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??Abstract??Objective To investigate the general characteristics?? undrelying causative factors and prognosis of bronchiectasis in chilren. Methods A total of 172 chilren diagnosed with bronchiectasis were included in the study. Demographics?? clinical data?? HRCT?? lung function?? and underlying etiology were collected and retrospectively analyzed. Part of the patients were followed up. Results Cough and expectoration were the two most common symptoms. Chest X-ray was significantly less sensitive ??4.42%?? than HRCT ??100%?? scan in diagnosis of bronchiectasis. Immunodeficiency?? bronchiolitis obliterans and primary ciliary dyskinesia were the three main etiology causing widespread distribution in HRCT ??P??0.05??. Lung functions in 76.92% of the patients were abnormal. The underlying etiologies were identified in 113 patients ??65.70%???? and pulmonary infecion??31.40%?? was the most common. It was found that FEV1 decreased by 1.28% per year with the extension of the course in the follow-up patients. There was improvement of HRCT in one patient whose symptom and lung fuction were also improved. Conclusion Bronchiectasis usually presents nonspecific symptoms. Most patients can be found with the underlying causes?? and pulmonary infection is the most common cause. HRCT is needed as early as possible in children with chronic respiratory symptoms. FEV1% declines slowly with the course extended. Part of the bronchiectasis children can be improved.     

儿童溃疡性结肠炎172例

目的分析中国儿童溃疡性结肠炎（UC）患者的临床资料为其诊治提供帮助。方法采用CBM、CNKI及VIP数据库检索1995~2004年国内发表的儿童UC的文献,对符合纳入标准者及同期本院确诊UC患儿172例的临床特征、X线和内镜表现、组织学特点、治疗及预后进行分析。结果172例中男女之比是1.07：1.0;就诊年龄2个月~15岁,≤3岁36.7%;病程3d~4年;阳性家族史1例。儿童UC以慢性腹泻、黏液血便或脓血便、腹痛为主要表现,发热、体质量减低或营养不良、贫血等全身表现常见,肠外表现及并发症少见,病变范围以全结肠为主,病情以中重型居多。以水杨酸偶氮磺胺吡啶（SASP）或5-乙酰水杨酸（5-ASA）为基础,配合糖皮质激素治疗在短期内可获得临床缓解,但长时程随访完全缓解者不足1/3。结论婴幼儿UC患者并不少见,儿童UC临床特征有异于成人,3岁前后亦不尽相同。儿童UC误诊率高,治疗困难。制定一套适合中国儿童UC的诊疗规范十分必要。     

儿童支气管扩张症172例临床研究

目的 探讨儿童支气管扩张症临床表现、 影像学及肺功能方面特点, 分析病因构成, 了解疾病预后。方法 回顾性总结 172 例支气管扩张症患儿的临床表现、 影像学、 肺功能特点及病因, 对部分患儿进行随访。结果 支气管扩张症患儿临床主要表现为咳嗽、咯痰、生长发育受限、杵状指等。胸部X线诊断支气管扩张症的检出阳性率（4.42%）显著低于高分辨率CT（HRCT）的100%。存在免疫缺陷、闭塞性细支气管炎及原发性纤毛运动障碍的患儿在HRCT中弥漫性支气管扩张较其他病因更显著（P＜0.05）。76.92%的患儿肺功能异常。65.70%的患儿可以发现潜在病因,以肺部感染（31.40%）为主。门诊随访患儿FEV1%随病程的延长以每年1.28%的速度下降。其中1例临床症状、HRCT、肺功能均有好转。结论 儿童支气管扩张症临床表现没有明显特异性。大部分患儿可以发现潜在病因,以肺部感染最为常见。对于临床怀疑支气管扩张症的患儿应尽早进行HRCT检查,积极寻找潜在病因。支气管扩张症患儿的肺功能可随着病程的进展缓慢下降。部分患儿在临床症状、HRCT及肺功能上可有好转。     

172名健康学龄前儿童空腹呼气氢测定

目的　检测健康学龄前儿童空腹呼气氢 (FH2 )值。方法　选择 4周内未患消化道疾病及未应用过抗生素、胃肠动力药物的 172名某幼儿园 3～ 6岁健康学龄前儿童为检测对象 ,其中 <3岁组 6 0名、～ 4岁组5 8名、～ 5岁组 31名、～ 6岁组 2 3名。采集被测儿童清晨空腹平静呼吸时终末呼出气置于特制采气袋内。即刻注入HD -I型测氢法消化道疾病检测仪内 ,每气样标本测定 2次 ,取均值 ,氢气含量单位以 10 -6L/L(体积分数 )表示。结果　本组FH2 测定结果为非正态分布 ,采用矩法作正态型检验 ,男 :3岁、～ 4岁、～ 5岁及 6岁组FH2 中位数分别为 8.6、13.3、19.75及 2 5 .2 5 ( 10 -6L/L) ,女各组FH2 中位数分别为 10 .8、12 .8、19.9及 2 5 .4( 10 -6L/L)。结论　本组 3～ 6岁健康学龄前儿童空腹FH2 含量波动于 ( 10 .2 5～ 2 5 .4 0 )× 10 -6L/L ,各年龄组间有显著性差异 ,随年龄增长FH2 含量有增高趋势 ,可能与各年龄组间不同食物性底物产H2 量不同有关。     

小儿细菌性肝脓肿172例诊断及治疗

本文报道我院1971～1984年14年间收治小儿细菌性肝脓肿172例。年龄以6～12岁最多,计134例(77.9%)。病变位于右叶142例,左叶28例,左右叶均受累2例。应用A型超声诊断67例,B型超声94例。手术引流127例,术前均在超声监测下定位。强调超声诊断的重要性。全组治愈169例(98.3%),死亡3例(1.7%)。共作脓液细菌培养118例,其中113例有细菌生长,以金黄色葡萄球菌,白色葡萄球菌多见,大肠杆菌次之。     

172例新生儿血象及其中46例贫血调查分析

172例新生儿,日龄为2小时～28天,其中正常者126例,贫血者46例。126例正常新生儿血象:Hb(17.5±1.6g/dl)、RBC(554.3±8.9万/mm~3)、HCt(58.2±6.7%)、MCV(105.2土17.9μm)、MCH(31.7±5.2μμg)、MCHC(30、4±3.9%)、WBC(13333.4±3084.7/mm~3、N(50.7±10.7%)、L(49.1±10.7%)。46例贫血儿,患病率26.7%,小于10天组9人(9.2%),10天～28天组37人(50%),不同的日龄间贫血患病率有显著差异(p<0.005),贫血程度随出生时体重而改变,体重越低贫血程度越重,贫血的原因有脐带松解、前置胎盘、颅内出血、低出生体重、早产、双胎、各种感染及孕母有中度以上的贫血等。     

172例TEL/AML1阳性急性淋巴细胞白血病生物学特征分析

目的分析伴有TEL/AML1融合基因阳性急性淋巴细胞白血病(ALL)儿童群体生物学特征。方法回顾性分析2008年1月—2015年4月就诊的172例年龄≤18岁初诊伴有TEL/AML1阳性ALL患儿(根据MICM标准诊断分型)临床特点。结果 TEL/AML1阳性患儿占同期新诊断(n=940) ALL患儿的18. 3%。发病中位年龄为4(1～13)岁。完成染色体检查的160例患儿中均未发现有t(12; 21)(p13; q22)异位。行多参数流式细胞仪检查163例,结果提示免疫表型均为B细胞表型。在诊断时没有患者发生中枢神经系统白血病。在中位随访时间为65(1～123)个月时,27例复发(复发组),145例(84. 3%)获得持续完全缓解(非复发组)。分析复发组和非复发组的生物学特征,结果发现两组发病时在激素预治疗的敏感性、危险度分组以及染色体异常诸方面有统计学显著差异。结论 TEL/AML1阳性患儿发病年龄小;免疫分型为B细胞型、激素预治疗敏感、核型分析未发现t(12; 21)(p13; q22)异位;发病时多无中枢神经系统白血病的发生;复发组患者激素预治疗不敏感者多、危险度分组为高危组的患者比例高,非随机次要染色体异常的患者多见。     

Analysis of renal biopsies performed in children with abnormal findings in urinary mass screening

Background: School urinary mass screening tests are performed to make early diagnosis and provide proper treatment for chronic renal diseases. However, very few systemic analyses or studies have been reported regarding final diagnosis made on children with abnormal urinary screening results. Aim: To study the cases of renal biopsy in children detected in urinary screening. Methods: We retrospectively analysed 461 cases of renal biopsy performed on children referred to us with abnormal school urinary mass screening results who satisfied indications for renal biopsy. Results: Pathologically abnormal findings were observed in 285 (61.8%) patients. Thin glomerular basement membrane disease was detected in 127 (27.5%) cases and IgA nephropathy in 121 (26.2%) cases. Among those 461 children, microscopic haematuria was observed in 289 (62.7%) patients, proteinuria in nine (2.0%), and both in 163 (35.4%). In addition, a statistically higher rate of pathological abnormalities on renal biopsy was noted in the group with microscopic haematuria combined with proteinuria and also in cases with more severe haematuria.

Conclusion: School urinary mass screening has greatly contributed to diagnosing chronic renal diseases. Continuous medical observation is required when abnormal urinalysis is observed, and a more aggressive medical approach such as renal biopsy should also be performed if necessary.     

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??Abstract?? Hematuria is a common problem in children?? and a high prevalence of asymptomatic haematuria was found in early screening for chronic kidney disease ??CKD??. The causes of disease are complex?? so long-term follow-up and management are very important. Meanwhile?? renal biopsy indication of asymptomatic microscopic haematuria is ancontroversial issue. Epidemiological studies will provide scientific information with guiding roles and application values for asymptomatic haematuria?? especially CKD.     

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??Objective??To explore and conclude the pathological categories and clinical data of childhood renal diseases and understand the importance of renal biopsy in childhood renal diseases. Methods??Totally 753 patients who underwent percutaneous renal biopsy from 1995 to 2015 were selected as study subjects??and their clinical and pathological information was analyzed retrospectively. Results??Among 753 patients who underwent percutaneous renal biopsy??428 cases??56.84%?? had primary glomerular disease??306 cases??40.64%?? had secondary glomerular disease??17 cases??2.26%?? had heritage glomerular disease??and 2 cases??0.27%?? had renal tubular interstitial disease. The most common clinical diagnosis were primary nephritic syndrome. The most common clinical diagnosis and pathological category in primary glomerulary disease were primary nephritic syndrome and IgA nephropathy respectively?? and in secondary glomerulary disease they were purpura nephritis and mesangial proliferative glomerulonephritis respectively. Thin basement membrane disease and Alport’s syndrome are the most common pathological category of the heritage glomerular disease. The 8 repeated renal biopsies showed pathological and clinical progression. Conclusion??The primary glomerulary disease is the main type of childhood glomerulary diseases??The most common clinical diagnosis and pathological category are primary nephrotic syndrome and mesangial proliferative glomerulonephritis respectively. The repeated renal biopsy is beneficial to control the transformation of pathological types and adjust new treatments timely.     

无症状肾小球性血尿63例临床与病理

目的探讨小儿无症状肾小球性血尿的临床与病理关系。方法符合条件者行肾穿刺活检进行光镜、电镜和免疫荧光镜检查。结果临床分为持续镜下血尿（A）13例,发作性肉眼血尿（B）6例,A＋B28例,持续肉眼血尿（C）16例。病理以IgA肾病最常见（44．4％）。不同临床表现类型的血尿之病理类型无明显差异,但B之病理以IgA肾病更常见。病变程度似以A＋B和C表现者相对偏重。结论IgA肾病是小儿无症状肾小球性血尿的主要疾病之一。掌握肾治检的适应证对无症状肾小球性血尿患儿进行肾病理检查,对明确诊断、指导治疗、估计预后有重要意义。     

30例儿童毛细血管内增生性肾小球肾炎临床和病理分析

为了解毛细管内增生性肾小球肾炎的特殊临床表现,探讨肾穿刺活检术在急性肾小为中的应用,采用回顾分析法,对３０例毛细血管内增生 性肾小球肾炎临床、病理进行统计分析,结果显示,临床表现为肾病综合征８例,急性肾功能衰竭８例,ＩｇＡ肾病６例,急性肾炎６例,隐匿性肾炎２便。病理学表明,以肾病型、肾衰型病变重,分别有不同程度的新月体形成。肾病型有５例免疫荧光示ＩｇＧ、Ｃ３沉积呈“花环状”,提示毛细血管内增生性肾     

1000例肾活检儿童临床与病理分析

目的探讨肾活检儿童的临床、病理特点及两者之间的关系。方法回顾性分析1 000例肾脏病患儿临床与肾脏病理资料。结果 1 000例患儿肾活检取材成功962例,成功率96.2%。患儿术后出现轻度并发症170例,并发症发生率为17%。临床表现与病理大部分符合,但有20例临床与病理诊断不符。结论儿童肾脏病的临床表现轻重与病理改变轻重并不平行,仅凭临床表现进行诊治,有误诊、过度治疗、延误治疗的可能,病理诊断对临床治疗及预后评价具有重要的指导意义。     

IgA肾病患儿临床病理的动态变化:附5例重复肾活检分析

目的 探讨IgA肾病患儿的临床表现和病理改变的动态变化。方法 分析5例重复肾活检的IgA肾病患儿的临床表现和病理改变的动态变化。结果 5例患儿中,4例进行2次肾活检,1例进行3次肾活检。5例患儿的基本临床表现均为持续镜下血尿伴间断肉眼血尿,并出现不同程度的蛋白尿及肾功能改变,其基本病理改变为不同程度的系膜增生。重复肾活检提示每例患儿的病理类型均有进展,且伴临床变化。结论 IgA肾病的临床表现及病理改变呈多样性、多变性,且病理类型可以转变。IgA肾病在出现病情反复或加重时,应进行重复肾活检以利于指导治疗,并提出IgA肾病时重复肾活检的指征。     

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??Objective??To analyze the clinical characteristics??pathological features and treatment responses of 6 pediatric patients with C3 glomerulonephritis??C3GN????in order to improve the understanding and treatment of this disease for pediatricians. Methods??Analyze the clinical manifestations??pathological features??therapies??prognosis of patients who were diagnosed with C3 glomerulonephritis from September??2010 to June??2016 retrospectively. Results??Clinical characteristics and laboratory examination??2 patients’ first symptom was hematuria??4 patients’ first symptom was hematuria and proteinuria??3 patients presented as acute nephritic syndrome??one presented as nephrotic syndrome. All the patients showed that the level of serum complement C3 was reduced??while sernm complement C4 was normal. Pathological character??6 patients showed strong positive complement C3 deposition under immunofluorescence. Lightmicroscopy showed mesangial proliferative glomerulonephritis in 5 cases??1 case was diagnosed as endocapillary proliferative glomerulonephritisin??and 3 patients presented electrondense depositionin under electron microscope. Treatment and prognosis??after conventional treatment??2 patients who were with crescent were treated with glucocorticoid .After a follow-up from 6 months to 42 months ??the prognosis was pretty good. Conclusion??Children with C3GN are usually presented with hematuria and ??or??proteinuria??characterized by strong positive C3 deposition. Lightmicroscopy always shows mesangial proliferative glomerulonephritis. Electron microscope show electron dense deposition??and short-term prognosis is pretty good.