目的 探讨急性白血病患儿化疗后血流感染的临床特点、病原菌分布和耐药性.方法 选取化疗后发生血流感染的64例急性白血病患儿,收集临床资料,分析病原菌分布特点、药敏试验结果及预后.结果 64例患儿发生血流感染时均处于粒细胞缺乏期.64株病原菌中以革兰阴性(G-)菌为主,占51.6％(33/64),革兰阳性(G+)菌占45.3％(29/64),真菌占3.1％(2/64).G-菌中前3位依次是肺炎克雷伯菌肺炎亚种、大肠埃希菌、产酸克雷伯菌,G+菌中前4位依次是G+杆菌、表皮葡萄球菌、人葡萄球菌人亚种、草绿色链球菌.51例急性淋巴细胞白血病(ALL)患儿的病原菌中G-菌占51.0％(26/51),G+菌占45.1％(23/51),真菌占3.9％(2/51);13例急性髓细胞性白血病(AML)患儿的病原菌中G-菌占53.8％(7/13),G+菌占46.2％(6/13).64株病原菌中耐药菌占32.8％(21/64),其中G-菌中检出产超广谱β内酰胺酶大肠埃希菌占21.2％(7/33),产超广谱β内酰胺酶肺炎克雷伯菌占15.2％(5/33),耐碳青霉烯类肺炎克雷伯菌(CRKP)占9.1％(3/33);G+菌中检出耐甲氧西林凝固酶阴性葡萄球菌占34.5％(10/29),耐克林霉素葡萄球菌占17.2％(5/29),未检出耐利奈唑胺、万古霉素等葡萄球菌属菌株及肠球菌属菌株.G-菌感染和G+菌感染急性白血病患儿的降钙素原(PCT)水平比较,差异无统计学意义(P﹥0.05).G-感染的急性白血病患儿白细胞介素-6(IL-6)水平明显高于G+菌感染患儿,差异有统计学意义(P﹤0.01).63例患儿感染得到完全控制,1例放弃后续化疗出院.结论 急性白血病患儿血流感染以G-菌为主,ALL及AML患儿中G+菌与G-菌分布相当,IL-6在G-菌感染患儿中较G+菌感染患儿明显升高.

目的 分析儿童急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)诱导期合并败血症患儿的临床特点及预后因素.方法 收集2018年6月 ～2021年6月入住复旦大学附属儿科医院安徽医院(安徽省儿童医院)血液科的初诊ALL患儿共192例,对诱导期合并败血症的52例患儿的临床资料进行回顾性分析,包括临床特点、病原学资料、实验室检查及治疗转归等.结果 52例败血症患儿中,男性31例,女性21例,中位年龄为4(1～13)岁,病程中均有发热症状.最常见的血流外感染部位是肺部,其次是口腔黏膜及消化道.共分离出G+菌16株、G-菌36株,分别以肺炎链球菌、大肠杆菌为主要菌种.经积极抗感染治疗后最终存活43例,死亡9例.存活组和死亡组患儿的实验室检查指标血清白蛋白、C反应蛋白(CRP)、降钙素原(PCT)水平及诱导期粒细胞缺乏持续天数比较,差异有统计学意义(P分别为0.012、0.001、0.037、0.040).结论 ALL诱导期合并败血症者患儿以G-菌感染为主,白蛋白、CRP、PCT水平及粒细胞缺乏持续天数可能与不良预后相关.

目的:分析3例ANKRD26基因突变所致遗传性血小板减少症2型(THC2)的临床特征与基因突变家系谱,探讨ANKRD26基因突变类型与THC2临床表型之间的关系.方法:回顾性分析2017年5月-10月在医院血液肿瘤科诊治的3例THC2患儿临床资料与一般实验室检查结果,使用二代技术(NGS)进行全外显子检测(WES)、基因数据和致病突变验证三个主要步骤对先证者及其家庭成员ANKRD26基因进行测序、筛查和分析.结果:3例患儿初诊时均PLT<20×109/L,皆有淤点瘀斑,初诊时丙球及激素效果欠佳.基因分析结果均存在ANKRD26基因突变,突变位点分别位于第22号外显子、第1号外显子启动区与第30号外显子,突变类型均为杂合突变.结论:THC2是一组异质性疾病,其不同基因突变类型预后差别很大,ANKRD26基因多态性可能为影响THC2患儿临床表现的关键因素.

目的 总结中国抗癌协会小儿肿瘤专业委员会CCCG-RMS-2016方案中期研究数据.方法 本研究为多中心前瞻性临床研究,对符合入组条件的新确诊RMS患儿,按照CCCG-RMS-2016 方案进行治疗、评估及随诊,采集数据进行统计学分析.结果 共213例新诊断的RMS患儿纳入研究,男122例(57.3％),女91例(42.7％).中位年龄47.5(3～155)个月.低危组31(14.6％)例,中危组100(46.9％)例,高危组42(19.7％)例,中枢侵犯组40(18.8％)例.中位随访时间25(1～61)个月.4年总生存率为73.5±3.6％,4年无进展生存率为63.0±4.0％,其中低、中、高危组及中枢侵犯组4年OS分别为:100％、(80.9±4.5)％、(56.1±8.6)％及(32.2±15.1)％;4年 PFS 分别为:(93.5±3.2)％、(69.4±5.6)％、(39.5±8.8)％及(32.2±15.1)％.患儿的OS及PFS与原发部位、病理亚型、淋巴结转移、浸润程度及手术切除程度相关(P＜0.05).多因素COX回归分析显示:危险度分组及肿瘤浸润(T)程度是影响预后的独立危险因素(P＜0.05).结论 CCCG-RMS-2016中期报告数据反应了我国当前RMS的诊治现状.

目的:探讨3例Wiskott-Aldrich综合征(WAS)的基因检测结果与临床特征.方法:分析3例WAS患儿的临床资料,使用第二代基因测序(NGS)对先证者及其家庭成员WAS蛋白(WASP)基因进行测序、筛查和分析.结果:3例患儿均有湿疹、反复感染及血小板减少,其中仅1例患儿表现为血小板体积减小.3例患儿均行NGS检测,发现WAS基因突变,其中2例突变点突变,1例为插入突变;家系验证结果显示,2例为X连锁遗传,1例为新生突变.结论:对月龄较小的血小板减少症男性患儿,如常规治疗效果欠佳,同时伴发免疫缺陷、湿疹,应及时完善WASP基因检测明确诊断,造血干细胞移植(HSCT)是有效的治疗手段.

Objective:To investigate the prognostic factors of patients with stages Ⅰ-]Ⅲ left-sided colon cancer (LCC) versus right-sided colon cancer (RCC) receiving radical surgery.Methods:A retrospective analysis of clinical data from 332 patients with stages Ⅰ-Ⅲ colorectal cancer (CRC) who underwent radical surgery in Anhui Provincial Hospital,Anhui Medical University between February 2008 and February 2012 was conducted.The differences in clinicopathological characteristics by tumor location (RCC vs LCC) were examined by using x2 test.The comparisons of overall 5-year survival rate between RCC and LCC within each stage and for all stages were done by using Kaplan-Meier method.The univariate analysis of prognosis was performed by using log-rank test,and the multivariate analysis was performed by using COX regression model.Results:The overall 5-year survival rate of all patients was 69.9％.The LCC patients had significantly higher overall 5-year survival rate than RCC patients (72.6％ vs 66.9％,P =0.020).The stage Ⅲ LCC patients had significantly higher overall 5-year survival rate than the stage Ⅲ RCC patients (62.5％ vs 52.2％,P =0.018),but no significant difference in overall 5-year survival rate was found between stage Ⅰ or Ⅱ RCC and LCC patients (P ＞ 0.05).There were significant differences in T stage,histologic type,degree of differentiation,tumor size,hemoglobin,albumin,fibrinogen and carcinoembryonic antigen (CEA) between RCC and LCC patients (all P ＜0.05).The univariate analysis showed that tumor location,T stage,N stage,histologic type,degree of differentiation,tumor size,hemoglobin,albumin,fibrinogen and CEA were significantly correlated with overall 5-year survival rate of CRC patients (all P ＜ 0.05).Multivatiate analysis showed that N stage,histologic type,degree of differentiation,hemoglobin,albumin,fibrinogen and CEA were independent prognostic factors of CRC (all P ＜ 0.05).Conclusion:For patients with stages Ⅰ-Ⅲ CRC treated with radical surgery,the factors of higher tumor N stage,mucinous adenocarcinoma/signet ring cell carcinoma,poorly differentiated carcinoma,anemia,hypoproteinemia,fibrinogen level more than 4 g/L and CEA level more than 10 ng/mL indicate a poor prognosis.The significant differences in clinicopathological characteristics and prognosis are found between RCC and LCC,but the tumor location is not an independent prognostic factor.

食管癌是消化道常见的恶性肿瘤之一，食管癌根据组织形态学特点主要分为食管鳞状细胞癌和食管腺癌。目前有较多的研究发现，食管癌的发生发展与炎症因素密切相关。环氧化酶-2（COX-2）、前列腺素E2（PGE2）、表皮生长因子受体（EGFR）等均在食管癌中存在过度表达，并与肿瘤的侵袭和转移及预后密切相关。选择性COX-2抑制剂的开发和应用有望为食管癌预防和治疗开辟新的途径。在临床上可能为食管癌分子靶向治疗提供一定的科学依据。

目的:探讨影响初诊即确诊发生肝和(或)肺转移食管鳞癌患者的预后因素.方法:回顾性分析了2013年1月—2015年1月由安徽医科大学附属省立医院和安徽省肿瘤医院收治的56例初诊时已确诊发生肝和(或)肺转移的食管鳞癌患者的临床资料,应用Kaplan-Meier法计算总生存(overallsurvival,OS)时间,log-rank法对影响预后的因素进行单因素分析,COX回归模型对影响预后的因素进行多因素分析.结果:全组患者中位生存时间为11.8个月.单因素分析结果显示,美国东部协作肿瘤组(Eastern Cooperative Oncology Group,ECOG)体能状况(performance status,PS)评分、脏器转移部位和治疗方法与OS有关(P值均＜ 0.05),而性别、年龄、肿瘤的分化程度、病灶转移数目、有无远处淋巴结(锁骨上淋巴结和腹腔干淋巴结)转移以及癌胚抗原(carcinoembryonic antigen,CEA)的表达水平均与OS无关(P值均＞0.05).多因素分析结果显示,ECOG PS评分和脏器转移部位为影响OS的独立因素.结论:对于初诊时已发生肝和(或)肺转移食管鳞癌患者中,ECOG PS评分和脏器转移部位是影响远期生存的独立预后因素.

Objective To analyze prognostic-related factors of 46 cases of gastric cancer with bone metastasis as an&nbsp;initial presentation. Methods The retrospective study included 64 cases of gastric cancer with bone metastasis as an initial presentation. The Kaplan-meier was used to evaluate survival. The median overall survival( OS) was ana-lyzed among different clincal pathological parameters, including sex, age, differentiation degree, the number of bone metastases, bone metastasis site andother parts, radiotherapy or not, ECOG score, bone related events, serum calcium,alkaline phosphatase, the tumor markers carcinoembryonic antigen and carbohydrate antigen 199. The COX model was employed to analyze the independent factors of patients. Results The median OS of 64 gastric cancer patients was 6. 13 months. The single factor analysis showed that the degree of differentiation, the number of bone metastasis, bone metastasis site, extraosseous metastasis or not, ECOG score and the tumor markers CEA and CA199 were related to the prognosis of patients with gastric cancer ( P <0. 05 ) . The multivariate analysis showed that the independent factors of OS were bone metastasis(OR=0. 524, 95% CI:0. 275~0. 996, P=0. 049), ex-traosseous metastasis(OR =2. 343, P =0. 003,95% CI:1. 338 ~4. 101), the ECOG score(OR =2. 914,P =0. 003, 95% CI:1. 422~5. 972). Conclusion Bone metastasis site and extraosseous metastasis, ECOG score at first visit of gastric cancer with bone metastasis as an initial presentation may be the independent prognostic predic-tors with long-term survival.