Nonconvulsive status epilepticus (NCSE)is a peculiar kind of status epilepticus,which is not un-common in children,but it always be misdiagnosed due to its unnoticeable clinical signs.So far,there is no international unifying diagnose standard of NCSE.Therefore,electroencephalography(EEG)monitoring is recommended.Now,the progress in this field of clinical manifestation and EEG features of NCSE was reviewed.

Objective To evaluate the safety and clinical efficacy of transcatheter arterial chemoembolization (TACE) in treating primary hepatic carcinoma (PHC) complicated by tumor thrombus in inferior vena cava and right atrium (IVC-RA).Methods A total of 17 patients with PHC complicated by tumor thrombus in IVC-RA were included in this study.After the tumor-feeding arteries were confirmed with selective arteriography,TACE was carried out.The used embolization materials included chemotherapy drug-lipiodol emulsion and particle type embolic materials,and the target arteries included branches of hepatic artery,right inferior phrenic artery,branches of left gastric artery,etc.All patients were periodically followed up,and further treatment would be conducted if it was necessary.Results A total of 45 interventional procedures were performed in the 17 patients and all procedures were successful without any significant complication.Explicit blood supply arteries of IVC-RA tumor thrombus were observed in all the 17 patients,including hepatic artery branches (n=12) and extra-hepatic arteries (n=9),which included left gastric artery (n=1) and right inferior phrenic artery (n=8).CT reexamination showed that lipiodol deposition in IVC-RA tumor thrombus was found in 15 patients.In the 17 patients,the median survival time was 12 months,and the one-year and 2-year overall survival rates were 52.9％ and 29.4％ respectively.Conclusion IVC-RA tumor thrombus has rich blood supply,and its main blood supply arteries include hepatic artery and right inferior phrenic artery.For the treatment of PHC associated with IVC-RA tumor thrombus,TACE is safe and effective.

Objective To summarize the phenotypic features of an unrecognized leukoencephalopathy in infants sharing same clinical features,and to better understand the disease and provide new evidence for identification of new leukoencephalopathy. Methods Clinical and follow-up data of 13 patients with unrecognized infantile leukoen-cephalopathy were collected from Peking University First Hospital from January, 2006 to December, 2014. Results (1) There were 7 male and 6 female. The average age of onset was 11 months (4-25 months). Thirty-eight percent (5/13 cases) of patients had incentives before the onset;all of the cases had acute onset and rapid motor function regression. Fifteen percent (2/13 cases) of the patients suffered from seizures in the course of the disease. Patients′condition became stable,and cognition and motor function improved gradually 1 month after onset. No patient died till the last follow-up. (2) Imaging features:magnetic resonance imaging (MRI) of the patients was characterized by im-plicating deep white matter,presenting T1 hypointense,T2 and fluid attenuated inversion recovery ( FLAIR) hyperin-tense in the periventricular area. All of MRI showed massive and symmetric lesions with heterogeneous signal and cystic degeneration. DWI showed patch or massive hyperintense in some of the lesions. The follow-up MRI showed the original lesions decreased in 88% ( 8/9 cases ) of patients, and white matters atrophied in 55% ( 5/9 cases ) of patients;the cystic degeneration still existed and even expanded;DWI showed regional linear or spot hyperintense in 88% (8/9 cases) of patients,which was smaller than before,and distributed around the original lesions. Conclusions The patients with leukoencephalopathy caused by unknown pathogenic gene were much likely to be mitochondrial leukoencephalopathy. This study provided evidence for further exploration of new pathogenic genes causing leu-koence-phalopathy.

Objective To analyze the clinical characteristics of 3 unrelated boys with paroxysmal nonkinesigenic dyskinesia and developmental delay caused by de novo mutation in KCNMA1,and to expand the knowledge of clinical phenotype of KCNMA1 mutation.Methods Clinical data of patients were collected,including gender,age,condition of the perinatal period,personal history,and family history.And the features of genotype data were collected including features of attack,developmental milestones,physical examinations,treatments,and responses to treatment.The data including blood biochemical results,results of metabolic screening and genetic testing and the pedigree validation were collected,while the relationship between phenotype and genotype was analyzed.Results (1)Phenotypic features:3 unrelated boys were diagnosed.The ages of disease onset were 20 days,7 months and 13 months,respectively.All the patients manifested paroxysmal nonkinesigenic dyskinesia and were characterized by the episodes that occurred during wakefulness,presented with sudden onset of asymmetric limb dystonic posture,sometimes with nystagmus and strabismus,or sudden decrease of voluntary movement of limbs with hypotonia and occasional esotropia and yawning.There was no loss of awareness during attack.No precipitating factors were observed before attacks.The developmental milestones were delayed.Three children had no response to anti-epilepsy drug before diagnosis.After diagnosis,2 cases used Clonazepam and 1 case showed less attack.There was not any epileptic seizure until the last follow-up at the ages of 3 years and 6 months old,7 years old,and 5 years and 8 months old,respectively.The frequency of attacks was decreased.The episodes were recorded during video-electroencephalogram(EEG) monitoring,which showed normal ictal and interictal EEG.(2)Genotypic features:all 3 children were detected to have KCNMA1 genetic heterozygous missense mutation,while c.2650G>A (p.Glu884Lys) mutation was identified in 1 patient,and c.3158A>G(p.Asn1053Ser)mutation in the other 2 patients,but no such mutation was found in their parents.Conclusion This finding expands the phenotype of KCNMA1mutation.KCNMA1 should be considered as one of the candidate genes for screening in patients with early onset of paroxysmal nonkinesigenic dyskinesia without triggers,or early-onset of developmental delay,with or without epilepsy.

Objective To summarize our experience with surgical management of renal neoplasm involving inferoir veno cava.Methods We review the data of 115 patients,including 74 male patients and 41 female patients,with renal neoplasm involving venous system between March 1993 and December 2015.The mean age was 52 years old,ranging 22 to 77 years old.The tumor was found in right side in 77 cases and left side in 38 cases.There were 15 patients (13.0％) with renal vein thrombus,38 (33.1％) with infrahepatic thrombus,29 (25.2％) with low retrohepatic thrombus,20 (17.4％) with high retrohepatic thrombus,and 13 (11.3％) with supradiaphragmatic thrombus.The mean age was 52 years old,ranging 22 to 77 years old.The tumor was found in right side in 77 cases and left side in 38 cases.All patients accepted the radical nephrectomy.Primary outcomes were overall survival (OS),and cancer special survival (CSS) in patients with renal cell carcinoma (RCC) estimated by Kaplan-Meier method.Secondary outcome included operative and oncological features,past-operative complications and hospital mortality.Cox proportional hazard model was used to univariate and multivariate analysis for risk factor impacting on OS of RCC patients.Results Complete resections of renal neoplasm with tumor thrombus were achieved in 113 patients (98.3％),2 patients died intraoperatively due to the dropping of thrombus.Postoperative complication rate was 16.8％.Hospital mortality was 2.6％.Mean follow-up interval was 48 months.OS rates at 5-,and 10-year in RCC patients were 66.5％,and 61.8％,respectively.Metastasis was an independent risk factor affecting on OS (P =0.000).However,the level of thrombus was not an risk factor affecting the prognosis.Conclusions Radical resection of renal tumor and caval thrombus is an effective treatment for prolonging survival in patients with RCC extending into venous system.Retrohepatic caval tumor thrombus below or above the main hepatic vein should be dichotomized and managed respectively with diverse techniques.Metastatic rather than the level of thrombus was a risk factor impacting on RCC patient survival.

Liquid biopsy is a technology that exhibits potential to detect cancer early,monitor therapies,and predict cancer prognosis due to its unique characteristics,including noninvasive sampling and real-time analysis.Circulating tumor cells(CTCs)and extracellular vesicles(EVs)are two important components of circu-lating targets,carrying substantial disease-related molecular information and playing a key role in liquid biopsy.Aptamers are single-stranded oligonucleotides with superior affinity and specificity,and they can bind to targets by folding into unique tertiary structures.Aptamer-based microfluidic platforms offer new ways to enhance the purity and capture efficiency of CTCs and EVs by combining the advantages of microfluidic chips as isolation platforms and aptamers as recognition tools.In this review,we first briefly introduce some new strategies for aptamer discovery based on traditional and aptamer-based micro-fluidic approaches.Then,we subsequently summarize the progress of aptamer-based microfluidics for CTC and EV detection.Finally,we offer an outlook on the future directional challenges of aptamer-based microfluidics for circulating targets in clinical applications.