系统性红斑狼疮患儿霉酚酸酯血药浓度与疾病活动、疾病复发及不良反应的关系

目的探讨吗替麦考酚酯(MMF)治疗儿童系统性红斑狼疮(SLE)的霉酚酸血药浓度时间曲线下面积(MPA AUC)与疾病活动、疾病复发及药物不良反应的关系。方法回顾分析2017年1月至2019年6月行MMF血药浓度检测的SLE患儿的临床资料。纳入患儿近半年的治疗方案为激素联合MMF、羟氯喹,MMF使用剂量为20~40 mg/(kg·d)。根据患儿SLE疾病活动度评分(SLEDAI)、是否复发以及有无不良反应分组,比较各组间MPA AUC水平;根据MPA AUC水平分为3组,比较三组间疾病活动度及不良反应情况。结果本研究共纳入81例患儿,男20例、女61例,中位年龄为13.1(11.0~14.4)岁。根据SLEDAI-3个月分组,非活动组67例,活动组14例。与非活动组相比,活动组的起病年龄偏小,白蛋白和肌酐水平较低,估算肾球滤过率较高,差异均有统计学意义(P<0.05)。非活动组随访3个月及6个月的MPA AUC0-12h均高于活动组,差异有统计学意义(P<0.05)。低、中、高AUC三组之间随访3个月以及6个月后的SLEDAI评分差异均有统计学意义(P<0.05),均以低AUC组的SLEDAI评分为较高。复发组MPA AUC水平低于未复发组,差异有统计学意义(P=0.001)。利用ROC曲线预测患儿复发时MPA AUC的截断值为43.03 mg/(h·L),曲线下面积为0.89(P=0.001),灵敏度100%,特异度69.8%。结论MMF治疗儿童SLE,监测MPA AUC有重要意义。MPA AUC与疾病活动度呈负相关,血药浓度43.03 mg/(h·L)以上可维持SLE在低活动度水平及减少疾病复发,过高浓度对疾病活动并无改善。     

合并过敏性鼻炎对儿童系统性红斑狼疮病情及治疗的影响

目的探讨过敏性鼻炎(AR)及其干预对儿童系统性红斑狼疮(JSLE)病情和治疗用药的影响。方法收集96例确诊的JSLE患儿临床资料,按是否合并过敏性鼻炎或其他过敏性疾病分为过敏性鼻炎组(44例)和非鼻炎过敏组(20例),以及非过敏组(32例),过敏性鼻炎组又随机分为鼻炎干预组和非干预组(各22例)。所有患儿均进行规范JSLE治疗。比较各组患儿的SLE疾病活动性指数(SLEDAI)和激素、免疫抑制剂用药情况。结果合并过敏性鼻炎组和非鼻炎过敏组的JSLE患儿治疗前的SLEDAI评分、糖皮质激素日累积剂量、免疫抑制剂用药种数均高于非过敏组(P0.05),而过敏性鼻炎组和非鼻炎过敏组间的差异无统计学意义(P0.05)。治疗1个月后,JSLE合并过敏性鼻炎患儿干预组的SLEDAI评分及激素日累积剂量低于非干预组(P0.05),两组患儿免疫抑制剂用药种数的差异无统计学意义(P0.05);治疗3个月及6个月后,干预组的SLEDAI评分、激素日累积剂量、免疫抑制剂使用种类均少于非干预组(P0.05)。结论 JSLE合并AR等过敏性疾病可能对SLE病情及治疗产生不利影响;针对AR给予干预有利于病情控制。     

硫酸羟氯喹治疗肺表面活性蛋白C基因突变致婴儿间质性肺病1例并文献复习

目的：报道硫酸羟氯喹治疗肺表面活性蛋白C基因（SFTPC）突变致婴儿间质性肺病的疗效,提高对该病诊断和治疗的认识。方法：总结分析1例SFTPC突变致婴儿间质性肺病的临床特点、诊断过程和硫酸羟氯喹的疗效,并进行文献复习。结果：患儿女,2月龄,因“生后反复咳嗽伴气促2个月”于2015年9月9日就诊。患儿在新生儿期即发生呼吸窘迫,持续无法离氧。影像学示肺部渗出,病原学检查均阴性,常规抗感染治疗无效,否认肺部疾病家族史。基因检测发现SFTPC基因外显子4有1个杂合错义突变位点（c.T337C:p.Y113H）,目前尚无报道。患儿13月龄时开始硫酸羟氯喹治疗,治疗6个月后,呼吸窘迫、生长发育情况和胸部CT影像学表现明显改善。在PubMed、Web of Science、中国知网、维普数据库和万方数据库中检索SFTPC基因突变的间质性肺病,检索时间均从建库至2016年12月1日,共检索到相关文献12篇,均为英文文献。总结包括本文1例患儿在内的51例SFTPC基因突变致间质性肺病病例使用硫酸羟氯喹的治疗情况,随访0.3~15.8年,其中单用硫酸羟氯喹治疗的有12例,均取得良好疗效,未提及或未发现药物不良反应;全身糖皮质激素合用硫酸羟氯喹治疗39例,33例（84.6%）有效,2例（5.1%）无改善,4例（10.3%）恶化（1例死亡）。结论：对于SFTPC基因突变的婴儿间质性肺病,早期发现和早期诊断很重要,及早使用硫酸羟氯喹治疗可以改善临床症状、体征和生长发育情况,减少终末肺的发生。     

托吡酯添加治疗儿童癫癎临床疗效观察

目的　观察托吡酯 (TPM )添加治疗儿童癫疒间 的疗效。方法　采用不同剂量 (3 2～ 12 0mg·kg-1·d-1)的托吡酯添加治疗 35例儿童部分性和 (或 )全身性癫疒间(基线发作次数的中位值 6次 /月 )的开放性研究。TPM治疗的平均时间为 (44 7± 14 2 )d (范围 2 8～ 5 82d) ,TPM持续治疗 3个月和 6个月的平均剂量为 6 75mg·kg-1·d-1。结果　 >70 %的患儿发作频率减少≥ 5 0 % ,38% (13/34)的患儿无发作持续 3个月以上 ,4 3%(14 /32 )的患儿无发作持续 6个月以上。接受不同剂量TPM的治疗反应大致相同 (P >0 0 5 )。而最常见的不良反应为中枢神经系统的表现。经过 1 5年的治疗 ,2 6 %的患儿因不良反应和发作未得到适当的控制而未继续用药。结论　TPM添加治疗儿童癫疒间具有良好的耐受性和安全性 ,并可作为基础抗癫疒间 药物失败后长期控制癫疒间 发作的广谱抗癫疒间药。     

利妥昔单抗治疗儿童系统性红斑狼疮12例临床研究

目的探讨利妥昔单抗(Rituximab RTX,商品名:美罗华)治疗儿童系统性红斑狼疮(SLE)的疗效及安全性,为传统治疗不耐受和疗效不佳者提供新的治疗途径。方法采用罗氏药厂生产的RTXCD20单克隆抗体,静脉注射。初始剂量188mg/m(体表面积),2周后进行以375mg/m2第2次注射。每次注射前30min给地塞米松25mg,异丙嗪10mg各1次,预防药物过敏。结果 12例SLE患儿用药后4周通过外周血细胞流式仪检测10例(85.71%)B淋巴细胞去除为0,并持续6～8个月逐渐回升。IgG、IgM和IgA血浆检测与用药前差异无统计学意义。12例SLE治疗后1年,临床表现及体征通过SLE活动指数评分由治疗前的(16.0±2.95)分下降到治疗后的(8.67±1.83)分。抗核抗体(ANA)、抗双链DNA抗体(ds-DNA)、补体C3、C4均有不同程度改善。6例狼疮性肾炎(LN)患儿用药6个月,尿蛋白明显改善;4例用药6个月后,血浆肌酐和尿素氮恢复正常。1例合并严重肺部感染。结论 RTXCD20单克隆抗体治疗儿童SLE尤其是重症和LN患儿临床效果较为明显。RTX为传统泼尼松和环磷酰胺治疗不耐受和临床效果不佳者提供了新的治疗途经,但其合并严重感染问题不可忽视。RTX单抗能否替代传统的儿童SLE治疗药物,仍需进一步深入研究。     

胆道闭锁术后大剂量类固醇的疗效及安全性

目的探讨胆道闭锁Kasai术后大剂量类固醇应用的有效性和安全性。方法回顾分析复旦大学附属儿科医院1997年1月~2003年10月胆道闭锁术后52例患儿药物治疗方法及随访资料,比较地塞米松短期冲击治疗(A组)与大剂量甲基强的松龙长期用药(B组)在自体肝生存率、胆汁清除情况,反流性胆管炎发生率及激素并发症等方面的差异。结果平均(32±3.23)个月的随访期内,患儿自体肝总生存率为71.14%。A组自体肝1年生存率为74.75%,2年生存率为68.52%,B组自体肝1年生存率84.97%,2年生存率为67.97%,两组生存率曲线差异无显著性意义(P>0.05)。两组患儿术后6个月黄疸清除率分别为44.0%(11/25)和74.48%(20/27),差异有显著性意义(P<0.05)。A组术后胆管炎发生率为56.0%(14/25),B组为25.92%(7/27)(P<0.05)。B组中1例患儿术后1个月激素治疗疗程中出现消化道出血。结论较短期冲击治疗相比,大剂量长期使用类固醇(甲基强的松龙)可以改善胆道闭锁患儿的术后近期的胆汁引流,减少胆管炎的发生率,但并不能延长自体肝的生存年限。激素使用过程中的并发症的存在影响着其安全性,尚需进一步观察和评价。     

114例神经母细胞瘤患儿13-顺式维甲酸维持治疗用药安全性观察

目的评价13-顺式维甲酸(13-CRA)的安全性,为临床安全有效地使用该药提供依据。方法连续纳入应用维甲酸维持治疗神经母细胞瘤(NB)患儿,采用问卷调查法收集NB患儿在13-CRA用药期间,发生的任何不良事件,判断不良事件与药物的关系,并计算不良反应发生率。结果2018年3月-12月期间,共纳入114名NB患儿,男65例,女49例,年龄11个月～14岁,中位年龄4岁5个月。其中高危组94例,中危组20例,原发瘤灶为腹膜后及肾上腺区76例,后纵膈38例。患儿服用13-CRA剂量160mg/(m~2·d),分两次,连用14d停14d为1个周期,共6～12个周期。总体药物不良反应发生率为100%,其中轻度60例(52.6%),中度36例(31.6%),重度18例(15.8%),无严重不良反应发生。不良反应主要涉及皮肤黏膜系统(114例,100%),其他可见于肌肉骨骼系统(14例,12.3%)、消化系统(18例,15.8%)、神经精神系统(9例,7.9%)及血液系统(6例,5.3%)。结论 13-CRA用于NB患儿维持治疗总体药物安全性较好,药物不良反应以皮肤干燥为主,当前剂量疗程下患儿可耐受。     

儿童不典型系统性红斑狼疮的诊断与治疗

儿童系统性红斑狼疮（SLE）临床症状较成人严重,病情发展快,预后差,但临床表现常不典型,且变化多端,常表现为发热,ESR增快和不能解释的多器官损害。对不典型儿童SLE,需谨慎对症处理。对仅有免疫学异常,而无临床症状,无血象、尿蛋白及尿红细胞异常患儿,可严密观察病情变化,暂不用药。对有免疫学异常和仅有面颊部红斑、而无脏器损害全身表现者,可试用不含氟的糖皮质激素局部治疗,或加用氯喹或羟氯喹。儿童不典型SLE在临床上必须引起重视,对于有上述不典型临床表现患儿需积极加强随访。     

抗中性粒细胞胞浆抗体与儿童风湿性疾病

目的探讨抗中性粒细胞胞浆抗体(ANCA)与儿童风湿性疾病包括系统性红班狼疮(SLE)、过敏性紫癜(HSP)及混合性风湿性疾病(MRD)肾损伤的关系.方法采用间接免疫荧光法(IIF)和酶联免疫吸附试验(ELISA)检测105例患儿于疾病急性期血清ANCA,检测并比较这些患儿肾损伤情况与ANCA的相关性.结果血清ANCA阳性率为SLE7/23(30.43%)、HSP15/64(23.44%)、MRD6/18(33.33%),其中核周型即P-ANCA18例(SLE5例、HSP9例、MRD4例);胞浆型即C-ANCA7例(SLE2例、HSP4例、MRD1例);未确定型即A-ANCA3例(HSP2例、MRD1例).比较患儿疾病急性期肾功能受损与血清ANCA水平的相关性,提示血清ANCA阳性患儿早期肾损伤率明显高于ANCA阴性患儿(P<0.05).结论本文提示ANCA可能对鉴别小儿风湿性疾病肾损伤具有重要意义,并可能成为该类疾病针对其肾损伤给予早期评价预后、选择积极治疗的重要免疫学血清指标.     

奥卡西平治疗儿童良性癫(癎)伴中央颞区棘波的临床观察

目的观察奥卡西平(OXC)治疗儿童良性癫癎伴中央颞区棘波(BECT)的疗效和安全性。方法用OXC治疗17例BECT患儿,分析单药治疗后1、2、4、6个月的疗效和不良反应。OXC起始剂量为5～10mg/(kg·d),每隔1周增加1次剂量5～10mg/(kg·d),维持剂量20～30mg/(kg·d)。结果本组总有效率为88.24%,服药6个月时累积控制率为70.59%,留存率为94.12%,均呈现较高比率。结论OXC治疗BECT的疗效明显,不良反应轻,安全性高。     

A nationwide surveillance study of rheumatic diseases among Japanese children

To estimate the number of children with rheumatic diseases, a questionnaire was distributed to the pediatrics department of 1290 hospitals in Japan in June 1994. From this survey, 1606 cases with juvenile rheumatoid arthritis (JRA), 906 cases with systemic lupus erythematosus (SLE), 320 cases with dermatomyositis/polymyositis (DM/PM), 28 cases with scleroderma (PSS), 70 cases with Sjögren's syndrome (Sjs), 93 cases with mixed connective tissue disease (MCTD), 25 cases with aortitis syndrome, 20 cases with polyarteritis (PN) and 51 cases with Behçet disease were reported. The crude annual incidence rates per 100 000 among the childhood population were estimated as JRA, 0.83; SLE, 0.47; DM/PM, 0.16; PSS, 0.01; Sjs, 0.04; MCTD, 0.05; aortitis syndrome, 0.01; PN, 0.01; and Behçet disease, 0.03. The present study reveals that there are more children with rheumatic diseases than are estimated from the reported cases in the literature and the number of children who are receiving Assistance Medical Costs Insurance covered by the Japanese government.     

Cardiac Operations for North American Children with Rheumatic Diseases: 1985–2005

Certain pediatric rheumatic diseases are known to affect the heart, sometimes requiring surgical intervention. The Pediatric Cardiac Care Consortium database was used to characterize cardiac surgical intervention among children with rheumatic diseases from 1985 to 2005. From this large database, the records for patients younger than 21 years who underwent cardiac surgery for any rheumatic disorder were extracted. The data collected included the type of procedure performed, the age at the time of the procedure, and the year the procedure was performed. The 261 pediatric patients identified underwent 361 cardiac surgical procedures for complications of rheumatic heart disease (RHD; 160 patients), neonatal lupus (NLE; 53 patients), Kawasaki disease (KD; 28 patients), systemic lupus erythematosus (SLE; 13 patients), and juvenile rheumatoid arthritis (JRA; 7 patients). Multiple procedures were performed for 23% of the patients. The most common procedures included pacemaker implantations among infants with NLE, coronary artery bypass grafts for KD primarily in 5- to 15-year-olds, and cardiac valve operations among adolescents with RHD, SLE, and JRA. Six perioperative deaths occurred. The proportion of annual pediatric cardiac surgical volume attributable to rheumatic diseases did not change during the period studied. Despite advances in their medical care, children with rheumatic diseases continue to sustain measurable morbidity and mortality due to the cardiovascular manifestations of their disease.     

儿童系统性红斑狼疮细胞膜DNA抗体间接免疫荧光检测研究

目的 间接免疫荧光法检测抗细胞膜DNA抗体(抗mDNA抗体),探讨抗mDNA抗体对儿童系统性红斑狼疮(SLE)的诊断价值.并与儿童SLE诊断的金指标抗dsDNA抗体和抗Sm抗体进行比较.方法 选取44例SLE患儿,30例非SLE自身免疫性疾病患儿作为对照组(包括幼年类风湿关节炎9例、幼年脊柱关节病13例、幼年皮肌炎4例、幼年干燥综合征2例、幼年血管炎2例).分别以早幼粒白血病细胞系HL60细胞和马疫锥虫为底物,用间接免疫荧光法检测抗mDNA抗体及抗dsDNA抗体;联合应用免疫双扩散法和免疫印迹法检测抗Sm抗体.结果 44例SLE患儿中34例抗mDNA抗体阳性,30例疾病对照组中抗mDNA抗体阳性者6例.抗mDNA抗体对儿童SLE诊断的敏感度和特异度分别为77.27%和80.00%,阳性预测值和阴性预测值分别为85.00%和70.59%.抗mDNA抗体、抗dsDNA抗体和抗Sm抗体对儿童SLE诊断的ROC曲线下面积分别为0.786、0.716和0.557,以抗mDNA抗体最高.在抗dsDNA抗体和抗Sm抗体阴性的儿童SLE患者中抗mDNA抗体的阳性率分别为68.00%(17/25)、79.49%(31/39).结论 抗mDNA抗体是一种诊断儿童SLE新的自身抗体,具有较高的敏感度和特异度,有助于抗dsDNA抗体和抗Sm抗体阴性的儿童SLE的诊断,检测方便,技术成熟,有一定的临床应用价值.     

Demographic features and compliance of children with allergic diseases receiving sublingual immunotherapy北大核心CSCD

目的探讨标准化舌下免疫治疗(sublingual immunotherapy,SLIT)过敏性疾病患儿的人口学特征,并分析依从性的影响因素。方法纳入江西省儿童医院2018年1月1日—2020年12月31日接受SLIT的1789例过敏性疾病患儿为研究对象,回顾性分析患儿的人口学特征和随访资料,分析SLIT的依从性及其影响因素。结果共1789例SLIT患儿,其中男性1271例(71.05%),女性518例(28.95%),年龄4~14岁;完全依从777例(43.43%);脱落1012例(56.57%),因自觉使用不便脱落354例(34.98%),因疗效欠佳脱落346例(34.19%),因症状改善自行停用253例(25.00%),因不良反应停用的59例(5.83%)。脱落主要集中在开始治疗后1~4个月(74.31%,752/1012)。女性患儿依从率低于男性患儿,患单一疾病者依从率低于患多种疾病者(P<0.05)。多因素分析结果显示,与男性相比,女性发生脱落的风险增加(OR=0.811,95%CI:0.658~0.998,P<0.05);相对于患多种疾病者,患单一疾病者发生脱落的可能性增加(OR=1.313,95%CI:1.005~1.715,P<0.05)。结论过敏性疾病患儿SLIT依从性不理想,与患儿性别、所患疾病数量等特征有关;主要脱落原因为自觉使用不便和疗效欠佳。     

Usefulness of antinuclear antibody testing to screen for rheumatic diseases

OBJECTIVE: To assess the usefulness of the indirect immunofluorescence antinuclear antibody test (FANA) using human laryngeal epithelial carcinoma cells as nuclear substrate, to screen for childhood rheumatic diseases. STUDY DESIGN: A review of all FANA tests performed on children at British Columbia's Children's Hospital between 7 March 1991 and 31 July 1995. RESULTS: FANA tests were positive at titres of 1:20 or greater in 41% of all subjects tested, and in 65% of all subjects in whom the diagnosis was obtained. FANA positivity occurred in 67% of those with a rheumatic disease, compared with 64% of those with a non-rheumatic disease (p = 0.4). More girls had high titre FANA positivity than boys independent of whether or not they had a rheumatic disease (p = 0.05). At a screening serum dilution of 1:40 a positive test has a sensitivity of only 0.63, and a positive predictive value of only 0.33 for any rheumatic disease. For systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), or overlap syndrome at a screening dilution of 1:40 the test has a very high sensitivity of 0.98, but a very low positive predictive value of only 0.10, the test having slightly better characteristics for boys than girls. CONCLUSION: Although a negative FANA test makes a diagnosis of SLE or MCTD extremely unlikely, a positive test even at moderately high titres of 1:160 or higher is found so frequently in children without a rheumatic disease that a positive result has little or no diagnostic value. It is suggested that a screening serum dilution of 1:160 or 1:320 would increase the usefulness of the test, by decreasing false positive tests, without significantly increasing false negative tests for SLE or MCTD, and would have the potential for considerable cost savings.     

Usefulness of antinuclear antibody testing to screen for rheumatic diseases

OBJECTIVE—To assess the usefulness of the indirect immunofluorescence antinuclear antibody test (FANA) using human laryngeal epithelial carcinoma cells as nuclear substrate, to screen for childhood rheumatic diseases.
STUDY DESIGN—A review of all FANA tests performed on children at British Columbia''s Children''s Hospital between 7 March 1991 and 31 July 1995.
RESULTS—FANA tests were positive at titres of 1:20 or greater in 41% of all subjects tested, and in 65% of all subjects in whom the diagnosis was obtained. FANA positivity occurred in 67% of those with a rheumatic disease, compared with 64% of those with a non-rheumatic disease (p=0.4). More girls had high titre FANA positivity than boys independent of whether or not they had a rheumatic disease (p=0.05). At a screening serum dilution of 1:40 a positive test has a sensitivity of only 0.63, and a positive predictive value of only 0.33 for any rheumatic disease. For systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), or overlap syndrome at a screening dilution of 1:40 the test has a very high sensitivity of 0.98, but a very low positive predictive value of only 0.10,the test having slightly better characteristics for boys than girls.
CONCLUSION—Although a negative FANA test makes a diagnosis of SLE or MCTD extremely unlikely, a positive test even at moderately high titres of 1:160 or higher is found so frequently in children without a rheumatic disease that a positive result has little or no diagnostic value. It is suggested that a screening serum dilution of 1:160 or 1:320 would increase the usefulness of the test, by decreasing false positive tests, without significantly increasing false negative tests for SLE or MCTD, and would have the potential for considerable cost savings.

     

Abnormalities in serum osteocalcin values in children with chronic rheumatic diseases

We studied bone mineral metabolism prospectively in 113 children with chronic rheumatic diseases (juvenile arthritis, systemic lupus erythematosus, and juvenile dermatomyositis) to determine the relationship of serum levels of osteocalcin to rheumatic disease activity and corticosteroid usage, and to determine, in part, the cause of osteopenia in this population. Disease activity was quantitated by historical, clinical, and serologic means and an activity score derived. The 113 children were divided according to the expression of their disease, which was active (group 1: mean score 3.42, mean erythrocyte sedimentation rate 28 mm/hr) or inactive (group 2: score 1.7, erythrocyte sedimentation rate 15 mm/hr) (p less than 0.02 group 1 vs group 2 for each value), or which remitted during the study (group 3). We found that serum levels of osteocalcin, but not those of ionized calcium, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D, and parathyroid hormone, were reduced in group 1 children even before corticosteroid therapy was employed. Children in both group 2 and group 3 had normal osteocalcin levels despite the use of corticosteroids. The reduced levels of osteocalcin were predictive of a reduction in bone mass measured by photon absorptiometry in 16 of 19 children so studied. We conclude that skeletal abnormalities that result in a reduced bone mass occur in the clinical course of the majority of children with active chronic rheumatic diseases, are associated with reduced osteocalcin levels, and are not related to the use of corticosteroids. Serum osteocalcin levels may be a sensitive marker for reduced osteoblast activity and bone formation in children with chronic rheumatic diseases.     

中国儿童间质性肺疾病的临床研究

目的 了解中国儿童间质性肺疾病( ILD)的病因谱.方法 以2009年来自全国11家医院就诊的93例儿童ILD患者为研究对象,采用统一登记表格,详细记录其病史、症状、体征;所有病例均进行胸CT或高分辨率CT;住院期间常规检查项目包括血常规、血沉、C反应蛋白、血气分析,据病情轻重和年龄尽可能进行肺功能检查;具有诊断意义的项目包括呼吸道分泌物细菌培养、呼吸道病毒抗原检测、血清的病毒和支原体抗体检查,以及血清学的免疫抗体检查,痰、胃液、肺泡灌洗液涂片找含铁血黄素细胞等;对25例病例进行了支气管镜检,对14例病例进行了肺活检.由全国儿童弥漫性实质性肺疾病/间质性肺疾病协作组成员对每一病例进行集体讨论诊断.14例病例按临床-放射-病理(C-R-P)诊断模式达成诊断共识,79例病例根据临床及典型影像学改变、辅助检查做出最后诊断.结果 93例ILD患儿年龄8个月～14岁,平均年龄为4岁11个月,其中男53例,女40例.(1)93例ILD的病因谱为:闭塞性细支气管炎39例;特发性肺含铁血黄素沉着症39例;特发性间质性肺炎7例(其中非特异性间质性肺炎4例,急性间质性肺炎1例,淋巴细胞间质性肺炎1例,特发性肺纤维化1例);继发性的间质性肺炎2例(其中继发于系统性红斑狼疮的急性间质性肺炎1例,HIV感染引起的淋巴细胞间质性肺炎1例);外源性过敏性肺泡炎2例;闭塞性细支气管炎伴机化性肺炎1例,肺泡微石症1例,弥漫性泛细支气管炎1例,类脂性肺炎1例.(2)临床特点:72例有咳嗽,24例有气促,5例有三凹征,43例肺内可闻及湿啰音,8例有杵状指趾.(3)影像学特征:肺CT显示56例肺部有磨玻璃影;30例肺部有马赛克灌注征,其中11例伴有支气管扩张,8例伴有支气管壁增厚;1例弥漫性小叶中心性结节,伴小支气管和细支气管扩张;1例为弥漫性的细小结节影;1例为弥漫性网格影和泡性肺气肿.结论 儿童ILD是一组异质性疾病,本组共诊断出11种疾病,其中例数最多的前三位疾病为闭塞性细支气管炎、特发性肺含铁血黄素沉着症、特发性间质性肺炎.     

全身型幼年特发性关节炎合并肺间质病变概述

全身型幼年特发性关节炎是儿童常见的风湿性疾病之一,肺间质病变是指以肺间质和远端气隙重构为特征,进而导致异常气体交换的疾病.近年来,国内外对全身型幼年特发性关节炎合并肺间质病变的报道有增多趋势.该文总结了近年来报道中全身型幼年特发性关节炎儿童合并肺间质病变的情况,并对儿童肺间质病变的临床表现,影像学表现,诊断和治疗进行综...