Objective:To investigate the clinical manifestation, therapy, and prognosis of Susac syndrome and enhance the understanding of this disease.Methods:A case summary was made.The clinical data of two siblings with Susac syndrome treated at Children′s Medical Center, Peking University First Hospital in January 2024 were summarized.Reported cases of pediatric Susac syndrome were reviewed.Results:The onset of the disease in the two siblings was at the age of 3.00 and 6.75 years, with recurrent headaches, tinnitus, hearing loss and encephalopathy symptoms.Cranial magnetic resonance imaging showed multiple cerebral microbleeding and microinfarction lesions, " snowball like" in the corpus callosum and diffuse white matter edema in the brain.Audiometry revealed sensorineural hearing loss.In one case, ophthalmic fluorescein angiography revealed ischemic changes due to branch retinal artery occlusions.No pathogenic variants were detected in gene testing.This child was diagnosed with Susac syndrome, and the symptoms were improved after treatment with Corticosteroids and Rituximab.No relapse was observed during the 9-month follow-up.A total of 20 pediatric cases of Susac syndrome were retrieved, including 18 reported previously and 2 cases from this study.There were 2 boys and 18 girls, with the age of onset ranging from 2.5 to 17.0 years.The common initial symptoms included headache (19 cases), vertigo and tinnitus or hearing loss (9 cases), and vision impairment or visual field defect (4 cases). The symptoms were improved after immunotherapy.Conclusions:With a low incidence, Susac syndrome is rare in children and difficult to diagnose.There may be a genetic predisposition in such disease.Early diagnosis and immunotherapy can low the relapse and improve the prognosis.

Objective:To investigate the clinical manifestation, therapy, and prognosis of Susac syndrome and enhance the understanding of this disease.Methods:A case summary was made.The clinical data of two siblings with Susac syndrome treated at Children′s Medical Center, Peking University First Hospital in January 2024 were summarized.Reported cases of pediatric Susac syndrome were reviewed.Results:The onset of the disease in the two siblings was at the age of 3.00 and 6.75 years, with recurrent headaches, tinnitus, hearing loss and encephalopathy symptoms.Cranial magnetic resonance imaging showed multiple cerebral microbleeding and microinfarction lesions, " snowball like" in the corpus callosum and diffuse white matter edema in the brain.Audiometry revealed sensorineural hearing loss.In one case, ophthalmic fluorescein angiography revealed ischemic changes due to branch retinal artery occlusions.No pathogenic variants were detected in gene testing.This child was diagnosed with Susac syndrome, and the symptoms were improved after treatment with Corticosteroids and Rituximab.No relapse was observed during the 9-month follow-up.A total of 20 pediatric cases of Susac syndrome were retrieved, including 18 reported previously and 2 cases from this study.There were 2 boys and 18 girls, with the age of onset ranging from 2.5 to 17.0 years.The common initial symptoms included headache (19 cases), vertigo and tinnitus or hearing loss (9 cases), and vision impairment or visual field defect (4 cases). The symptoms were improved after immunotherapy.Conclusions:With a low incidence, Susac syndrome is rare in children and difficult to diagnose.There may be a genetic predisposition in such disease.Early diagnosis and immunotherapy can low the relapse and improve the prognosis.

Microspheres are a novel drug delivery system,which provides a new approach for cancer therapy.Anti-cancer agents loaded in microspheres can be released in a controlled and sustained pattern,thereby enhancing the therapeutic efficacy and reducing the side effects and toxicity.The preparation methods for drug delivery microspheres include solvent evaporation,phase separation,spray drying,and microfluidic technology,each of these have advantages and limitations.Based on the preparation materials,drug delivery microspheres can be categorized into natural polymer microspheres,synthetic polymer microspheres and bioceramic microspheres.Natural polymer micro-spheres have good biocompatibility and degradability;synthetic polymer microspheres exhibit superior mechanical properties;bioceramic microspheres have good biocompatibility and specific biological functions,which are widely used in bone tissue engineering.Drug delivery microspheres are used for cancer treatment in various modalities,including photothermal therapy,photodynamic therapy,radioembolization,and immunotherapy,as well as chemotherapy.This article reviews the recent progress of microspheres as nano drug delivery system in cancer treatment to provide a reference for further clinical and translation research.

【Objective】 To study the effect of air inlet modes on the temperature variation, fluctuation, uniformity and coefficient of variation(CV), so as to evaluate the stability and uniformity of the temperature field in refrigerated warehouse for blood. 【Methods】 The temperature changes of blood refrigerated warehouse under different modes of air inlet during compressor operation were analyzed. The stability of the temperature field in the storehouse was evaluated by the changes, fluctuation, uniformity, CV and deviation of temperature at each testing point. 【Results】 The average value of temperature in the storehouse, adopting air inlet via straight blow, was (4.98±0.92)℃, while that of air inlet via micro-hole mode was(4.15±0.25)℃, with significant differences between each other(P<0.05). As to the CV of temperature, air inlet via straight blow was significantly different from that via micro hole(P<0.01). The fluctuation, uniformity and deviation of temperature created by straight blow and micro hole were 1.85±1.11 vs 0.49±0.38, 1.00±0.68 vs 0.47±0.37, and 0.61±0.45 vs 0.27±0.21, respectively, with significant differences between each other(P<0.01). 【Conclusion】 Compared with straight blow, the mean temperature created by micro hole was closer to the median value (4℃) of the temperature range, i. e.(4±2)℃, during blood storage. Otherwise, micro hole demonstrated a smaller CV of temperature, and superior performance in fluctuation, uniformity and deviation of the temperature at the testing points, which was conducive to ensure the stability of storehouse temperature field.

DNA damage response (DDR) is essential for maintaining genome stability and protecting cells from tumorigenesis. Ubiquitin and ubiquitin-like modifications play an important role in DDR, from signaling DNA damage to mediating DNA repair. In this report, we found that the E3 ligase ring finger protein 126 (RNF126) was recruited to UV laser micro-irradiation-induced stripes in a RNF8-dependent manner. RNF126 directly interacted with and ubiquitinated another E3 ligase, RNF168. Overexpression of wild type RNF126, but not catalytically-inactive mutant RNF126 (CC229/232AA), diminished ubiquitination of H2A histone family member X (H2AX), and subsequent bleomycin-induced focus formation of total ubiquitin FK2, TP53-binding protein 1 (53BP1), and receptor-associated protein 80 (RAP80). Interestingly, both RNF126 overexpression and RNF126 downregulation compromised homologous recombination (HR)-mediated repair of DNA double-strand breaks (DSBs). Taken together, our findings demonstrate that RNF126 negatively regulates RNF168 function in DDR and its appropriate cellular expression levels are essential for HR-mediated DSB repair.
Carrier Proteins ; metabolism ; Cell Line, Tumor ; DNA Breaks, Double-Stranded ; DNA Repair ; genetics ; DNA-Binding Proteins ; metabolism ; Genomic Instability ; HeLa Cells ; Histones ; metabolism ; Humans ; Nuclear Proteins ; metabolism ; RNA Interference ; RNA, Small Interfering ; genetics ; Signal Transduction ; Tumor Suppressor p53-Binding Protein 1 ; metabolism ; Ubiquitin ; Ubiquitin-Protein Ligases ; genetics ; metabolism ; Ubiquitination

Objective The purpose of study was to evaluate the safety and effectiveness of theblood-letting and herbal-cupping therapy for lumbar spinal stenosis.Methods A multi-center prospective case series was performed.The LSS patients meeting the inclusion criteria received 8 treatments as a course and 4 courses in total.The primary outcomes were the symptom severity and physical function scale ofthe Swiss Spinal Stenosis Measurement (SSM,total score 0-5 for each domain).The secondary outcomes were thethe 12-item short form health survey (SF-12,total score 0-100),and Oswestry disability index (ODI,total score 0-100) at time of baseline,completion of last treatment of each course.The minimal clinically important differences (MCIDs) were calculated for estimating the percentage of improvement in the population.The adverse events were reported at any time of the intra-and post-operation.This was a phrase analysis of the studyat seven months.Results Forty-eight patientswere included,with 64.6％ (31/48) of LSS showing neurogenic claudication (walking distance ≤200 m).The average age was 63.1 ± 11.7 years,19 (39.6％) female,and the average BMI was 25.3 ± 3.3 kg/m2.The scores of symptom severity scale of SSM were 2.8 ± 0.6,2.6 ± 0.7,2.3 ± 0.6,1.9 ± 0.2 at baseline,1st,2nd,3rd course,and the scores of physical function scale were 2.5 ± 0.8,2.4 ± 0.7,2.1 ± 0.5,1.8 ± 0.3,and all the changes between baseline and each course showed significant improvement.The patient satisfaction of SSM,ODI and SF-12 showed significantimprovements after the 1st,2nd,3rd course (P＜0.05).The SF-12 subgroup physical composite scores after 3rd course and mental composite score after 1st showed no significant improvement.The minimal clinically important difference for the “SymptomSeverity scale” in the SSM was achieved withimprovement of 18.8％,40.6％,83.3％ in the LSS patient population after 1st,2nd,3rd course;and the "physical function scale" in SSM was achieved withimprovement of 22.9％,31.3％,50.0％.A total of 15 patients felt pain when they were micro-punctured with little blood at first time,but the symptom wereimmediately relieved without any treatment.Conelusions The Blood-letting and herbal-cupping therapy could benefit patients with lumbar spinal stenosis after third course of treatment in the fields of symptom relief and quality of life with no severe adverse event.However,this was a phrase analysis,so more evidence of this study and large comparative researches should be warranted in future.

Objective The objective of this study was to investigate the clinicopathological features and rational treatment of primary fallopian tube cancer(PFTC).Methods The clinical and pathological data of 39 patients with primary fallopian tube cancer from January 2006 to July 2016 in Beijing Tongren Hospital of Capital University were retrospectively analyzed.Results There were 39 cases of undifferentiated carcinoma,including 1 case of undifferentiated and transitional cell carcinoma,37 cases of undifferentiated adenocarcinoma,or 29 cases of simple adenocarcinoma and 10 cases of mixed type.Among them,7 cases were treated with clear cell carcinoma,endometriosis in 2 cases,combined with transitional cell carcinoma in 1 case.Extra-pelvic metastases were the most common site of the omentum with 17 cases.The preoperative test CA125 was increased in 13 cases and 38 patients received postoperative chemotherapy.The 5-year overall survival rate of 39 patients was 51.3%.Univariate analysis showed that postoperative pathologic stage(Ⅰ~Ⅱ vs.Ⅲ,P<0.001),intraoperative residual lesion size(P<0.001),omentum metastasis(P<0.001),ovarian metastasis(P=0.034),retroperitoneal lymph node metastasis(P=0.018)and preoperative CA125 elevation(P=0.002)were associated with prognosis,while age(P=0.310)and pathological grade(P=0.663)were not associated with prognosis.Multivariate analysis showed that the number of patients with postoperative lymph node metastasis(P=0.018)and preoperative CA125 elevation(P=0.002)were correlated with prognosis(HR=1.202,95% CI:2.354~63.290,P=0.003) and pathological stage(HR=3.810,95% CI:1.202~12.079,P=0.023).They were associated with prognosis as independent prognostic factors.Conclusion Tumor pathologic staging and omentum metastasis are important prognostic factors influencing the prognosis of patients with primary fallopian tube cancer.Early diagnosis and complete operation can improve the prognosis of patients.

Objective To analyze the clinical value of 1.5T MRI DWI and SWI sequence in the diagnosis of cerebral infarction and cerebral hemorrhage.Methods A total of 28 patients with cerebral infarction were selected as control group,and 28 patients with cerebral hemorrhage were selected as observation group.The PHILPS 1.5T magnetic resonance imaging system was applied.The patients were examined by T1WI and T2WI sequence scanning,and DWI and SWI sequence images were obtained.In the two groups,lesions around the center and different characteristics of SWI and DWI were observed and compared,and the measurement of peripheral lesions were measured for calculating the central area of the PV and ADC.Results There was no significant difference between T1WI and T2WI in patients with cerebral hemorrhage and cerebral infarction,but there was significant difference between DWI sequence and DWI sequence.MRI routine examination showed that there were no significant differences in detection rates of T1WI sequence and T2WI sequence between two groups,but the detection rate of DWI sequence in the observation group was significantly lower than the control group,while detection rate of DWI sequence was significantly higher than the control group(P<0.05).The central area of ADC in the observation group was significantly better than that in the control group,and the PV value of the hematoma area was significantly lower than that of the control group(P<0.05).Conclusion Collections of DWI and SWI images of cerebral infarction and cerebral hemorrhage by magnetic resonance prove that there are significant differences between characteristics of two images.

Objective To analyze the clinical value of 1.5T MRI DWI and SWI sequence in the diagnosis of cerebral infarction and cerebral hemorrhage.Methods A total of 28 patients with cerebral infarction were selected as control group,and 28 patients with cerebral hemorrhage were selected as observation group.The PHILPS 1.5T magnetic resonance imaging system was applied.The patients were examined by T1WI and T2WI sequence scanning,and DWI and SWI sequence images were obtained.In the two groups,lesions around the center and different characteristics of SWI and DWI were observed and compared,and the measurement of peripheral lesions were measured for calculating the central area of the PV and ADC.Results There was no significant difference between T1WI and T2WI in patients with cerebral hemorrhage and cerebral infarction,but there was significant difference between DWI sequence and DWI sequence.MRI routine examination showed that there were no significant differences in detection rates of T1WI sequence and T2WI sequence between two groups,but the detection rate of DWI sequence in the observation group was significantly lower than the control group,while detection rate of DWI sequence was significantly higher than the control group(P<0.05).The central area of ADC in the observation group was significantly better than that in the control group,and the PV value of the hematoma area was significantly lower than that of the control group(P<0.05).Conclusion Collections of DWI and SWI images of cerebral infarction and cerebral hemorrhage by magnetic resonance prove that there are significant differences between characteristics of two images.

Ubiquitin-fold modifier 1 (UFM1) is one of the newly-identified ubiquitin-like proteins. Similar to ubiquitin, UFM1 is conjugated to its target proteins by a three-step enzymatic reaction. The UFM1-activating enzyme, ubiquitin-like modifier-activating enzyme 5 (UBA5), serves as the E1 to activate UFM1;UFM1-conjugating enzyme 1 (UFC1) acts as the E2 to transfer the activated UFM1 to the active site of the E2;and the UFM1-specific ligase 1 (UFL1) acts as the E3 to recog-nize its substrate, transfer, and ligate the UFM1 from E2 to the substrate. This process is called ufmylation. UFM1 chains can be cleaved from its target proteins by UFM1-specific proteases (UfSPs), suggesting that the ufmylation modification is reversible. UFM1 cascade is conserved among nearly all of the eukaryotic organisms, but not in yeast, and associated with several cellular activities including the endoplasmic reticulum stress response and hematopoiesis. Furthermore, the UFM1 cascade is closely related to a series of human diseases. In this review, we summarize the molecular details of this reversible modification process, the recent progress of its functional studies, as well as its implication in tumorigenesis and potential therapeutic targets for cancer.