血尿、糖尿、肾功能不全

病史摘要患儿 ,男 ,10岁。因蛋白尿、血尿9年 ,发现肾功能不全1月入院。患儿于9年前无明显诱因出现肉眼血尿。查尿常规中尿蛋白 ++～ +++,潜血 +++,肉眼血尿持续20d后转为镜下血尿至今。患儿在外口服中草药 (名称不详 )治疗9年 ,镜下血尿、蛋白尿未减轻。近1个月来查血BUN9.5mmol/L ,Cr130μmol/L。家族史 :患儿母亲11岁曾发现尿常规异常 ,现尿蛋白100mg/dL ,潜血 ++,镜检红细胞8～11/HP ,肾功能正常 ;其父亲尿常规、肾功能正常 ;其舅舅6岁发现有蛋白尿 ,予激素治疗 ,7岁痊愈 ,现查尿常规、肾功能正常。入院时体重26kg,血压105/65mmHg。…     

血浆置换联合连续性血液净化治疗儿童重症溶血尿毒综合征

目的：探讨床旁血浆置换（ plasma exchange,PE）联合连续性静-静脉血液滤过透析（ continuous veno-venous hemodiafiltration,CVVHDF）在抢救儿童重症溶血尿毒综合征（ hemolytic uremic syndrome,HUS）中的作用。方法总结本院PICU收治的HUS患儿的临床特点,采用儿童危重病例评分和儿童死亡危险评分Ⅲ评估病情的危重程度。对4例重症HUS患儿采用床旁PE联合CVVHDF治疗,观察病情演变与转归情况。结果2012年6月至2015年5月我院PICU收治重症HUS8例,经利尿剂、输血等保守治疗12～24 h后,病情加重的4例危重患儿进行PE＋CVVHDF序贯式血液净化治疗。 PE采用Prisma TPE 2000膜式血浆分离器,CVVHDF应用AN69 M60滤器,PE每次新鲜冰冻血浆50～70 ml／kg,连续3～6次,CVVHDF置换液50 ml／（ kg· h）。4例重症患儿治疗后均存活,血生化指标和肾功能指标改善。治疗前后血肌酐平均值（318μmol／L vs．162μmol／L）、乳酸脱氢酶（1963 U／L vs．407 U／L）明显下降,血小板计数明显升高（40×109／L vs．97×109／L）。结论 PE和CVVHDF治疗重症HUS,可以迅速改善患儿血生化指标,稳定内环境,阻断溶血和改善肾功能,可作为重症HUS抢救的重要手段。     

溶血性尿毒症综合征急性期后治疗探讨

目的探讨溶血性尿毒症综合征（HUS）患儿渡过急性期后如何促进肾功能的修复、延缓肾损害进程,探讨HUS急性期后的治疗方法。方法分析1993年至2005年我科收治的17例HUS患儿的临床资料。13例接受急性期后治疗患儿除用血管紧张素转化酶抑制剂（ACEI）和限制蛋白质摄入外,参照中华医学会儿科学分会肾脏病学组制定的“小儿肾小球疾病的临床分类、诊断及治疗”（方案）,按临床分型、对泼尼松治疗的反应及病理类型拟定治疗方案。2例临床表现为肾小球肾炎的患儿中1例应用雷公藤多甙。11例表现为肾病综合征的患儿均用泼尼松;其中5例泼尼松治疗不缓解或部分缓解者,加用环磷酰胺冲击（4例）或甲泼尼龙冲击（1例）治疗;3例因肾组织病理改变为膜增殖＋／-局灶节段性肾小球硬化、新月体形成,加用甲泼尼龙冲击。结果随访2个月～8年,轻型4例（1例复发1次）血压、血尿素氮（BUN）、血肌酐（Cr）及尿常规均正常。重型9例中6例血压、BUN、Cr、尿常规正常;3例持续尿检异常,肾功能不全,且没有坚持治疗,分别于病程的第3、9和13个月死亡。4例（均为重型）放弃治疗者分别于病程的第27～48天死亡。结论对渡过急性期的HUS患儿依据其临床分型和肾病理改变参照肾脏病学组制定的方案治疗有望改善预后。除急性期病情轻重、治疗的合理性影响预后外,患儿家长对治疗的依从性也是一个重要因素。     

血浆置换治疗小儿危重病的临床观察

目的 探讨对危重患儿进行血浆置换治疗的安全性和效果.方法 回顾性分析应用BM25连续肾脏替代治疗机配合血浆分离器对14例患儿进行27次血浆置换治疗的临床资料.结果 血浆置换量为50～100 ml/(kg·次),血流速度为3.5～8.5 ml/(kg·min),每次治疗时间约2～3 h.26例次治疗顺利完成,1例次因患儿血流缓慢堵塞滤器滤过膜后未能顺利完成.血浆置换治疗期间无1例出血.经血浆置换和综合治疗,1例急性脊髓炎、1例吉兰-巴雷综合征、2例脓毒症并多器官功能障碍顺利脱离呼吸机,治愈出院;1例毒草中毒并严重肝损害治愈出院;5例重症系统性红斑狼疮、1例抗中性粒细胞胞浆抗体相关性肾炎、1例紫癜性肾炎并急性肾功能衰竭和1例溶血尿毒综合征患儿肾功能恢复正常,尿蛋白减少,临床症状好转;1例肝功能衰竭、肝豆状核变性并溶血性贫血死亡.结论 血浆置换是治疗扎童危重病有效和安全的方法 .     

儿童紫癜性肾炎治疗进展

紫癜性肾炎是最常见的儿童继发性肾小球疾病。其临床表现不一,轻者仅表现为镜下血尿、微量蛋白尿,重者可逐渐进展为肾功能不全,直至终末期肾脏病而需要依靠长期肾脏替代治疗,严重影响患儿生存质量。近年来过敏性紫癜发生率不断提高,需予以重视。关于紫癜性肾炎的治疗,目前国内外有较多的研究,但结果不尽一致,因此至今未有定论。我们提倡阶梯式疗法,即根据患儿临床表现、肾脏病理选择合适的方案,而对于重症紫癜性肾炎,我们推荐多药强化治疗并联合血浆置换等方法以缓解病情。     

小儿溶血尿毒综合征10例临床分析

目的探讨小儿溶血尿毒综合征(HUS)的临床特点及诊治经验。方法总结分析7a间收治的10例溶血尿毒综合征患儿的临床表现、辅助检查和治疗过程及疗效。结果10例HUS均具备不同程度溶血性贫血、血小板减少和急性肾功能不全。部分病例有上呼吸道感染病史(40%)或腹泻病史(20%)。该病的诊断必须对病史、临床表现以及实验室检查结果进行综合分析,治疗的关键是早期改善肾功能不全、及时纠正贫血及血小板减少。糖皮质激素疗效不佳,不应作为首选,丙种球蛋白对于部分患儿有效,对于重症和不典型HUS患儿,早期联合应用血浆置换可以迅速控制病情,改善症状。结论早诊断、早治疗,选择正确的治疗方案是该疾病取得良好预后的关键因素。     

不同剂量静脉丙种球蛋白或加甲泼尼龙治疗无反应川崎病患儿疗效观察

目的:探讨不同剂量静脉丙种球蛋白（IVIG）或加甲泼尼龙治疗无反应川崎病（KD）患儿疗效观察。方法:回顾性收集2002至2010年首都医科大学附属北京儿童医院IVIG无反应KD住院患儿的临床资料,根据归纳的6种IVIG或加甲泼尼龙的给药方法分为IVIG 2 g组、IVIG 1次1 g组和IVIG 2次1 g组（1次1 g组不敏感病例再次IVIG 1次1 g组方案）。以接受不同剂量IVIG或加甲泼尼龙（2 mg·kg-1×3 d）治疗后48 h患儿体温降至38℃以下定义为敏感,以接受不同剂量IVIG或加甲泼尼龙治疗2周后超声心动图判断冠状动脉是否损伤。结果:9年间在KD急性期接受规范首剂IVIG 2 g·kg-1治疗无反应KD的发生率为18.3%（230/1 257）。IVIG 2 g组40例,36例敏感（90.0%）,4例加用甲泼尼龙敏感;IVIG 1次1 g组190例,123例敏感（64.7%）,7例加用甲泼尼龙敏感;IVIG 2次1 g组60例,25例敏感,35例加用甲泼尼龙敏感。不加甲泼尼龙时,IVIG 2 g组与IVIG 1次1 g组敏感率差异有统计学意义（P<0.01）;IVIG 2 g组与IVIG 1次1 g组、IVIG 2次1 g组敏感率之和差异无统计学意义（P=0.082）。不加甲泼尼龙时,3组中IVIG敏感184例,发生冠状动脉损伤44.0%;IVIG不敏感46例,加甲泼尼龙治疗后均敏感,发生冠状动脉损伤32.6%;加或不加甲泼尼龙治疗的KD患儿发生冠状动脉损伤差异无统计学意义（P=0.183）。3组发生冠状动脉损伤差异无统计学意义（P=0.623）。加或不加甲泼尼龙治疗时,IVIG 1 g·kg-1给药冠状动脉损伤的结局不比IVIG 2 g·kg-1给药差,在药费上减少了一半,如仍不敏感还可选择甲泼尼龙或再次IVIG 1 g·kg-1给药。结论:IVIG无反应的KD患儿中,IVIG 2 g·kg-1比IVIG 1 g·kg-1治疗效果好,因经济条件等所限不能行IVIG 2 g·kg-1再治疗者,可选择先行IVIG 1 g·kg-1治疗,仍不敏感时可选择甲泼尼龙,也可选择再次IVIG 1 g·kg-1治疗。     

血浆置换治疗儿童溶血尿毒综合征(附四例报告)

目的 探讨血浆置换治疗在重症溶血尿毒综合征患儿的应用价值和治疗方案.方法 应用金宝-PRISMA床旁血滤机和TPE2000膜式血浆分离器对4例重症溶血尿毒综合征患儿进行13次血浆置换治疗;以新鲜冰冻血浆作置换液,置换量40 ml/(kg次),血泵速度为50～120 ml/min,治疗时间2～3 h/次.结果 13次血浆置换治疗均顺利成功实施,无明显并发症出现;4例患儿在治疗后临床症状及生化指标明显好转,其中3例痊愈,1例好转出院.结论 血浆置换可以应用于重症溶血尿毒综合征患儿,疗效显著.     

血浆置换在溶血尿毒综合征治疗中的应用

目的 探讨血浆置换在治疗溶血尿毒综合征(hemolytic uremic syndrome,HUS)中的意义.方法 对本院2008年1月至2010年12月收治的16例HUS患儿进行血浆置换治疗;以新鲜冰冻血浆作置换液,治疗时间每次2～3h.结果 16例患儿经血浆置换治疗均存活,无明显并发症出现.全部患儿首次治疗后病情明显缓解;12 -72 h,黄疸消失,血肌酐[(385.0±189.4)μmol/L vs( 100.0±19.3)μmol/L]、乳酸脱氧酶明显下降[(799.3±289.8)U/L vs (300.0±100.4) U/L],血小板计数[(45.0±18.8)×109/L vs( 120.0±20.0)×109/L]、血红蛋白回升[(59.3±15.3) g/L vs (120.0±18.3) g/L],差异均有统计学意义(P＜0.05).出院时15例患儿症状消失,14例实验室检查正常.住院时间为15 ～57 d.结论 血浆置换可以有效缓解病情,清除血浆致病物质,及时阻断HUS的病理过程,补充血浆中有效成分.推荐血浆置换作为治疗HUS的首选治疗方案.     

小儿局灶节段性肾小球硬化38例临床表现与病理特点

目的　了解小儿局灶节段性肾小球硬化 (FSGS)的临床与病理特点。方法　 38例临床诊断为原发性肾病综合征 (NS)或蛋白尿 ,病理确诊为原发性FSGS的患儿 ,进行回顾性分析、总结。结果　FSGS年龄 ( 8.9± 3.7)岁 ,男∶女比为 1 92 ,临床诊断为孤立蛋白尿 3例 ,蛋白尿伴血尿 1例 ,NS34例 (单纯型 16例 ,肾炎型 18例 )。临床伴血尿 2 4例 ( 6 3% ) ,高血压 11例 ( 2 9% ) ,肌酐清除率降低7例 ( 18% )。病理分型为门型 17例 ,周围型 14例 ,Tip型 7例。 38例FSGS中 ,2 1例伴有系膜细胞增生。临床疗效 :34例NS中 ,激素初治敏感 12例 ,观察期末对激素治疗敏感者 6例。 4例非NS中 ,3例激素治疗无效 ,1例不详。应用CTX共 18例次 ,4 4 %缓解或部分缓解 ;应用甲泼尼龙加环磷酰胺冲击或口服环孢霉素A治疗 12例 ,83%缓解或部分缓解。结论　小儿FSGS学龄儿童多见 ,临床多表现为NS ,血尿常见 ,高血压及肾功能不全相对少见 ,病理可表现为多种病变 ,甲泼尼龙加环磷酰胺冲击或口服环孢霉素A治疗方案相对好     

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??Abstract: Objectives??To investigate the clinical characteristics of the children with hemolytic-uremic syndrome??HUS?? and to verify the effect of plasma exchange in HUS. Methods??We analyzed 9 patients with HUS in renal service in Shengjing Hospital of China Medical University from July 2001 to September 2007. All patients were analyzed with main symptoms??lab examinations?? treatment efficiency of steroid and IVIG and plasma exchange??PE??. All patients had been followed up from 0.5??6 years with treatment of oral administration of steroid. Results??It seemed that there was no difference in the clinical characteristics and the lab examinations between the patients with D+ HUS and D- HUS. The patients should be treated with plasma exchange as soon as possible if there was no effect with steroid or IVIG. Blood dialysis or plasma exchange would be added if there was no obvious effect with PE for the first time. Steroid would be orally administered by the HUS patients with proteinuria after PE?? which was effective for proteinuria. Among the following 9 patients?? 1 patient was followed with proteinuria and was done with re-renal biopsy?? which showed increased antigen and renal interstitial injury?? 1 patient with hypertention. There was no abnormal signs for the other 7 patients. Conclusion??It is effective for the patients with HUS treated with plasma exchange. It seems that oral administration of steroid is effective for the HUS patients with proteinuria. All patients with HUS should be paid more attention to in the long-term follow up.     

Pulse methylprednisolone therapy in children with membranoproliferative glomerulonephritis

The effects of pulse methylprednisolone (PM) therapy were studied in 15 patients (aged 3–14 years) with biopsy proven membranoproliferative glomerulonephritis (MPGN). Patients were treated with intravenous PM 30 mg/kg (max 1 g) given over 30 min every other day for a mean of 9.8 days (3–15 days). Oral prednisolone therapy was continued at a dose of 1 mg/kg/24 h for 1 month and subsequently tapered off the following month. Eight patients had hematuria and six had medically controlled hypertension. Serum C₃ levels were low in 11 patients and all of the patients had proteinuria. Following PM therapy proteinuria was significantly reduced from 2602.9 ± 1852.5 mg/24 h to 1871.2 ± 2090.8 mg/24 h (P < 0.05) and at final evaluation, proteinuria was 774.33 ± 1225.67 mg/24 h which was significantly lower than pre- and post-PM therapy values (P < 0.05). Serum creatinine levels were high in five patients before PM therapy and remained high in one of the patients who progressed to end-stage renal failure. After PM therapy, high serum creatinine levels normalized in three patients and was reduced, but still above normal, in one. One patient, with initially normal serum creatinine, had elevated levels afterwards. Nine of the patients were considered responsive and six non-responsive according to our tentatively defined criteria. Mean follow-up period was 27.4 ± 24.1 months (6–84 months). Three patients were lost for follow-up, and 12 were re-evaluated. At final evaluation, all of the patients except one with end-stage renal failure had normal creatinine levels. There was no correlation between the clinical and laboratory features at onset and the outcome of the disease in the responder or non-responder patients. Results of our study show that PM therapy reduced proteinuria and may affect renal function positively in patients with MPGN. However, a prospective controlled study with repeat biopsies is required to draw a conclusion.     

Neuropsychological sequelae of haemolytic uraemic syndrome. Investigators of the HUS Cognitive Study.

BACKGROUND: Severe haemolytic uraemic syndrome (HUS) in childhood can cause stroke, hemiplegia, cortical blindness, and psychomotor retardation. These outcomes are evident at the time of discharge immediately after the acute illness. Less is known about the neuropsychological outcomes of less severely affected children who recover from acute HUS. AIMS: This multicentre case control study investigated the hypothesis that children who survive an acute episode of HUS without recognizable neurological injuries have greater impairment of cognitive, academic, and behavioural functions than controls. DESIGN: Children with HUS were eligible if they had no evidence of severe neurological dysfunction when discharged from one of six Canadian hospitals. Controls had been admitted to hospital for a non-HUS illness and were matched by age, sex, first language, and socioeconomic status. All subjects underwent evaluation of behaviour, academic achievement, cognitive function, and verbal abilities using standardised tests administered by a psychometrist blinded to the case or control status. RESULTS: Ninety-one case control pairs were enrolled. No important differences between patients with HUS and paired controls were evident on tests of IQ, behaviour, verbal abilities, or academic achievement. There was no increased risk of attention deficit disorder among patients with HUS. There was no correlation between the severity of acute renal failure and neuropsychological measures, although scores on some verbal ability tests were lower in those with the highest serum creatinine concentrations during illness. CONCLUSIONS: Children discharged from hospital without apparent neurological injury after an episode of acute HUS do not have an increased risk of subclinical problems with learning, behaviour, or attention.     

儿童新月体性IgA肾病临床与病理分析

目的 了解儿童原发性新月体性IgA肾病的临床、病理和免疫病理的特征 方法 分析9例儿童原发 性新月体性IgA肾病患儿的临床、病理和免疫病理资料,进行疗效观察及随访 结果 临床表现为肾病综合征5例, 急进性肾炎3例,无症状性血尿和蛋白尿1例。尿蛋白均>2 g／d,其中>3 g／d者7例:有持续性肉眼血尿8例,其中>2 周者7例;肾功能减退苦8例,其中5例仅内生肌酐清除率(CCr)轻度下降者;伴高血压7例 9例肾病理均有不同程 度的弥漫性系膜增生和小管-间质病变,平均74.5％肾小球有新月体形成,半数病例可见球囊粘连、小球硬化、节段内 皮增生和间质灶状纤维化。免疫荧光无1例单纯IgA型,IgA M和IgA M G型占55.6％,4例呈"满堂亮"。8例经大剂 量甲基泼尼松龙冲击2~4个疗程治疗,肉眼血尿、高血压全部消失,肾功能恢复正常,蛋白尿有不同程度改善 7例随 访3~18个月,3例尿蛋白正常,2例轻度蛋白尿(<1 g／d),尿蛋白>3 g／d者2例 结论 本组儿童原发性新月体性IgA 肾病临床以肾病综合征为主,持续性肉眼血尿和大量蛋白尿突出,肾功能减退程度不一;肾小球、小管和间质均有急、 慢性病理改变,球囊粘连、小球硬化和间质纤维化易见,免疫病理以IgA合并IgM、IgG沉积为主,可见"满堂亮"现象 及时大剂量甲基泼尼松龙冲击治疗,短期疗效好。     

Neuropsychological sequelae of haemolytic uraemic syndrome

BACKGROUND—Severe haemolytic uraemic syndrome (HUS) in childhood can cause stroke, hemiplegia, cortical blindness, and psychomotor retardation. These outcomes are evident at the time of discharge immediately after the acute illness. Less is known about the neuropsychological outcomes of less severely affected children who recover from acute HUS.AIMS—This multicentre case control study investigated the hypothesis that children who survive an acute episode of HUS without recognisable neurological injuries have greater impairment of cognitive, academic, and behavioural functions than controls.DESIGN—Children with HUS were eligible if they had no evidence of severe neurological dysfunction when discharged from one of six Canadian hospitals. Controls had been admitted to hospital for a non-HUS illness and were matched by age, sex, first language, and socioeconomic status. All subjects underwent evaluation of behaviour, academic achievement, cognitive function, and verbal abilities using standardised tests administered by a psychometrist blinded to the case or control status.RESULTS—Ninety one case control pairs were enrolled. No important differences between patients with HUS and paired controls were evident on tests of IQ, behaviour, verbal abilities, or academic achievement. There was no increased risk of attention deficit disorder among patients with HUS. There was no correlation between the severity of acute renal failure and neuropsychological measures, although scores on some verbal ability tests were lower in those with the highest serum creatinine concentrations during illness.CONCLUSIONS—Children discharged from hospital without apparent neurological injury after an episode of acute HUS do not have an increased risk of subclinical problems with learning, behaviour, or attention.     

Renal Granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature

We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leucocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1–11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Conclusion Renal failure, proteinuria, leucocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure. Received: 4 December 1997 / Accepted in revised form: 22 June 1998     

甲基丙二酸尿症的肾脏损害五例报告

目的探讨甲基丙二酸尿症肾脏损害的临床特点,以期提高认识,达到早期诊断、早期干预和改善预后的目的。方法对尿有机酸分析确诊的5例有肾脏受累的甲基丙二酸尿症患者的临床表现、生化特点以及诊疗情况进行分析。结果5例患者均有不同程度的肾小球及肾小管受累表现,其中2例有肾功能不全,肾脏受累可先于、同时或晚于神经系统受累。5例均为钻胺素反应型,治疗后肾脏损害得到不同程度的改善。结论对于不明原因的血尿、蛋白尿、肾功能下降的患者,特别应注意有无合并神经系统体征,尽早行代谢病筛查及尿有机酸分析,以免延误诊断。     

Clinical features and treatment of children with hemolytic uremic syndrome caused by enterohemorrhagic Escherichia coli O157:H7 infection: Experience of an outbreak in Sakai City, 1996

Presented is a study of 15 patients (seven males and eight females ranging between 5 and 10 years of age) with hemolytic uremic syndrome (HUS) associated with hemorrhagic colitis that was caused by enterohemorrhagic Escherichia coli (EHEC) O157:H7, encountered during the outbreak in Sakai City in July, 1996. The complete form of HUS, which includes the three characteristics hemolytic anemia, thrombocytopenia and acute renal dysfunction, was noted in eight patients, while an incomplete form of HUS, which did not include all three characteristics, was noted in seven patients. Regarding treatment, intravenous gamma-globulin was administered in nine patients and dialysis was performed in five patients (two males and three females) with the complete form of HUS. In three of these five patients, plasma exchange was also performed. Weaning from dialysis was accomplished by the 15th day of disease in all patients. Some patients developed pancreatitis, central nervous system symptoms, fundal hemorrhage and elevation of transaminase, although these abnormalities subsided uneventfully. Renal biopsy, which was performed in two patients who recovered from acute renal failure but still had mild proteinuria and a decrease in creatinine clearance, showed moderate changes in the glomeruli and tubulointerstitium. One year after onset of disease, hematological and urological findings were within normal limits in all patients except one with the complete form of HUS, who still had slightly decreased creatinine clearance.