Background Bietti crystalline dystrophy (BCD) is a congenital and autosomal recessive hereditary eye disease characterized by multiple glistening intraretinal crystals scattered over the fundus.Studies determined abnormality of fatty acid metabolism probably is associated with BCD.However,the study on the alteration of blood lipid level in BCD patients is rare.Objective This trail was to study the change of serum lipids in BCD patients.Methods A total of 50 patients with bilateral BCD and 50 matched healthy volunteers were included from November 2011 to March 2013 in Beijing Tongren Eye Center with the approval of Ethic Committee of Beijing Tongren Hospital.Written informed consent was obtained from each subject before any medial examination.Peripheral blood of 3 ml was collected from the subjects.The serum concentrations of triglyceride (TG),total cholesterol (TC),low density lipoprotein cholesterol (LDL-C) and high density lipoprotein cholesterol (HDL-C) were measured and analyzed.The examination outcome was identified based on the criteria of China Adult Dyslipidemia Prevention Guideline (Version 2007).Results Abnormality of serum lipid content was detected in 58.00％ patients (29/50),and hypertriglyceridemia and hypercholesteremia were in 34.48％ (10/29),respectively,and mixed hyperlipidaemia was in 27.59 ％ (8/29).The serum levels of TG,TC and LDL-C were (1.63± 1.19) mmol/L,(5.10±1.05) mmol/L and (3.27±0.97) mmol/L in the BCD group,which were significantly higher than (0.93± 0.33) mmol/L,(4.33 ±0.56) mmol/L,(2.63 ±0.51) mmol/L of the normal group (t =4.036,4.496,4.095,all at P=0.000).Conclusions The serum lipid levels elevate in BCD patients,which might be related to the occurrence of BCD.

BACKGROUND: Documents recorded that the correlation between micro emulsion Ciclosporin peak concentration (C_2) and area under curve was best with maximum individual difference. According to C_2, dose of Ciclosporin can be adjusted indMdually to decrease acute rejection and Ciclosporin toxicity, which has widely used in perioperative stage of renal transplanted recipients. However, some transplantation center still used tough concentration (C_0) to adjust the dose of Ciclosporin in stable stage of renal transplanted recipients. OBJECTIVE: To analyze the efficacy and safety of changing from monitoring C_0 to C_2 in stable stage recipients following renal transplantation. METHODS: Totally 65 patients with renal transplantation were enrolled in this study, including 31 males and 34 females, aged 20-57 (39.4±15.3) years. Within 3 months prior to this study, all patients did not suffered from rejection, and their serum creatinine and urea nitrogen were stable (creatinine ≤180 μmol/L). They were in stable stage after renal transplantation. Their period of transplantation and function of allograft were recorded. Their C_0 and C_2 of Ciclosporin were assayed. According to the target C_2 value 500-600 μg/L, the patients were prospectively and randomly divided into 3 groups. In the high C_2 group (n=17), the dose of Ciclosporin was decreased. In the target C_2 group (n=23), the dose of Ciclosporin was remained. In the low C_2 group (n=25), the dose of Ciclosporin was increased. All of the patients were followed-up for 12 months. The grafts function and the complications of heart, lung and brain were compared. RESULTS AND CONCLUSION: According to the target concentration of Ciclosporin C_2, the dose of Ciclosporin in the high C_2 group was decreased by 575.0 mg. The Creatinine and urea nitrogen of 88% patients were stable, while blood pressure, blood fat and blood uric acid decreased in parts of patients. In the target C_2 group, the levels of creatinine, urea nitrogen, Co and C_2 of patients were stable, no complications of heart, lung and brain occurred. According to the target concentration of Ciclosporin C_2, the dose of Ciclosporin in low C_2 group was increased by 755.0 mg. The creatinine and urea nitrogen of 84% patients were stable. All of the patients were no complications of heart, lung and brain. It is safe and effective to adjust Ciclospori dose under C_2 monitoring according to the target peak concentration (500-600 μg/L) in most stable stage recipients following renal transplantation.

Objective To investigate the clinical features of bilateral native pelvic and ureteral transitional cell carcinoma (TCC) in renal transplant patients. Methods A retrospective analysis was carried out on 16 patients with bilateral native pelvic and ureteral TCC after kidney transplantation.The mean time between transplantation and diagnosis of upper urinary TCC was 56. 2 ± 33. 0 months.Two patients were suffered from bilateral upper urinary TCC at the same time. The mean interval between 2 upper urinary tract operations of the remaining 14 cases was 8. 6 ± 6. 7 months. Hematuria and hydronephrosis of native kidneys were the main symptoms and targets in checkup. Intravesical chemotherapy was postoperatively given. Results All operations were performed successfully. All specimens obtained from the operations were pathologically diagnosed as TCC. The TCC location involved pure native pelvis (n = 4), pure native ureter (n = 9), and pelvis combined with ureter (n = 19). Pelvic TCC pathological grades included grade 1 in 8 cases, grade 2 in 11 cases, and grade 3 in 4 cases; Ureteral TCC grades included grade 1 in 6 cases, grade 2 in 10 cases, and grade 3 in 12 cases.Patients were followed up for 26. 8 ± 25. 1 months. One patient died of lung metastasis. (One case of lumbar soft tissue transfer was given local excision. The remaining patients had no recurrence and metastasis. Conclusion Renal transplant patients with hematuria and native renal hydronephrosis should be highly vigilant of the occurrence of upper urinary tract TCC. TCC after renal transplantation is invasive. Prophylactic contralateral nephroureterectomy should be performed on the recipients having TCC at the bladder and one side of native upper urinary tract.

Objective To explore the expression of anti-MICA antibodies and evaluate its influence on acute rejection and renal function in early period after renal transplantation. Methods A total of 29 sensitized subjects (PRA＞20 %) were enrolled in this study. All the patients underwent protein A immunoabsorption treatment and the expression of anti-MICA antibodies was detected before and after treatment. Triple immunosuppressive regimen consisting of tacrolimus, mycophenolate mofetil (MMF) and steroid was given to prevent graft rejection. The correlation between the expression of anti-MICA antibodies and acute rejection or serum creatinine (SCr) level was analyzed.Results The expression of anti-MICA antibodies was detected in 8 candidates (27. 6 % ,8/29) ,and 6 kinds of anti-MICA antibodies simultaneously expressed were found in one individual, 3 kinds in one case,and sole kind in 6 patients. There was no significant difference in acute rejection rate between positive anti-MICA antibodies group and negative group [37.5 % (3/8) vs 38. 1% (8/21), P＞0.05). The positive expression rate of anti-MICA antibodies in the recipients with PRA ≥40% was higher than that in those with PRA ＜40% [43. 8 % (7/16) vs 7. 7 % (1/13),P＜0.05]. The SCr level in patients positive for anti-MICA antibodies was markedly higher than that in those negative anti-MICA antibodies at the 1st week postoperatively ( 135.4 ± 21.4 vs 108. 6 -+ 31.6 μmol/L, P＜0.05). The SCr level in the patients with positive anti-MICA antibodies, however, was reduced to the normal range at the 2nd week after surgery (P＞0.05). The levels of anti-MICA antibodies were continuously decreased in the candidates undergoing protein A irnmunoadsorption treatment. Conclusion Higher expression of anti-MICA antibodies exists in sensitized recipients and possesses an influence on the recovery of renal function in early postoperative period. Protein A immunoadsorption can eliminate anti-MICA antibodies effectively in sensitized recipients.

Objective To summarize the experience of the clinical research network operation mechanism in typical countries,and provide reference for the construction of China's national clinical research network.Methods Literature research method and the inductive analysis method were used to analyze the information on official website and literature materials of the clinical research network in typical countries to summarize their characteristics and experience.Results The clinical research network in typical countries had developed an optimized and efficient operational mechanism,including the development of information platforms,development of tools and templates,streamlined review processes,implementation of membership systems,establishment of sharing mechanisms,and complementary models of clinical centers and research institutes,stable sources of funds,establishing reward and punishment mechanisms,etc.Conclusions China's national clinical research network is still at the initial stage,government should make full use of the information platform,strengthen human resource input,and accelerate the research and exploration of more innovative management mechanisms.

Objective:To investigate the factors associated with delay in anticoagulant therapy in patients with cerebral venous sinus thrombosis (CVST) and its effect on outcome.Methods:Patients with CVST admitted to Changhai Hospital, Naval Medical University from January 2010 to August 2021 were retrospectively enrolled. Patients were divided into early anticoagulation group and late anticoagulation group by the median time interval from first symptom to initiation of anticoagulation. The modified Rankin Scale was used for outcome assessment at 90 d after onset. 0-2 scores were defined as good outcome and 3-6 were defined as poor outcome. Demographic and clinical data were compared for the early versus late anticoagulation group and for the good versus poor outcome groups. Multivariable logistic regression was used to identify independent influencing factors of delay in anticoagulation and the correlation of delay in anticoagulation with poor outcome. Results:A total of 131 patients were included, their age was 40.07±15.11 years old, and 68 (51.91%) were male. Of these, 65 patients (49.62%) were in the early anticoagulation group and 14 (10.69%) were in the poor outcome group. Compared with the late anticoagulation group, the early anticoagulation group had a significantly higher proportion of patients with seizures and brain parenchymal damage as well as higher D-dimer levels on admission, while the proportion of patients with visual impairment/papilloedema was significantly lower (all P<0.05). Compared with the good outcome group, the poor outcome group had significantly higher proportions of patients with seizures, dyskinesia, impaired consciousness, low Glasgow Coma Scale score, and brain parenchymal damage as well as higher D-dimer, total cholesterol and low density lipoprotein cholesterol levels, sites of thrombus involvement were more common in the superior sagittal and straight sinuses, and significantly lower proportions of patients with headache and lower albumin levels on admission (all P<0.05). Multivariate logistic regression analysis showed that visual impairment/papilloedema (odds ratio [ OR] 0.119, 95% confidence interval [ CI] 0.030-0.473; P=0.002) and brain parenchymal damage ( OR 1.341, 95% CI 1.042-1.727; P=0.023) were independently associated with a delay in anticoagulation treatment, and a delay in anticoagulation treatment ( OR 6.102, 95% CI 1.185-30.504; P=0.030) and D-dimer level on admission ( OR 1.299, 95% CI 1.141-1.480; P<0.001) were the independent predictors of poor outcome in patients with CVST. Conclusions:Visual impairment/papilloedema and absence of brain parenchymal damage on cranial imaging are the independent risk factors for delay in anticoagulation in patients with CVST. The delay in anticoagulation is strongly associated with the poor outcome in patients with CVST.

Objective:To evaluate the effect of stroke volume variation (SVV)-guided fluid therapy on perioperative haemodynamics and tissue perfusion in the patients with end-stage renal disease (ESRD) undergoing parathyroidectomy.Methods:One hundred and twenty-one patients of either sex, aged 18-64 yr, of American Society of Anesthesiologists Physical Status classification Ⅲ, with body mass index of 18-28 kg/m 2, with ESRD undergoing elective parathyroidectomy, who received haemodialysis treatment within 24 h before surgery, were enrolled in this study. The patients were divided into standard restrictive fluid therapy group (group SRT, n=61) and goal-directed fluid therapy group (group GDT, n=60) using a random number table method. Group SRT received restrictive fluid therapy, with a continuous infusion of 0.9% normal saline at a rate of 4 ml·kg -1·h -1. Group GDT received goal-directed fluid therapy guided by SVV, and when the SVV≥10% lasted for 5 min, the 0.9% normal saline 3 ml/kg was infused within 5 min until SVV<10%. Systolic blood pressure (SBP) was maintained at ≥90 mmHg or mean arterial pressure(MAP) at ≥65 mmHg throughout the perioperative period in both groups. The intraoperative volume of fluid infused, usage rate and consumption of intraoperative vasoactive drugs were recorded, and arterial blood lactate (Lac) level, MAP, heart rate, cardiac output, and inferior vena cava collapse index (IVC-CI) after removal of endotracheal tube at the end of surgery were measured. MAP was continuously recorded within 12 h after surgery, and MAP variability (CV MAP) was calculated. The occurrence of cardiovascular and cerebrovascular events within 30 days after operation was also recorded. Results:Compared with group SRT, the intraoperative volume of fluid infused was significantly increased, the usage rate of ephedrine and norepinephrine was decreased, the consumption of ephedrine was reduced, and the percentage of postoperative IVC-CI<50% and cardiac output were increased, the percentage of Lac≥2.0 mmol/L and CV MAP were decreased ( P<0.05), and no significant change was found in the incidence of cardiovascular and cerebrovascular events within 30 days after surgery in group GDT ( P>0.05). Conclusions:Compared with restrictive fluid therapy, SVV-guided fluid therapy can optimize the perioperative hemodynamics and tissue perfusion in the patients with ESRD undergoing parathyroidectomy.