Hisense计算机辅助系统辅助腹腔镜精准手术治疗儿童肾上腺区巨大肿瘤

目的探讨计算机辅助手术系统(computer-aided system,CAS)对于腹腔镜手术切除肾上腺巨大肿瘤(瘤体直径≥5 cm)的指导价值。方法回顾性分析青岛大学附属医院2015年9月至2018年6月收治的13例肾上腺区巨大肿瘤病例,13例术前均完善相关辅助检查并行Hisense CAS三维重建,依据三维重建结果指导完成腹腔镜手术。结果13例肾上腺区巨大肿瘤患儿术前和术中在Hisense CAS三维重建结果的指导下均顺利完成腹腔镜手术。术后病理检查:神经母细胞瘤7例,节细胞性神经母细胞瘤2例,节细胞神经瘤1例,嗜铬细胞瘤2例,肾上腺皮质癌1例。结论Hisense计算机辅助系统能够清晰显示肿瘤位置及其与周围重要脏器结构的毗邻关系,可用于指导手术治疗儿童肾上腺区巨大肿瘤。     

3D腹腔镜手术治疗小儿阑尾周围脓肿

目的总结3D腹腔镜手术治疗小儿阑尾周围脓肿的临床经验。方法回顾性分析三峡大学第一临床医学院普外科2013年1月至2016年8月26例行3D腹腔镜手术治疗的小儿阑尾周围脓肿病例资料,均采用三孔法腹腔镜手术,局部分离脓腔并清除脓液,分离粘连后找到阑尾,超声刀离断阑尾系膜,双重结扎或夹闭阑尾根部,切除阑尾,经操作孔取出,右侧盆腔留置引流管。结果26例均完成3D腹腔镜下阑尾切除术,无一例中转开腹手术。术中出血量(15.5±3.1)m L,手术时间(45.6±7.8)min,术后住院时间(7.8±1.6)d。操作孔感染1例,腹腔脓肿2例,炎性肠梗阻1例,均经保守治疗痊愈。出院后随访2~24个月,中位随访时间14个月,无一例出现粘连性肠梗阻、营养不良等远期并发症。结论3D腹腔镜手术治疗小儿阑尾周围脓肿术中操作过程的立体感更强,操作更精细,整体安全可行。     

婴幼儿多发性大动脉炎14例临床特点分析

目的通过分析总结14例婴幼儿多发性大动脉炎(TA)的临床特点,并结合文献复习总结,以提高对该病的认识及诊疗水平。方法回顾性分析2016年7月至2019年5月在首都儿科研究所附属儿童医院住院的TA婴幼儿的临床资料及随访情况,并结合文献,分析总结该病的临床特点。结果14例患儿年龄为1个月23 d^28个月,男6例,女8例。临床表现中最常见的是发热[10例(71.4%)],高血压9例(64.3%),脉弱或无脉5例(35.7%)。按照病变血管部位的临床分型,广泛型11例(78.5%),头臂动脉型3例(21.4%),本组患儿无胸腹主动脉及单纯肺动脉型。14例TA患儿中,12例有颈总动脉、颈动脉、锁骨下动脉及冠状动脉及其分支(前降支、回旋支)受累(85.7%);11例肾动脉受累(78.6%);9例腋动脉受累(64.2%);8例腹主动脉受累(57.1%);6例降主动脉受累(42.9%);6例胸主动脉受累(42.9%);6例肠系膜上动脉受累(42.9%);5例股动脉受累(35.7%);5例肺动脉受累(35.7%);4例肱动脉受累(28.6%)。14例患儿中,误诊11例,诊断不清3例,误诊时间为18 d^2个月。误诊病例中,8例误诊为不典型川崎病。14例患儿中,7例治疗后大部分病变血管范围逐渐减少,受累较轻血管甚至可以完全恢复正常血管状态。4例患儿血管影像学检查较前无明显加重或好转。9例出现高血压患儿应用降压药血压能控制在正常范围,但不能停用降压药。5例查体发现脉弱或无脉患儿均未改善。14例患儿中7例生长发育同正常同龄儿,7例落后于正常同龄儿身高体质量第25百分位。14例患儿随访2~22个月,均规律治疗,未出现复发。结论3岁以内TA累及血管较多,病情严重,误诊率高,经过治疗病情很快能够控制,但容易遗留血管病变,部分患儿预后不佳。     

重型血友病A患儿关节出血与关节评估的相关性研究

目的了解血友病患儿出血与关节病变之间的关系,为临床治疗和预后判断提供理论依据。方法选取2016年6月至2017年1月于首都医科大学附属北京儿童医院及成都市新世纪妇女儿童医院就诊的1~7岁重型血友病A且有关节出血记录的患儿,以患儿关节出血为研究关节,收集前3个月研究关节的出血次数,应用超声、X线、磁共振成像(MRI)和血友病关节健康评分(HJHS)评分系统对关节进行评估。将关节出血次数与超声、X线、MRI和HJHS评分进行相关性分析,并对超声、X线、MRI和HJHS评分间的相关性进行分析。结果1.共收集重型血友病A患儿18例,年龄(5.6±1.8)岁,共30个出血关节,中位年关节出血次数为4次(4~16次),中位年靶关节出血次数为8次(4~16次)。2.关节出血次数与超声、X线评估有相关性(r=0.390,P=0.033;r=0.517,P=0.008),与HJHS评估、MRI评估均无相关性(均P>0.05)。3.超声、X射线、HJHS和MRI两两之间均呈极显著正相关[r=0.815(超声与X线),r=0.510(超声与HJHS),r=0.812(超声与MRI),r=0.666(X线与HJHS),r=0.911(X线与MRI),r=0.781(HJHS与MRI),均P<0.01]。4.关节超声和/或MRI评估异常的关节中,出血次数与关节评估间均无相关性(P>0.05)。结论关节出血与关节评估获得的关节病变程度结果不一致,因此准确判断关节病变情况需综合评估关节结构、功能等,以便更加全面地指导血友病患儿的治疗。     

中晚期儿童肾透明细胞肉瘤10例的诊治及预后分析

目的探讨中晚期儿童肾透明细胞肉瘤(CCSK)的临床特点、诊治经过及预后特点。方法收集2014年1月至2017年12月在首都医科大学附属北京同仁医院儿科住院治疗的10例中晚期CCSK患儿的临床资料,对其临床特征、诊疗经过及预后情况等进行回顾性分析。结果1.临床特点:10例CCSK病例中男6例,女4例;中位发病年龄为32个月;7例为左侧CCSK,3例为右侧CCSK。初次诊断时Ⅲ期9例,Ⅳ期1例;其中4例初次诊断时误诊为肾脏其他肿瘤(40%,4/10例)。5例Ⅲ期病例治疗及随访过程中出现肿瘤复发及转移,主要远处转移部位为肺、骨、肝脏及脑。2.治疗及预后:10例中给予手术联合放疗及化疗者7例,未规范化治疗放弃者3例。中位随访时间33.5个月,7例存活,3例死亡,3年总生存率为65.6%。Ⅲ期患儿3年总生存率为74.1%,Ⅳ期患儿3年总生存率为0,Ⅲ期与Ⅳ期预后比较差异有统计学意义(χ^2=9,P=0.003)。5例复发病例中仅1例完全缓解,2例部分缓解,1例进展,1例死亡;3例无复发病例均完全缓解,且均为给予手术、化疗及放疗规范化治疗者。结论儿童CCSK初诊误诊率高,Ⅲ期病例治疗及随访过程中复发及远处转移风险高;Ⅲ期病例积极给予手术、化疗及放疗的规范化治疗,预后良好,而发生复发及远处转移者死亡率高。     

去甲乌药碱对实验性心力衰竭的治疗作用

去甲乌药碱(DMC)是中药附子的有效成分之一。静脉滴注DMC2μg/kg/min共5min,使豚鼠正常心脏的收缩力明显加强,LVSP和LV dP/dt_max分别增加58±7和25±7%;心衰后,LVSP和LV dP/dt_max分别下降到心衰前的40±5和30.5±2.8%。DMC可使之恢复到79±14和75±9.9%,DMC也能加强离体豚鼠衰竭心脏的收缩力。DMC的强心作用与ISO相似,但前者作用较弱,作用维持时间较长,这可能与他们的作用机制不同有关。     

Impact of operative start time on surgical outcomes in patients undergoing primary cytoreduction for advanced ovarian cancer

Objectives

To evaluate the impact of operative start time (OST) on surgical outcomes in patients with advanced ovarian cancer.

Methods

All stage IIIB-IV serous ovarian cancer patients who underwent primary surgery at our institution from 1/01 to 1/10 were identified. Fourteen factors were evaluated for an association with surgical outcomes including OST and OR tumor index (1 point each for carcinomatosis and/or bulky [≥ 1 cm] upper abdominal disease). Univariate logistic regression considering within-surgeon clustering was performed for cytoreduction to ≤ 1 cm versus > 1 cm residual disease. In patients with ≤ 1 cm residual disease, univariate logistic regression considering within-surgeon clustering was performed for 1-10 mm residual disease versus complete gross resection (CGR, 0 mm residual). A multivariate logistic model was developed based on univariate analysis results in the ≤ 1 cm residual disease cohort.

Results

Of 422 patients, residual disease was: 0 mm, 144 (34.1%); 1-10 mm, 175 (41.5%); > 10 mm, 103 (23.3%). OST was not associated with cytoreduction to ≤ 1 cm residual disease on univariate analysis. In the ≤ 1 cm residual disease cohort, albumin, CA-125, ascites, ASA score, stage, OR tumor index, and OST were associated with CGR on univariate analysis. Earlier OSTs were associated with increased rates of CGR. On multivariate analysis, CA-125 was independently associated with CGR. OST was associated with CGR in patients with an OR tumor index of 2 but not an OR tumor index < 2.

Conclusions

OST was not associated with cytoreduction to ≤ 1 cm residual disease in patients with advanced serous ovarian cancer. In the cohort of patients with ≤ 1 cm residual disease, later OSTs were associated with reduced rates of CGR in patients with greater tumor burden.     

Abnormal DNA methylation in oocytes could be associated with a decrease in reproductive potential in old mice

Purpose

This study was designed to evaluate DNA methylation and the expression of DNA methyltransferases (Dnmt1, Dnmt3a, Dnmt3b and Dnmt3L) in metaphaseII (MII) oocytes and the DNA methylation of pre-implantation embryos during mouse aging to address whether such aging-related changes are associated with decreased reproductive potential in aged mice.

Methods

Oocytes (MII) from 6 to 8 weeks old female mice are referred to as the ‘young group’; oocytes from the same group that were maintained until 35–40 weeks old are referred to as the ‘old group.’ The oocytes were fertilized both in vitro and in vivo to obtain embryos. The DNA methylation levels in the oocytes (MII) and pre-implantation embryos were assessed using fluorescence staining. The expression levels of the Dnmt genes in the oocytes (MII) were assessed using Western blotting.

Results

The DNA methylation levels in the oocytes and pre-implantation embryos (in vivo and in vitro) decreased significantly during the aging of the mice. The expression levels of all of the examined Dnmt proteins in the old group were lower than young group. Both the cleavage and blastocyst rate were significantly lower in the oocytes of the older mice (69.9 % vs. 80.9 %, P < 0.05; 33.9 % vs. 56.4 %, P < 0.05). The pregnancy rate of the old mice was lower than that of the young mice (46.7 % vs. 100 %, P < 0.05). The stillbirth and fetal malformation rate was significantly higher in the old group than in the young group (17.2 % vs. 2.9 %, P < 0.05).

Conclusions

The decreased expression of Dnmt1, Dnmt3a, Dnmt3b and Dnmt3L in oocytes (MII) and the change of genome-wide DNA methylation in oocytes and pre-implantation embryos due to aging may be related to lower reproductive potential in old female mice.     

A case series of 130 neonates with congenital syphilis: preterm neonates had more clinical evidences of infection than term neonates

Background: The early clinical manifestations of congenital syphilis (CS) vary from asymptomatic to florid lesions, involving multisystem damage. But little is known about the differences of early clinical features between preterm and term neonates with CS. Objectives: To compare the clinical characteristics between preterm and term neonates with CS and analyze the possible underlying reasons for the differences. Methods: Consecutive medical charts of infants at risk for CS from January 1999 to December 2009 were retrospectively reviewed. Neonates with positive 19S-IgM in serum were included in the study. Results: Among the 1,670 cases at risk for CS, 130 neonatal cases with positive 19S-IgM in serum were included in the analysis, including 58 preterm ones and 72 full-term ones. Compared with term neonates with CS, preterm ones were more likely to have characteristic skin rash (36.2 vs. 9.7%, p < 0.001), hepatomegaly (51.7 vs. 25%, p = 0.02), splenomegaly (32.8 vs. 15.4%, p = 0.02), PRP titer ≥1:8 (96.6 vs. 70.8%, p < 0.001), thrombocytopenia (43.1 vs. 23.6%, p = 0.018), elevated CRP (65.5 vs. 36.5%, p = 0.002), and abnormal long bone X-ray results (94.6 vs. 68.1%, p < 0.001). Fewer mothers of preterm neonates with CS received treatment for syphilis (15.5 vs. 40.3%, p = 0.003). The rate of withdrawal of care was higher in preterm neonates with CS (31 vs.12.5%, p = 0.036). Conclusions: Preterm neonates with CS had more clinical evidences and suffered more than term ones.     

Phase II study of MLN8237 (alisertib), an investigational Aurora A kinase inhibitor, in patients with platinum-resistant or -refractory epithelial ovarian, fallopian tube, or primary peritoneal carcinoma

ObjectivesAurora A kinase (AAK), a key mitotic regulator, is implicated in the pathogenesis of several tumors, including ovarian cancer. This single-arm phase II study assessed single-agent efficacy and safety of the investigational AAK inhibitor MLN8237 (alisertib), in patients with platinum-refractory or ‐resistant epithelial ovarian, fallopian tube, or primary peritoneal carcinoma.MethodsAdult women with malignant, platinum-treated disease received MLN8237 50 mg orally twice daily for 7 days plus 14 days' rest (21-day cycles). The primary endpoint was combined objective tumor response rate per Response Evaluation Criteria in Solid Tumors (RECIST) and/or CA-125 criteria. Secondary endpoints included response duration, clinical benefit rate, progression-free survival (PFS), time-to-progression (TTP), and safety.ResultsThirty-one patients with epithelial ovarian (n = 25), primary peritoneal (n = 5), and fallopian tube carcinomas (n = 1) were enrolled. Responses of 6.9–11.1 month duration were observed in 3 (10%) patients with platinum-resistant ovarian cancer. Sixteen (52%) patients achieved stable disease with a mean duration of response of 2.86 months and which was durable for ≥ 3 months in 6 (19%). Median PFS and TTP were 1.9 months. Most common drug-related grade ≥ 3 adverse events were neutropenia (42%), leukopenia (23%), stomatitis, and thrombocytopenia (each 19%); 6% reported febrile neutropenia.ConclusionsThese data suggest that MLN8237 has modest single-agent antitumor activity and may produce responses and durable disease control in some patients with platinum-resistant ovarian cancer. MLN8237 is currently undergoing evaluation in a phase I/II trial with paclitaxel in recurrent ovarian cancer.