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目的探讨液质联用串联质谱技术在筛查以黄疸为主要表现的肝病患儿中的临床意义。方法选取2010年4月至2011年4月首都医科大学附属北京儿童医院肝病门诊就诊和消化内科住院且临床表现为黄疸、肝大、肝酶增高的儿童75例。收集其干血滤纸片进行液质联用串联质谱检测,并且进行数据分析。结果 75例患儿中有49例患儿的串联质谱分析结果正常;26例患儿出现不同程度的氨基酸谱或酰基肉碱谱的异常,其中包括有确诊遗传代谢性疾病5例和非遗传代谢性疾病15例,6例患儿尚未明确诊断。结论临床上患儿出现不明原因的肝大、黄疸或肝酶增高等,尤其伴有血氨(NH3)或血清乳酸(LAC)升高时,应该考虑遗传代谢性疾病的可能。一些非遗传代谢性疾病患儿也可因肝脏继发损伤出现氨基酸谱或酰基肉碱谱的异常。     

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??Objective??To analyze the influence of perinatal period factors??feeding strategies??probiotics usage??complications and mechanical ventilation before the onset of the disease on neonatal necrotizing enterocolitis??NEC????in order to explore the risk factors for NEC. Methods??Totally 76 infants with NEC were classified as the case group??who were hospitalized in the First Neonatal Department of Shengjing Hospital of China Medical University from January 2011 to January 2016??80 infants without NEC who were admitted at the same time were randomly selected as the control group. A retrospective analysis was performed on the clinical data of these patients??including general conditions of newborns??perinatal period factors??feeding strategies??probiotics usage??complications and mechanical ventilation before the development of NEC. Single and multiple factor analysis was performed on 19 items using the SPSS analysis software. Results??Single factor analysis revealed that the incidence of asphyxia??sepsis??hypoglycemia and mechanical ventilation was higher??while the incidence of oral probiotics usage was lower in the case group than the control group??with statistically significant difference??P??0.05??. According to the logistic multivariate regression analysis??sepsis??OR??6.531????hypoglycemia??OR??2.785?? and mechanical ventilation??OR??3.448?? were risk factors of NEC??while oral probiotics usage was protective factor of NEC??OR??0.417??. Conclusion??Sepsis??hypoglycemia and mechanical ventilation are risk factors of NEC??and oral probiotics usage is protective factor of NEC. We should pay attention to the prevention and intervention of NEC by reasonably analyzing these factors??in order to improve the prognosis of these children.     

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目的了解冬春季呼吸道感染住院患儿中7种常见呼吸道病毒的感染状况。方法采集2006年11月至2007年5月及2007年11月至2008年5月于新疆维吾尔自治区人民医院儿科住院的共479例呼吸道感染患儿的鼻咽分泌物,用直接免疫荧光方法,检测呼吸道合胞病毒(RSV)、腺病毒(ADV)、甲型流感病毒(IfuA)、乙型流感病毒及副流感病毒Ⅰ、Ⅱ、Ⅲ(ParaⅠ、Ⅱ、Ⅲ)型7种常见呼吸道病毒抗原。结果 2006—2007年冬春季7种病毒阳性检出率为24.3%,2007—2008年冬春季阳性检出率为41.8%。2年冬春季7种病毒中RSV检出率均占首位,分别为20.1%及32.3%。结论 RSV感染是新疆维吾尔自治区人民医院冬春季呼吸道感染住院患儿的最主要呼吸道病毒病原。ADV及ParaⅢ型感染位居第二、三位,而其他4种病毒感染少见。     

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??Objective To analyse the treatment effects of bronchoscopy lavage treatment for RMPP??and to study the proper time to do the operation. Methods A total of 76 cases??from June to December 2013??were enrolled in the retrospective study. According to the the course of the disease??there were divided into three groups??≤2 weeks????2??3 weeks and ??3 weeks. The febrile period??duration of hospital stay??WBC??N%??L%??CRP??the chest radiography and the bronchoscopic findings were compared with each other. Results The febrile period??≤2 weeks group it was 13.9±3.8?? in ??2??3 weeks group??14.1±5.0?? in ??3 weeks group??14.1±5.0?? they all had significant difference between each other. The chest radiographic changes were divided into complete absorption??partial absorption and no change. Compare the chest radiography at the 7th day after operation?? the ratio was 14.3%??5.4% and 0??85.7%??75.8% and 76.0%??0??18.9% and 24.0%. At one month??the ratio was 41.4%??26.3% and 11.3%??42.4%??65.7% and 75.1%??0??7.9% and 13.6%. According to the bronchoscopic findings we analysed??the airway mucosal or bronchial wall’s lesions in RMPP mainly were white mucosa covered??mucosal erosions and roughness. Lumen stenosis??dilator??obliteration and mucosa plug were seen more in the third group. Conclusion In the treatment of RMPP children??earlier bronchoscopy therapy can help shorten the fever course and promote chest radiography improvement. The mycoplasma infection of long time could cause lumen stenosis??dilator??obliteration and mucosa plug.     

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??Abstract?? Allergic disorders and rheumatic diseases are two common diseases of dysregulated immune system in children?? with the feature of long disease course and easy relapse??and the incidence of the two diseases is increasing. All these features are bad for the prognosis of children with the diseases. Prior Th1/Th2 theory deemed that Th1-mediated autoimmune diseases and Th2-mediated allergic conditions are complementary both on the mechanism and incidence?? which improve patients’ outcomes with a new balance of T cell subsets. Recent studies?? however?? have served to challenge the idea that the presence of allergy and autoimmunity are mutually exclusive states and suggest that allergic diseases and rheumatic diseases are risk factors for each other. Clear understanding of the relationship between children’s rheumatic diseases and allergic disorders will have important implications for clinical treatment and diagnosis. This article clarifies the influence of allergic diseases on children’s rheumatic disease.     

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??Children often visit their general practitioner for treatment of chronic cough. Recently?? pediatricians have paid more attention to protracted bacterial bronchitis??PBB?? as it is one of the most common causes of chronic wet cough in children. PBB is associated with persistent or protracted bacterial infection of the conducting airways. Clinical diagnosis is very important. Haemophilus influenzae?? especially non-typable H. influenza strains?? streptococcus pneumoniae and moraxella catarrhalis are the three most commonly identified bacteria. The occurrence of PBB is related to bacterial biofilm formation in the airway??impaired mucociliary clearance??systemic immune function defects??and airway anomalies. The common symptoms of PBB include wet cough??wheezing??and stridor. Pulmonary signs are moist rales and/or wheezing. The current international clinical guidelines recommend antibiotic treatment as appropriate management for PBB. A two-week amoxicillin/clavulanate regimen will achieve cough relief in a significant number of children with PBB.     

Improved outcomes in pediatric liver transplantation for acute liver failure

Miloh T, Kerkar N, Parkar S, Emre S, Annunziato R, Mendez C, Arnon R, Suchy F, Rodriguez‐Laiz G, Del Rio Martin J, Sturdevant M, Iyer K. Improved outcomes in pediatric liver transplantation for acute liver failure.
Pediatr Transplantation 2010: 14:863–869. © 2010 John Wiley & Sons A/S. Abstract: OLT is a life‐saving option for ALF. Aim: To evaluate our outcomes in pediatric OLT for ALF. Methods: Retrospective review of our data between 1992 and 2007. Results: Of 142 children with ALF, 126 were listed, of which 40 spontaneously improved, nine died, and 77 underwent OLT (median waiting time four days). Fifty‐three children received deceased donor grafts (34 whole and 19 split grafts), and there were 24 living donor grafts. The one‐ and five‐yr patient survival was 87% and 80%, and graft survival 83% and 79%, respectively. Thirteen patients died after OLT, and there were nine retransplants in seven patients. Patient weight, length of stay, creatinine, and infection were significantly associated with death; increased weight and black ethnicity were associated with graft loss on univariate analysis, but not on multivariate analysis. There were no significant differences in patient survival (one and five yr), graft loss, or other complications between the groups. Conclusion: We report the largest single‐center study of OLT in pediatric ALF, demonstrating no difference in outcomes between different graft types. Our liberal use of segmental grafts may allow earlier OLT in this high‐risk cohort and contribute to our excellent outcomes.     

儿童急性肝衰竭120例病因和预后分析

目的探讨儿童急性肝衰竭(PALF)的病因、预后及预后影响因素,为临床治疗提供依据。方法收集2008年5月至2018年5月在湖南省儿童医院住院并确诊为PALF患儿的临床资料,对其病因和预后进行分析,并根据预后分为死亡组和存活组,比较2组生化指标,根据不同资料分别采用t检验、Wilcoxon检验和χ2检验进行统计学分析。结果120例PALF患儿中,男68例,女52例;婴儿36例,幼儿34例,学龄前期22例,学龄期28例。病因方面:脓毒症20例(16.7%)、遗传代谢性疾病19例(15.8%)、中毒18例(15.0%)、病毒感染12例(10.0%)、药物6例(5.0%)、胆道息肉1例(0.8%)、肿瘤性疾病1例(0.8%)和病因不明43例(35.9%)。在已知病因中,婴儿以感染和遗传代谢性疾病为主,幼儿以感染和药物/毒物为主,而学龄期和学龄前期以药物/毒物和遗传代谢性疾病为主;PALF患儿病死率为50.0%;EB病毒相关噬血细胞综合征、脓毒症、希特林蛋白缺乏症和酪氨酸血症合并PALF病死率高;与存活组比较,死亡组总胆红素(TB)[159.00(73.05,274.00)μmol/L比62.75(2.65,221.75)μmol/L]、结合胆红素(DB)[83.00(41.43,160.00)μmol/L比38.74(10.98,128.75)μmol/L]、凝血酶原时间(PT)[39.60(24.93,62.60)s比24.65(21.43,29.83)s]、国际标准化比值(INR)[3.40(2.30,6.74)比2.09(1.85,2.84)]、血氨(NH3)水平[109.50(85.25,149.75)μmol/L比80.00(60.25,102.75)μmol/L]升高,差异均有统计学意义(均P<0.05);死亡组清蛋白(ALB)[(28.72±5.88)g/L比(33.69±4.96)g/L]、丙氨酸转氨酶(ALT)[586.50(223.25,1082.00)U/L比1434.00(615.00,3334.50)U/L]和天冬氨酸氨基转移酶(AST)[827.50(545.00,2024.00)U/L比1663.50(821.00,4886.75)U/L]水平降低,差异均有统计学意义(均P<0.05),而血糖和胆固醇水平无明显变化。结论PALF病死率高,不同年龄段病因不同,TB、DB、PT、INR、NH3水平升高及肝性脑病发病率升高,ALB、ALT和AST降低提示预后不良。     

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??The true incidence of fulminant hepatic failure in the pediatric population is unknown?? though fulminant hepatic failure is a rare but devastating syndrome??such as hepatic encephalopathy??DIC??MODS?? that results in the death of most children affected. The mainstay of the treatment is liver transplantation??however?? organ shortage limits its use.The purpose of this review is to introduce the current situation of fulminant liver failure in children??focusing on assessing the application of artificial liver??which is considered to play a pivotal role in the treatment of fulminant liver failure.According to its classification??we mainly discussed the application of non-biological artificial liver?? including its indications??advantages and disadvantages??especially the MARS. At present?? the artificial liver treatment also faces many problems. None of the ELS techniques has yet been evaluated systematically in children??and survival benefits have not yet been demonstrated.     

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??Early and accurate prognosis assessment of patients with fulminant liver failure is difficult?? but critically important for optimum clinical pathway?? improving survival rate and appropriate utilization medical resources?? especially liver transplantation. To date in the pediatric field there is no ideal prognosis assessment in children with fulminant liver failure. This article review the variety of prognostic criteria and scores and clinical common biochemical indicator widely used in adult and application status in pediatrics.     

Renal function outcome in pediatric liver transplant recipients

The orthotopic liver transplantation (OLT) allows survival of children followed for severe hepatic injury, provided that the immunosuppressive treatment is prolonged. The nephrotoxicity of cyclosporine predicts the long-term outcome of the adult patients receiving a liver transplant. The aim of this study was to determine the long-term outcome of renal function in children receiving OLT. This study included 12 children, with a median for age of 7.1 yr (2-15 yr) at the time of OLT. The duration of follow-up was at least 4 yr, being 7 yr in 10 patients and more than 10 yr in seven. Renal function was evaluated with the serum level of creatinine, calculated glomerular filtration rate (cGFR), and measurement of glomerular filtration rate using chrome 51 ethylenediaminetetraacetate ((51)Cr EDTA) clearance performed at least once during follow-up. The doses and the serum concentrations (C(0)) of cyclosporine were reported at each study time. The cGFR decreased significantly 2 yr after the OLT [median (range): 106 mL/min/1.73 m(2) (71-150) at the time of OLT vs. 85 mL/min/1.73 m(2) (57-128) 2 yr after the OLT, p = 0.03], and decreased again between 7 and 10 yr after OLT [median (range): 99 mL/min/1.73 m(2) (76-125) 7 yr after OLT vs. 81 mL/min/1.73 m(2) (66-140) 10 yr after OLT, p = 0.04]. Six patients developed chronic renal failure (cGFR from 57 to 80 mL/min/1.73 m(2)) 2 yr after OLT associated with high doses of cyclosporine [median (range): 8.8 mg/kg/day (3.5-13)]. The cGFR overestimated renal function by 16% compared with the isotopic measurement of GFR (p = 0.03). Using the (51)Cr EDTA measurement, six of seven patients followed up more than 10 yr after OLT presented mild (n = 3) or moderate (n = 3) chronic renal failure. In our study, the majority of OLT recipients developed a chronic renal failure 10 yr after transplantation. Cyclosporine seems to be the most important factor responsible for the impairment of renal function. The use of the mycophenolate mofetil, a new immunosuppressive agent, allowing a reduction in the dose of cyclosporine, could minimize renal dysfunction. While awaiting the results of a prospective long-term study, close drug monitoring is advised.     

Serum alpha-NH-butyric acid may predict spontaneous survival in pediatric acute liver failure

Abstract:  ALF is a serious, often fatal condition. Up to half of PALF patients do not survive without liver transplantation; however, early identification of those least likely to survive spontaneously remains difficult. Clinical experience suggests that recovery from ALF depends on the ability of the liver to regenerate. Based on this, we hypothesized that bio-markers of hepatic regeneration could have utility as predictors of recovery from PALF. In the studies reported here, we used comprehensive amino acid analysis to search for novel metabolomic markers of liver regeneration in mice subjected to partial hepatectomy. This analysis identified α-NH₂-adipic acid and α-NH₂-butyric acid as significantly increased in liver and plasma samples from mice subjected to partial hepatectomy compared to controls. Next, we tested whether serum levels of these markers were associated with clinical outcomes in PALF patients. This examination, performed on the initially collected serum samples from 40 randomly selected patients enrolled in the PALF Study Group, showed increased α-NH₂-butyric-acid (Aab) and Aab:leucine (Aab:Leu) ratio in patients who survived without transplantation compared to those who were transplanted or died. These data indicate that Aab and the Aab:Leu ratio may predict clinical outcomes in PALF.     

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??The causes of acute liver failure ??ALF?? in children differ from those observed in adults. They can be schematically grouped into six categories?? metabolic?? infective?? toxic?? autoimmune?? vascular and malignancy-induced ALF. Although the mechanisms of liver injury contributing to ALF vary due to different causes and are incompletely understood?? they can be summarized into two categories?? direct damage and immune-mediated liver injury. Cellular necrosis and apoptosis are the final changes of pathology. This article will review the current knowledge of the epidemiology and mechanisms of ALF in children.     

Hodgkin disease relapse discovered at the time of liver transplant for acute liver failure

Lymphoma is a recognized cause of liver damage and in rare instances presents as ALF. In such cases, the underlying malignancy is often difficult to detect. Historically, the prognosis has been poor. Cure has occasionally been achieved with chemotherapy alone. LT in this setting is controversial, but has contributed to successful outcomes, as in the case of the five-yr-old girl reported here.     

Liver transplantation for acute liver failure due to toxic agent ingestion in children

Abstract: ALF is characterized by sudden onset, impaired liver function, jaundice and encephalopathy, without previous liver disease. We analyzed the patients who underwent LT due to toxic agent induced ALF to raise community awareness about preventing the toxic agent induced ALF. Five children (three boys, two girls) underwent LT due to toxic agent ingestion. Toxic agents were mushroom poisoning (n = 2), Datura stramonium (n = 1), yellow phosphorous (n = 1) and INH (n = 1). On admission, one patient had stage IV, two had stage III and two had stage II hepatic encephalopathy but worsened during the follow‐up. One patient had renal failure, and three patients required mechanical ventilation. Three patients underwent LRLT and others from a DD. Post‐operative complications were managed by supportive managements successfully, and overall all the patients are alive (100% survival) without any organ sequelae. Although outcome of these patients are excellent, ALF may be prevented in these cases by educating the public about consuming mushrooms and toxic effects of wild plants, prohibiting fireworks and serial liver enzyme measurements after initiating INH.     

Hypogammaglobulinemia in pediatric liver transplant recipients

Hypogammaglobulinemia has been reported after solid organ transplantation in adults, however immunoglobulin replacement [intravenous immunoglobulins (IVIG)] is only necessary in a minority of affected patients. We here present three pediatric patients with severe post-transplant hypogammaglobulinemia following liver transplantation (LTx) receiving a cyclosporine-based standard immunosuppression. Patient 1 was transplanted at the age of 10 months for biliary atresia. Eight weeks post-Ltx the serum IgG was 1.7 g/L. Patient 2 was transplanted at the age of 12 yr for acute liver failure. Four weeks post-Ltx the IgG dropped to 2.6 g/L. Patient 3 was transplanted at the age of 4 months for biliary atresia. Ten weeks post-Ltx severe hypogammaglobulinemia (IgG < 1.48 g/L) was diagnosed during a severe infectious complication. Patients 1 and 3 received a steroid bolus therapy for acute graft rejection. All patients had normal IgG concentrations prior to Ltx and lymphocyte subsets were post-operatively in the normal range. There was no extensive loss of protein by ascites. IGIV were replaced in the three patients monthly without further complications. In two of the patients (1 and 3) IVIG therapy was discontinued 8 and 10 months after Ltx when the immunosuppression has been reduced and serum IgG concentrations were found in the normal range without further immunoglobulin replacement. Severe hypogammaglobulinemia is a rare phenomenon following pediatric LTx and seems to be mainly caused by immunosuppressive drugs, however, the exact underlying mechanisms are unclear. A screening for hypogammaglobulinemia is useful after pediatric LTx, especially in patients with an intensified immunosuppression. Moreover, further immunologic research in affected patients is necessary.