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??Objective??To investigate the clinical features??diagnosis??treatment and prognosis of eosinophilic cystitis in pediatric population. Methods??The records of four patients who had been diagnosed and treated for eosinophilic cystitis from January 2012 to May 2015 in Shengjing Hospital were retrospectively reviewed. Results??All the four patients were boys whose age ranged from 6 to 8 years. The main symptoms of the 4 cases were frequent micturition??odynuria??dysuria??suprapubic pain and hematuria.All of the 4 cases had significant peripheral eosinophilia and increased bladder wall thickness. All of the patients were diagnosed with biopsy. Bladder interstitial eosinophil infiltration was revealed by histopathology. The clinical symptoms??peripheral eosinophilia and bladder imaging changes were relieved after steroids and antihistamines treatment. Three cases developed recurrence. Total course of oral corticosteroids ranged from 3 months to 18 months. One case remained persistent remission for 2 years??two cases had are recurrence and one case had two recurrences. Conclusion??Bladder biopsy is essential to establishing the diagnosis of eosinophilic cystitis. Patients with peripheral eosinophilia and the increased bladder wall thickness should be considered with eosinophilic cystitis. Steroids is effective as medical therapy for eosinophilic cystitis and close long-term follow-up is necessary.     

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??Objective To investigate the clinical characteristics and treatment of hypereosinophilic syndrome??HES?? in children. Methods The clinical manifestations?? laboratory examinations??gastroscopy and imaging features??pathological results and therapy experience in 13 HES children admitted in Beijing Children’s Hospital??Capital Medical University from January 2009 to February 2016 and related literatures were analyzed retrospectively. Results Of the 13 patients evaluated?? 7 were male??54%?? and 6 were female??46%??. The median age at diagnosis was 9.6 years old??ranging 3 to 14 years old??. The median course of disease was 23 months??ranging 1 to 72 months??. The peripheral eosionophil counts ranged ??4.5—29.2??×109/L??mean 13.5×109/L??. Gastrointestinal tract was the most commonly involved organ?? and was reported in 92%??12/13?? of patients. It was followed in frequency by urinary system??84%????pulmonary??53%????cardiac??23%????and skin??8%?? and liver??8%??. A total of 2 sites were involved in 6 patients??3 sites were involved in 5 patients and 4 sites were involved in 2 patients. Treatment of oral prednisone therapy was given and follow-up of 10 patients had no clinical symptoms??2 patients had stopped prednisone??. But eosionophil counts still increased to varying degrees. Three patients were lost to follow-up. Conclusion HES in children is more common in school age and adolescent children. Gastrointestinal tract??urinary system and pulmonary involvement are more common. Glucocorticoid treatment is effective??which requires to be maintained in small dose in the long term.     

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现将我院2001年12月至2004年6月收治的5例慢性炎症性脱髓鞘性多发性神经病(CIDP)进行分析。本组5例,男2例,女3例,年龄2~13岁,病程2~12个月,病前有上呼吸道感染史2例,3例无明显诱因。2例为慢性复发型,另3例均为缓慢起病,进行性加重。临床表现:首发症状以行走不稳起病2例,双下肢乏力起病1例,四肢活动障碍起病1例,双侧髋关节活动受限1例。体检:对称性双下肢肌力减退4例,肌力2~4级,另1例双上肢肌力减退,肌力0级;双下肢肌萎缩1例。5例均有腱反射减弱或消失,双侧巴氏征阴性,1例咽反射减弱。所有病例均无明显感觉障碍及自主神经功能障碍。实验室…     

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目的 探讨血浆脑钠素(BNP)、肌钙蛋白Ⅰ(cTnI)质量浓度的变化在心力衰竭(简称心衰,CHF)患儿的临床诊断、预后评估中的意义.方法 2003-02-2005-02于青岛市海慈医院住院诊断心衰的患儿41例,同时选健康体检者41例作为正常对照组,采用放射免疫分析法分别检测其血BNP及cTnI的水平.结果 心衰早期BNP、cTnI即开始升高,心衰期达高峰,恢复期逐渐下降,但仍高于正常对照组(P＜0.01);心衰早期、恢复期BNP阳性率(92.7%,90.2%)较cTnI阳性率(63.4%,58.5%)差异有显著性(P＜0.01);BNP持续高于500 ng/L的心脏事件发生率(66.7%)较BNP＜500 ng/L的心脏事件发生率(21.4%)高,差异有显著性(P＜0.01).结论 心衰患儿血浆BNP、cTnI质量浓度明显升高,BNP、cTnI可反映其心肌损伤;血浆BNP在心衰早期诊断方面,其敏感性及特异性均优于cTnI,可作为诊断小儿心衰的一项重要指标;血浆BNP则可作为判断预后的一个重要参考依据.     

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目的通过复习文献、结合病例总结儿童髓质海绵肾的临床特点,提高对本病的认识,以达早期诊断和治疗,改善预后。方法对苏州大学附属儿童医院2005年1月至2010年12月收治的11例儿童髓质海绵肾患儿的临床资料进行回顾分析。结果 11例患儿中男3例,女8例。年龄48d至17岁,其中<1岁6例,>3岁5例。临床表现无特异性,均由影像学确诊为双侧髓质海绵肾。表现反复尿路感染5例、远端肾小管酸中毒7例、肾结石1例、高血压2例、发育迟缓6例、肾衰竭2例、肾上腺皮质功能亢进1例。结论髓质海绵肾儿童期发病临床症状无特异性,表现形式多样化,早期诊断依靠辅助检查,影像学检查B超、CT可作首选,存在合并症时合理治疗可改善预后。     

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??Objective??To investigate the clinical diagnosis treatment and prognosis of young children infected by leishmania with hemophagocytic syndrome ??HPS?? in Xinjiang. Methods??During December 2007and December 2009??16 patients of young children with HPS were hospitalized in the Pediatric Department of the People’s Hospital??Xinjiang Uyghur Autonomous Region. The data of patients’ habitation?? clinical presentation and laboratory results were reviewed and analyzed. Results??All the 16 patients came from the Tarim Basin in Xinjiang and the diagnosis was supported by laboratory tests. In nine patients??Lidou body ??Leishmania donovani?? were found in bone marrow??and in fourteen patients??serum rK39-ELISA ??rK39?? Leishmania donovani gene expression product?? ELISA?? enzyme-linked immunosorbent assay?? test was positive. The first two cases among these16 patients were misdiagnosed as familial HPS??who had no response to the treatment and died. The other 14 cases were diagnosed as Assam fever caused by visceral leishmania??and sodium antimony gluconate was administered. Fourteenpatients were cured. Conclusion??In the Tarim Basin??in young patients with HPS the visceral leishmania infection should first be considered. Serum rK39-ELISA test is helpful in the diagnosis of Leishmania infection. The HPS Assam fever in young children is often emergent and severe ??the children would die quickly unless an early diagnosis were made and appropriate treatment were given.     

小儿血液透析应用特点和临床分析

探讨血液透析（HD）在儿科应用的特点及小儿肾功能衰竭HD的临床意义。方法对20例1a～14a危重肾功能衰竭及药物中毒患儿进行67次HD分析。结果所有病例在HD后肾功能明显改善,尿毒症症状减轻。痊愈、好转各6例,自动出院、死亡各4例,无1例死亡与透析直接有关。主要并发症为失衡综合征（11．9％）和低血压（10．5％）。结论HD成功的关键在于血管通路的建立、血容量的稳定及并发症的防治。HD抢救危重肾功能衰竭患儿疗效迅速、安全、可靠。     

小儿血液透析267例次临床分析

报道我院自１９７８年１０月￣１９９４年１１月为３７例肾功能衰竭患儿进行２６７例次血液透析,占同期我院血液透析中心总数１９４８４例次的１．３％,结果：痊愈１４例,好转１６例,死亡７例,主要并发症为失衡综合征（１９．１％）,低血压（１８．０％）和心力衰竭及肺水肿（１．９％）,通过血液透析,降低了小儿明功能衰竭的死亡率。根据临床实践及有关实验研究,提出有关小儿血液透析必须遵循的特点及做法。     

Cardiovascular risk assessment in children following kidney transplantation

Dégi A, Kerti A, Kis é, Cseprekál O, Tory K, Szabó AJ, Reusz GS. Cardiovascular risk assessment in children following kidney transplantation. Abstract: CV diseases are the leading cause of death among patients with ESRD. RTX decreases the CV risk; however, it still remains definitely higher than that of the general population. Large multicenter and longitudinal studies are difficult to perform and hard end-points of CV events are usually missing among pediatric population. Thus, appropriate estimation of CV risk is of crucial importance to define the potential hazards and to evaluate the effect of treatments aimed to reduce the risk. A number of validated non-invasive methods are available to assess the extent of CV damage in adults, such as calcification scores, cIMT, aPWV, 24-h ABPM, AASI, and HRV; however, they need adaptation, standardization, and validation in pediatric studies. cIMT and PWV are the most promising methods, as pediatric normative values are already present. The up-to-date treatment of ESRD aims not only to save life, but to offer the patient a life expectancy approaching that of the healthy population and to ensure a reasonable quality of life.     

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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.     

儿童可调钠透析的临床应用

目的 观察可调钠透析（PHD）在透析患儿中应用的安全性，确证PHD是否对儿童血液透析的并发症有预防作用以及是否会增加患儿的钠负荷。方法 28例血液透析（HD）的肾功能衰竭患儿，年龄1．3～15．1岁（男11例，女17例），其中6例应用普通透析（CHD），匀速脱水；4例应用PHD，脱水先快后慢；18例患儿应用了2种透析方法；共进行HD319例次，其中PHD129例次，CHD190例次。结果 PHD和CHD两组间透析前、后血钠浓度、尿素氮、肌酐、渗透压及钠清除差异均无显著性（P〉0．05）。PHD组中低血压和失衡综合征的患病率分别为5．4％和9．3％，明显低于CHD组的12．1％和17．9％（P〈0.05）。结论 PHD结合先快后慢的程序超滤与CHD相比，在毒素清除率相同的基础上可减少透析低血压和失衡综合征的患病率，有更好的血容量维持作用，且不伴有钠负荷的增加。     

Causes and outcome of late referral of children who develop end-stage kidney disease

Aims: This study aims to characterise the timing of referral to a paediatric nephrology unit of children who develop end‐stage kidney disease (ESKD). This study also aims to determine whether late referral (LR) influences outcomes and to explore factors that may lead to LR. Methods: A retrospective case review of all incident patients with ESKD who received renal replacement therapy (RRT) at a single paediatric centre. Time between referral to a paediatric nephrologist and commencement of RRT, demographic and clinical data were collated. Estimated glomerular filtration rate (eGFR) at referral was calculated using height and creatinine. LR was defined as having an eGFR ≤30 mL/min/1.73 m² when first seen by a paediatric nephrologist. Results: RRT was initiated for 74 patients <18 years of age between 1988 and 2010. The median age at referral was 2.0 years (birth–15.9 years) and age at RRT was 10.0 years (6 days–17.4 years). Children referred before age 1 year (41%) had a more prolonged course before ESKD. Median (interquartile range) eGFR at referral of children >1 year was 27.2 (9.0–52.0) mL/min/1.73 m². Twenty‐two (55%) of these children were referred late (LR) with an eGFR ≤30 mL/min/1.73 m². LR patients were more likely to have glomerulonephritis or haemolytic uraemic syndrome and to live in a remote or outer regional area. LR patients had higher urea, lower haemoglobin and were more likely to receive haemodialysis via a vascular catheter. Conclusions: A significant proportion of children who develop ESKD are referred late to nephrology units with potentially preventable complications. Aetiology of renal disease and geographic isolation contribute to LR.     

Effect of hemodialysis on carnitine levels in children with chronic renal failure

BACKGROUND: Impaired structural and metabolic integrity of the kidney in chronic renal failure (CRF) effects carnitine metabolism by means of many factors. Depletion due to hemodialysis (HD) is one of the major concerns. The aim of the study was to investigate the effects of chronic renal failure and HD on plasma free carnitine (FC) concentrations in children. METHODS: Plasma FC concentrations were measured in age-matched 14 undialyzed patients, 20 dialyzed patients and 12 healthy children. In the HD group, measurements were done pre- and postdialysis and an hour after ceasing HD. None of the children have been receiving exogenous l-carnitine replacement. RESULTS: Plasma FC concentrations on either HD or conservative treatment were found to be decreased as compared to the healthy subjects (P < 0.001 and P = 0.001, respectively). The patients on HD had lower levels of plasma FC at the predialysis period than those on conservative treatment (P = 0.01). The FC levels significantly dropped at the postdialysis period as compared to those at the predialysis period (P < 0.001), but recovered at 1 h after ceasing HD. The mean duration of HD did not correlate with plasma FC levels at predialysis period. CONCLUSIONS: Children with CRF, either dialyzed or undialyzed, have decreased plasma FC levels. Hemo-dialysis treatment significantly depletes plasma FC concentrations during the procedure, but predialysis levels are reached 1 hr after ceasing HD.     

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目的探讨连续性静-静脉血液透析/滤过(CVVHDF)对小儿急性肝功能衰竭的治疗作用。方法 2009年5月至2010年5月期间,采用CVVHDF辅助治疗上海交通大学附属儿童医院重症监护病房(PICU)收治的急性肝功能衰竭患儿共5例。其中男2例,女3例。年龄10个月至5岁。入院时发病时间3～14d。药物性肝损(对乙酰氨基酚)3例,严重脓毒症合并肝损1例,阑尾炎术后门静脉炎引起的肝损1例。肝性脑病分级:2级1例,3级2例,4级2例。观察治疗前后病情及肝功能指标,并观察处理并发症。结果 CVVHDF治疗开始时间为入院后(2～24h),治疗时间为(24～144h),5例患儿中治愈3例,自动出院1例,死亡1例。CVVHDF治疗后12h,丙氨酸转氨酶(ALT)从(3888.76±2373.60)U/L下降至(3284.80±1974.80)U/L,治疗后24～48h继续下降,差异有统计学意义(F=3.58,P<0.05);CVVHDF治疗后12h,血氨从(209.00±53.61)μmol/L下降至(158.80±60.93)μmol/L,差异有统计学意义,以后继续好转(F=3.75,P<0.05)。CVVHDF治疗后12...     

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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.