Objective:To explore the clinical features of Heidenhain variant Creutzfeldt-Jakob disease (HvCJD) to deepen understanding and recognition of this disease.Methods:Clinical data of 1 case of HvCJD admitted to Hainan General Hospital on August 4, 2022, were collected, whose clinical characteristics were reviewed and analyzed, and literature review was conducted.Results:The 59 years old male patient initially experienced symptoms of blurred vision, followed by a rapid decline in cognitive function. Mini-Mental State Examination score was 21/30 and Montreal Cognitive Assessment score was 11/30. Diffusion-weighted magnetic resonance imaging demonstrated the presence of mild ribbon-like hyperintensity within the bilateral frontal-parietal-occipital-insular cortex. T 2WI fluid attenuated inversion recovery imaging exhibited slight hyperintensity within the bilateral parietal cortex. The electroencephalography showed atypical triphasic waves. The examination of cerebrospinal fluid demonstrated 14-3-3 protein with a positive result. Two months after onset of illness, follow-up revealed new symptoms of myoclonus in the patient. Finally, the patient was not effectively treated and died about 2.5 months after onset. Thirty-six relevant literatures of HvCJD were reviewed. Most Heidenhain variant patients exhibited occipital cortical diffusion weighted imaging hyperintensity. Posterior cortical damage may affect primary and higher-level visual processing, leading to various visual disturbances. The early symptoms were mainly visual symptoms, such as visual reduction, blurred vision, visual field defect and color vision impairment. Conclusions:HvCJD patients only present with various visual disorders at the onset, followed by other neurological symptoms. The disease progresses rapidly, and patients often die in a short period of time. The disease is very rare and is often misdiagnosed. Currently, there is no feasible and effective treatment for HvCJD.

Objective:To analyze the long-term prognosis of IgA nephropathy (IgAN) with focal segmental glomerulosclerosis (FSGS) and the risk factors related to renal prognosis in children with IgAN-FSGS.Methods:A retrospective study was concluded in IgAN-FSGS children who were followed up for more than 5 years and diagnosed by renal biopsy for the first time in the Eastern Theater General Hospital from January, 2004 to December, 2018. The end-point events of the study were entering end-stage kidney disease (ESKD) or estimated glomerular filtration rate (eGFR) decreased by ≥50% from baseline, which were defined as poor renal prognosis. Baseline clinicopathologic data of IgAN-FSGS children were compared between the end-point event group and the non-end-point event group. The cumulative renal survival rate of IgAN-FSGS children was calculated by Kaplan-Meier survival analysis. The influencing factors of poor renal prognosis in IgAN-FSGS children were analyzed by Cox proportional hazards model, and the diagnostic value was evaluated by the receiver operating characteristic curve (ROC curve) and area under the curve (AUC). The diagnostic value was verified by time dependent-ROC and time dependent-AUC.Results:A total of 204 IgAN-FSGS children were enrolled in this study, of whom 132 cases were males (64.7%). The median age of renal biopsy was 16 (14, 17) years old. During a median follow-up time of 90.7 (71.7, 114.8) months, 57 cases (27.9%) reached the end-point events. Compared with the non-end-point event group ( n=147), the end-point event group ( n=57) had higher proportions of males and hypertension, higher levels of 24-hour urinary protein, serum creatinine, serum uric acid, urinary N-acetyl-β- D-glucosaminidase, urinary retinol binding protein, higher proportions of glomerular segmental sclerosis (S1) ≥25% and tubular atrophy/interstitial fibrosis (T1/T2), and lower levels of serum albumin, serum IgA, and serum IgG (all P<0.05). There was no statistical difference between the two groups in treatment (all P>0.05). Kaplan-Meier survival analysis showed that with entry of ESKD or eGFR decreased by ≥50% from baseline as the end-point events, the 5-year, 10-year, and 15-year cumulative renal survival rates in IgAN-FSGS children were 88.7%, 67.6%, and 50.7%, respectively. Multivariate Cox regression analysis showed that proteinuria >1 g/24 h ( HR=3.702, 95% CI 1.657-8.272, P=0.001), hyperuricemia ( HR=3.066, 95% CI 1.793-5.245, P<0.001), S1≥25% ( HR=2.017, 95% CI 1.050-3.874, P=0.035), T1/T2 ( HR=1.863, 95% CI 1.021-3.158, P=0.016) were the independent related factors for poor renal prognosis. ROC curve analysis showed that S1≥25% ( AUC=0.605, P=0.021, sensitivity 26.3%, specificity 94.6%), T1/T2 ( AUC=0.624, P=0.006, sensitivity 43.9%, specificity 81.0%), hyperuricemia ( AUC=0.658, P<0.001, sensitivity 52.6%, specificity 78.9%), proteinuria>1 g/24 h ( AUC=0.670, P<0.001, sensitivity 87.7%, specificity 46.3%) could accurately predict the renal outcome of IgAN-FSGS. Time dependent-ROC curve validation showed that the combined diagnosis of S1≥25%, T1/T2, hyperuricemia and proteinuria>1 g/24 h had a good predictive value for renal prognosis (3-year AUC=0.846 and 5-year AUC=0.777, respectively). Conclusions:During a median follow-up of 90.7 months, 27.9% of IgAN-FSGS children have poor renal prognosis, and the 5-year, 10-year, and 15-year cumulative renal survival rates are 88.7%, 67.6%, and 50.7%, respectively. Urinary protein >1 g/24 h, hyperuricemia, T1/T2, and S1 ≥25% are the risk factors for renal prognosis in IgAN-FSGS children.

【Objective】 To investigate the effect of sinomenine hydrochloride （SH） on angiogenesis and the underlying mechanism in breast cancer. 【Methods】 The 4T1 orthotopic tumor model of breast cancer was utilized， and mice were treated with different dosage of SH to investigate the effect of SH on tumor growth. IHC staining of CD31 was used to evaluate angiogenesis within tumors under different treatment. ELISA was performed to measure the bFGF level within tumor extracellular fluid （TEF） and tumor cells to analyze the effect of SH on bFGF secretion and production. RT-qPCR was utilized to evaluate the effect of SH on the mRNA expression of bFGF in tumor cells. Kaplan-Meier Plotter was analyzed online to investigate the relationship between bFGF expression and the survival of patients with breast cancer. 【Results】 SH at 100 mg/kg could inhibit 4T1 orthotopic tumor growth compared with saline （P<0.05）， but SH showed little cytotoxicity in vitro under the tested concentrations. SH at 100 mg/kg suppressed the vessel area compared with saline （P<0.01）， and the concentration of angiogenic factor bFGF in TEF and tumor cells was decreased by SH treatment （P<0.05）， while the mRNA expression of bFGF in tumor cells was also downregulated by SH treatment （P<0.05）. Kaplan-Meier Plotter online analysis showed that elevated mRNA expression of bFGF in the primary tumor was associated with poorer OS， RFS and DMFS in patients with breast cancer （P<0.01）. 【Conclusion】 SH inhibits breast cancer growth by suppressing bFGF-induced angiogenesis， which enriches the pharmacological effects of this traditional Chinese medicine.

Objective To compare H_p(3) calibration with a homemade (A) thermoluminescent dosimeter (TLD) and an imported (B) TLD in a standard X-ray RQR radiation field, to explore the different responses of A and B, and to provide foundation for the calibration of H_p(3). Methods A column mode was selected. H_p(3) calibration was performed using A and B in a standard X-ray RQR radiation field in the Secondary Standard Dosimetry Laboratory, National Institute for Radiological Protection, China Center for Disease Control and Prevention. Angle response, energy response, and linear response were calibrated with RQR4 (60 kV), RQR7 (90 kV), and RQR9 (120 kV), respectively. Results In terms of angle response, the calibration results of A were relatively high, while the calibration results of B were relatively low. In terms of energy response, the calibration results showed a similar pattern to angle response. In terms of linear response, the calibration results of both A and B were satisfactory. Conclusion Both A and B can be used for normal calibration of H_p(3) in a standard X-ray RQR radiation field. However, in actual monitoring, attention should be paid to the energy and angle response values of TLDs.