标准化尘螨特异性皮下免疫治疗尘螨过敏性哮喘伴变应性鼻炎患儿的长期随访研究

目的：探讨标准化尘螨特异性皮下免疫治疗(SCIT)对尘螨过敏性哮喘伴变应性鼻炎患儿远期疗效的影响。 方法：本文为干预效果的长期随访的病例系列报告。对尘螨过敏性哮喘伴变应性鼻炎患儿,开始接受标准化SCIT治疗（T0）至疗程≥30个月治疗结束（T1）,在T0、T1、T2（T1后的3年）和T3（T1后的6年）,均在哮喘专科门诊或通过电话随访,分别以哮喘症状评分（ASS）、鼻炎症状评分（RSS）、鼻炎与哮喘症状总评分（TSS）、药物评分（TMS）、症状药物总评分（SMS）、视觉模拟量表(VAS)评价和病情改善自我评价量表进行病情估计,ASS、RSS、TSS、TMS评估时点为晚近4周内情况、VAS和病情改善自我评价为患儿或其家长自我感受评估时点为晚近1个月的情况。 结果：2006年4月至2011年6月在哮喘专科门诊诊断为尘螨过敏性哮喘伴变应性鼻炎患儿、且接受了标准化SCIT治疗≥30个月,T1时点56例,男41例,女15例,平均年龄7.1（5~12）岁。均回顾性收集到T0时点病情评估指标ASS、RSS、TSS和VAS,并计算TSS和SMS。随访至T2时点有51例（男38例）和T3时点有45例（男33例）采集到了本文全部病情评估指标。T1时点不同病情评估指标较T0时点差异均有统计学意义。反映病情评估的2个组合指标（TSS和SMS）前后相临时点差异均有统计学意义。病情改善自我评价量表中,变应性鼻炎与哮喘T1时点（18.5% vs 75.0%）、T2时点（31.2% vs 84.0%）、T3时点（39.5% vs 80.0%）比较差异均有统计学意义,病情改善自我评价量表病情评估变应性鼻炎明显差于哮喘。T3时点女生的RSS、TSS、SMS明显低于男生,差异有统计学意义。 结论：标准化SCIT治疗能使尘螨过敏哮喘伴变应性鼻炎患儿的哮喘、鼻炎症状明显减轻,用药减少,VAS评分降低,在停止治疗后的6年仍能维持长期疗效。女性患儿比男性患儿在鼻炎哮喘症状以及症状用药评分方面疗效更加显著。     

发热危险度评分在门诊无明显感染灶发热婴幼儿中的应用研究

目的评估发热危险度评分对指导门诊处理无明显感染灶急性发热儿童的临床价值。方法根据发热时间、生活状况、每日退热药应用次数、毛细血管再充盈时间、外周血白细胞计数、中性粒细胞计数、C反应蛋白值建立发热危险度评分表,前瞻性评估并随访体温≥38℃无明显感染灶的3个月~5岁急性发热儿童839例,计算该评分的灵敏度、特异度及阳性和阴性预测值。结果 839例患儿中发热危险度评分0分者94例,均无严重疾病,皆居家治疗,其中使用口服抗生素24例(25.54%);评分1~3分者474例,诊断严重疾病141例,留观或住院治疗112例(23.63%),其中使用抗生素248例(52.32%);评分≥4分者271例,诊断严重疾病167例,危重症17例,全部住院或留观治疗,其中使用抗生素250例(92.25%)。0~3分组和≥4分组严重疾病发生率、抗生素使用率差异均有统计学意义(P均0.01)。发热危险度评分≥1分对严重疾病的诊断灵敏度为100%,特异度17.70%,阴性预测值100%,阳性预测值41.34%;评分≥4分对严重疾病的诊断灵敏度为100%,特异度69.10%,阴性预测值100%,阳性预测值6.27%。结论发热危险度评分可为门诊3个月~5岁无明显感染灶急性发热儿童的病情判断提供参考。     

血浆1,3-β-D葡聚糖检测对儿童侵袭性真菌感染诊断价值

目的 评价血浆1,3-β-D葡聚糖（BG）检测（G试验）对儿童侵袭性真菌感染（IFI）的诊断价值。方法 回顾性分析2008年1月至2011年8月在首都医科大学附属北京儿童医院住院,抗生素应用时间>10 d,并行G试验患儿的临床资料,排除拟诊IFI者。根据IFI诊断标准,将研究对象分为IFI组和非IFI组。G试验测定采用GKT-5M Set动态真菌检测试剂盒,血浆BG浓度≥10 pg·mL-1判定G试验阳性。采用四格表计算G试验诊断IFI的敏感度、特异度、阳性预测值和阴性预测值。对G试验结果进行受试者特征工作曲线（ROC曲线）分析,并计算曲线下面积。结果 研究期间共有525例患儿行G试验,排除拟诊IFI者129例,最终396例患儿纳入分析。IFI组43例,非IFI组353例。IFI组G试验阳性31/43例,阳性率为72.1%;阴性12/43例,假阴性率为27.9%。非IFI组G试验阳性48/353例,假阳性率为13.6%。G试验诊断IFI的敏感度、特异度、阳性预测值和阴性预测值分别为72.1%、 86.4%、39.2%和96.2%。根据G试验结果绘制ROC曲线,曲线下面积为0.815（95%CI：0.732~0.898）。结论 G试验对儿童IFI具有中等诊断价值。适当提高诊断界值或连续监测可很大程度消除假阳性。     

结核菌素皮试和全血γ干扰素测定试验的儿童结核感染诊断应用研究

目的 评价结核菌素（PPD）皮试和全血γ干扰素（IFN-γ）测定试验诊断儿童结核病的准确性。方法 选择2006年7月至2010年4月首都医科大学附属北京儿童医院住院临床诊断结核和呼吸系统疾病的患儿为研究对象。根据患儿所暴露的结核感染危险因素分为5组：A组：无结核病密切接触史的非结核病的呼吸系统疾病患儿;B组：有活动性结核病患者密切接触史的非结核病的呼吸系统疾病患儿;C组：无结核病密切接触史的临床诊断结核病患儿;D组：有活动性结核病患者密切接触史的临床诊断结核病患儿;E组：病原学或病理学确诊的活动性结核病患儿。患儿于入院当日行PPD皮试,入院后1~7 d采集外周静脉血行全血IFN-γ测定。以敏感度、特异度、阴性预测值、阳性预测值和似然比评价PPD皮试和全血IFN-γ测定对结核病的诊断价值。结果 125例患儿进入分析。A组40例,B组11例,C组29例,D组27例,E组18例。①PPD皮试取硬结≥10 mm为阳性判断标准时,诊断结核病的敏感度为77.0%,特异度为70.6%;取硬结≥15 mm为阳性判断标准时,诊断结核病的敏感度为50.0%、特异度为80.2%;全血IFN-γ测定的敏感度为85.1%、特异度为94.1%。②PPD皮试取硬结≥10 mm为阳性判断标准诊断结核病时,<3岁患儿PPD皮试的敏感度和特异度均显著低于≥3岁患儿,城区和郊区患儿的敏感度和特异度接近;全血IFN-γ测定诊断结核病的敏感度和特异度在不同年龄、居住地间差异无统计学意义。③全血IFN-γ测定阳性率与结核感染暴露因素的相关性优于PPD皮试（取硬结≥10或15 mm为阳性判断标准时）。结论 潜伏结核感染筛查时以硬结≥15 mm作为PPD皮试阳性判断标准,可提高诊断的特异度;临床疑似结核病的诊断以硬结≥10 mm作为PPD皮试阳性判断标准,可提高诊断的敏感度。全血IFN-γ测定诊断结核病的敏感度和特异度均较好。     

应用ROC评价Alvarado改良评分联合高频超声回盲部定位法对小儿不同病理分型阑尾炎的诊断价值

目的应用受试者工作特征曲线(ROC)评价Alvarado改良评分联合高频超声回盲部定位法对小儿不同病理分型阑尾炎诊断的应用价值。方法选择2014年10月至2016年1月在本院收治的阑尾炎患儿158例,经手术病理证实为阑尾炎者为阳性诊断,术中未发现阑尾有炎症或发现其它病变者为阴性诊断。术前对患儿单独应用Alvarado改良评分法,单独应用高频超声回盲部定位法以及两种方法联合检查对患儿进行评价。分别计算Alvarado改良评分法、高频超声回盲部定位法和联合诊断3种方法诊断阑尾炎的准确率、敏感度和特异度,并绘制ROC曲线评价3种方法对阑尾炎的诊断价值。采用Kappa检验评估超声对于小儿不同病理分型阑尾炎的诊断结果与金标准的一致性。结果经过手术的158例阑尾炎患儿,Alvarado改良评分法诊断阑尾炎的准确率、灵敏度、特异度、假阳性率、假阴性率、阳性预测值和阴性预测值分别为85.44%、85.33%、62.50%、37.50%、14.67%、97.71%和18.51%。高频超声回盲部定位法诊断阑尾炎的准确率、灵敏度、特异度、假阳性率、假阴性率、阳性预测值和阴性预测值分别为91.14%、92.00%、75.00%、25.00%、8.00%、98.57%和33.33%。Alvarado改良评分联合高频超声回盲部定位法诊断阑尾炎的准确率、灵敏度、特异度、假阳性率、假阴性率、阳性预测值和阴性预测值分别为95.56%、97.3%、62.5%、37.5%、4.67%、97.99%和41.67%。ROC评价显示曲线下面积比较结果:二者联合方法(0.854)高频超声定位法(0.851)Alvarado改良评分法(0.762),二者联合方法较单一方法诊断准确(P0.05)。超声诊断小儿不同病理分型阑尾炎的结果与手术病理金标准一致性较好(Kappa=0.791,P0.01)。结论 Alvarado改良评分联合高频超声回盲部定位法有助于小儿阑尾炎不同病理分型的准确诊断,为临床做出正确的治疗方案提供重要依据。     

儿童克罗恩病10 例临床分析

目的 探讨儿童克罗恩病（CD） 的临床特点,并对其治疗进行初步探讨。方法 回顾性分析2005 年1 月至2013 年12 月间10 例确诊为CD 患儿的临床资料,包括其临床表现、内镜表现及内镜下黏膜活检病理特点、腹部B 超、实验室检查结果及治疗情况等。结果 患儿临床表现以腹痛、腹泻、便血为主,多伴有不同程度的生长发育障碍及营养障碍,肠外表现以发热为主。肠镜检查常见表现为非连续性阶段性黏膜充血、糜烂、黏膜呈鹅卵石样增生病变、黏膜溃疡等。腹部B 超检查示肠壁的不均匀阶段性增厚。病理表现主要为固有膜内较多淋巴细胞、嗜酸性粒细胞、浆细胞浸润,黏膜腺体部分萎缩。疾病缓解期CRP 明显低于急性发作期及疾病复发期（PP结论 儿童CD 的临床表现及实验室检查均缺乏特异性。ESR 及CRP 可作为疾病进展评估的指标。5-ASA 在儿童CD 的诱导和维持缓解中有一定的疗效。治疗转归与疾病初期PCDAI 评分的高低有一定的相关性。     

结核分枝杆菌多抗原蛋白芯片对儿童结核病的诊断价值

目的 探讨结核分枝杆菌(MTB)多抗原蛋白芯片对儿童结核病的诊断价值。方法 选取2005年4月至2006年4月在首都医科大学附属北京儿童医院诊断为结核病的住院患儿作为结核病组。选取同期住院，患感染性疾病，同时除外结核病的患儿作为非结核病组；选取体检纯化蛋白衍生物（PPD）试验阳性，既往无结核病史，无明显结核中毒症状，胸部影像学及腹部B超检查未见结核病灶的儿童作为结核感染组；选取同期行健康体检，卡疤试验阳性，无基础疾病，无结核接触史的儿童为健康对照组。各组留取血清标本。计算结核病组PPD试验阳性率及细菌学检查阳性率。应用MTB多抗原蛋白芯片同时检测标本中脂阿拉伯甘露糖（LAM）、相对分子质量16 000和38 000蛋白IgG抗体，通过蛋白芯片阅读仪判断结果，其中任意1种或1种以上抗体检测阳性，即判为蛋白芯片检测阳性。分别计算各组抗体检测阳性率，并计算该方法检测儿童结核病的灵敏度、特异度、阳性预测值和阴性预测值等指标。应用Logistic回归及χ2检验分析蛋白芯片检测阳性率与患儿年龄、病程、抗结核治疗时间、激素使用以及结核病类型的关系。结果 研究期间共纳入结核病组79例，非结核病组33例，结核感染组15例，健康对照组30例。蛋白芯片检测结核病组的阳性率为34.2%（27/79），低于PPD试验阳性率（84.8%，67/79），高于细菌学检查阳性率（12.7%，10/79）。在非结核病组阳性率为6.1%（2/33），结核感染组和健康对照组阳性率为0。蛋白芯片检测结核病组的灵敏度为34.2%，特异度为97.4%。阳性预测值93.1%，阴性预测值58.5%。Logistic回归发现蛋白芯片检测阳性率仅与病程相关，且随病程延长而阳性率升高。病程<1个月，蛋白芯片检测阳性率为18.8%（6/32），病程在～3个月，蛋白芯片检测阳性率为21.6%（8/37），病程>3个月，蛋白芯片检测阳性率为100%（13/13）。目前的统计结果尚未发现蛋白芯片检测阳性率与患儿年龄、抗结核治疗时间、激素使用情况有显著相关关系。结核病分型不同所造成的蛋白芯片检测阳性率的差异主要是由于其病程分布不同所致。结论 多抗原蛋白芯片对儿童结核病的诊断有一定价值，可作为一种诊断补充手段。但该方法灵敏度较低，不适用于早期诊断。     

英夫利西单抗治疗儿童克罗恩病的临床分析

目的 探讨英夫利西单抗(IFX)治疗儿童克罗恩病(CD)的临床疗效与安全性。方法 2011年1 月至2014 年12 月确诊为CD 的患儿13 例,在常规综合治疗基础上,加用IFX(5 mg/kg)治疗,回顾性分析所有患儿治疗前及治疗30 周后的临床表现、实验室指标及儿童克罗恩病活动指数(PCDAI)的变化,同时内镜下进行疗效评估。结果 所有患儿腹痛、腹泻、便血等临床症状在治疗后短期内迅速缓解并未见复发,治疗30 周后白细胞计数、红细胞沉降率、C 反应蛋白及PCDAI 较治疗前明显降低,血红蛋白较治疗前明显升高(P结论 IFX 治疗儿童CD 临床疗效显著,无明显的不良反应,可作为治疗儿童CD 的首选方案之一。     

他克莫司治疗儿童重症肌无力的疗效研究

目的 评价他克莫司治疗儿童重症肌无力（MG）的疗效及安全性。方法 采用他克莫司治疗28例MG儿童,运用重症肌无力日常生活（MG-ADL）量表于治疗1、3、6、9、12个月时评估他克莫司的疗效及安全性。结果 他克莫司治疗1、3、6、9、12个月时的MG-ADL绝对评分与基线水平相比均降低（P < 0.05）,且呈逐渐降低趋势。他克莫司治疗1、3、6、9、12个月时的有效率分别为59%、81%、84%、88%、88%。他克莫司治疗6、9、12、18个月时累计停用泼尼松4、13、14、15例。所有患儿治疗期间均未出现病情反复。主要不良反应/事件有无症状血镁降低（5例）;尿潜血阳性（1例）,后自行转为阴性;均未因不良反应/事件停药。1例患儿因反复呕吐自行停药。根据CYP3A5基因型分为慢代谢组（慢代谢型,n=19）、非慢代谢组（快代谢型+中间型,n=9）,慢代谢组患儿他克莫司剂量低于非慢代谢组（P < 0.05）,非慢代谢组他克莫司血药谷浓度低于慢代谢组（P < 0.05）,慢代谢组有效率高于非慢代谢组（P < 0.05）。结论 他克莫司治疗儿童MG疗效显著、安全性好,是需要免疫抑制剂治疗的MG患儿的良好选择。CYP3A5基因型对他克莫司使用剂量有一定的指导意义。     

他克莫司治疗儿童重症肌无力的疗效研究

目的 评价他克莫司治疗儿童重症肌无力（MG）的疗效及安全性。方法 采用他克莫司治疗28例MG儿童,运用重症肌无力日常生活（MG-ADL）量表于治疗1、3、6、9、12个月时评估他克莫司的疗效及安全性。结果 他克莫司治疗1、3、6、9、12个月时的MG-ADL绝对评分与基线水平相比均降低（P < 0.05）,且呈逐渐降低趋势。他克莫司治疗1、3、6、9、12个月时的有效率分别为59%、81%、84%、88%、88%。他克莫司治疗6、9、12、18个月时累计停用泼尼松4、13、14、15例。所有患儿治疗期间均未出现病情反复。主要不良反应/事件有无症状血镁降低（5例）;尿潜血阳性（1例）,后自行转为阴性;均未因不良反应/事件停药。1例患儿因反复呕吐自行停药。根据CYP3A5基因型分为慢代谢组（慢代谢型,n=19）、非慢代谢组（快代谢型+中间型,n=9）,慢代谢组患儿他克莫司剂量低于非慢代谢组（P < 0.05）,非慢代谢组他克莫司血药谷浓度低于慢代谢组（P < 0.05）,慢代谢组有效率高于非慢代谢组（P < 0.05）。结论 他克莫司治疗儿童MG疗效显著、安全性好,是需要免疫抑制剂治疗的MG患儿的良好选择。CYP3A5基因型对他克莫司使用剂量有一定的指导意义。     

Inflammatory myofibroblastic tumor of the liver due to <Emphasis Type="Italic">Μycobacterium tuberculosis</Emphasis> in an immunocompetent girl

The case of a 9-year-old girl with a Mycobacterium tuberculosis inflammatory myofibroblastic tumor (IMT) of the left lobe of the liver is reported. The tumor was surgically excised and had histological features diagnostic of IMT, a positive Ziehl-Nielsen staining for acid-fast bacilli and a positive polymerase chain reaction for Mycobacterium tuberculosis. Surgical excision of the tumor followed by anti-tuberculosis treatment for 9 months resulted in full recovery. The patient had no apparent immune disorder, and there was no evidence of extrahepatic tuberculosis. These findings make this case exceptional because IMTs, due mostly to atypical mycobacteria, have been described only in immunocompromised patients.     

Orocaecal transit time in patients with Crohn disease

Orocaecal transit time (OCTT) using the lactulose hydrogen breath test was investigated in 16 patients (age 10–19.4 years) with active Crohn disease (CD). Disease activity was assessed by the paediatric CD activity index (PCDAI). OCTT was prolonged in all patients (mean 149.9 min, SD 32.7) relative to healthy age-matched controls (mean 56.9 min, SD 11.1). PCDAI was increased (median 48.8, range 32.5), indicating moderate to severe disease in all patients. A close correlation between OCTT and PCDAI (Spearmanr=0.90) was observed. Following nutritional therapy with a semi-elemental diet over a period of 6 weeks OCTT and PCDAI declined, OCTT becoming normal in 4 and PCDAI in 4/12 patients respectively. OCTT provides a sensitive, noninvasive method for the assessment and followup investigations in patients with CD.     

Pediatric Crohn Disease Activity Index: responsive to short-term change

BACKGROUND/AIMS: Despite documented feasibility, reliability, and validity, the Pediatric Crohn's Disease Activity Index (PCDAI) has yet to be demonstrated to be sensitive to change in the time frame of acute treatment trials. We evaluated short-term responsiveness and determined the minimal change in PCDAI score associated with a clinically meaningful improvement in disease activity. METHODS: Standardized effect size (SES) and standardized response mean (SRM) were calculated as measures of responsiveness among pediatric patients being treated for acute exacerbations of Crohn disease 1) in a regular clinical practice setting and 2) as part of a multicenter, randomized controlled trial (RCT). Receiver operating characteristic (ROC) curves were constructed to determine the minimal PCDAI score change associated with significant clinical improvement used as the gold standard in 1) physician global assessment of change and in 2) change in adult Crohn disease activity index (CDAI). RESULTS: Among responders, the SES and SRM of the PCDAI were 1.78 and 1.41 (95% CI: 0.89-1.92) and 2.10 and 1.95 (95% CI: 1.70-2.20) in the clinical practice setting and RCT setting, respectively. The optimal minimal PCDAI change score associated with clinically significant change in physician global assessment was determined to be -12.5 (sensitivity 83.3%, specificity 92.3%). In the RCT setting a change in PCDAI of -10 corresponded to a change in CDAI of not greater-than-or-equal 70 points. CONCLUSIONS: The PCDAI is responsive to improvement in disease activity in Crohn disease patients over a short interval. As such, the PCDAI is an appropriate instrument to use in pediatric acute treatment trials.     

Elemental versus polymeric enteral nutrition in paediatric Crohn's disease: a multicentre randomized controlled trial

AIM: To compare the efficacy and safety of an elemental and a polymeric diet as the primary therapy for active Crohn's disease in children. METHODS: In a randomized, non-blind, multicentre, controlled trial in Sweden, 16 children with Crohn's disease received Elemental 028 Extra (E028E) and 17 Nutrison Standard (NuS). Remission rates (Paediatric Crohn's Disease Activity Index (PCDAI) < 10 or a PCDAI decrease of 40% or 15 points of initial level) were compared at 6 wk. RESULTS: There was no significant difference between the two groups in remission rate at 6 wk (intent-to-treat analysis): E028E 11/16 (69%) and NuS 14/17 (82%) (p = 0.438). There was no difference in the decrease in PCDAI and CDAI between patients treated with E028E and those treated with NuS from 0 to 6 wk. Patients treated with NuS gained significantly more weight than patients treated with E028E (+2.5 kg; 95% CI 0.9, 4.1; p = 0.004), this difference remained when adjusting for maximum caloric intake per kilogram bodyweight (+2.9 kg; 95% CI 1.4, 4.5; p = 0.001). Concomitant disease, complications and side effects were seen in 5/33 patients (pyelonephritis, pneumonia, intraabdominal abscess, perianal abscess and borborygmi). CONCLUSION: E028E and NuS did not differ in terms of remission rate. Patients treated with NuS gained more weight than patients with E028E. Polymeric diet may be superior to elemental diet in the treatment of paediatric Crohn's disease where the primary aim is to increase the patient's weight.     

Correlation between CD4+ lymphocyte counts, concurrent antigen skin test and tuberculin skin test reactivity in human immunodeficiency virus type 1-infected and -uninfected children with tuberculosis.

BACKGROUND: HIV-infected children are at high risk of developing tuberculosis after infection by Mycobacterium tuberculosis. Emphasis is placed on tuberculin skin testing (TST) for diagnosing tuberculosis in children; however, its value in HIV-infected children is controversial. OBJECTIVES: To determine whether concurrent antigen testing and/or CD4+ lymphocyte counts help in the interpretation of the TST in children with tuberculosis. METHODS: Children eligible for the study were diagnosed as having tuberculosis on clinical criteria. CD4+ lymphocyte counts and delayed-type hypersensitivity (DTH) test, using the CMI Multitest were performed when tuberculosis was diagnosed. RESULTS: One hundred thirty children were enrolled. Tuberculin reactivity was lower in HIV-infected children at all cutoff levels than in HIV-uninfected children (P < 0.0001). The positive predictive value of normal CD4+ lymphocyte counts in predicting tuberculin reactions of > or =5 mm (in HIV-1-infected) and > or =10 mm (in HIV-uninfected patients) were 50 and 80.3%, respectively (P < 0.0001). An intact DTH reaction to the CMI Multitest in predicting reactions of > or =5 mm and > or =10 mm to tuberculin in HIV-infected and -uninfected children were 55 and 76%, respectively (P < 0.001). Kwashiorkor was responsible for 53.3% of false-negative TST in HIV-uninfected children with normal CD4+ lymphocyte counts. CONCLUSION: TST is of limited value as an adjunct in diagnosing tuberculosis in HIV-infected children. CD4+ lymphocyte counts and concurrent DTH testing are not useful for predicting tuberculin reactivity in HIV-infected patients with tuberculosis.     

儿童结核性脑膜炎抗结核治疗中发生类赫反应的危险因素

目的:探讨儿童结核性脑膜炎(TBM)抗结核治疗中出现类赫反应(PR)的临床特征、危险因素及对预后的影响。方法:回顾性分析2013年1月至2018年12月在遵义医科大学附属医院儿科收治的TBM患儿的临床资料及随访情况;根据是否发生PR分组,采用单因素分析选出PR的影响因素后,引入多因素 Logistic回归分...     

Evaluation of the pediatric crohn disease activity index: a prospective multicenter experience

BACKGROUND AND OBJECTIVES: Longitudinal assessment of disease activity is necessary for studies of therapeutic intervention in children with Crohn disease. The Pediatric Crohn Disease Activity Index (PCDAI) was developed a decade ago for such a purpose, but it function has only been examined in a small number of studies with a limited number of patients. The primary objectives of the present study were to develop cut scores reflecting disease activity as determined by physician global assessment (PGA) and to evaluate the responsiveness of the PCDAI to changes in patient condition after therapeutic interventions. METHODS: Data were derived from a prospective database of newly diagnosed children with inflammatory bowel disease established in 2002 at 18 pediatric gastroenterology centers in the United States and Canada. At diagnosis, at 30 days and 3 months after diagnosis, and quarterly thereafter, children (<16 years of age) with Crohn disease had disease assessment performed by PGA and PCDAI. Disease management was provided according to the dictates of the attending gastroenterologist and not by predetermined protocol. RESULTS: 181 patients had concomitant PGA and PCDAI performed at diagnosis, and 95 of these had similar assessment at short-term follow up. Mean +/- SD PCDAI scores for mild, moderate, and severe disease by PGA at diagnosis were 19.5 +/- 10.4, 32.2 +/- 12.7, and 47.8 +/- 14.9, respectively (P < 0.001 for all comparisons). Mean +/- SD PCDAI for inactive disease after treatment was 5.2 +/- 5.4. Receiver operating characteristic (ROC) curve analysis suggested that: 1) activity of moderate/severe disease was best reflected by a PCDAI of > or = 30 points, 2) clinical response (moderate/severe disease improving to mild/inactive) was best reflected by a decrease in PCDAI of > or = 12.5 points, and 3) a PCDAI < 10 best reflected inactive disease. CONCLUSIONS: PCDAI scores accurately reflect disease activity as assessed by physician global assessment. A PCDAI score of > or = 30 has acceptable sensitivity and specificity to indicate disease of moderate/severe activity. A PCDAI decrease of 12.5 points or greater following therapeutic intervention accurately reflects a clinically significant response. The PCDAI is an appropriate tool for intervention trials in Crohn disease in children.     

Neutrophil CD64 expression: a sensitive diagnostic marker for late-onset nosocomial infection in very low birthweight infants

This study aims to evaluate the diagnostic utilities of four leukocyte surface antigens-two lymphocyte antigens (CD25 and CD45RO) and two neutrophil antigens (CD11b and CD64)-for identification of late-onset nosocomial bacterial infection in preterm, very low birthweight infants, and to define the optimal cutoff value for each marker so that it may act as a reference with which future studies can be compared. Very low birthweight infants in whom infection was suspected when they were >72 h of age were eligible for the study. A full sepsis screen was performed in each episode. IL-6, C-reactive protein, and leukocyte surface antigens (CD25, CD45RO, CD11b, and CD64) were measured at 0 (at the time of sepsis evaluation), 24, and 48 h by standard biochemical methods and quantitative flow cytometric analysis. The diagnostic utilities including sensitivity, specificity, and positive and negative predictive values of each marker and combination of markers for predicting late-onset neonatal infection were determined. One hundred twenty-seven episodes of suspected clinical sepsis were investigated in 80 infants. Thirty-seven episodes were proven infection. The calculated optimal cutoff values for CD25, CD45RO, CD11b, and CD64 were 3,100, 2,900, 10,450, and 4,000 phycoerythrin-molecules bound per cell, respectively. An interim analysis of data after 68 episodes suggested that CD25 and CD45RO were poor predictors of neonatal infection with sensitivity or specificity <75% during a single measurement. Thus, these two markers were excluded from further investigation. In the final analysis, CD64 has the highest sensitivity (95-97%) and negative predictive value (97-99%) at 0 and 24 h after the onset. The addition of IL-6 or C-reactive protein (0 h) to CD64 (24 h) further enhanced the sensitivity and negative predictive value to 100%, and has the specificity and positive predictive value exceeding 88% and 80%, respectively. Neutrophil CD64 expression is a very sensitive marker for diagnosing late-onset nosocomial infection in very low birthweight infants. If further validated, the use of CD64 as an infection marker should allow early discontinuation of antibiotic treatment at 24 h without waiting for the definitive microbiologic culture results. The quantitative flow cytometric analysis applied in this study could be developed into a routine clinical test with high comparability and reproducibility across different laboratories.     

Laryngeal tuberculosis in childhood

Laryngeal tuberculosis is an extremely rare condition in childhood, although probably less so in the underdeveloped world. We have described two cases treated concurrently in our wards. The first case showed features of a pharyngopalatotonsillar membrane, an exquisitely painful edematous pharynx and larynx and was initially sputum-negative for acid-fast bacilli. This presentation fits the hematogenous form of disease and stresses that: laryngeal tuberculosis is not confined to cases of far advanced pulmonary tuberculosis; tuberculosis should enter the differential diagnosis of pharyngeal, tonsillar and palatal lesions (especially membranoulcerative lesions); and there is a common association between laryngeal and abdominal tuberculosis. Her treatment included a 1-month course of steroids and to date (12 months after onset) she shows no signs of complications. The edematous form of laryngeal tuberculosis may be yet another indication for the use of steroids in tuberculosis. Our second patient presented with prolonged chest symptoms, initial positive sputum for acid-fast bacilli and localized granulomatous laryngeal disease, features of far advanced "adult" disease and bronchogenic laryngeal spread. Laryngeal tuberculosis usually responds rapidly to antituberculosis chemotherapy. This was clinically and endoscopically confirmed in both our cases.