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06 May 2017, Volume 32 Issue 5 Previous Issue    Next Issue

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Meeting summary of  the Founding Congress of Chinese National Pediatric Allergy，Immunology and Rheumatology Commitee of Beijing Medical velopment 　Foundation RONG Zan-hua，XIAO Ji-hong，ZENG Hua-song，et al 2017, 32(5): 321-322.  DOI: 10.19538/j.ek2017050601 Abstract ( )

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Resent progress in the clinical application of transcutaneous oxygen and carbon dioxide partial pressure monitoring in NICU WANG Ting-ting，FU Jian-hua 2017, 32(5): 323-327.  DOI: 10.19538/j.ek2017050602 Abstract ( )   PaO2 and PaCO2 monitoring is the most important indicators of ventilation and oxygenation in the NICU. The results of PaO2 and PaCO2 are from blood gas obtained by collecting arterial or peripheral blood samples. Repeated blood sampling is frequently needed in critically ill neonates，which may increase the risk of neonatal infection, iatrogenic anemia and pain stress. TcPCO2 and TcPO2，as the noninvasive monitors，have been emerging as non-invasive blood gas monitoring in the clinical care of critically ill neonates，especially the VLBW and ELBW infants，during neonatal oxygen therapy，mechanical ventilation and shock requiring perfusion monitoring.

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Application of amplitude-integrated EEG in neonatal ICU LUO Fang，DU Li-zhong 2017, 32(5): 328-332.  DOI: 10.19538/j.ek2017050603 Abstract ( )   Amplitude integrated EEG（aEEG）is derived from limited channels （usually P3-P4 or C3-P3，C4-P4） and is filtered，rectified and time-compressed to serve as a real-time，noninvasive，bedside cerebral function monitoring. It’s simple application and interpretation has resulted in increasing use in neonatal intensive care units across the world. Current evidence demonstrates that aEEG is useful to monitor cerebral background activity，diagnose and treat seizures and predict neurodevelopmental outcomes for preterm and term infants.  Validation investigation with full array EEG have shown reliable results in interpretation of cerebral electric background and electrographic seizures，especially when used with the simultaneously displayed raw EEG trace.

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Clinical application of therapeutic hypothermia in neonatal hypoxic-ischemic encephalopathy ZHANG Peng，ZHOU Wen-hao 2017, 32(5): 332-336.  DOI: 10.19538/j.ek2017050604 Abstract ( )   Hypoxic ischemic encephalopathy（HIE） is the most important reason for morbidity and mortality in term-born infants. Neonatal HIE is seen approximately in 2-3/1000 live births. Moreover， in the developing countries， its frequency has risen up to 26/1000 live births. Among the remaining living newborns， cerebral palsy， epilepsy， autism， visual and auditory problems are diagnosed. Therapeutic hypothermia is now well-established as standard treatment for infants with moderate to severe hypoxic-ischemic encephalopathy. In this review， the clinical appilication of therapeutic hypothermia in neonatal hypoxic-ischemic encephalopathy will be discussed.

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Application of 24-hour gastric and esophageal double pH monitoring in neonatal gastroesophageal reflux diseases ZHANG Shu-cheng 2017, 32(5): 337-341.  DOI: 10.19538/j.ek2017050605 Abstract ( )   The 24-hour intraesophageal pH monitoring is a widely used method in diagnosis of gastroesophageal reflux diseases. It has been regarded as the diagnositic standard of these diseases. Due to the technical limitation， the traditional pH monitoring can only show the acid refluxes in the lower esophagus instead of the mixed and alkaline ones. As a result， there is inevitably a proportional rate of misdiagnosis in the traditional pH monitoring. The 24-hour gastric and esophageal double pH monitoring can perfectly solve this problem. In this method， both the gastric and esophageal pH is recorded for 24 hours. Both the acid refluxes in the lower esophagus and the duodenogastric reflux and the mixed and alkaline refluxes can be clearly demonstrated. With improvement of accuracy， the 24-hour gastric and esophageal double pH monitoring has been used in adults and adolescent， but rarely in the neonates. In this paper，the key point of this method and its application in neonates and prematures was reviewed and introduced.

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Clinical application of continuous renal replacement therapy in the newborn ZHANG Jin-jing*，SHEN Ying，WANG Ya-juan 2017, 32(5): 341-344.  DOI: 10.19538/j.ek2017050606 Abstract ( )   Acute kidney injury（AKI） is common in newborns，especially in preterm and low birth weight infants，with high mortality rate and poor prognosis. Renal replacement therapy is the effective treatment for AKI，including peritoneal dedialysis（PD），continuous renal replacement therapy（CRRT） and intermittent hemodialysis（HD）. CRRT was widely used in clinical practice in recent decades，but there was less experience in newborn. Indication，monitoring and care of CRRT in newborn were introduced in this paper.

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Application of noninvasive cardiac output measurement in neonates ZHOU Wei 2017, 32(5): 345-348.  DOI: 10.19538/j.ek2017050607 Abstract ( )   Cardiac output measurement is a very important content in the rescue management of  critically ill neonates，especially in patients with hemodynamic instability，such as neonatal severe infection，septic shock，multiple organ failure，congenital heart disease，etc. The monitoring methods include invasive measurement，noninvasive measurement and wearable or portable dynamic measurement，etc. The current clinical noninvasive continuous measurement of neonatal cardiac output mainly adopts ultrasonic cardiac output monitor（USCOM） technique. By monitoring the cardiac output，we can accurately estimate the patient’s cardiac function and systemic perfusion，which has important significance in patient’s condition assessment，clinical medicine guidance and prognosis judgement.

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Analysis of the clinical features，treatment options and clinical outcomes of Fanconi anemia patients CHANG Li-xian，SUN Cong-cong，ZHANG Jing-liao，et al 2017, 32(5): 349-352.  DOI: 10.19538/j.ek2017050608 Abstract ( )   Objective　To analyze the clinical characteristics of children with Fanconi anemia（FA） and their responses to different drug treatments，and to study the clinical outcomes of different drug treatments. Methods　A total of 43 children with definite diagnosis of FA in Institute of Hematology and Blood Diseases Hospital，Chinese Academy of Medical Sciences & Peking Union Medical College（From October 2003 to December 2014） were included. Fisher’s exact test was used to analyze the potential difference of different treatments. Results　Of 43 cases，the median age of onset was 5 years（50 days to 14 years），and there were 23 male cases（53.5%） and 20 female cases（46.5%）. The male to female ratio was 1.15∶1. There were 30 cases（69.8%） of malformation，and the incidence of hand deformity was the highest（34.3%）. No family history was observed in 27 cases（62.8%）. The most common first symptoms were anemia and thrombocytopenia. The authors further analyzed the efficacy of cases with the follow-up time≥6 months，and found that there was significant efficacy difference between the androgen +/-glucocorticoid/rabbit anti-human thymocyte immunoglobulin（ATG）+cyclosporin A group and androgen group as well as androgen+glucocorticoid groups（P＜0.05）. Conclusion　The incidence of malformations in children with FA is inconsistent with some other international reports. The deformity or family history does not exist in all children with FA. The poor clinical prognosis and progression of FA are associated with the treatment of cyclosporin A.

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Risk factors for mortality of septic shock in pediatric patients with hematological malignancies LIU Ting-hua，TANG Xi-kang，CHEN Ze-kai，et al 2017, 32(5): 353-356.  DOI: 10.19538/j.ek2017050609 Abstract ( )   Objective　To study clinical characteristics and risk factors for mortality of septic shock patients with hematological malignancies in pediatric intensive care unit（PICU）. Methods　A retrospective analysis of records of pediatric patients with hematological malignancy and septic shock was performed. In our study，43 cases admitted to PICU in Sun Yat-sen Memorial Hospital between March 2012 and September 2015 were included. Results　Among the 43 cases，16 died，while the other 27 survived. The percentage of mortality was 37.2%. Pulmonary infection （74.4%） and gastrointestinal tract infection（39.5%） were the common causes of septic shock in these patients. Etiological examination revealed 43 kinds of pathogenic bacteria in 26 cases. The percentage of gram-positive bacteria，gram-negative bacteria and fungus was 11.6%，72.1% and 16.3%，respectively. Escherichia coli was found to be the main pathogen（6/43，14.0%）. Between the survival group and the mortality group，there were significant differences in the parameters of age，chemotherapy courses，time from infection to shock，time from neutropenia to shock，multiple organs dysfunction，pulmonary infection，hypocalcemia，oxygenation index and serum lactic acid concentration after 6 hours of fluid resuscitation，requirement of dopamine and mechanical ventilation （P＜0.05）. By logistic regression analysis，mortality was associated with the variables of serum lactic acid concentration after 6 hours of fluid resuscitation and requirement of mechanical ventilation. Conclusion　The risk factors for mortality of septic shock in pediatric patients with hematological malignancies are hyperlactacidemia after 6 hours of fluid resuscitation，hypocalcemia and requirement of mechanical ventilation.

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Analysis of 9 cases of complete left bundle branch block after interventional treatment for ventricular septal defect CAO Li-ming*，WANG Feng-ming，QIN Yu-ming，et al 2017, 32(5): 357-360.  DOI: 10.19538/j.ek2017050610 Abstract ( )   Objective　To investigate the risk factors and prognosis of CLBBB complicated with VSD after interventional therapy. Methods　The general data and follow-up results of 9 cases of complete left bundle branch block（CLBBB） after interventional treatment for ventricular septal defect（VSD） were reviewed from April 2010 to December 2015 in our hospital heart center. The age of children，type of VSD and occluder，the occurrence time and prognosis of postoperative CLBBB and the change of LVEDD and LVEF were analyzed. Results　Age：1 case＞5 years and 8 cases＜5 years；VSD and occluder type：perimembranous VSD with pseudo aneurysm in 3 cases and intracristal VSD in 6 cases；there was 1 case of symmetry occluder，2 cases of small waist big edge type occluder，and 6 cases of eccentric type occluder. Postoperative CLBBB occurrence time and prognosis：CLBBB occurred in postoperative 3 days in 8 cases，including 4 cases recovered to normal after drug treatment and 2 cases again had CLBBB in postoperative 1 month；1 case began to appear CLBBB in postoperative 1 month and returned to normal after receiving surgery the next day. Left ventricle end-diastolic dimension（LVEDD） and ejection fraction of LV（LVEF） changes：2 cases had increased LVEDD in 6 and 12 months after operation，respectively；the other 7 cases did not have obvious LVEDD increase；all the 9 cases did not have decreased LVEF. Conclusion　VSD interventional therapy should strictly follow the expert consensus and standardized operation；VSD interventional occlusion therapy for young children（＜5 yrs） should be cautious；in the premise of ensuring the occlusion effect of interventional therapy，the symmetric type occluder should be selected first. CLBBB complicated with VSD after interventional therapy has a good recent prognosis，but in some cases the long-term LVEDD increases；CLBBB complicated with VSD after interventional therapy cases should be strictly followed up.

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A prospective study of multi-target immunosuppressive therapy for children with steroid-resistant nephritic syndrome XU Da-liang*，FU Ming-li，DONG Yang，et al 2017, 32(5): 361-365.  DOI: 10.19538/j.ek2017050611 Abstract ( )   Objective　To observe the efficacy and safety of multi-target immunosuppressive therapy in treatment of children with steroid-resistant nephritic syndrome（SRNS）. Methods　A total of 48 children with SRNS were enrolled in this multicenter prospective study. Based on the same comprehensive treatment，the children were randomly divided into two groups： （1）observation group： they were orally given CSA［3～4 mg/（kg·d）］ and MMF［20 mg/（kg·d）］ multi-target immunosuppressive therapy；（2）control group：they were orally given CSA［4～6 mg/（kg·d）］. The side effects were closely observed. The plasma concentrations of CSA，urine protein to creatinine ratios，liver and kidney function，blood routine and urine β2-microglobulin were respectively compared between the two groups after 2 weeks，1 month，3 months and 6 months of treatment. Results　The average plasma concentrations of CSA in the observation group was （88.86±16.94） μg/L，and in the control group it was （152.96±19.20） μg/L（P＜0.001）. The urine protein to creatinine ratios in the observation group after 1 month and 3 months of treatment were lower than the control group in the same time period（P＜0.05）. The serum albumin in the observation group after 1 month and 3 months of treatment was higher than the control group in the same time period（P＜0.05）. The urine β2-microglobulin in these two groups had no differences during the treatment（P＞0.05）. The overall remission rate of the observation group was 88%，and in the control group it was 87%. The remission rate of the observation group after 2 weeks and 1 month of treatment was better than the control group（P＜0.05）. The main side effect during therapy was infection，gastrointestinal reaction，crinosity，hypertension and leukocyte decrease. The side effect of the observation group was less than the control group（P＜0.05）. Conclusion　The multi-target therapy in children with SRNS by CSA and MMF results in early remission and can keep long-term remission with mild side effect.

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A study on platelet indices and platelet membrane glycoprotein in children with acute lymphocytic leukemia　　 HUANG Zhe*，LIU Wen-jun，CAO Bian-chuan，et al 2017, 32(5): 366-370.  DOI: 10.19538/j.ek2017050612 Abstract ( )   Objective　To investigate the change of the platelet state and function in acute lymphocytic leukemia. Methods　The changes of platelet indices（PLT，PCT，MPV，PDW）、immature platelet fraction（IPF%），immature platelet counts（IPC），granule membrane glycoprotein of platelet （CD62p） and PAC-1 were obtained by using automatic blood cell analyzer and whole blood flow cytometry（FCM） respectively，in children with acute lymphocytic leukemia（ALL），ALL in first complete remission （ALL-CR1） and children undergoing elective surgical procedure（control group）. Results　（1）Without addition of platelet agonists ADP，expression of platelet surface activated CD62p and PAC-1 in ALL was higher than that in control group（P＜0.05），while expression of platelet surface activated PAC-1 in ALL-CR1 was higher than that in control group（P＜0.05），and lower than that in ALL（P＜0.05）；（2）With addition of platelet agonists ADP，expression of platelet surface activated CD62p and PAC-1 in ALL was lower than that in control group（P＜0.05），while expression of platelet surface activated PAC-1 in ALL-CR1 was lower than that in control group（P＜0.05），and higher than that in ALL（P＜0.05）；（3） PLT，PCT and MPV in ALL was lower than that in control group and ALL-CR1 （P＜0.05）；（4） IPF% in ALL was higher than that in control group and ALL-CR1（P＜0.05），and IPC was lower than that in control group and ALL-CR1（P＜0.05）. Conclusion　The children who are newly diagnosed with acute lymphocytic leukemia have peripheral platelet reactivity and platelet function defects. Platelet indices and platelet membrane glycoprotein can be used as effective indicators to judge the effect on ALL.

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Diagnostic value of the ratio of α1-microglobulinuria to microalbuminuria in Dent disease HE Guo-hua&，ZHANG Hong-wen，WANG Fang，et al 2017, 32(5): 371-373.  DOI: 10.19538/j.ek2017050613 Abstract ( )   Objective　To explore the diagnostic value of the ratio of α1-microglobulinuria to microalbuminuria in Dent disease. Methods　Data were collected from 8 patients with childhood Dent disease from Jan.1，2014 to Dec.31，2015. The ratio of α1-microglobulinuria to microalbuminuria，and urine protein electrophoresis were monitored and analyzed. Results　In all the 8 cases，α1-microglobulinuria was increased significantly，the ratio of α1-microglobulinuria to microalbuminuria was much higher than 1，and was closely related to the percentage of low molecular weight proteinuria in urine protein electrophoresis （P＜0.05）. Conclusion　The ratio of α1-microglobulinuria to microalbuminuria over 1 can be used as a diagnostic criteria for tubuloproteinuria.

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Clinical and pathological analysis of C3 glomerulonephritis in 6 children WANG Li-li，ZHAO Kai-shu，LI Qing-mei，et al 2017, 32(5): 374-378.  DOI: 10.19538/j.ek2017050614 Abstract ( )   Objective　To analyze the clinical characteristics，pathological features and treatment responses of 6 pediatric patients with C3 glomerulonephritis（C3GN），in order to improve the understanding and treatment of this disease for pediatricians. Methods　Analyze the clinical manifestations，pathological features，therapies，prognosis of patients who were diagnosed with C3 glomerulonephritis from September，2010 to June，2016 retrospectively. Results　Clinical characteristics and laboratory examination：2 patients’ first symptom was hematuria；4 patients’ first symptom was hematuria and proteinuria；3 patients presented as acute nephritic syndrome，one presented as nephrotic syndrome. All the patients showed that the level of serum complement C3 was reduced，while sernm complement C4 was normal. Pathological character：6 patients showed strong positive complement C3 deposition under immunofluorescence. Lightmicroscopy showed mesangial proliferative glomerulonephritis in 5 cases，1 case was diagnosed as endocapillary proliferative glomerulonephritisin，and 3 patients presented electrondense depositionin under electron microscope. Treatment and prognosis：after conventional treatment，2 patients who were with crescent were treated with glucocorticoid .After a follow-up from 6 months to 42 months ，the prognosis was pretty good. Conclusion　Children with C3GN are usually presented with hematuria and （or）proteinuria，characterized by strong positive C3 deposition. Lightmicroscopy always shows mesangial proliferative glomerulonephritis. Electron microscope show electron dense deposition，and short-term prognosis is pretty good.

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Two cases of microscopic polyangiitis in children and the literature review ZHAO Xing，MA Xiao-xue，JIANG Hong 2017, 32(5): 379-381.  DOI: 10.19538/j.ek2017050615 Abstract ( )

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Clinical analysis of 298 cases of right bundle branch block in children LIU Tong*，QIN Fang，ZHENG Zhi-jie，et al 2017, 32(5): 382-383.  DOI: 10.19538/j.ek2017050616 Abstract ( )

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Diagnosis and treatment progress in vasovagal syncope in children SONG Jing-yuan，JIN Hong-fang，DU Jun-bao 2017, 32(5): 384-388.  DOI: 10.19538/j.ek2017050617 Abstract ( )

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Pathogenesis of chronic refractory thrombocytopenia in children FU Pan，YE Qi-dong 2017, 32(5): 389-392.  DOI: 10.19538/j.ek2017050618 Abstract ( )

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Application of interferon in myeloid leukemia XU Wei，LIU Wen-jun 2017, 32(5): 393-397.  DOI: 10.19538/j.ek2017050619 Abstract ( )

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Next generation sequencing in the diagnosis of dystrophic epidermolysis bullosa in the newborn：A report of one case JI Ai-hua，GAO Min，ZHANG Kai-hui，et al 2017, 32(5): 398-400.  DOI: 10.19538/j.ek2017050620 Abstract ( )