Therapeutic plasma exchange in pediatric patients with acute demyelinating syndromes of the central nervous system: A single-center experience

BackgroundTherapeutic plasma exchange (TPE) is an extracorporeal treatment that can be used in adult and pediatric patients with acute demyelinating syndromes of the central nervous system. In this study, the efficacy and safety of TPE was evaluated in 10 pediatric patients who underwent TPE that were unresponsive to corticosteroid treatment.MethodsRecords of 10 pediatric patients who underwent TPE in our pediatric intensive care unit (PICU) between May 2017 and June 2020 were used. Expanded Disability Status Scale (EDSS), Gait Scale (GS), and Visual Outcome Scale (VOS) were applied to the patients before and after TPE.ResultsOf the 10 patients who underwent TPE, five were diagnosed with multiple sclerosis (MS), three with transverse myelitis (TM), and two with acute disseminated encephalomyelitis (ADEM). The median age of the patients was 13.3 years (IQR 8-15), and the median day from symptom onset to onset of TPE was 12.5 days (IQR 7-28). A total of 104 TPE sessions were performed successfully. While no complications were encountered in three patients during the sessions, the most common complication was hypofibrinogenemia. The decrease in EDSS and GS scores was found to be consistent with the clinical response of the patients. There was no statistically significant decrease in the VOS.ConclusionsWith this study, we can say that TPE is a feasible, effective, and safe treatment modality in children with acute demyelinating syndromes of the central nervous system.     

35例新型冠状病毒肺炎患者中医临床特征分析

目的分析广东省新型冠状病毒肺炎患者临床资料及中医证候,为该病的防治提供科学依据。方法将2020年1月23日至2020年2月14日本院收治的35例新型冠状病毒肺炎患者纳为研究对象,收集患者的基本情况、流行病学史及疾病史,通过血液生化分析及胸部CT检查患者临床情况,由5名高级职称中医师对患者舌象进行诊断,并根据《广东省新型冠状病毒感染的肺炎中医药治疗方案(试行第一版)》进行辨证。结果新型冠状病毒肺炎患者年龄分布较广,平均年龄为(44.00±15.17)岁,以男性居多,57.14%的患者有武汉及周边地区旅居史,42.86%的患者有确诊患者接触史;50%以上的患者淋巴细胞绝对值降低,CD3、CD4和、CD8降低以及C反应蛋白增加;胸部CT显示,80%以上的患者出现肺部病变,其中双肺病变占60%,病变类型以斑块状阴影居多;多数患者伴有发热(85.71%)、咳嗽(80.00%)、肌肉酸痛(42.86%)、乏力(40.00%)等临床症状;患者的证型主要是湿邪郁肺(45.71%),其次为邪热壅肺(37.14%),病机特点以湿为主,其次为热;舌苔以腻苔为主,舌质以红和淡红为主。结论新型冠状病毒肺炎患者的中医证型以湿邪郁肺为主,致病特点以"湿"为主,可针对性地采取中医治疗。     

Progress in Targeting the TGFβ Pathway

Signaling by the transforming growth factor-β (TGF-β) superfamily is important in the regulation of hematopoiesis and is dysregulated in myelodysplastic syndromes (MDS), contributing to ineffective hematopoiesis and clinical cytopenias. TGF-β, activins and growth differentiation factors exert inhibitory effects on red cell formation by activating canonical SMAD2/3 pathway signaling. SMAD2/3 overactivation is seen in numerous subtypes of MDS. Furthermore, reduced levels of inhibitory SMAD7 are

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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.     

基于聚类分析和因子分析的肺小结节患者中医证候特点研究

目的分析肺小结节患者中医证候分布特点,为中医治疗肺小结节提供参考依据。方法收集385例肺小结节患者中医四诊信息,运用聚类分析、因子分析方法对肺小结节患者的中医证候特点和分布规律进行分析。结果肺小结节患者中医症状以虚为主,神疲乏力、夜寐不安、舌红、咳嗽、口渴、口干咽燥、面萎黄、气短、脉细弱、食少纳呆等居多。中医证候以肺脾气虚证最多,占52.2%,其余依次为肺阴亏虚证、气虚血瘀证、肝肾不足证。肺脾气虚证主症为喘息、大便稀溏、腹胀、食少纳呆、神疲乏力、自汗、舌淡红、齿痕舌、苔薄白、脉细弱,次症为夜寐不安、面萎黄;肺阴亏虚证主症为口干咽燥、口渴、咳嗽、痰少难咳、盗汗、痰中血丝、裂纹舌、苔少、脉细,次症为烦躁易怒、面红润、舌红;气虚血瘀证主症为头晕、胸痛、气短、面晦黯、舌黯红,次症为心悸;肝肾不足证主症为胸胁隐痛、喜太息、脉细弦、苔白、腰膝酸软、胸闷、耳鸣、便秘,次症为浮肿。结论肺小结节中医证候以肺脾气虚证、肺阴亏虚证、气虚血瘀证、肝肾不足证为主,正气亏虚是肺小结节变化发展的主要病机。     

亚急性甲状腺炎超声特征与中医证型相关性评价

目的:探讨亚急性甲状腺炎超声特征与中医证型的相关性。方法:将甲状腺穿刺活检确诊为亚急性甲状腺炎的65例患者纳入研究,对患者实施中医辨证和超声(超声弹性成像)检查,观察不同中医证型患者的超声表现,并对患者的超声检查图像进行分级,比较不同超声分级患者的中医证型。结果:中医证型以风热痰凝证为主,风热痰凝证与肝郁气虚证比较,差异有统计学意义(χ^2=4.680,P=0.031)。风热痰凝证与气阴两虚兼痰凝证比较,差异有统计学意义(χ^2=8.613,P=0.003)。患者主要超声表现包括甲状腺肿大、片状低回声区、低回声区周围有丰富血流信号、甲状腺上动脉峰值流速>40 cm/s。风热痰凝证各超声表现与气阴两虚兼痰凝证比较,差异有统计学意义(P <0.05);风热痰凝证甲状腺肿大、片状低回声区与肝郁气虚证比较,差异有统计学意义(P <0.05)。风热痰凝证各超声表现(除甲状腺肿大外)与气阴两虚兼痰凝证比较,差异有统计学意义(P <0.05)。超声弹性成像分级Ⅱ级构成比与0级比较,差异有统计学意义(χ^2=15.577,P=0.000);Ⅱ级构成比与Ⅰ级比较,差异有统计学意义(χ^2=5.969,P=0.015);Ⅱ级构成比与Ⅲ级比较,差异无统计学意义(χ^2=1.851,P=0.174);Ⅱ级构成比与Ⅳ级比较,差异无统计学意义(χ^2=2.459,P=0.117)。不同超声弹性成像分级中医证型分布情况比较,0级:风热痰凝证与肝郁气虚证比较,差异有统计学意义(χ^2=4.800,P=0.028);Ⅱ级:风热痰凝证与肝郁气虚证、气阴两虚兼痰凝证比较,差异有统计学意义(χ^2=7.765,6.017,P=0.005,0.014);Ⅳ级:风热痰凝证与肝郁气虚证、气阴两虚兼痰凝证比较,差异有统计学意义(χ^2=7.337,5.250,P=0.007,0.022);其他证型两两比较,差异无统计学意义(P> 0.05)。结论:亚急性甲状腺炎患者的超声特征与中医证型存在密切联系,不同中医证型患者的超声表现存在明显差异,不同超声弹性成像分级患者的中医证型分布也存在明显差异,而超声弹性成像分级更高。     

约束隐结构研究冠心病痰湿证的定量化辨证规则＊

目前在中医界已发布的冠心病痰湿证辨证标准是以主症、次症形式定性地给出，存在主观性较强的问题。本文引入约束隐结构分析，该方法将主症、次症的语义作为约束条件加入隐结构分析过程，得到含有主症、次症语义约束的定量化中医证候辨证规则。使用该方法对冠心病痰湿证患者556条无标签数据的分析，得到其约束隐结构模型，最后建立定量化痰湿证辨证规则，舌胖边有齿痕（3.16）、苔腻（3.12）、苔白滑（4.72）、胸闷（1.73）、脉濡或滑（6.04）；次症：肢体困重（0.48）、口黏（0.63）、体胖（0.49）、大便粘滞（1.38）、脘腹痞满（0.97）、面色晦浊（0.79）、嗜睡（1.18）、纳差（1.07）。与经典隐结构模型得到规则和中医界已发布的定性化辨证规则相比，约束隐结构得到的规则客观性强，具有可重复性。在证候类大小、规则的量化合理度上较好地反映了主症、次症的特点，得到的规则切合中医实际，为冠心病痰湿证辨证标准的定量化研究提供帮助和参考。     

Oral and dental findings in emanuel syndrome

Emanuel Syndrome (ES; OMIM# 609029) is a rare disorder caused by an unbalanced chromosomal translocation [supernumerary der(22)t(11,22)] and characterized by multiple congenital abnormalities. With limited published cases and low prevalence (1:110 000), detailed ES‐associated oro‐dental findings have not previously been reported. This is a case report of a 14‐year‐old boy with ES who presented with congenital cardiac, renal, auditory, musculoskeletal problems, and global developmental delay. The patient was managed with risperidone, melatonin, omeprazole, guanfacine, and oxcarbazepine. Anxiety‐associated self‐injurious behaviour was seen along with stereotypic hand movements. Consistent with previous reports, microcephaly and micrognathia were noted. Oro‐facial cleft or gross asymmetry, however, was not observed. Significant oro‐dental findings included delayed eruption of primary and permanent teeth, oligodontia (two erupted and five unerupted permanent teeth), and short‐root anomaly of central incisors. The patient demonstrated anxiety‐triggered bruxism with generalized attrition. This case report provides a comprehensive list of systemic ES findings along with oro‐dental manifestations, which have previously not been reported in detail.