2016年广西肿瘤登记地区恶性肿瘤发病和死亡分析

目的 分析2016年广西肿瘤登记地区恶性肿瘤发病和死亡情况。 方法 对2016年广西31个肿瘤登记处的肿瘤登记数据进行分析，按城乡、性别、年龄别对数据进行分层，分别计算恶性肿瘤发病和死亡的粗率、标化率、累积率（0～74岁）和前10位恶性肿瘤构成。人口标准化率采用2000年全国普查标准人口年龄构成和Segi's 世界标准人口年龄构成计算。 结果 纳入分析的31个登记处共覆盖人口11 628 368人（其中城市6 698 487人，农村4 929 881人），恶性肿瘤新发病例28 140例，发病粗率、中标率、世标率和累积率（0～74岁）分别为241.99/10万、196.89/10万、192.26/10万、21.90%；无论是中标率还是世标率，男性均高于女性，城市地区均高于农村地区。恶性肿瘤发病在40岁以后快速上升，在80岁年龄组时达到高峰。发病前10位恶性肿瘤依次为肝癌、肺癌、乳腺癌、结直肠癌、宫颈癌、胃癌、鼻咽癌、前列腺癌、子宫肿瘤和脑瘤，占全部恶性肿瘤发病的75.65%。恶性肿瘤死亡病例17 647例，死亡粗率、中标率、世标率和累积率（0～74岁）分别为151.76/10万、119.05/10万、117.31/10万、13.27%；无论中标率还是世标率，男性均高于女性，城市地区均低于农村地区。恶性肿瘤死亡在40岁以后快速上升，在85岁年龄组时达到高峰。死亡前10位恶性肿瘤依次为肝癌、肺癌、结直肠癌、胃癌、乳腺癌、鼻咽癌、宫颈癌、食管癌、前列腺癌和白血病，占全部恶性肿瘤死亡的81.74%。结论  2016年广西肿瘤登记地区恶性肿瘤发病率和死亡率略高于2015年，肝癌、肺癌、结直肠癌、乳腺癌和鼻咽癌是重点防控的癌种。     

Sensory evoked potentials in patients with Rett syndrome through the lens of animal studies: Systematic review

ObjectiveSystematically review the abnormalities in event related potential (ERP) recorded in Rett Syndrome (RTT) patients and animals in search of translational biomarkers of deficits related to the particular neurophysiological processes of known genetic origin (MECP2 mutations).MethodsPubmed, ISI Web of Knowledge and BIORXIV were searched for the relevant articles according to PRISMA standards.ResultsERP components are generally delayed across all sensory modalities both in RTT patients and its animal model, while findings on ERPs amplitude strongly depend on stimulus properties and presentation rate. Studies on RTT animal models uncovered the abnormalities in the excitatory and inhibitory transmission as critical mechanisms underlying the ERPs changes, but showed that even similar ERP alterations in auditory and visual domains have a diverse neural basis. A range of novel approaches has been developed in animal studies bringing along the meaningful neurophysiological interpretation of ERP measures in RTT patients.ConclusionsWhile there is a clear evidence for sensory ERPs abnormalities in RTT, to further advance the field there is a need in a large-scale ERP studies with the functionally-relevant experimental paradigms.SignificanceThe review provides insights into domain-specific neural basis of the ERP abnormalities and promotes clinical application of the ERP measures as the non-invasive functional biomarkers of RTT pathophysiology.     

Mutation status and burden can improve prognostic prediction of patients with lower‐risk myelodysplastic syndromes

Patients with lower‐risk myelodysplastic syndromes (LR‐MDS) as defined by the International Prognostic Scoring System (IPSS) have more favorable prognosis in general, but significant inter‐individual heterogeneity exists. In this study, we examined the molecular profile of 15 MDS‐relevant genes in 159 patients with LR‐MDS using next‐generation sequencing. In univariate COX regression, shorter overall survival (OS) was associated with mutation status of ASXL1 (P = .001), RUNX1 (P = .031), EZH2 (P = .049), TP53 (P = .016), SRSF2 (P = .046), JAK2 (P = .040), and IDH2 (P = .035). We also found significantly shorter OS in patients with an adjusted TET2 variant allele frequency (VAF) ≥18% versus those with either an adjusted TET2 VAF <18% or without TET2 mutations (median: 20.4 vs 47.8 months; P = .020; HR = 2.183, 95%CI: 1.129‐4.224). After adjustment for IPSS, shorter OS was associated with mutation status of ASXL1 (P < .001; HR = 4.306, 95% CI: 2.144‐8.650), TP53 (P = .004; HR = 4.863, 95% CI: 1.662‐14.230) and JAK2 (P = .002; HR = 5.466, 95%CI: 1.848‐16.169), as well as adjusted TET2 VAF ≥18% (P = .008; HR = 2.492, 95% CI: 1.273‐4.876). Also, OS was increasingly shorter as the number of mutational factors increased (P < .001). A novel prognostic scoring system incorporating the presence/absence of the four independent mutational factors into the IPSS further stratified LR‐MDS patients into three prognostically different groups (P < .001). The newly developed scoring system redefined 10.1% (16/159) of patients as a higher‐risk group, who could not be predicted by the currently prognostic models. In conclusion, integration of the IPSS with mutation status/burden of certain MDS‐relevant genes may improve the prognostication of patients with LR‐MDS and could help identify those with worse‐than‐expected prognosis for more aggressive treatment.     

52例结肠炎性息肉患者的中医证型分布规律

目的文章将主要针对于结肠炎性息肉患者进行中医辨证分型。方法选取2017年8月-2018年12月在本院脾胃科320例结肠息肉患者进行发病率统计。结果在320例结肠肉患者中,炎性息肉,占据16.25%;其中,男性与女性的占比分别为67.3%和32.7%,男性老年患者与女性老年患者的比例分别为51.4%和52.9%;在炎性息肉中医证型分布中,以湿热淤阻证型最高,占据比例为48.1%,其中男性比例为60.0%,年龄分布上,以青中年患者居高。结论结肠炎性息肉在人群的年龄性别分布规律上,以男性老年患者最高,中医证型中以湿热淤阻为主。而在湿热淤阻炎性息肉患者分布中,以男性青中年患者为主要发病人群。     

Epileptogenic modulation index and synchronization in hypsarrhythmia of West syndrome secondary to perinatal arterial ischemic stroke

ObjectivePerinatal arterial ischemic stroke (PAIS) is associated with epileptic spasms of West syndrome (WS) and long term Focal epilepsy (FE). The mechanism of epileptogenic network generation causing hypsarrhythmia of WS is unknown. We hypothesized that Modulation index (MI) [strength of phase-amplitude coupling] and Synchronization likelihood (SL) [degree of connectivity] could interrogate the epileptogenic network in hypsarrhythmia of WS secondary to PAIS.MethodsWe analyzed interictal scalp electroencephalography (EEG) in 10 WS and 11 FE patients with unilateral PAIS. MI between gamma (30–70 Hz) and slow waves (3–4 Hz) was calculated to measure phase-amplitude coupling. SL between electrode pairs was analyzed in 9-frequency bands (5-delta, theta, alpha, beta, gamma) to examine inter- and intra-hemispheric connectivity.ResultsMI was higher in affected hemispheres in WS (p = 0.006); no differences observed in FE. Inter-hemispheric SL of 3-delta, theta, alpha, beta, gamma bands was significantly higher in WS (p < 0.001). In WS, modified Z-Score of intra-hemispheric SL values in 3-delta, theta, alpha, beta and gamma in the affected hemispheres were significantly higher than those in the unaffected hemispheres (p < 0.001) as well as 0.5–4 Hz (p = 0.004).ConclusionsThe significantly higher modulation in affected hemisphere and stronger inter- and intra-hemispheric connectivity generate hypsarrhythmia of WS secondary to PAIS.SignificanceEpileptogenic cortical-subcortical transcallosal networks from affected hemisphere post-PAIS provokes infantile spasms.