儿童慢性肾脏病5期病因分析及并发症研究

目的了解儿童慢性肾脏病(CKD)5期的病因、并发症及影响疾病预后的因素。方法病例系列研究, 回顾性分析2012年4月至2021年4月于重庆医科大学附属儿童医院确诊CKD 5期并住院治疗的174例患儿的一般情况、临床表现、实验室检查、基因检测及随访情况(截至2022年10月)等临床资料, 根据年龄、性别和病因分组比较患儿并发症的特点;根据有无左心室肥厚(LVH)分为LVH组和非LVH组, 分析发生心血管疾病的影响因素;根据随访时情况分为死亡组和存活组、腹膜透析组和血液透析组, 分析影响预后的相关因素。组间比较采用χ2检验、独立样本t检验、Fisher确切概率法、Mann-WhitneyU检验及Kruskal-Wallis检验, 采用二元Logistic回归分析与疾病预后相关的因素。结果 174例CKD 5期患儿中男96例、女78例, 初诊年龄11.2(8.2, 13.0)岁。主要病因为先天性肾脏和尿路畸形(CAKUT)(84例, 48.3%)及肾小球疾病(83例, 47.7%), 其中遗传性肾小球病28例(16.1%)。主要并发症依次为贫血(98.2%, 165/168), 慢性肾脏病合...     

1002例慢性肾脏病患儿临床及病理分析

目的 了解慢性肾脏病(CKD)儿童的临床及病理特点.方法 回顾分析1002例住院CKD患儿的临床及病理资料.结果 1 002例CKD患儿中,男635例,女376例;中位发病年龄7岁;CKD Ⅰ期973例,CKD Ⅱ期4例,CKDⅢ期7例,CKD Ⅳ期6例,CKD V期12例;病因以后天获得性肾小球疾病为主,原发性肾病综...     

慢性肾脏疾病患儿维生素D水平研究进展

儿童慢性肾脏疾病(chronic kidney disease,CKD)是威胁儿童正常生长发育的主要疾病之一,在我国主要病因以肾小球疾病为主,常并发心血管疾病、肾性贫血、肾性骨病等,严重影响儿童生活质量.CKD患儿由于肾脏病变25-羟维生素D[25-(OH) Vit D]羟化受到影响、尿毒症导致的皮肤变化使骨化三醇合成减少等因素常引起25-(OH) Vit D水平低下.该文就CKD患儿25-(OH) Vit D基础水平和影响因素作一综述.     

先天性肾积水与肾间质纤维化研究进展

先天性肾脏和尿路畸形(CAKUT)是引起儿童慢性肾脏病(CKD)的主要病因,先天性肾积水是CAKUT常见类型,通常在胎儿期通过超声检查被发现。严重的先天性肾积水会导致肾实质损伤及最终发生终末期肾病(ESRD)的不良后果。先天性肾积水的成因复杂,涉及多种基因的异常表达。人体组织标本及动物模型研究显示,氧化应激、慢性炎症及凋亡等机制导致肾小管萎缩及肾纤维化。通过蛋白质组学等先进的生物学技术,新的生物学标志物不断涌现,为早期诊断监测此类患儿的病情提供了有力手段。     

应用Schwartz公式和99Tcm-DTPA肾动态显像评估肾小球滤过率的一致性研究

目的 评价应用Schwartz公式评估肾小球滤过率（GFR）的准确性,为儿科临床准确和便捷地评估GFR提供参考。方法 选择2002年 4月至2006 年12月在复旦大学附属儿科医院肾病科诊断为慢性肾脏病（CKD）的患儿为研究对象。患儿于入院后第2日早晨空腹采血,以碱性苦味酸法测定血肌酐（SCr）水平,测量身高,采用Schwartz公式计算GFR（eGFR）。入院后第2或3日行99Tcm-DTPA肾动态显像,获得GFR（mGFR）。采用NKF-K/DOQI推荐的CKD分期标准,依据mGFR分为CKD 1~5期。mGFR与eGFR的相关性采用Pearson相关分析;eGFR估算mGFR的准确性采用相对预测误差（MPE）和绝对预测误差(MAE)表示,eGFR和mGFR的一致性采用Bland-Altman检验。结果 170例CKD患儿进入分析,其中男100例,女70例;年龄2.3～17.8（9.3±3.9）岁。肾病综合征75例,肾小球肾炎28例,泌尿道感染49例,急性肾功能衰竭10例和慢性肾功能衰竭8例。CKD 1期 80例,2期 40例,3期 27例,4期 17例,5期6例。①eGFR和mGFR总体上有显著相关性（r＝0.871）;CKD 1期患儿的eGFR和mGFR呈显著弱相关性,CKD 2~4期患儿eGFR和mGFR无显著相关性。②eGFR预测mGFR的MPE和MAE随肾功能损害程度的加重呈增高趋势,CKD 1期79/80例（98.8%）、CKD 2期32/40例（80.0%）患儿eGFR预测mGFR的MPE均落在±30％内;CKD 3~5期患儿eGFR预测mGFR的准确性较差。③Bland-Altman检验结果提示,eGFR和mGFR的一致性CKD 1和2期患儿较好,CKD 3和4期患儿较差。结论 Schwartz公式对于肾功能损害较轻的CKD 1和2期患儿预测mGFR的准确性较高,CKD 3～5期患儿的准确性较差。对于CKD 3~5期患儿仍应行99Tcm-DTPA肾动态显像以获得准确的GFR。     

单中心6年先天性肾脏和尿道畸形病因构成分析

目的 分析2005至2010年复旦大学附属儿科医院先天性肾脏和尿道畸形（CAKUT）诊断情况,为CAKUT的早期诊断提供线索。方法 系统提取住院诊断为CAKUT病例的病史资料,将三聚氰胺事件结石筛查作为特殊暴露因素,与正常暴露因素下进行比较,分析CAKUT病种构成比、首次入院的病因和影像学检查。结果 6年间1 358例CAKUT患儿进入分析,在重复入院只计1次的前提下CAKUT占同期住院患儿的1.33%（1 358/102 442）。男913例,女445例,男女比例为2.05∶1;首次入院时平均年龄为（3.0±3.4）岁。①正常暴露因素下CAKUT的住院构成比2005至2010年呈缓慢增高趋势（P<0.05）, CAKUT的住院构成比特殊暴露因素较正常暴露因素下高0.67%和0.26%,2008至2009两个年度多筛查出112例达到手术干预指征的CAKUT病例,占同期正常因素暴露下达到手术干预指征CAKUT病例的24.4％［112/（227＋232）］。②CAKUT构成比排序前4位的病种分别为肾盂输尿管连接处梗阻（PUJO）、 膀胱输尿管反流(VUR)、双集合系统和输尿管膀胱连接部梗阻(UVJO),占总CAKUT病例的79.6％;③首诊通过产前B超检查发现异常406例（29.9%）,以PUJO、UVJO和输尿管膨出比例最高;48.5%（658/1 358）CAKUT患儿有泌尿系统症状和体征（腹部症状、血尿、排尿异常、尿路感染伴发热和尿路感染）;尿路感染伴发热占41%（270/658）,为CAKUT病种主要的症状, <2岁患儿是≥2岁患儿的3.2倍（206/64）;其他症状和体征均为≥2岁患儿明显多于<2岁患儿。 98.0%的CAKUT患儿进行B超检查,除VUR的B超异常检出率为68.5%外,其他CAKUT病种的B超异常检出率均>94.0%;根据B超检查和临床表现,选择性进行了功能状态检查（DTPA、DMSA）1 203例,形态学检查（MR、CT或IVP）1 009例,反流情况（MCU）400例。结论 CAKUT构成比以PUJO、VUR、双集合系统和UVJO最为常见,泌尿系统B超筛查CAKUT的准确性较好。在人群中常规筛查可检出CAKUT,其成本效益有待进一步研究。     

基于一家国家儿童医学中心住院病案首页肾脏专科疾病诊断谱及其一般特征的横断面调查

目的 探索基于住院病案首页专科医疗数据的分析方法,描述复旦大学附属儿科医院(我院)肾脏专科的医疗服务特色。方法 横断面调查。以医院信息系统(HIS)住院病案首页2014年1月1日至2018年12月31日肾脏专科全样本数据为基础。数据脱敏后提取本研究目标字段建立备份数据库,行数据清洗(ICD-10编码国标新旧版映射、现住址简约为省市),建立肾脏(相关)专科疾病字典库(DASF-PEKDI)并分类(原发性肾小球疾病、继发性肾脏疾病、遗传性肾小球疾病、肾小管疾病、泌尿系感染、先天性肾脏和尿路畸形、尿石症、肾血管病变、夜遗尿、肾脏肿瘤、肾功能不全/肾衰竭、肾脏替代治疗、血尿/蛋白尿、其他)。结果 在我院HIS系统中检索住院病案首页肾脏专科标识性符号的住院病案7 898份,从DASF-PEKDI导出1091条国标新版ICD-10诊断编码,其中肾脏(相关)专科疾病诊断编码共285条,逐条审读剔除非明确的肾脏疾病诊断编码、合并症编码、手术状态编码,专科疾病诊断编码共227条,同时还纳入3条肾脏穿刺术操作编码(ICD-9-CM3)。我院肾脏科出院3 963例(7 898例次);肾脏(相关)专科疾病占90.1%(3 571例),中位年龄5.8岁(0~20岁),男61.3%,肾脏(相关)专科疾病人次占90.5%(7 144人次),5年间上海市以外的病例由2014年72.1%上升至2018年85.7%。年均住院总费用每人次13 716元,平均住院9.4 d。原发性肾小球疾病以原发性肾病综合征为主(78.1%),继发性肾脏疾病以紫癜性肾炎和狼疮性肾炎为主(共占78.4%),先天性肾脏和尿路畸形(CAKUT)诊断构成比由2014年的9.3%上升至2018年的15.3%,肾功能不全/肾衰竭以慢性肾脏病2~5期为主(共占90.7%),肾脏替代治疗腹膜透析占62.2%、血液透析占12.6%、肾移植占25.2%。结论 住院病案首页的肾脏专科疾病诊断谱首先需要基于ICD-10编码的标化,并建立肾脏(相关)专科疾病字典库和分类,基于此才能准确反映肾脏专科住院疾病的人口学和专科化特点。     

基于一家国家儿童医学中心住院病案首页肾脏专科疾病诊断谱及其一般特征的横断面调查

目的 探索基于住院病案首页专科医疗数据的分析方法,描述复旦大学附属儿科医院(我院)肾脏专科的医疗服务特色。方法 横断面调查。以医院信息系统(HIS)住院病案首页2014年1月1日至2018年12月31日肾脏专科全样本数据为基础。数据脱敏后提取本研究目标字段建立备份数据库,行数据清洗(ICD-10编码国标新旧版映射、现住址简约为省市),建立肾脏(相关)专科疾病字典库(DASF-PEKDI)并分类(原发性肾小球疾病、继发性肾脏疾病、遗传性肾小球疾病、肾小管疾病、泌尿系感染、先天性肾脏和尿路畸形、尿石症、肾血管病变、夜遗尿、肾脏肿瘤、肾功能不全/肾衰竭、肾脏替代治疗、血尿/蛋白尿、其他)。结果 在我院HIS系统中检索住院病案首页肾脏专科标识性符号的住院病案7 898份,从DASF-PEKDI导出1091条国标新版ICD-10诊断编码,其中肾脏(相关)专科疾病诊断编码共285条,逐条审读剔除非明确的肾脏疾病诊断编码、合并症编码、手术状态编码,专科疾病诊断编码共227条,同时还纳入3条肾脏穿刺术操作编码(ICD-9-CM3)。我院肾脏科出院3 963例(7 898例次);肾脏(相关)专科疾病占90.1%(3 571例),中位年龄5.8岁(0~20岁),男61.3%,肾脏(相关)专科疾病人次占90.5%(7 144人次),5年间上海市以外的病例由2014年72.1%上升至2018年85.7%。年均住院总费用每人次13 716元,平均住院9.4 d。原发性肾小球疾病以原发性肾病综合征为主(78.1%),继发性肾脏疾病以紫癜性肾炎和狼疮性肾炎为主(共占78.4%),先天性肾脏和尿路畸形(CAKUT)诊断构成比由2014年的9.3%上升至2018年的15.3%,肾功能不全/肾衰竭以慢性肾脏病2~5期为主(共占90.7%),肾脏替代治疗腹膜透析占62.2%、血液透析占12.6%、肾移植占25.2%。结论 住院病案首页的肾脏专科疾病诊断谱首先需要基于ICD-10编码的标化,并建立肾脏(相关)专科疾病字典库和分类,基于此才能准确反映肾脏专科住院疾病的人口学和专科化特点。     

儿童先天性肾脏及尿路畸形的研究现状

先天性肾脏及尿路畸形(congenital abnormalities of the kidmey and urinary tract,CAKUT)是由于各种原因所致的,以先天性泌尿系统解剖学异常为临床特征的表型多样的一组疾病,发生率较高.CAKUT是导致儿童慢性肾脏病(chronic kidney disease,CKD)的最主要原因,部分患者可隐匿进展至终末期肾病(end-stage renal disease,ESRD).儿童CAKUT的研究对优生优育有重要意义,其发病机制尚不完全明确,但不少研究证明该病的发病与基因突变、拷贝数变异及环境等多因素有关.另外,采用彩色多普勒超声检查对于发现儿童CAKUT具有明显优势,是筛查的首选方法,进而实行早期相应治疗干预,必将大大降低患儿肾功能的损伤程度,提高患儿的生存率,对于疾病预防意义重大.该文综述近年来儿童CAKUT的流行病学研究、病因学研究、彩超筛查及干预治疗,旨在使医务人员更全面地了解儿童CAKUT,并采取积极的干预措施,延缓CKD的进展从而防止ESRD发生.     

1 316例小儿肾脏病临床与病理分布特点

目的：了解小儿肾小球疾病肾脏病理改变的特点及其与临床表现的关系。方法：对近20年间该院的1 316例患儿的肾活检资料,进行了回顾性分析。结果：1 316例患儿中临床主要表现为肾病综合征(383例,占29.09% )、急性肾炎综合征(291例,占22.00%)、孤立性血尿(224例,占17.21%)、紫癜性肾炎（209例,占15.87%）、乙型肝炎病毒相关肾炎(96例,占7.30% )等。病理改变主要为系膜增生(756例,占57.45%)、IgA肾病(113例,占8.59% )、毛细血管内增生（112例,占8.51%）、膜性肾病(66例,占5.02%)、微小和轻微病变(59例,占4.48%)等。通过超微结构检查,使Alport综合征、薄基底膜病、先天性肾病、纤维样肾小球病、Fabry病等,得以明确诊断。通过肾活检组织免疫病理学检查,使IgA肾病、IgM肾病及C1q肾病得以确诊。肾活检患儿中原发性肾小球疾病最为常见（915例,占69.53%）。原发性肾小球疾病病因以原发性肾病综合征为最常见（375/915例,占41.0%）,而继发性肾小球疾病病因以紫癜性肾炎为最常见（209/344例,占60.8%）。结论： 在该次调查的肾活检资料中,原发性肾小球疾病最常见,肾病综合征是最常见的临床诊断,病理改变则以系膜增生最为多见。［中国当代儿科杂志,2007,9（2）：117-121］     

超声筛查26989名儿童中先天性肾脏和尿路畸形的临床分析

目的 了解泌尿系超声筛查儿童先天性肾脏和尿路畸形(CAKUT)的发生率及其中各种畸形的发病诊疗情况,探讨超声在筛查诊断儿童CAKUT的重要性.方法 对2008年底北京儿童医院超声筛查26 989名儿童发现的489例儿童泌尿系异常病例进行随访,核实是否进一步明确筛查发现的泌尿系异常情况,对于未复查或者仍未确诊者统一者安排到北京儿童医院复查超声,进行总结分析.结果 ①本次筛查中(26 989例)估算CAKUT发生率约为1.67%,绝大部分无临床表现.②489例病例中随访到320例,失访169例,随访组(320例)中明确CAKUT 295例,其中16例确诊结果与超声筛查结果不完全一致.③肾积水137例(0.78%),输尿管膀胱连接部梗阻33例(0.19%),肾发育不良30例(0.17%),肾囊肿26例(0.15%);重复肾24例(0.14%);孤立肾22例(0.12%);输尿管囊肿16例(0.09%);融合肾10例(0.06%);肾发育不全9例(0.05%);异位肾5例(0.03%);多囊肾3例(0.02%);原发膀胱输尿管反流、后尿道瓣膜、肾旋转不良、分支肾盂、肾外型肾盂各1例.④114例畸形病例需手术治疗,占随访组中CAKUT患儿的38.6%(114/295),只有1例肾盂输尿管连接部梗阻病例手术失败.结论 儿童CAKUT的发生率较高,且早期并无明显临床表现而不易发现;超过三分之一的CAKUT需要外科干预;超声检查对发现儿童CAKUT具有明显优势,是筛查的首选方法,可以作为儿童的常规体检项目.

Abstract：

Objective To investigate the proportion of children with congenital abnormalities of the kidney and urinary tract(CAKUT)among those who received screening,analyze the rate, diagnosis and treatment of each abnormality, and evaluate the value of ultrasonogrphy in the CAKUT diagnosis.Method Totally 489 of 26 989 children who received screening for urinary tract abnormalities were found to have CAKUT and were followed up by telephone interviews and were asked whether they had defined diagnosis of CAKUT, had clinical symptoms and received treatment. Children who had been operated on were also asked about the operation time, surgical procedure, postoperative diagnosis, and outcome. Children who had never been reviewed or still had no diagnosis were arranged to Beijing Children's Hospital for ultrasound follow-up. The final data were summed up for a retrospective analysis. Result ① The proportion of children with CAKUT was 1.67%. Most children with CAKUT had no clinical manifestations. ② Of the 489 cases, 320 were followed up, and 169 were lost. Of the cases who were followed up, 16 out of 295 cases who had clear diagnosis showed inconsistent results in ultrasound screening.③Hydronephrosis was identified in 137 cases (0.78%),among whom 111 were males and 26 were females,in 98 cases the hydronephrosis was on the left, in 28 on the right and in 11 were bilateral. Ureterovesical junction obstruction occurred in 33 cases (0.19%),25 males and 8 females,in 22 cases on the left, in 10 on right and bilateral in 1 case. Renal dysplasia was shown in 30 cases(0.17%),22 males and 8 females;renal cysts in 26 cases(0.15%); duplex kidney in 24 cases (0.14%) ;solitary kidney in 22 cases(0.12%);ureterocele in 16 cases(0.09%);fused kidney in 10 cases(0.06%);renal hypoplasia in 9 cases(0.05%);ectopic kidney in 5 cases(0.03%);polycystic kidney in 3 cases(0.02%).Primary vesicoureteral reflux, posterior urethral valves, renal malrotation, branch renal pelvis, extrarenal pelvis in 1 case each.④Totally 114 of the 295 cases (38.6%)who were followed up for CAKUT in screening test required surgical treatment.Only one case of ureteropelvic junction obstruction(UPJO)failed in surgery.Conclusion The proportion of children with CAKUT was high.CAKUT is difficult to detect since there is no significant clinical manifestations in early ages.More than one third of patients with CAKUT needed surgical treatment.Ultrasonography showed obvious advantages in detecting children's CAKUT, proven to be the most preferred method of screening, thus could be recommended as a routine of children's physical examination.     

The association between the congenital heart diseases and congenital anomalies of the kidney and the urinary tract in nonsyndromic children

Several congenital heart diseases (CHD) are present in children with congenital anomalies of the kidney and the urinary tract (CAKUT) in syndromic or nonsyndromic patterns. The purpose of this study was to identify nonsyndromic children with CAKUT who required echocardiographic evaluation and to find the prevalence of CHD in these patients. Retrospective chart review of nonsyndromic patients with CAKUT between 2012 and 2018 was conducted. Types of congenital anomalies of the kidney and urinary tract and echocardiogram reports of the patients, age, gender, prematurity, prenatal diagnosis of CAKUT, and family history were noted. Among 806 children with CAKUT, 135 patients underwent transthoracic echocardiographic evaluation and CHD was detected in 91 (11.2%). The most common type of CHD was secundum type atrial septal defect that was found in 73 (80.2%) patients. On the other hand, the most frequent types of congenital anomalies of the kidney and the urinary tract were isolated hydronephrosis and primary vesicoureteral reflux (61.4% and 12.8%, respectively). Our results showed that 75.8% of the patients with congenital heart diseases had isolated hydronephrosis. Finally, only 18 (2.2%) cases out of 806 patients that were evaluated via transthoracic echocardiography due to the clinical findings and family history had a CHD that required close (<6 months) follow-up. Congenital heart diseases that required close follow-up are rarely observed in nonsyndromic patients with CAKUT. Therefore, we suggest echocardiography should be performed in cases of clinical suspicion and family history rather than routinely in this patient group.     

Renal development in the fetus and premature infant

Congenital abnormalities of the kidney and urinary tract (CAKUT) are one of the leading congenital defects to be identified on prenatal ultrasound. CAKUT represent a broad spectrum of abnormalities, from transient hydronephrosis to severe bilateral renal agenesis. CAKUT are a major contributor to chronic and end stage kidney disease (CKD/ESKD) in children. Prenatal imaging is useful to identify CAKUT, but will not detect all defects. Both genetic abnormalities and the fetal environment contribute to CAKUT. Monogenic gene mutations identified in human CAKUT have advanced our understanding of molecular mechanisms of renal development. Low nephron number and solitary kidneys are associated with increased risk of adult onset CKD and ESKD. Premature and low birth weight infants represent a high risk population for low nephron number. Additional research is needed to identify biomarkers and appropriate follow-up of premature and low birth weight infants into adulthood.     

Chronic Kidney Disease (CKD): An Observational Study of Etiology,Severity and Burden of Comorbidities

Objectives

To study the etiology and burden of comorbidities across stages of chronic kidney disease (CKD).

Methods

Children, 2–16 y of age with CKD Stages II- IV were recruited over 12 mo. The etiology, clinical presentation and severity of complications were studied.

Results

Among 78 children [Stage II (n = 21), Stage III (n = 26), Stage IV (n = 31)], congenital anomalies of the kidney and urinary tract (CAKUT) was the commonest etiology and 28 were newly diagnosed in Stage III /IV. High prevalence of comorbidities was observed with growth retardation (65%), hypertension (59%), hyperphosphatemia (32%), vitamin D deficiency (92%), dyslipidemia (64%), left ventricular hypertrophy (45%) and hyperparathyroidism (56%). While most comorbidities correlated with the estimated glomerular filtration rate and severity of CKD, hypertension, vitamin D deficiency and cardiovascular morbidity were prevalent even in early stages.

Conclusions

CAKUT was the commonest cause of CKD. Late detection and high prevalence of comorbidities even in early stages of CKD were observed.

     

Conduite à tenir devant un rein unique

Solitary functioning kidneys form an important subgroup of congenital anomalies of the kidney and urinary tract (CAKUT). A solitary kidney can be congenital or acquired after unilateral nephrectomy and is often associated with ipsilateral urogenital anomalies. Both types of solitary functioning kidney are associated with an increased risk of chronic kidney disease (CKD). A low functional nephron number results in compensatory glomerular hypertension and enlargement of remnant nephrons, indicating glomerular hyperfiltration. Glomerular hyperfiltration may lead to glomerulosclerosis, which further results in hypertension, proteinuria, and decline of the glomerular filtration rate (GFR) in the long run. About 20–30% of patients with solitary functioning kidney have hypertension, proteinuria, or reduced GFR during childhood, especially those with associated CAKUT. Regular and lifetime monitoring (including growth, blood pressure, serum creatinine, proteinuria or microalbuminuria, and renal ultrasound) is required. The frequency and modality of follow-up should be adapted to individual risk for CKD. Early detection of renal injury and timely nephroprotective measures are critical.     

比较不同肾小球滤过率计算方法评价儿童慢性肾脏病肾功能的适用性

目的 肾小球滤过率(GFR)是评价肾功能的最好指标,可用直接检测法和公式估算法获得GFR,后者在临床实践中尤为常用,如24 h内生肌酐清除率、Schwartz公式以及Filler公式,但少有对这些计算方法在我国儿童慢性肾脏病(CKD)患者的适用性的研究.方法 选择2002年至2005年在我院住院、且符合入选标准和排除标准的CKD患儿为研究对象.将24 h内生肌酐清除率、Schwartz公式以及Filler公式估算的GFR(eGFR)与99mTc-DTPA肾动态显像(Gates法)测得的GFR(rGFR)进行比较.结果 入选30例患儿(男18例、女12例),平均年龄为9.4岁.CKD1期13例、CKD2期8例、CKD3期8例、CKD4期1例.CKD病因包括结构异常、肾小球肾炎、血管性疾病、肾病综合征和泌尿系结核.Bland-Altman分析显示24 h内生肌酐清除率的eGFR与rGFR的一致性相对最好;由24 h肌酐清除率和Schwartz公式得出的eGFR的准确性较Filler公式好.CKD1期时,由24 h内生肌酐清除率和Schwartz公式得出的eGFR过高估计rGFR,而由Filler公式得出的eGFR过低估计rGFR;CKD2期时,由24 h内生肌酐清除率得出的eGFR过低估计rGFR,而由Schwartz公式和Filler公式得出的eGFR过高估计rGFR.结论 3种公式获得的GFR的估计值与标准方法测得的GFR值之间存在显著差异,有待于今后开发更适用于我国儿童的肾功能估算方法.     

儿童侵袭性肺炎链球菌病的临床特征及耐药性分析

目的 总结儿童侵袭性肺炎链球菌病(IPD)的临床特征及耐药性资料,以提高对该病的诊疗水平.方法 回顾性分析2004年1月-2009年6月55例IPD患儿的临床资料;采集患儿的血液、脑脊液、腹水、纵隔及软组织引流液标本,经实验室培养、分离、鉴定出64株肺炎链球菌(SP),检测其对青霉素等抗生素的敏感性.结果 55例IPD患儿中男32例,女23例,男女比例为1.39:1.年龄47 d～12岁,其中2岁以下占62%.临床诊断败血症38例(69%);化脓性脑膜炎9例(16%);臀部或颈部脓肿7例(13%);化脓性腹膜炎1例.13例(24%)有基础疾病,以白血病最多见(31%).3例(5%)有外科手术史;3例(5%)合并病毒感染,2例(4%)合并支原体感染.发病以冬春季为主(73%),89%系社区获得性感染.临床治愈40例,好转12例,死亡3例(5%);9例(16%)出现神经系统并发症.各年度侵袭性SP检出率间差异有统计学意义(χ~2=33.93,P<0.01);青霉素中介SP和青霉素耐药SP检出率分别为30%和41%;SP对红霉素和氯林可霉素的耐药率高达94%和88%;多重耐药率达89%.结论 IPD好发于5岁以下尤其是2岁以下儿童,24%患儿存在基础疾病.临床疾病以败血症和化脓性脑膜炎最常见.     

Retrospective study of the renal function using estimated glomerular filtration rate and congenital anomalies of the kidney-urinary tract in pediatric Turner syndrome

Although Turner syndrome (TS) is frequently associated with congenital anomalies of the kidney-urinary tract (CAKUT), which is a major cause of pediatric chronic kidney disease, renal function in TS is usually considered normal. The present study aimed to analyze the frequency of renal dysfunction and CAKUT in pediatric patients with TS. Our study included 122 patients with TS between the ages of 2 and 18 years from 30 hospitals across Japan. Clinical data related to renal function and CAKUT were retrospectively collected. The estimated glomerular filtration rate (eGFR) was calculated using the serum creatinine-based formula recommended by the Japanese Society for Pediatric Nephrology. An eGFR <90 mL/min/1.73 m² for two consecutive years was defined as renal dysfunction. Fifteen (13.5%) of 122 patients had CAKUT, and four patients had renal dysfunction (3.2%, 95% confidence interval: 0%-6.7%). Three of the four did not have CAKUT. Of the CAKUT manifestations, horseshoe kidney, renal hypodysplasia, and multicystic dysplastic kidney were seen in nine, two, and one patient, respectively. Eight of the nine patients with horseshoe kidney had a normal renal function; however, the remaining patient with renal hypodysplasia had renal dysfunction. A small percentage of patients with pediatric TS may had an eGFR below 90 mL/min/1.73 m² which was not necessarily associated with CAKUT.