原发性肾病综合征患儿足量激素治疗前后血、尿白细胞介素-6检测的意义

目的探讨原发性肾病综合征(PNS)患儿足量激素治疗前后血、尿白细胞介素-6(IL-6)水平变化的意义。方法初治PNS患儿38例,分别于足量激素治疗前和治疗8周后(或尿蛋白转阴2周后)用ELISA方法检测血、尿IL-6含量。比较激素敏感组和耐药组血、尿IL-6变化。结果激素治疗前,激素敏感组和耐药组血IL-6均较对照组显著升高(P均<0.01),两组比较无显著性差异(P均>0.05)。激素治疗前激素敏感组和耐药组尿IL-6均较对照组显著升高(P均<0.01),两组比较有显著性差异(P<0.01)。激素治疗8周后激素敏感组血、尿IL-6均降低,与对照组比较无显著差异(P>0.05),而耐药组血、尿IL-6水平无明显降低,与对照组比较仍有显著性差异(P<0.01)。结论血、尿IL-6可作为PNS患儿激素敏感性和预后估计的参考指标之一。     

小儿原发性肾病综合征足量糖皮质激素治疗前后血尿白介素-6检测及其临床意义

目的：白介素-6(IL-6)是原发性肾病综合征有肯定意义的细胞因子之一。该文探讨原发性肾病综合征患儿足量糖皮质激素治疗前后血、尿白细胞介素-6水平变化及其临床意义。方法：初治的原发性肾病综合征患儿38例,分别于足量激素治疗前和足量激素治疗8周后(或尿蛋白转阴2周后)用ELISA方法检测血、尿中白细胞介素-6的水平。比较激素敏感组和激素耐药组血、尿白细胞介素-6的含量。结果：激素治疗前,激素敏感组和激素耐药组血IL-6分别为118.74±31.18 ng/L和129.62±28.14 ng/L,均较对照组35.13±16.21 ng/L显著升高(P<0.05),激素敏感组和激素耐药组之间比较差异无显著性(P>0.05)。在激素治疗前激素敏感组和激素耐药组尿IL-6分别为14.19±4.87 ng/L和22.54±5.35 ng/L,均较对照组3.62±1.87 ng/L显著升高(P<0.05),而激素耐药组和激素敏感组比较差异有显著性(P<0.05)。激素治疗8周后,激素敏感组血、尿IL-6分别为41.68±18.94 ng/L和5.11±1.31 ng/L,均较治疗前显著降低(P<0.05),与对照组比较差异无显著性,而激素耐药组血、尿IL-6水平分别为115.53±24.65 ng/L,20.74±3.21 ng/L,与治疗前比较差异无显著性(P>0.05)。结论：血、尿IL-6对原发性肾病综合征激素敏感性的判断和预后估计有一定的参考价值。     

激素中长程疗法治疗70例肾病综合征的疗效观察和远期随访

1978～1992年收治原发性肾病综合征70例,男55例,女15例;年龄15个月～13岁半。临床分型:单纯型59例,肾炎型11例。初治52例,再治18例。强的松用量按1.5～2.0mg/kg·d。治疗2周尿蛋白转阴后续用8周,尿蛋白持续阴性为激素敏感共50例(71.4％);尿蛋白有减少,但仍在(++)～(+)为部分敏感,共4例(5.7％);虽浮肿减轻尿蛋白始终在(+++)以上为不敏感共16例(22.9％)。不敏感组中肾炎型占9例,单纯型占7例。单纯型加用CTX4例,加用CB3例,待尿蛋白转阴后,强的松改为2mg/kg·d隔日治疗,逐渐减     

激素、钙调磷酸酶抑制剂和吗替麦考酚酯三联用药治疗激素耐药型肾病综合征患儿的疗效和安全性

背景少数病初即表现为激素耐药型的儿童原发性肾病综合征,临床治疗较困难,对于无明确遗传因素证据患儿,临床多采用激素联合一种或多种免疫抑制剂进行治疗,但目前尚无统一的药物添加原则或规范的治疗方案。目的观察激素联合钙调磷酸酶抑制剂、吗替麦考酚酯对初治激素耐药型肾病综合征患儿的治疗效果和安全性。设计回顾性非随机对照研究。方法纳入2014年1月至2020年12月北京大学第一医院儿科收治的初治激素耐药型肾病综合征患儿,除外遗传因素后,分为A组(激素+钙调磷酸酶抑制剂+吗替麦考酚酯三联治疗,三种药按先后顺序依次添加)、B组(激素+钙调磷酸酶抑制剂+吗替麦考酚酯三联治疗,激素联合钙调磷酸酶抑制剂治疗3个月以上无效,改为激素联合吗替麦考酚酯治疗3个月以上仍无效,最后三者联用)、C组(钙调磷酸酶抑制剂+吗替麦考酚酯治疗,因类固醇性糖尿病或青光眼停用激素)和D组(激素+钙调磷酸酶抑制剂+美罗华),比较各组的治疗效果。主要观察指标尿蛋白转阴时间、尿蛋白阴性时间百分比、平均复发次数。结果39例患儿纳入分析,A、B、C、D组分别为16、8、3、12例。A、B、C、D组尿蛋白转阴率分别为75.0%(12/16)、75.0%(6/8)、100%(3/3)和75.0%(9/12),组间比较差异无统计学意义(P>0.05)。A、D组平均尿蛋白转阴时间低于B、C组,尿蛋白阴性时间百分比高于B、C组,差异均有统计学意义(P<0.05)。A和D组间平均尿蛋白转阴时间和尿蛋白阴性时间百分比差异均无统计学意义(P>0.05)。各组患儿用药期间未报告药物相关不良反应。结论对于少数原发性激素耐药型肾病综合征患儿,激素联合钙调磷酸酶抑制剂、吗替麦考酚酯三联治疗有较好的效果。     

肾病综合征患儿尿视黄醇结合蛋白测定及分析

临床资料 :1999年 8月至 2 0 0 0年5月本院收治的 5 2例肾病综合征 (NS)患儿 ,诊断符合 1981年全国小儿肾脏病科研协作组制定的标准。单纯性肾病组 2 8例 ,肾炎性肾病组 2 4例。其中 ,极期组 17例 ,初次发病 ,未用激素治疗 ;未缓解组 13例 ,应用激素 4～ 8周 ,尿蛋白仍 (+)～ (+++) ;缓解组2 2例 ,经治疗尿蛋白转阴 2周以上。正常对照组 2 3例 ,系健康查体儿童。方法 :取晨尿 10mL ,采用双抗体夹心ELESA法测定尿视黄醇结合蛋白(RBP) ,试剂购自上海德波生物技术公司 ,同时测定尿 β2 微球蛋白 (β2 MG)、尿白蛋白 (Alb)和尿IgG。统…     

甲基泼尼松龙冲击治疗小儿激素敏感肾病综合征的临床随机对照研究

目的客观评价甲基泼尼松龙(甲泼尼龙)冲击与传统的口服足量泼尼松对小儿肾病综合征的疗效及不良反应,为临床应用提供可参考的证据。方法以原发性肾病综合征、激素敏感复发或初发初治的病例为研究对象,采用分层区组随机有效对照,开放试验,以甲泼尼龙冲击为治疗组,口服泼尼松治疗为对照组,比较两者的疗效及不良反应。结果甲泼尼龙组13例患儿中2例冲击治疗期间因感染停止治疗,11例完成治疗并缓解,尿蛋白转阴时间为3～7d,中位时间5d;泼尼松组11例患儿均完成治疗并缓解,尿蛋白转阴时间为5～28d,中位时间8d(P=0.0008);甲泼尼龙冲击治疗组患儿2周内的不良反应有感染、情绪改变、消化道症状和浮肿加重,不良反应的发生率较泼尼松组高;缓解后3个月内的再次复发两组无差别;治疗后3个月的体质量和皮下脂肪厚度,泼尼松组较治疗前增加,身高和骨密度两组治疗前后均无差别;全部患儿的肾上腺皮质功能治疗后2周均受到抑制,至治疗后3个月绝大多数仍未恢复。结论与口服足量泼尼松治疗相比,甲泼尼龙冲击治疗显示了可使激素敏感的原发性肾病综合征患儿尿蛋白更快转阴的趋势,但治疗期间可能出现感染等不良反应;甲泼尼龙冲击治疗不能减少缓解后3个月内的再次复发,对患儿体质量和皮下脂肪分布的影响可能更小。     

强的松中长程治疗小儿肾病的疗效观察

本文总结了47例小儿肾病综合征的激素中长程治疗方法,发现单纯性肾病对激素有效应达87.2%,于每日用药4周内尿蛋白即可转阴。疗程结束时44例缓解;占93.6%,随访6月～3年,有9例复发,占27.6%,经第二次治疗,皆又获得缓解。对每日应用激素6～8周尿蛋白尚不能转阴的病例可考虑加用免疫抑制剂.     

肾病综合征尿视黄醇结合蛋白与激素效应的关系

尿视黄醇结合蛋白 (简称ＲＢＰ)可敏感地反映肾近曲小管的损伤。肾病综合征 (ＮＳ)时尿ＲＢＰ可升高。为探讨ＮＳ时尿ＲＢＰ对激素效应的关系 ,本文对 6 4例原发性ＮＳ尿ＲＢＰ进行检测 ,并与激素治疗效应作了比较 ,报告如下。对象和方法一、对象　 6 4例平均年龄 6 .3ａ(1.8～ 15ａ) ,男 5 0例 ,女14例。平均病程 6 .2个月 (1个月～ 1.3ａ)。单纯性肾病 5 3例 ,肾炎性肾病 11例。二、分组　入院后均予足量强的松 1.5ｍｇ/ (ｋｇ·ｄ)口服 ,治疗 8周 ,按疗效分为Ａ ,Ｂ两组 :Ａ组为激素效应组 33例 (尿蛋白转阴 ,生化恢复正常 )。Ｂ组为激素…     

激素中长程疗法治疗小儿原发性肾病的临床观察

根据南京会议提出的激素中长程疗法，本文介绍23例小儿原发性肾病综合征临床情况。资料及方法一、对象男16例，女7例，年龄1岁半～14岁，初发19例，复发4例。来院前病程＜6月18例，～1年4例，＞1年1例。单纯性好病18例，肾炎性肾病5例，随访时间为6月～5年。二、治疗方法强的松1．5～2mg／（kg·d），口服4～8周尿蛋白转阴后改隔日疗法，即原最隔日晨顿服，以后每隔二周减去隔日量5mg或2．5mg，根据临床分型，单纯性肾病用药至少6个月，肾炎性肾病9个月以上，才能逐渐停用。每日用药4周内尿蛋白转阴者16例，8周内转阴者5例，隔日疗法用药半…     

甲基泼尼松龙冲击疗法治疗小儿肾病综合征复发疗效观察

目的 初步分析甲基泼尼松龙冲击治疗激素敏感的原发性肾病综合征的疗效和不良反应。方法 对2000～2004年应用甲基泼尼松龙冲击治疗的激素敏感的11例原发性肾病综合征住院患儿临床资料进行回顾性分析，并与既往相似情况下口服足量泼尼松治疗的效果进行自身对照分析。结果 11例中男9例，女2例；甲基泼尼松龙冲击时年龄3～12岁，平均（7．5±3．1）岁；病程3～38个月，平均（15．1±10．4）个月；3例为不频复发，8例为频复发。甲基泼尼松龙冲击治疗1疗程后11例中尿蛋白转阴10例，尿蛋白转阴时间为1～18d，平均（7．4±4．4）d；其中7例较口服足量泼尼松治疗尿蛋白转阴的时间缩短，但对前后2次复发时病情基本相同的6例患儿尿蛋白转阴时间进行配对样本t检验，差异无统计学意义。甲基泼尼松龙冲击治疗期间，出现不良反应6例。结论 与口服足量泼尼松治疗相比，甲基泼尼松龙冲击治疗对小儿激素敏感的原发性肾病综合征的疗效尚待定论，且治疗期间可能出现不良反应，需慎重使用，其有效性及安全性有待进一步作前瞻性随机对照研究证实。     

Resilience in Families with a Child with Cancer

The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.     

Colonization with Clostridium difficile in Children with Cancer

Objective

Clostridium difficile is a gram-positive, anaerobic, spore-forming bacillus. Usually it does not cause disease unless a patient who is colonized with toxin-producing strains has been treated with antibiotics, particularly those that change the anaerobic flora of the large intestine.

Methods

We investigated in a prospective study intestinal colonization of C. difficile and its toxins in children with malignancy that used different antibiotics and cytotoxic drugs.

Findings

One hundred fifty-two patients were included in this prospective study. Stool samples were obtained within the first 48 hours after admission and cultured for C. difficile; cytopathic effect of C. difficile was detected on HELA cells, also ELISA test was performed for detection of toxins A and B. 25% of patients had positive culture for C. difficile; 36/38 (92%) revealed positive cytopathic effect on HELA cells. No significant relation was found between age, gender, history of antibiotic consumption and C. difficile positive culture and cytopathic effect on HELA cells. The only relation was seen between cotrimoxazol usage and cytopathic effect on HELA cells (P=0.03).

Conclusion

Although the rate of C. difficile colonization (25.6%) and toxigenic strains (23.7%) in admitted children in hematologic ward is high, the rate of ELISA positive test for toxin A+B was not correspond with culture and cytopatic effect on HELA cell. With respect to sensitivity and specificity of ELISA test, possibility for existence of toxin C with cytopathic effect is high in this type of patients.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Juvenile rheumatoid arthritis with myelofibrosis with myeloid metaplasia

Myelofibrosis with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoesis and varying degree of myelofibrosis. It may be idiopathic or secondary to a large number of conditions. Here is a rare case of myelofibrosis occurring in a patient with juvenile rheumatoid arthritis.     

Therapeutic experience with hepatoblastoma associated with trisomy 18

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease‐free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.