Objective To study the diagnosis and treatment of spontaneous perirenal hemorrhage(SPH). Methods The clinical data of 31 cases of SPH were reviewed retrospectively.In the 31 cases,flank pain,flank mass,shock,gross hematuria,abdominal bleeding occured in 31,10,8,3 and 3 cases,respectively.The final diagnosis was established on CT scan,B-ultrasonography and IVU were 18,22 and 7 cases. Results In 31 cases,nephrectomy were performed in 18,radical nephrectomy in 1 case,nephron spare surgery in 4,renal artery embolization in 2,inspiration and drainage of hemorrage in 1 and careful watching in 5 cases.There were 11 cases of angiomyolipoma,6 renal cyst,4 of renal cell carcinoma,3 infectious diseases,2 aneurysm of renal artery and 2 hydronephrosis. Conclusions The clinical presentation may vary greatly depending on the degree and duration of bleeding,and the severe pain in the upper abdomen of abrupt onset is the common sign and symptom.The spontaneous rupture of renal cell carcinoma and angiomyolipoma are the most common cause of SPH.CT scan and B-ultrasonography are the most valuable in the diagnosis of SPH.It is demanded that management of SPH depends on the histopathology and the degree of bleeding.

Objective To measure the expression of activin receptor-like kinases 1(ALK1)in dermal fibroblasts from patients with systemic scleroderma(SSc)and to estimate its role in the production of fibronectin and plasminogen activator inhibitor-1(PAI-1).Methods Dermal fibroblasts were isolated from the lesions of 12 patients with SSc as well as the normal skin of 14 healthy controls,and subjected to a primary culture.The third-passage fibroblasts were used in the next experiment.Western blot and indirect immunofluorescence technique were utilized to quantify the expression of ALK1.A specific siRNA targeting ALK1 was designed,constructed,and transiently transfected into the control dermal fibroblasts,which were then classified into 2 groups to be cultured with or without the presence of transforming growth factor(TGF)-β1 for 72 hours followed by the detection of fibronectin and PAI-1 expression with Western blot.Results As Western blot and direct immunofluorescence technique showed,both control and SSc fibroblasts showed an expression of ALK1 in the cytoplasm and membrane,and the expression intensity of ALK1 in SSc fibroblasts was significantly higher than that in the control fibroblasts(1.97 ± 0.05 vs.1.12 ± 0.03,t =50.96,P ＜ 0.05).The expression of ALK1,fibronectin and PAI-1 was decreased by 90％,58％ and 31％ respectively in specific siRNA-transfected SSc fibroblasts compared with the control siRNA-transfected fibroblasts.TGFβ1 significantly increased the expression of ALK1,fibronectin and PAI-1 in the control siRNA-transfected fibroblasts,but the increase was markedly inhibited by the siRNA-targeting ALK1.Conlusion TGFβ1 can promote the production of fibronectin and PAI-1 via ALK1 in fibroblasts,and ALK1 may be involved in the development of sclerosis in SSc.

PAK1 plays an important role in the development of tumors. It is of great significance to screen and develop new PAK1 inhibitors as targeted drugs for cancer treatment. The traditional PAK1 inhibitor screening method has the problems of high cost and low efficiency. Computer virtual screening can reduce the cost of finding active lead compounds and improve the screening efficiency. In this study, a kind of PAK1 candidate compound was screened by computer assisted virtual screening combined with Z′lyteTM high flux kinase screen. In vitro enzyme activity screening showed that compound 18(K788)had good PAK1 inhibitory activity(inhibition rate was 42. 7%). Furtherly by MTT detection, it was found that K788 had significant PAK1 positive tumor killing activity, which was even better than the positive drug IPA-3. Flow cytometry and Western Blot showed that K788 could activate caspase apoptosis pathway and induce apoptosis of colon cancer cell DLD-1 by inhibiting PAK1 expression and activation. K788 has great potential for clinical development and application, and can be used as a PAK1 target for further research.

Objective To determine the relationship between serum soluble Klotho (sKL) level and adverse outcome in maintenance hemodialysis (MHD) patients.Methods One hundred and twenty nine cases of MHD patients were collected prospectively.Serum sKL was detected by ELISA.Abdomen lateral plain was used as a criterion to determine the abdominal aortic calcification.The abdominal aortic calcification score (AAC) was calculated.Cox regression analysis was used to determine the risk factor of cardiovascular death (CVD) in MHD patients.Kaplan-Meier showed the relationship between sKL and CVD in MHD patients.Results There were 27 cases (20.9％) of allcause death and 19 cases (14.7％) of cardiovascular death.The median sKL was 612.6(379.2-816.6) nig/L,and log[iPTH] was an independent factor of sKL concentration.Low sKL had high AAC and CVD death rate.Kaplan-Meier method showed that the all-cause death rate was similar between two groups,and CVD death rate increased significantly in low sKL patients (P=0.036).Cox regression indicated that lower sKL level was associated with high CVD death rate [OR=0.352,95％CI(0.127-0.977),P=0.045].After adjustment for the general condition,biochemical indicators,the relationship still existed [OR=0.331,95％ CI (0.117-0.933),P=0.037].In no or mild vascular calcification patients (AAC ≤4),compared with high sKL patients,low sKL patients had no significant difference rate in all-cause mortality.The CVD mortality was significantly higher in high sKL (P=0.035) compared with low sKL.In severe calcification group (AAC ＞ 4),all-cause death and CVD death rates were similar between different sKL groups (P=0.991 and 0.522,respectively).Conclusions Lower sKL has the high CVD death rate and sKL level decreasing is an independent risk factor for CVD death in MHD patients.The lower sKL concentration in MHD patients with no or mild calcification may predict CVD mortality.This study suggests that sKL levels may be helpful in predicting the outcome of patients with MHD.

Objective To evaluate the impact of hepatobiliary surgery in a tertiary public hospital on its core medical operation indicators after the implementation of subspecialty reform.Methods The Department of Hepatobiliary Sur-gery,which implemented the subspecialty reform,was the experimental group,and six surgical departments with-out subspecialties were the control group.Seven indicators in the dimensions of medical quality,medical efficiency,and rational use of medication were collected from September to December 2022,and the difference between the experimental group and the control group was analyzed by the differences-in-differences method using the Septem-ber data before the implementation of sub-specialty reform as the baseline value.Results After the subspecialty re-form,the changes of the indicators of the percentage of discharged patients for surgery,intensity of antimicrobial drug use and average hospital stay in hepatobiliary surgery were better than those of the control group;the percentage of discharged patients for minimally invasive surgery and the percentage of medical service revenue were inferior to those of the control group;the intergroup differences of the two indicators of the percentage of discharged patients for level IV surgery and the case-mix index were not significant.Conclusion By setting up similar control departments in the same period and excluding the influence of external confounding factors on the study results,overall,the core medical operation indexes of hepatobiliary surgery were slightly better than those of the control group,but the advantage was not obvious,which might be related to the short observation time after the subspecialty reform,and its long-term effects need to be continuously followed up and analyzed.

OBJECTIVETo investigate the impact of pre-operative uric acid on acute kidney injury (AKI) after cardiac surgery in elderly patients.

METHODSClinical data were collected from 936 elderly patients (age ≥ 60 years) undergoing cardiac surgery with cardiopulmonary bypass in Guangdong General Hospital between January 2005 and May 2011. The baseline serum creatinine was defined as the latest serum creatinine before surgery, and AKI was diagnosed according to RIFLE criteria. Patients were divided into three groups according to the sex-specific cutoff values of serum uric acid tertiles (group A: ≤ 384.65 µmol/L in men, and ≤ 354.00 µmol/L in women; group B:384.66-476.99 µmol/L in men and 354.01-437.96 µmol/L in women; group C: ≥ 477.00 µmol/L in men and ≥ 437.97 µmol/L in women). Multivariate logistic regression analysis was used to analyze the independent risk factors for AKI.

RESULTSAmong 936 elderly patients, 576 cases (61.5%) developed AKI. Mean uric acid concentration was higher in AKI patients than in Non-AKI patients ( (436.6 ± 119.1) µmol/L vs. (398.0 ± 107.2) µmol/L, P < 0.001). The incidence of AKI was 56.1% (175/312) in group A, 56.3% (175/311) in group B, 72.2% (226/313) in group C (P < 0.001). Multiple logistic regression analysis showed that, after adjusted for age, gender, co-morbidities(hypertension, diabetes mellitus, cerebrovascular disease, chronic obstructive pulmonary disease), previous cardiac surgery, eGFR<60 ml×min(-1) ×1.73 m(-2), heart function ≥ 3 (NYHA), positive urine protein, combination of coronary artery bypass grafting and valvular surgery, cardiopulmonary bypass operation time, aortic cross-clamping time, pre-operative angiotensin converting enzyme inhibitor or angiotensin II receptor blockers and lipid-lowering drugs use, early postoperative angiotensin converting enzyme inhibitor or angiotensin II receptor blockers, diuretics and digoxin use, post-operation central venous pressure, risk of post operative AKI was significantly higher in group C than in group A (OR:1.897, 95%CI: 1.270-2.833, P = 0.002).

CONCLUSIONPre-operative elevated uric acid is an independent risk factor of AKI after cardiac surgery in elderly patients.

Acute Kidney Injury ; etiology ; Aged ; Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors ; Cardiac Surgical Procedures ; adverse effects ; Cardiopulmonary Bypass ; Coronary Artery Bypass ; Female ; Humans ; Incidence ; Kidney Function Tests ; Male ; Middle Aged ; Predictive Value of Tests ; Risk Factors ; Uric Acid ; blood

Objective To evaluate the reliability, validity and feasibility of the Chinese version of SF-36 health survey questionaire in long-term survivors of nasopharyngeal carcinoma (NPC). Methods A total of 85 long-term NPC survivors completed the Chinese version of SF-36 by either telephone or mail survey. Correlation analysis, reliability analysis and factor analysis were performed to evaluate the reliability and validity of the scale. Results The Chinese version of SF-36 was easy to complete. The split-half reliability was 0. 92 and the Cronbach's α coefficient among domains were all above 0. 70, which showed good reliability and discrimination capacity among domains. All the correlation coefficients between each item and its domain achieved or approached 0. 5, which were greater than those between the item and other domains. These results demonstrated that the Chinese version of SF-36 had good content validity and discriminatory validity. Six principal components were extracted from the scale, which could basically represent eight domains. The cumulative variance was 71.4%. Two common factors were extracted from the eight domains, which accounted for 73.3% of the variance. The Chinese version of SF-36 was able to detect differences in the quality of life between NPC patients and healthy populations. Conclusions The Chinese version of SF-36 has good feasibility, reliability and validity in evaluating the quality of life in long-term NPC survivors.

Objective To analyze the effect of quality of life (QOL) on the prognosis of long-term survivors with nasopharyngeal carcinoma (NPC). Methods A total of 192 NPC patients treated between 1999 and 2000 and with tumor-free survival before July 2003 were enrolled in this study. All patients received QOL measurement between July and August in 2003. Measurement scales included Chinese SF-36 questionnaire and a checklist consisting of fourteen items about self-rating symptoms. The median follow-up time was 7.9S years (range 2.67-9.55 years). The effects of QOL, social demographic and clinical factors on prognosis were analyzed. Results Univariate analysis showed that QOL affected the tumor-free survival and overall survival. Multivariate analysis showed that hypomnesia was an independent prognostic factor of tumor-free survival, while trismus, headache and age were independent prognostic factors of overall survival. The younger and the better QOL had better prognosis. The other sociodemographic and clinical factors had no prognostic value. Conclusion QOL is an important factor affecting the prognosis of long-term survivors with NPC, and it should be routinely evaluated during the follow-up.

OBJECTIVETo summarize the clinical features, biochemical change and genetic mutations of a neonate with congenital bile acid synthesis disorder type 2.

METHODSClinical features, blood biochemical index, gene analysis and treatment of the patient were reviewed.

RESULTSThe patient presented with the symptoms of jaundice 3 days after birth but without skin itching. Pale stool was noted. Subsequently, he presented with hepatomegaly, blood coagulation disorders, left cochlear nerve damage, liver cirrhosis and remarkable growth retardation. Serum biochemistries showed that bilirubin and transaminase were elevated, while γ -GT and total bile acid was normal. Abdominal ultrasonography indicated decline of gallbladder contraction. Cholangiography showed normal extra- and intrahepatic bile ducts and patent biliary tract. Liver biopsy showed intrahepatic cholestasis. Gene testing has identified a homozygous mutation in AKR1D1 gene.

CONCLUSIONCongenital bile acid synthesis disorder should be suspected when a neonate has presented with jaundice, elevated bilirubin and transaminase, normal or reduced TBA and γ -GT. Genetic testing and urine mass spectrometry analysis can diagnose congenital bile acid synthesis disorder. Early therapy is crucial to patients with congenital bile acid synthesis disorder.

Objective To validate the effect of Renji acute kidney injury score (RAKIS) on predicting patients with acute kidney injury (AKI) after cardiac surgeries,and make comparison with Cleveland score,simplified renal index (SRI) and acute kidney injury following cardiac surgery (AKICS).Methods Patients undergoing open heart surgery from 2008/01/01 to 2010/10/31 in Renji hospital were enrolled,and their scores of those four scoring models were calculated.AKI patients were diagnosed by KDIGO,and those scores of AKI patients and non-AKI patients were compared.Receiver operating characteristic (ROC) curve and area under curve (AUC) were used to decide the predictive values of those models.Results A total of 1126 patients were chosen in this cohort,with the average age of (58.43±14.88) years (rang from 18 to 88).The male to female ratio was 1.47:1.And 355(31.5％) patients were developed AKI.AKI stage Ⅰ,Ⅱ and Ⅲ were 65.4％,23.7％ and 11.0％ respectively.RAKIS was significantly higher in AKI patients than in non-AKI patients (17.5 vs 9.0,P ＜ 0.001).The AUCs of RAKIS to predict AKI,AKI Ⅱ-Ⅲ stages,renal replacement therapy (RRT)and in-hospital death were 0.818,0.819,0.800 and 0.784 respectively.The AUCs of Cleveland score and SRI were 0.659 to 0.710,lower than those of RAKIS and AKICS.AKICS had lower value for predicting AKI and AKI Ⅱ-Ⅲ stages (AUC 0.766 and 0.793),but good value in predicting RRT and inhospital death after surgery (AUC 0.804 and 0.835) as compared with RAKIS.Conclusions RAKIS is valid and accurate in the discrimination of KDIGO defined AKI patients,while for predicting the composite end point,AKICS may be more useful.