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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.     

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??Objective??To summarize the clinical characteristics of drug-resistant tuberculosis in children and improve the level of diagnosis and treatment of the disease. Methods??The clinical data of 5 children with drug-resistant tuberculosis treated in Beijing Children’s Hospital from March 2013 to March 2017 were retrospectively analyzed. Clinical data included general information??tuberculosis exposure history??clinical and imaging performance??etiological examination??drug sensitivity test??treatment protocol and outcome. All five patients??4 male and 1 female?? had pulmonary tuberculosis??two had tuberculous meningitis??four had bronchial tuberculosis. Results??All had laboratory-confirmed drug-resistant tuberculosis??two were with rifampicin-resistant tuberculosis??RR-TB?? confirmed by Xpert MTB/RIF??one had confirmed by multidrug-resistant tuberculosis??MDR-TB?? cervical lymph node puncture fluid culture??one had by pre-extensive drug- resistant tuberculosis??Pre-XDR?? sputum culture and the other case was MDR-TB confirmed by the positive culture of his father’s sputum. All patients received regimens recommended by WHO??containing second-line anti-tuberculosis drugs??after diagnosis of drug-resistant tuberculosis. Two children were cured??two children were improved??but still receiving anti-tuberculosis treatment. Treatment failed in one child. All children had no severe adverse events. Conclusion??The drug-resistant tuberculosis in children can be diagnosed by molecular technique Xpert MTB/RIF and mycobacterium tuberculosis culture. The molecular technique is of great value in early and rapid diagnosis of drug-resistant tuberculosis. Most children with drug-resistant tuberculosis have a favourable prognosis after timely and effective treatment. The incidence of serious adverse effects of second-line anti-tuberculosis drugs is low in children.     

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??Abstract?? Objective To investigate the diagnosis and management of congenital supravalvular aortic stenosis. Methods Data of 26 cases is collected and analyzed retrospectively?? among which there were mental retardation in 10 cases??growth retardation in 12 cases??positive family history in 2 cases and other heart abnormalities in 4 cases. Results Local supravalvular aortic stenosis was proved in 26 cases by thoracic echocardiography. Catheterization and angiography was performed in 2 cases??and one patient died because of respiration and circulation failure after angiography. Computed tomography angiography was done in 12 cases. Surgical operation was conducted in 13 cases??and 11 patients recovered uneventfully. Of the other two patients??one died during operation??and the other was complicated with brain problem. Conclusions Echocardiography is the basic method for diagnosis; computed tomography angiography and/or catheterization is needed necessarily in establishing diagnosis. Surgical operation is feasible in correcting supravalvular aortic stenosis with satisfied recovery in short-term follow-up??and sometimes pulmonary artery plasty performed if necessary.     

小儿肾病综合征合并结核病31例分析

目的　探讨肾病综合征 (NS)与结核病 (TB)之间的内在联系。方法　回顾分析自 1990年 1月至 2 0 0 1年 4月收治的 31例NS和TB同时存在的患儿 ,并对其相关资料行 χ2 检验统计学分析。结果　①同期收治 6 94例NS患儿中有 31例 (占 4 47% )合并TB ;其中 17例合并肺结核的患儿中仅有 4例 (2 3 5 % )患儿有结核中毒症状 ;②二者同时存在时 ,同时应用抗结核药物及糖皮质激素或免疫抑制剂治疗NS的缓解率 (79 8% ) ,高于单纯应用糖皮质激素或免疫抑制剂治疗 (2 5 0 % ) (P <0 0 1) ,且前者结核感染向活动性结核的转化率低于后者 (分别为 0和 75 0 % ,P <0 0 1) ,部分病例单纯应用后者NS甚至不能缓解 ;③二者同时存在时NS的病理类型多样 ,以毛细血管内增生性肾小球肾炎为主。结论　①NS与TB并存时应同时抗结核治疗 ,即使是结核感染也应预防性应用抗结核药物。②TB可能诱发NS ,且其病理类型多样 ,但以毛细血管内增生性肾炎多见     

Rituximab for troublesome cases of childhood nephrotic syndrome

Nephrotic syndrome (NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab (RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on PubMed and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome.     

环磷酰胺冲击治疗难治性肾病综合征疗效分析

目的　探讨环磷酰胺冲击疗法对难治性肾病综合征的疗效。方法　选择２０ 例难治性肾病综合征患儿,分别给予环磷酰胺（ＣＴＸ）每次１２ｍｇ／ｋｇ,加入１０ ％ 葡萄糖溶液２５０ｍｌ 中静滴,每隔３ ～４ 周１ 次,连用８～１０ 次,后改为每３ 个月１ 次,维持缓解半年至１ 年后停药。累积量１２０～１５０ｍｇ／ｋｇ,ＣＴＸ 冲击同时联合应用强的松方案常规治疗。结果　冲击治疗后,血浆总蛋白及白蛋白有不同程度的升高,２４ 小时尿蛋白定量有明显降低,冲击治疗前后有显著性差异（ Ｐ＜ ０－０１） 。血肌酐（ＳＣｒ）及内生肌酐清除率（ＣＣｒ） 冲击治疗前后无显著性差异（ Ｐ＞ ０－０５） 。随着冲击次数增加,完全及部分缓解例数增多。结论　环磷酰胺冲击疗法对难治性肾病可获较好疗效,尤其对频繁复发和激素依赖的患儿治疗效果好。     

Steroid resistant nephrotic syndrome

Steroid Resistant-Nephrotic Syndrome (NS) is a chronic, progressive disorder affecting upto 10% of all children with NS. It causes morbidity and mortality due to persistent edema, hypertension, hyperlipidemia, thrombosis and infection. Progression to renal failure was thought to be inevitable in survivors. Recent insights into the pathogenesis of the disease has identified several responsible genes and proteins. Studies have shown that long term aggressive therapy with combinations of steroids, alkylating agents and cyclosporine, cause complete or partial remission in 20–80% patients. The use of nonspecific renal protective agents such as the angiotensin converting enzyme inhibitors, angiotensin 2 receptor blockers, and anti-lipid agents retard disease progression. Although these are indications of significant improvement in outcome, further multicentre controlled studies are required to determine the optimum drugs and regimens to be used.     

Steroid resistant nephrotic syndrome

Steroid Resistant-Nephrotic Syndrome (NS) is a chronic, progressive disorder affecting upto 10% of all children with NS. It causes morbidity and mortality due to persistent edema, hypertension, hyperlipidemia, thrombosis and infection. Progression to renal failure was thought to be inevitable in survivors. Recent insights into the pathogenesis of the disease has identified several responsible genes and proteins. Studies have shown that long term aggressive therapy with combinations of steroids, alkylating agents and cyclosporine, cause complete or partial remission in 20-80% patients. The use of nonspecific renal protective agents such as the angiotensin converting enzyme inhibitors, angiotensin 2 receptor blockers, and anti-lipid agents retard disease progression. Although these are indications of significant improvement in outcome, further multicentre controlled studies are required to determine the optimum drugs and regimens to be used.     

小儿肾病综合征并发肺栓塞八例临床及影像学检查

为探讨小儿肾病综合征（ＮＳ）并发肺栓塞的发生情况及其临床特点，对２０例血Ｄ２聚体升高的ＮＳ患儿进行了肺灌注显像检查，检出８例（４０％）合并有肺栓塞：属原发ＮＳ者５例，继发ＮＳ者３例，肺段受累１～６个不等，绝大多数无任何相应临床症状及体征。给予肝素为主的抗凝治疗２周后，６例肺栓塞好转。提示肺栓塞是小儿ＮＳ中较常见的合并症，多数无临床表现，易漏诊，及时诊断治疗，预后良好。     

婴幼儿期肾病综合征临床特点分析

目的　探讨婴幼儿期原发性肾病综合征 (简称婴幼儿肾病 )的临床特点 ;分析婴幼儿肾病临床特点、免疫功能、病理分型和糖皮质激素 (简称激素 )疗效的关系。方法　对 31例婴幼儿肾病患儿进行临床观察 ;进行体液免疫和细胞免疫功能测定 ;14例接受肾穿刺活检 ;31例均采用激素中长程疗法 ,18例予以免疫抑制剂如环磷酰胺 (CTX)等联合治疗。结果　婴幼儿肾病临床以肾炎型肾病为主 ;体液免疫和细胞免疫功能下降 ;病理以非微小病变型为主 ;约 6 0 %患儿对激素治疗不敏感 ,需用激素与免疫抑制剂联合治疗。结论　婴幼儿肾病具有与其它儿童肾病综合征不同的特点 ,应当引起临床重视。     

激素耐药型和激素依赖型肾病患儿肾脏病理计量分析

目的 探讨病理计量分析评价激素耐药型（SR）和激素依赖型（SD）肾病综合征患儿的肾脏病理损害及其临床应用意义。方法 采用自行研制的评分法,对73例SR型和SD型肾病患儿的肾脏病理从病理类型、肾小球病变、小管间质病变、肾脏总的病理损害4个方面计量分析,并以血尿素氮为应变量,临床表现和肾脏病理计分为自变量进行逐步回归分析。结果 （1）病理类型评分结果：微小病变、局灶节段肾小球硬化、膜性肾病、系膜毛细血管性肾小球肾炎和系膜增生性肾小球肾炎五组间的年龄、病程、复发次数、血尿素氮、胆固醇、白蛋白、尿蛋白定量、肾脏总的病理损害的差异均无显著意义,而五组患儿的肾小球病变计分分别为1,5,5,6,5,微小病变组与其他各组比较差异有显著意义（H＝19．278,P＜0．01）。（2）73例患儿肾小球病变计分结果：正常2例（3％）,轻度53例（73％）,中度17例（23％）,重度1例（1％）。且正常、轻度、中度三组间血尿素氮、小管间质病变的差异有显著意义（H＝8．40,P,0．01;H＝11．56,P＜0．05）。（3）肾小管间质病变计分结果：轻度23例（34％）,中度37例（18％）,且轻、中、重三组间病程、复发次数、血尿素氮、肾小球病变计分的差异均有显著意义。（H值分别为25．016,38．775,14．944,10．625,P值均＜0．01）。（4）肾脏损害的总分结果：轻度33例（45％）,中度34例（7％）,重度6例（8％）,轻、中、重三组间病程、复发次数、血尿素氮的差异有显著意义（H值分别为19．42,14．335,18．923,P值均＜0．01）。（5）以血尿素氮为应变量行逐步回归分析,小管间质及肾小球损害对血尿素氮均有显著影响,小管间质病变的回归系数为0．862（P＜0．01）;肾小球病变的回归系数为0．212（P＜0．05）。结论 采用评分法对肾脏病理进行主分评价其病变程度,具有临床实用性,尤其对小管间质病变的计分评价对判断肾功能受损更有价值。     

Cortisone cataract in children with nephrotic syndrome

In a group of 16 children with idiopathic nephrotic syndrome treated with corticosteroids for longer than 12 months, 9 developed a posterior subcapsular cataract (PSC). No correlation between the frequency of PSC and the duration of treatment and the total dose of treatment with steroids was demonstrable. However, the patients with PSC had received considerably higher average daily doses than those without PSC. Two patients with normal ophthalmologic findings at the end of treatment showed PSC 6 and 9 months later respectively. Only one patient acquired a signficant impairement of visus.     

Genetics of idiopathic nephrotic syndrome

Nephrotic syndrome (NS) is a pathological entity characterized by massive proteinuria and has diverse etiology. Although it is one of the most common renal diseases in children, the etiological factors responsible for idiopathic NS/FSGS remain largely unknown. Previous studies had implicated a variety of factors including genetic factors, although NS is generally regarded as a sporadic disease. Familial cases of NS have however been reported periodically, and both autosomal dominant and recessive forms have been identified. Studies of familial NS /FSGS have led to the discovery of several genes that are expressed in podocytes and are associated with proteinuria. These discoveries have shifted the focus from glomerular basement membrane (GBM) to recognition of the central role of podocytes in maintaining glomerular perm selectivity and pathogenesis of NS/FSGS. Associations with various genes (NPHS1, ACTN4, NPHS2, WT-1) and linkage to several chromosomal regions (such as 19q13,11q21,11q24) have been reported in patients with familial NS/FSGS.