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小儿急性坏死性筋膜炎是一种严重的小儿外科感染疾病,为皮下筋膜进行性坏死,进展快,病情重,易致中毒休克及多器官衰竭。我院自1993年以来,共收治16例,道如下。一般资料:男10例,女6例,<3个月2例,3个月至岁10例,>3岁4例。发病部位包括臀部2例,腹股沟处例,腰背部5例,腋下6例。患儿卫生条件差,营养不良,部污染严重。典型临床表现为发病急骤、精神萎靡、食差、腹胀,高热或体温下升,病变区迅速扩展,皮肤发凉,苍白、感觉减退、橡皮样硬。6例合并中毒性休克,4例合喉喘鸣,1例合并多器官衰竭,8例合并代谢酸中毒,7.25～7.30,剩余碱-4～-10,实际碳酸氢盐…     

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??Abstract?? Objective To summarize the clinical characteristics of childhood necrotizing pneumonia due to bacterial infection. Methods Clinical features?? radiographic manifestations?? etiology?? treatment and prognosis of 16 cases diagnosed as childhood necrotizing pneumonia according to radiographic manifestations were analyzed retrospectively. Results There were 9 boys and 7 girls in all 16 cases??average age 1.7±1.5 years??. The majority of the patients included in our study are previously healthy. All patients??whose average hospitalization time was 21.4±11.2 days?? were suffering from cough?? sputum and fever ??heat peak 39.4±0.5 ?棬 fever process 16.4±8.0 days and fever clearance time after treatment 9.1±5.1 days??. The average hospitalization time was 21.4±11.2 days. The white blood cell count ??mean value 30.20±12.3×109/L?? neutrophils ratio 0.73±0.12?? were ??5×109/L in 3 cases?? ??5??12?? ×109/L in 3 cases?? and ??12×109/L in 10 cases. All cases showed a significant rise in C-reactive protein ??mean value 115.1±73.3mg/L??. In the period of 11.4±4.0 days?? cystic lesions in lung??which was more common on right lobe?? could be found by chest X-ray or CT. In 7 cases?? pleural fluid or blood culture was positive?? respectively Streptococcus pneumoniae ??2 cases???? Staphylococcus aureus ??2 cases?? and Pseudomonas aeruginosa ??3 cases?? one of which coinfection with Mycoplasma pneumoniae??. There were 13 cases which had Vancomycin and/or Imipenem treatment?? and 5 cases were added Azithromycin or Erythromycin. Closed thoracic drainage was used in 9 cases?? one of which had pleural abscess incision and drainage and fiberboard dissection. None of all cases had pulmonary lobectomy. The pulmonary lesions were mostly or practically absorbed in all cases with in 6 months in follow-up. Conclusion Long duration and fever process?? high level of WBC and severe radiographic performance are usually shown in childhood necrotizing pneumonia. The common pathogen of childhood necrotizing pneumonia are Streptococcus pneumoniae and Staphylococcus aureus?? but Pseudomonas aeruginosa is worth noticing as well. After timely anti-infective therapy?? most childhood necrotizing pneumonia has favorable long-term prognosis.     

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??Abstract:Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia ??PIL) in children. Methods Retrospectively analyze the clinical manifestations?? laboratory examinations?? gastroscopy and imaging features?? lymph radionuclide imaging?? pathological results?? therapy experience in 13 PIL children who were admitted to our hospital from January ??2007 to February?? 2012?? related literatures were reviewed. Results The youngest case was a 4-month-old infant ?? the oldest case was a 11-year-old child?? and 8 cases were within 1 year. Edema??13??100%?????infection??12??92.3%?????diarrhea??11??84.6%?????fever??10??76.9%?????and ascites??9??69.2%????were common manifestations?? hematochezia??3??23.1%????? pleural effusion??3??23.1%?????convulsions?? 3??23.1%????and abdominal mass?? 1??7.7%????were relatively rare. Infection was predominant manifestation?? including respiratory tract??blood??lower limbs fascia and esophagus?? pathogens include bacterium??virus??fungus and ectosarc. The laboratory findings in these patients were lymphocytopenia??13??100%????? hypoalbuminemia??13??100%????? hypogammaglobulinemia??13??100%????? the CD4 cell counts??9??90.0%???? and serum IgG levels ??10??100%?????which were significantly decreased?? while B cell and NK cell counts were reduced in one of ten cases. The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots??13??100%????. Pathological examinations indicated dilated lymphatic channel in mucosal and submucosal ??9??69.2%????. Lymph radionuclide imaging discovered abnormalities consistent with PIL in ten out of twelve patients?? including four cases that were negative for pathological examinations. The main ways of treatment for PIL were introduction of a low-fat and full-MCT diet?? supplement of albumin and globulin??and control of the infection. The temperature returned to normal and edema were alleviated in most patients after internal therapy. However??therapeutic efficacy of diarrhea was not satisfactory??Only three children recovered from hypoalbuminemia and six children recovered from lymphocytopenia. Two cases had a complete remission after surgical therapy without relapse until 2012-12-31. Conclusion Compared to adult IL??PIL in children has similar manifestations??but infection is the predominant clinical manifestation in these patients??lymph radionuclide imaging is suggested to be used as one of the valuable methods in diagnosing PIL in children??internal therapy is not satisfactory in child PIL??surgical therapy can be used if pathological focus is localized.     

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??Abstract??Objective To explore the feasibility of monitoring minimal residual disease ??MRD?? in childhood acute lymphoblastic leukemia ??ALL?? by detection of cloned IgH and TCRγ gene rearrangements using multiplex polymerase chain-reaction ??PCR?? and automated fragment analysis. Methods In this 1??3 matched case-control study?? 4 cases of very early bone marrow relapsed non-high risk ALL ??relapsed group?? and 12 cases of non-relapsed non-high risk ALL as control ??control group?? were enrolled in the First Affiliated Hospital of Sun Yat-sen University from Jan. 2009 to Dec.2011. All patients were treated with Guangdong 2008 ALL protocol. Bone marrow samples were collected at four time points: at diagnosis?? the end of induction?? the beginning of reinduction and the third month of maintenace treatment. Cloned IgH and TCRγ gene rearrangements were amplified by multiplex PCR. The clonality of PCR production was analyzed by GENEMAPPERID softwares. Detectable clonality of IgH/TCRγ was defined as MRD positive. Results At diagnosis?? the frequency of cloned IgH and TCRγ in all patients was 100% and 56%. The positive rate of MRD was found to have no statistical difference between two groups at the end of induction?? while the difference of the MRD positive proportion between the two groups was statistically significant at the beginning of reinduction and the third month of maintenane therapy?? which was much more higher in relapse group than that of control group. Conclusion Detection of monoclonal IgH/TCRγ gene rearrangements by multiplex PCR with automated fragment analysis can be used as a method to monitor MRD during treatment for childhood ALL.     

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??Objective To analyze the correlation of heart rate variability??HRV?? parameters with troponin??cTn?? and N-terminal pro-B-type natriuretic peptide??NT-proBNP?? in children with Kawasaki disease??KD??. Methods According to presence or absence of coronary artery lesion??CAL????the enrolled 118 children with KD as KD group were divided into CAL group??n??43?? and non-CAL??NCAL?? group??n??75??. Meanwhile??32 children without cardiovascular disease in convalescent period and 106 healthy children at the same term were respectively selected as non-KD group and control group. Long-term HRV parameters were detected in each group??and levels of cTn?? and NT-proBNP were detected in KD group and non-KD group. Results HRV parameters in KD group were all higher during recovery phase than those during acute phase??except for LF/HF value lower than that during acute phase ??P??0.05 or P??0.01??. HRV parameters in KD group were all lower than those in control group??except for LF/HF value higher than that in control group ??P??0.05 or P??0.01??. Each index in time domain of HRV and high frequency ??HF????low frequency ??LF?? and very low frequency ??VLF?? in CAL group were all lower than those in control group and non-KD group ??P??0.05 or P??0.01????and SDANN??SDNN and PNN50 were also lower than those in NCAL group ??P??0.01??. Besides??the LF/HF value in CAL group was higher than that in control group??non-KD group and NCAL group ??P??0.05 or P??0.01??. The levels of cTn?? and NT-proBNP in CAL group and NCAL group were significantly higher than those in non-KD group??in which the level of cTn?? in CAL group was also higher than that in NCAL group ??P??0.01??. The level of cTn?? was negatively correlated with SDNN and HF in children with KD??while positively correlated with LF/HF value ??P??0.01??. The level of NT-proBNP had a significantly negative correlation with SDANN??SDNN and HF ??P??0.01??. Conclusion The heart autonomic nervous function is damaged in children with KD. HRV parameters have a certain clinical value for assessment of CAL in children with KD.     

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目的了解单次小剂量(0.4g/kg)静脉输注免疫球蛋白(IVIG)提升初发免疫性血小板减少性紫癜(ITP)患儿血小板至安全范围(≥30×109/L)的作用。方法研究对象为北京大学第一医院儿科2008-04-01—2011-04-01收治初发ITP患儿62例,其中2008-04-01—2009-10-01收治的30例为激素组,初始接受常规剂量醋酸泼尼松治疗;2009-10-02—2011-04-01就诊的32例为IVIG组,初始接受0.4g/(kg·d)IVIG治疗1～5d,每天复查血常规,血小板升至安全范围则规范停用。比较两组治疗第1、3、5天时血小板升至安全范围比例及长期随访结果。结果治疗前,激素组和IVIG组血小板中位值分别是10×109/L和6×109/L。治疗1d后两组血小板升至安全范围的比例分别是3.33%和43.75%,差异有统计学意义(P<0.01)。随访7～42个月后激素组和IVIG组分别有3.45%和3.23%血小板未升至正常(≥100×109/L)。所有患儿均无颅内出血发生及死亡。结论单次小剂量IVIG可使近半数初治ITP患儿血小板升至≥30×109/L相对安全范围,明显高于常规剂量醋酸泼尼松疗效。     

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??Objective??To detect pathogenic genes of short stature with unknown etiology by a targeted next generation sequencing panel to analyze the correlation between genotypes and clinical phenotypes. Methods??A total of 77 children diagnosed with unexplained short stature were enrolled for the study. These children were treated in Ruijin Hospital of Shanghai Jiao Tong University from 2007 to 2015. To search for genetic variation in 187 candidate genes which were associated with growth?? we constructed a targeted next generation sequencing panel encompassing the coding regions of 187 genes. According to ACMG Guidelines??the sites of variation were determined. Sanger sequencing was used to verify the suspected pathogenic genes variation. The relationship between genotype and clinical phenotype was analyzed. Results??Including 5 pathogenic variants?? one likely pathogenic variant and one variant of uncertain significance?? we identified 7 heterozygous variants of 7 cases in 77 cases of short stature with unknown etiology. A pathogenic variant p.D2407fs of ACAN gene was found in a case with advanced bone age. There were 3 reported pathogenic variants?? including p.A72G?? p.I282V and p.P491S of PTPN11 gene?? which were diagnosed as Noonan syndrome. A case carrying known pathogenic variant COL2A1??p.R904C?? was diagnosed as Stickler syndrome. We still got one likely pathogenic variant COMP??p.D401N???? which could cause multiple epiphyseal dysplasia. There was a familial short stature of delayed bone age carrying a variant??p.S289Y?? of uncertain significance??in which the genotype was in accordance with the clinical phenotype. Conclusion??The ACAN gene defection is associated with the idiopathic short stature with advanced bone age. The likely pathogenic variant COMP??p.D401N?? may cause multiple epiphyseal dysplasia. The newly-found heterozygous varians??p.S289Y?? of GHSR gene may result in short stature??which needs further function verification.     

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目的了解青紫型先天性心脏病在新生儿期的临床表现、分布情况、治疗和转归。方法1999年11月至2004年7月上海儿童医学中心对156例青紫型先天性心脏病(CHD)新生儿进行临床分析。结果(1)青紫型CHD在新生儿期主要表现为中央性紫绀、心功能不全,个别可合并心律紊乱。(2)新生儿期青紫型CHD以完全性大动脉转位(TGA)居首位,其他依次为肺动脉闭锁(PA)、完全性肺静脉异位引流(TAPVC)和肺动脉狭窄(PS)。(3)内科治疗包括强心、利尿、扩血管药物和前列腺素E1(PGE1)的运用;156例中57例施行外科手术,其中以TGA最多。结论新生儿青紫型CHD心脏畸形复杂,病情多危重,在生后尽早明确诊断并给予适当的内外科治疗有助于提高其生存率和改善预后。     

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??Objective??To study the clinical value of the test of tidal breathing lung function and exhaled nitric oxide in asthmatic children aged 2 to 5. Methods??216 children with asthma treated from January 2012 to June 2015 in the Pediatric Department of Xijing Hospital of Fourth Military Medical University were listed in asthma group??and 128 normal children in the same period were selected as control group. Tidal breathing lung function and exhaled nitric oxide at the acute and remission stages of asthma were tested respectively??and compared with control group. Tidal breathing lung function were compared before and after bronchial dilation test. Correlation between exhaled nitric oxide level and tidal breath lung function parameters was analyzed. Results??The ratio of time to peak tidal expiratory flow to total expiratory time??TPEF/TE?? and ratio of volume to peak expiratory flow to total expiratory volume??VPEF/VE?? in acute stage of asthma group were significantly lower than those of the asthma group in the remission stage and the control group??P??0.05??. TPEF/TE and VPEF/VE had significantly improved in asthma group in remission stage??but were still significantly lower than those of the control group??P??0.05??. TPEF/TE and VPEF/VE had significantly improved after bronchial dilation test in the acute stage of asthma group??P??0.05??. Taking an improvement rate of ≥15% either for TPEF/TE or for VPEF/VE as an indicator of positive bronchial dilation test??thus the positive rate was 69.90%. The levels of FeNO in acute and remission stages of asthma group???35.50±14.13??×10-9????28.16±5.52??×10-9?? were significantly higher than those of the control group???12.77±7.00??×10-9??P??0.05?????and the levels of FeNO in acute stage of asthma group were significantly higher than those of asthma group in remission stage??P??0.05??. FeNO levels were not correlated with TPTEF/TE and VPEF/VE in acute or in remission stages of asthma group. Conclusion??Tidal breathing lung function??exhaled nitric oxide test and bronchial dilation test are complementary??combined applications can effectively improve the diagnosis and treatment in early childhood asthma.     

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??Objective??To analyze the clinical characteristics and outcomes of children with stage I testicular germ cell tumors??TGCT????and to discuss the treatment strategy for this disease after radical inguinal orchiectomy. Methods??Clinical data of 41 patients with stage I TGCT at Shanghai Children’s Medical Center??Shanghai Jiaotong University School of Medicine between June 2003 to December 2013 were retrospectively analyzed. Their clinical characteristics??therapy and outcomes were analyzed. Results????1??The median age at diagnosis was 18??3 to 43??months old. Among them??38 children??92.7%?? were younger than 3 years old.The pathological subtype included 35 cases of yolk sac??85.4%????4 immature teratoma??9.7%?? and 2 mixed germ cell tumors??4.9%??.??2??Serum AFP levels were elevated in 38 patients??92.7%??. AFP reduced to normal in 27 cases??71.1%??4 weeks after surgery and in 35 cases??92.1%?? after 2 courses of chemotherapy respectively??but 2 cases of them relapsed with AFP increasing again.??3??Forty out of 41 patients underwent surgery and 3??4 courses of chemotherapy with cyclophosvnamide??vincristine and dactinomycin D??VAC??. One patient received surgery alone. The median follow-up period was 64 months. One of the 32 patients who received adjuvant chemotherapy relapsed and then lost follow-up without treatment. The recurrence rate was 3.1% and the 5-year overall survival was 100%. No complication related to chemotherapy occurred during the follow-up. The only one patient treated with surgery alone relapsed at 5 months and achieved complete response after salvage treatment. Conclusion????1??The relationship between the decrease of serum AFP and the prognosis is uncertain and needs to be further studied.??2??Short course chemotherapy with mild toxic side effects can reduce the recurrence rate effectively?? so it can be used as a necessary treatment for patients with high risk of relapse??and it can also be used as a selective treatment for patients with low risk of recurrence.     

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目的探讨川崎病(KD)患儿合并冠状动脉损伤的危险因素。方法按照KD诊断标准,对北京儿童医院2000-01-01—2004-12-31收治的644例KD患儿临床资料、治疗方法以及实验室资料进行分析。结果Pear-son卡方检验显示,患儿性别、发热持续时间、丙种球蛋白使用时间、丙种球蛋白使用剂量、血沉及C反应蛋白与KD合并冠状动脉损伤有关(P<0·05);进一步多因素Logistic回归分析显示,性别、发热持续时间、丙种球蛋白使用剂量与冠状动脉扩张显著独立相关(P<0·05)。结论对男性、发热持续时间长的KD患儿应予足够重视,早期足量使用丙种球蛋白以减少或减轻冠状动脉并发症。