristics in the nccRCC group and progression-free survival. Results:In the matched cohort, the median follow-up time for the nccRCC group and ccRCC group were 28.0 (16.3, 45.3) months and 31.0 (18.0, 57.0) months, respectively. The pathological types in the nccRCC group included chromophobe renal cell carcinoma (20 cases, 34.5%), papillary renal cell carcinoma (20 cases, 34.5%), Xp11.2 translocation renal cell carcinoma (8 cases, 13.8%), mucinous tubular and spindle cell carcinoma (3 cases, 5.2%), and other or unclassified renal cell carcinoma (7 cases, 12.1%). There was no statistical significance between the nccRCC and ccRCC groups in terms of invasion of the renal vein without involvement of the vein wall (yes/no: 5/53 cases and 41/191 cases), vascular invasion (yes/no: 18/40 cases and 52/180 cases), invasion of the perirenal fat (yes/no: 15/43 cases and 39/193 cases), invasion of the renal pelvis and sinus (yes/no: 51/7 cases and 200/32 cases), or sarcomatoid differentiation (yes/no: 2/56 cases and 4/228 cases)(all P > 0.05). However, there was a statistically significant difference in lymph node involvement (yes/no: 3/229 cases and 9/49 cases, P < 0.01). The 5-year PFS and OS of nccRCC group were 67% (95% CI 52%-86%) and 70% (95% CI 55%-89%) respectively. While the 5-year PFS and OS of ccRCC group were 78% (95% CI 70%-86%) and 87% (95% CI 81%-93%) respectively. There was no statistically significant difference in PFS between the two groups ( P>0.05), but there was a statistically significant difference in OS ( P<0.01). Furthermore, within specific pathological types, the 5-year PFS and OS rates of chromophobe renal cell carcinoma were 88% (95% CI 67%-100%) and 86% (95% CI 63%-100%) respectively, followed by papillary renal cell carcinoma with 5-year PFS of 55% (95% CI 33%-91%) and 5-year OS of 65% (95% CI 44%-97%), and Xp11.2 translocation renal cell carcinoma with 5-year PFS of 38% (95% CI 9%-100%) and 5-year OS of 43% (95% CI 10%-100%). The difference in PFS and OS between ccRCC, chromophobe renal cell carcinoma, papillary renal cell carcinoma, and Xp11.2 translocation renal cell carcinoma was statistically significant ( P<0.01). In addition, the multivariate Cox regression analysis revealed that the independent risk factor for PFS in nccRCC patients is the invasion of the renal vein without venous wall involvement ( HR = 8.0, 95% CI 1.8-36.2, P<0.01). Conculsions:Compared to ccRCC, pT 3a nccRCC is more prone to lymph node metastasis. Among them, papillary renal cell carcinoma and Xp11.2 translocation renal cell carcinoma have a poorer prognosis, resulting in an overall lower survival period for pT 3a nccRCC patients. Among different pT 3a characteristics, invasion of the renal vein without invading the vein wall is an independent risk factor for PFS in nccRCC patients.

Objective:To summarize the surgical technique and clinical experience of robotic-assisted laparoscopic radical nephrectomy (RN) + venous tumor thrombectomy (VTTE) approach for renal tumor with Mayo grade 0-Ⅲ tumor thrombus, and to discuss its safety and efficacy.Methods:A retrospective analysis of the clinical data of 26 patients with renal tumor associated with Mayo 0-Ⅲ thrombus admitted to Peking University Third Hospital from October 2020 to September 2021. There were 17 male cases and 9 female cases. The mean age was (56.9±13.9) years. The mean body mass index (BMI) was (25.8±3.5) kg/m 2. The renal tumors were located on the left side in 12 cases and on the right side in 14 cases, with a mean tumor diameter of (7.8±2.9) cm. The tumors were graded by Mayo: Mayo 0 in 10 cases, Mayo Ⅰ in 3 cases, Mayo Ⅱ in 11 cases and Mayo Ⅲ in 2 cases. The American Society of Anesthesiology (ASA) graded 23 cases as grade 2 and 3 cases as grade 3. All 26 patients were treated by robotic-assisted laparoscopic approach with RN+ VTTE. Mayo 0 tumor thrombus was treated in the same way as radical nephrectomy. For Mayo Ⅰ tumor thrombus, the lateral wall of the IVC at the inferior vena cava (IVC) where the renal vein joins was clamped to partially block the IVC flow and then the thrombus was removed. For Mayo Ⅱ tumor thrombus, after blocking the flow in the IVC with three blocking bands, the wall of the IVC was dissected and the thrombus was removed. For Mayo Ⅲ tumor thrombus: cut the short hepatic vein, free the liver, expose the posterior IVC and follow the same procedure as for Mayo Ⅱ tumor thrombus. Results:All 26 patients in this group were successfully operated on, 1 of which was converted to open surgery. The median operative time was 148.5 (77.0-399.0) min, and the median intraoperative estimated bleeding volume was 300 (10-2000) ml. Postoperative pathological diagnosis: 18 cases of renal clear cell carcinoma, 2 cases of papillary renal cell carcinoma type Ⅱ, 2 cases of TEF gene fusion-related renal carcinoma, 1 case of unclassified renal cell carcinoma, 1 case of uroepithelial carcinoma and 2 cases of AML. In 2 of the 26 cases, segmental resection of the IVC was performed because the right renal VTT had extensively invaded the wall of the IVC. Due to the residual wall thrombus at the head of the tumour thrombus, 1 case underwent inferior vena cava dissection and the inferior vena cava was cut obliquely to preserve the left renal venous return. 6 patients underwent intraoperative lymph node dissection of the hilum, three of which had pathology suggestive of lymph node metastasis. 1 patient underwent adrenalectomy for tumor invasion of the ipsilateral adrenal gland. The median postoperative hospital stay was 7.2(4.0-22.0)d. According to the modified Clavien classification, there were 18 grade Ⅰ and 8 grade Ⅱ postoperative complications. 26 patients were followed up for 1-11 months, with a median follow-up time of 5.5 months. 3 cases developed distant metastases, including 1 case with tumour-specific death due to multiple metastases in the liver and retroperitoneum at 4 months of follow-up.Conclusions:Robotic-assisted laparoscopic RN+ VTTE is a safe and effective procedure for the treatment of renal tumours with Mayo 0 to Ⅲ tumour thrombus, with the advantages of delicate operation, minimal trauma and low incidence of serious postoperative complications.

Introducing the multidisciplinary cooperation model into the clinical teaching of residents has gradually been paid attention to, and the relevant multi-disciplinary teaching teams participate in and formulate teaching plan. The Department of Urology of the Peking University Third Hospital carries out multidisciplinary cooperative teaching of residents based on network platform to improve residents' autonomous learning ability and teaching effect. This model has certain advantages in mobilizing students' subjective initiative and cultivating learning interest. It is of great significance for the training of urology residents.

Objective:To summarize the clinical and laboratory characteristics of infectious mononucleosis (IM) in children.Methods:Clinical features and laboratory results of 270 cases with IM admitted to the Department of Pediatrics in Strategic Support Force Medical Center of People′s Liberation Army from January 2012 to December 2020 were retrospectively analyzed. χ2 test was used for comparison between groups. Results:IM mainly occurred in children aged 5 months to 18 years old in autumn and spring.The highest incidence rate (105 cases, 38.9%) was 3-<6 years old (preschoolers). There were 253 cases (93.7%) with fever, 266 cases (98.5%) with adenopharyngitis, 196 cases (72.6%) with tonsil pseudomembrane or exudation, 248 cases (91.9%) with cervical lymphadenopathy, 92 cases (34.1%) with eyelid edema, 202 cases (74.8%) with nasal obstruction, 124 cases (45.9%) with nasal obstruction and snoring, 24 cases (8.9%) with rash, and 112 cases (41.5%) with splenomegaly.A total of 225 cases (83.3%) presented with typical triplets of IM (fever, adenopharyngitis and cervical lymphadenopathy). Sixty-two IM patients were complicated with pulmonary infections and 3 cases with diarrhea.The main co-infection pathogens in children with IM were Mycoplasma pneumonia (MP) (79 cases, 29.3%), influenza A or B virus (34 cases, 12.6%), Streptococcus pneumonia (SP) (18 cases, 6.7%), adenovirus (22 cases, 8.1%) and cytomegalovirus (3 cases, 1.11%). A total of 46 cases (17.0%) had multiple infections.Laboratory test results suggested that absolute lymphocyte count ≥5.0×10 9/L was found in 199 cases (73.7%), and abnormal lymphocyte ratio >0.10 was found in 225 cases (83.3%). Some children had elevated transaminase levels.Epstein-Barr virus capsid antigen-immunoglobulin M (EBV-VCA-IgM) was positive in 249 cases (92.2%), Epstein-Barr virus capsid antigen-immunoglobulin G (EBV-VCA-IgG) was positive in 238 cases (88.1%), and Epstein-Barr virus nuclear antigen-immunoglobulin G (EBV-NA-IgG) was negative in all cases.EBV-VCA-IgG showed low affinity in all cases (<40%). EBV DNA tests of peripheral blood plasma were carried in 153 cases, of which 118 cases (77.1%) were positive. Conclusions:EBV related IM mainly attacks preschoolers.Most patients are presented with typical triplets of IM.Eyelid edema, nasal obstruction, snoring, splenomegaly and elevated transaminase levels are prevalent in IM children.Most cases have a favorable prognosis.

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.

Objective: To explore the clinical characteristics of renal angiomyolipoma (AML) with inferior vena cava (IVC) tumor thrombus and to improve the diagnosis and treatment of the disease. Methods: The clinical data of 3 patients with renal AML and inferior vena cava tumor thrombus was retrospectively reviewed. The patients were all female, aged 19 to 70 years. Among them, 2 patients presented with lumbago on the right side, and the other one was diagnosed by physical examination. The body mass index ranged from 18.4 to 24.6 kg/m^2, with a median value of 20.4 kg/m². According to the American Society of Anesthesiologists (ASA), they were classified as grade Ⅱ. Color doppler ultrasound examination of the kidney and IVC was performed in all the 3 patients, all of which showed hyperechoic solid mass in the right kidney. Color doppler ultrasound of IVC showed hyperechoic band in the IVC, indicating blood flow signals and the tumor thrombus. All the 3 cases showed irregular fat density or mixed density in the right kidney and multiple irregular fat density were observed in the right renal vein and inferior vena cava on CT. Two of them received MRI examination of IVC, which showed irregular lesions in the right kidney, short T1 and long T2 signals, low lipids, and no definite limited diffusion on DWI. Irregular fat signal were seen in the right renal vein and inferior vena cava. All 3 patients were diagnosed with right renal mass with IVC tumor thrombus, with 1 patient of Mayo grade Ⅲ tumor thrombus and the other 2 of Mayo gradeⅡtumor thrombus. One underwent laparoscopic radical nephrectomy and inferior vena cava tumor thrombectomy, another one underwent open right partial nephrectomy and tumor thrombectomy, and the third one suffered preoperative AML rupture, undergoing open radical nephrectomy and tumor thrombectomy. Results: The operation time was 168 to 659 min, with median of 220 min. Intraoperative blood loss ranged from 50 to 300 ml, with the median of 50 ml. Postoperative indwelling time of drainage tube was 5 to 11 days, with the median of 6 days. Postoperative hospital stay ranged from 7 to 14 days, with a median of 8 days. Postoperative follow-up ranged from 12 to 16 months, with a median follow-up of 13 months. All the three patients underwent operation without postoperative complications. Postoperative pathology proved to be right renal angiomyolipoma. After 3 months of follow-up, the patients showed no tumor recurrence or metastasis. Conclusions Renal AML is a benign lesion, which is rarely concurrent with inferior vena cava cancer thrombus. Enhanced CT examination is the main diagnostic method, surgical resection of the lesion is the preferred treatment, partial nephrectomy combined with thrombectomy can be performed in patients with AML, if permitted, and postoperative prognosis turns out to be propitious.

OBJECTIVETo analyze the hematological and genetic characteristics of unstable hemoglobin Rush (Hb Rush) and compound heterozygote of Hb Rush and thalassemia.

METHODSPeripheral blood samples and genomic DNA from three patients (including two ethnic Dai and one Han Chinese) with anemia of undetermined origin were collected. Hematological phenotypes of these patients were determined through red blood cell analysis and hemoglobin electrophoresis. Genotypes of alpha- and beta-globin genes, -158 XmnⅠ polymorphic site ofγ promoter region, and haplotypes of 7 polymorphic restriction sites in the beta-globin gene cluster were determined using PCR-based methods and DNA sequencing.

RESULTSAll patients have presented hypochromic microcytic anemia and hemoglobin fraction with significant increased measurement (30.5%-59.2%) in the region of fetal hemoglobin during alkaline medium electrophoresis. DNA analysis suggested that all patients have carried mutations leading to the unstable hemoglobin Rush (HBB codon 101, GAG>CAG, Glu>Gln). Two of them were compound heterozygotes of Hb Rush and thalassemia mutations of -α,CD17 and Hb E, respectively. Hb Rush mutation was associated with various haplotypes of the β-globin gene cluster. No significant association was found between increased abnormal hemoglobin fraction in the region of Hb F and the polymorphism ofγ promoter or large deletion of the beta-globin gene cluster.

CONCLUSIONThis study has confirmed the distribution of Hb Rush among various Chinese populations and is the third report of its kind. Hb Rush can result in increased measurement of hemoglobin fraction in the region of fetal hemoglobin (Hb F) during routine hemoglobin electrophoresis under alkaline condition. Hb Rush heterozygote alone can lead to hypochromic microcytic anemia and thalassemia-like phenotype. Prenatal diagnosis of Hb Rush is necessary for carriers.

Adult ; Base Sequence ; Blood Protein Electrophoresis ; methods ; Female ; Fetal Hemoglobin ; genetics ; metabolism ; Genotype ; Haplotypes ; Hemoglobins, Abnormal ; genetics ; metabolism ; Heterozygote ; Humans ; Infant ; Mutation ; Phenotype ; Polymorphism, Genetic ; Sequence Analysis, DNA ; methods ; Thalassemia ; blood ; diagnosis ; genetics ; Young Adult ; alpha-Globins ; genetics ; metabolism ; beta-Globins ; genetics ; metabolism