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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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??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.     

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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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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.     

Hematopoietic Progenitors in Children with End-Stage Renal Disease

Bone marrow and circulating eythroid progenitors (BFU-E) in six anemic children with end-stage renal disease (ESRD) were 2.0 and 1.9 times as abundant, respectively, as in six age-matched normal controls and were significantly more responsive in vitro to low concentrations of recombinant human etythropoietin (rHuEpo) than those from the controls. After 4 weeks of rHuEpo therapy, both the number and the in vitro rHuEpo response of circulating BFU-E in the ESRD patients returned to normal control values. The numbers of bone narrow and circulating granulocyte-monocyte progenitors in the ESRD patients before and after rHuEpo therapy were comparable to those of normal controls. There was 120 inhibition of in vitro erythropoiesis by either the patients' serum or medium conditioned by their mononuclear cells. These results demonstrate a significant abundance and an increased rHuEpo sensitivity of BFU-E in anemic children with ESRD with no evidence of the presence of uremic inhibitors to erythropoiesis.     

Hematopoietic Progenitors in Children with End-Stage Renal Disease

Bone marrow and circulating eythroid progenitors (BFU-E) in six anemic children with end-stage renal disease (ESRD) were 2.0 and 1.9 times as abundant, respectively, as in six age-matched normal controls and were significantly more responsive in vitro to low concentrations of recombinant human etythropoietin (rHuEpo) than those from the controls. After 4 weeks of rHuEpo therapy, both the number and the in vitro rHuEpo response of circulating BFU-E in the ESRD patients returned to normal control values. The numbers of bone narrow and circulating granulocyte-monocyte progenitors in the ESRD patients before and after rHuEpo therapy were comparable to those of normal controls. There was 120 inhibition of in vitro erythropoiesis by either the patients' serum or medium conditioned by their mononuclear cells. These results demonstrate a significant abundance and an increased rHuEpo sensitivity of BFU-E in anemic children with ESRD with no evidence of the presence of uremic inhibitors to erythropoiesis.     

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.     

Low bone mineral density and nutritional vitamin D deficiency in pediatric renal transplant recipients: Assessment of risk factors and response to oral vitamin D therapy

VitD deficiency and bone disease are common after Tx. Prevalence and risk factors for low VitD and BMD and response to VitD therapy were investigated in pediatric renal Tx recipients. 25-hydroxy VitD levels of 71 Tx were compared to 54 healthy AA children. DXA of 44 Tx were compared to 47 AA controls. Of Tx, 59% were AA. Majority (59.1%) of Tx were VitD deficient (23.9%) or insufficient (35.2%). Prevalence of low VitD levels was double in AA (73.9%) vs. non-AA Tx (37.7%), (p = 0.003). Low VitD among Tx was associated with AA ethnicity (p < 0.01), winter (p < 0.05), older age (p < 0.05), males (p < 0.05) and time <6 months post Tx (p < 0.05). Tx with low VitD were treated with oral ergocalciferol or cholecalciferol (23 each); 13% treated with ergocalciferol vs. 82.6% treated with cholecalciferol achieved repletion (p < 0.0001). Of 36 Tx with whole body DXA, 19.5% had BMD (z < -1) after height adjustment. AA Tx had 3.4-fold higher risk of low BMD vs. controls (p < 0.05). Low VitD and BMD are prevalent in children after renal Tx. Better repletion of VitD is achieved with cholecalciferol.