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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.     

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目的探讨应用维持性血液透析(maintenance hemodialysis,MHD)治疗的终末期肾病(end-stage renaldisease,ESRD)患儿的临床特点,为今后儿童MHD的治疗提供参考。方法首都医科大学附属北京儿童医院血液净化中心2000年1月至2011年3月因ESRD行MHD的患儿共23例,其中男12例,女11例;年龄2.5～15.6岁。对其原发病、退出原因、透析并发症及透析充分性等进行统计和回顾性分析。结果在MHD儿童中ESRD的原发病中慢性肾小球肾炎8例,居首位,占34.8%,原因不明者5例,占21.7%。HD开始时均以临时中心静脉双腔管为血管通路,87.0%成功建立动静脉内瘘。动静脉瘘组尿素清除指数(spKt/V)(1.52±0.39),尿素下降率为(70.70±10.52)%,明显优于临时静脉双腔管组的(1.08±0.50)和(56.44±17.33)%。透析并发症以高血压、低血压和透析失衡综合征为常见,高血压发生率达87.0%,而低血压和失衡综合征发生率仅为5.6%和4.7%。退出原因43.5%为回当地医院透析,17.4%肾移植,死亡1例(4.3%),放弃治疗2例(8.7%)...     

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目的提高对甲状旁腺机能减退症(HP)的认识,总结其诊断及治疗经验。方法对深圳市儿童医院2002年4月至2009年4月来诊的10例HP患儿临床资料进行回顾性分析。结果特发性甲状旁腺机能减退症(IHP)5例,假性甲状旁腺机能减退症(PHP)5例;全部病例均存在低血钙、高血磷(除1例血磷正常),IHP甲状旁腺激素(PTH)水平低下,PHP则PTH水平高;经补钙及维生素D治疗后,临床症状均消失,血生化基本恢复正常;婴儿期即诊断明确者,疗效好,无智力及认知功能障碍。结论对癫痫及反复手足搐搦者,应及时行血钙、血磷、血清PTH测定,并行头颅CT检查。用钙剂及维生素D可以控制症状。治疗期间应定期复查血钙、血磷及24h尿钙。     

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目的探讨纤维支气管镜(纤支镜)在小儿喘息性疾病诊断中的作用。方法对2010年9月至2011年3月在厦门大学附属第一医院儿科住院的38例经常规诊治无明显好转的患儿行纤支镜检查,并对其结果进行回顾分析。结果镜下见气管、支气管内膜炎14例;气管、支气管软化8例,气管支气管狭窄4例,气管、主支气管异物4例,管外压迫4例,先天性气管畸形(气管开口异常、气管性支气管等)3例,支气管赘生物1例。≤6个月的婴儿,以先天性气道病变为主(62.5%),而>6个月婴儿,以气管、支气管内膜炎症为主(56.3%)。结论纤支镜术在小儿喘息性疾病的诊断中有重要作用,值得临床推广。     

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??Abstract??Objective??To study the clinical characteristics of neonatal fungal septicemia?? pathogens and related factors in order to provide evidence for prevention and early treatment. Methods??A retrospective study including clinical data?? pathogens and drug sensitivity test?? risk factors for fungal infection?? treatment and prognosis?? was made in 22 cases of neonatal fungal septicemia in the Second Xiangya Hospital??Central South University??from July 2005 to September 2009. Results??The incidence of neonatal fungal septicemia in hospitalized children was 0.64%?? with 13.17% of neonatal septicemia. The most common pathogens were Candida ??86.4%???? such as Candida albicans?? Candida parapsilosis?? Candida Tropicalis and so on. Fungi were highly sensitive to antibiotics commonly used?? such as Fluconazole?? except that one strain was resistant to Itraconazole. Preterm?? low birth weight?? peripherally inserted central venous catheters?? mechanical ventilation?? dexamethasone and multi-antibiotic treatment were significant risk factors for fungal infection. There was significant relation between fungal infection and nosocomial infection. Conclusion??Candida is the main pathogen for neonatal fungal septicemia. Possible strategies to be considered include barrier nursing?? strict aseptic manipulation?? fluconazole prophylaxis?? early empirical antifungal treatment and rational use of antibiotics and hormones to prevent neonatal fungal septicemia and its complication.     

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目的分析儿童横纹肌肉瘤的临床特点,提高其诊治水平。方法对中国人民解放军总医院1998年1月至2002年10月收治的8例横纹肌肉瘤患儿临床资料进行分析。结果8例患儿中,男5例,女3例;发病年龄7个月至11岁,<5岁者3例;依据美国横纹肌肉瘤研究组(IRS)的分期标准I期2例、Ⅱ期2例、Ⅲ期1例,Ⅳ期3例;原发于头颈部4例、四肢2例,泌尿、消化系统各1例。病理类型胚胎型7例,腺泡型1例。临床表现随原发部位不同有很大差异,基本为肿瘤组织占位、侵蚀后引起。明确诊断依靠病理,治疗以手术、化疗和放疗相结合,放疗总量40～50Gy,化疗共有3种方案VDCA、VAC和VadrC。对Ⅰ、Ⅱ期手术彻底切除肿瘤患儿行VAC、VadrC方案交替共2年,Ⅲ、Ⅳ期患儿前14周用VDCA方案,后VAC、VadrC方案交替共2年。全部患儿2年生存率为37.5%(3/8)。结论儿童横纹肌肉瘤是多发于头颈部,病理多表现为胚胎型的极度恶性软组织肿瘤,早期诊断,合理应用手术、化疗与放疗结合的综合治疗,是提高该肿瘤患儿生存率的关键。     

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??Abstract?? Objective To accumulate knowledge of methylmalonic acidemia ??MMA?? screening by studying the clinical characteristics and laboratory characteristics of the disease. Methods The clinical manifestations and the results of laboratory tests of 95 cases of MMA were analyzed. Results The age of onset was from 1 day to 12 years. Six cases had the episodes of vomiting?? lethargy in 14 cases and recurrent difficulty with feeding in 4 cases?? seizures in 24 cases?? mental retardation and movement disorders in 18 cases?? dysarthria in 3 cases?? debilitation and BLE edemas in 10 cases?? jaundice in 7 cases?? complaints of precordium in 6 cases?? anaemia in 3 cases. The laboratory tests showed that the counts of white blood cells??WBC??were increased in 26 cases and the counts of red blood cells ??RBC??were decreased in 26 cases. The results of urine routine tests were abnormal in 37 cases. The results of renal function tests were abnormal in 18 cases. The results of hepatic function tests were abnormal in 17 cases. The results of myocardial enzyme were abnormal in 40 cases. The results of blood gas test were abnormal in 11 cases. The levels of glucose were decreased in 6 cases. The levels of lactic acid were increased in 62 cases. The levels of blood ammonia were increased in 50 cases. Remarkable elevation of urinary methymalonic concentration was confirmed in 97% patients by gas chromatography -mass spectrometry ??GC-MS??. Remarkable elevation of C3?? C3/C0 and C3/C2 concentrations was confirmed in 89% patients by liquid chromatography-tandem mass spectrometry ??LC-MS/MS??. Conclusion The main clinical features of MMA include lethargy?? seizure?? vomiting??developmental retardation or regradation?? hyperarmnonemia and hyperlactacidemia. Urine organic acids analysis by GS-MS and dry blood spots ??amino acids and acylcarnitine?? analysis by LC-MS/MS are very important to the diagnosis of MMA.     

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??Abstract??Objective To expore the clinical features of acute osteomyelitis of neonate. Methods A retrospective study was done about clinical symptoms??radiologic features and treatments in 13 neonates who were diagnosed with osteomyelitis in Children’s Hospital Affliated to Chongqing University of Medical Sciences from 2004 to 2012. Results There were eleven male and two female infants??10 babies were related to bacteraemia and 3 cases were due to infection nearby.Limb bones were most commonly compromised. Six babies had joint involvement. Local swelling and disorder of limb’s activity were the most common clinical symptoms. Twelve patients got positve culture results from blood??pus or joint fluid samples. The most common pathogens were Staphylococcus aureus and Gram-negative bacteria. All of the babies accepted antibiotic treatment while 4 cases also underwent surgeries. All osteomyelitis patients had good outcomes. None had any sequelae among the 7 cases during the following 2 months to 2 years. Conclusion Neonatal osteomyelitis is not a rare disease with atypical clinical features. Radiologic and bacteriologic examination should be carried out once osteomyelitis is suspected. Appropriate antibiotics and necessary surgery are important to avoid further damage as well as long-term sequelae.