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06 June 2017, Volume 32 Issue 6 Previous Issue    Next Issue

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Expert consensus on the diagnosis and treatment of atypical hemolytic uremic syndrome in Chinese children LIU Xiao-rong，SHEN Ying，FAN Jian-feng，et al 2017, 32(6): 401-404.  DOI: 10.19538/j.ek2017060601 Abstract ( )

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Interpretation of Expert Consensus on Nasal Intermittent Positive Pressure Ventilation for Premature Infants　 WANG Li，SHI Yuan 2017, 32(6): 405-407.  DOI: 10.19538/j.ek2017060602 Abstract ( )   Nasal intermittent positive pressure ventilation is a kind of respiratory support mode，which is based on the application of nasal continuous positive airway pressure. It can be used in premature infants with frequent apnea，as the initial model of respiratory support therapy and after extubation. It is of great clinical significance to reduce the incidence of apnea，tracheal intubation and mechanical ventilation. Analyzing “Expert consensus on nasal intermittent positive pressure ventilation in preterm infants” is helpful to the correct understanding and application of the consensus.

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Immunodynamics  and  immunosuppression  in  sepsis LI Ning，ZENG Qi-yi 2017, 32(6): 408-413.  DOI: 10.19538/j.ek2017060603 Abstract ( )   Sepsis is the leading cause of death among critically ill patients. The long-term immunosuppression after recovery of an acute sepsis event is a major reason for the high mortality rate in long term outcomes. Understanding immunodynamics of sepsis and its immunosuppression is very important to prevent and reduce the immunosuppression，decrease mortality. Hyperinflammation is in the early stage of the immunodynamics of sepsis. The exorbitant release of inflammatory mediator and cytokines impairs tissue cells in inflammatory reaction，and at the same time，also causes immune cells damage and immune suppression consequently，which is the basis of sepsis immunosuppression. Middle stage of sepsis is mixed immune status. During this period，inflammatory and anti-inflammatory mechanism compete with each other，while the inflammatory lesions continue and immune function is further restrained；in the later stage of sepsis，further deterioration and severer immunosuppression leads to immune dysfunction，and immunoparalysis in the end. There is no specific therapy for immunosuppression according to immunodynamics，and further researche is needed.

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Epidemiological trend and features of sepsis in children FANG Bo-liang，QIAN Su-yun 2017, 32(6): 414-416.  DOI: 10.19538/j.ek2017060604 Abstract ( )   In the third international consensus definitions for sepsis and septic shock，sepsis is defined as life-threatening organ dysfunction caused by a dysregulated host response to infection. This new definition is similar to severe sepsis in the past. This article describes the epidemiological  characteristics of this disease，including etiology，underlying diseases，organ dysfunctions，prevalence and prognosis.

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Early identification of septic shock REN Xiao-xu 2017, 32(6): 417-422.  DOI: 10.19538/j.ek2017060605 Abstract ( )   The pathogenesis of septic shock is complex. There is a lack of characteristic manifestations in early phase of septic shock . It can not be identified and clearly diagnosed by using a simple indicator. So its early identification has been a clinical challenge. Continuously train clinical staff and update relevant knowledge，let all relevant clinical staff be familiar with septic shock pathogenesis，diagnostic criteria and early clinical manifestations，let them get familiar with monitoring and assessment methods and maintain a high alert for septic shock. Any abnormal clues of septic shock should be monitored and cared. Hospital’ laboratories and information systems should have corresponding warning mechanism for septic shock. There is quality control system to improve the diagnostic and treatment deficiencies in septic shock. All the above-mentioned systematic approaches are the key to improving the early identification of patients with septic shock.

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Diagnosis and treatment of high-power septic shock ZHANG Chen-mei，YE Sheng 2017, 32(6): 422-425.  DOI: 10.19538/j.ek2017060606 Abstract ( )   Sepsis shock is also known as septic shock，which is common in children. Early standardized intervention can reduce mortality and improve the prognosis. High-power septic shock is also known as warm shock，which is often not found in time，so it can not actively intervened. The diagnosis and treatment level of high-power septic shock should be further improved.

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Talking about the fluid resuscitation in septic shock once again GAO Heng-miao 2017, 32(6): 425-430.  DOI: 10.19538/j.ek2017060607 Abstract ( )   Fluid resuscitation continues to be recommended as the first-line resuscitative therapy for patients with septic shock. The current acceptance of the therapy is based in part on long history and familiarity with its use in the resuscitation of other forms of shock，as well as on an incomplete and incorrect understanding of the pathophysiology of septic shock. Recently，the safety of intravenous fluids resuscitation in patients with septic shock has been called into question with both prospective and observational data suggesting improved outcomes with less fluid. The current evidence for the continued use of fluid resuscitation for septic shock remains contentious with no prospective evidence demonstrating benefit to fluid resuscitation as a therapy in isolation. The article reviews the pathophysiological rationale for the introduction of fluid resuscitation as treatment for septic shock and highlights a number of significant concerns based on current clinical evidence.

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Clinical application of vasoactive agents for septic shock HE Yan-xia，LI Tong-hui 2017, 32(6): 430-435.  DOI: 10.19538/j.ek2017060608 Abstract ( )   During resuscitation of septic shock in children, vasoactive agents are often needed because of failure of fluid resuscitation. Abnormal vasoconstriction and vasodilation and myocardial dysfunction are commonly seen in children with septic shock，which is dynamic. Rational use of vasoactive and inotropic agents is very important to improve survival and prognosis of patients with septic shock and it is also quite challenging to clinical doctors. In this article the characteristics and monitoring of hemodynamics and therapeutic use of vasoactive agents in children with septic shock are discussed.

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Hemofiltration and renal replacement therapy in pediatric sepsis MIAO Hui-jie，ZHANG Yu-cai 2017, 32(6): 436-439.  DOI: 10.19538/j.ek2017060609 Abstract ( )   Hemofiltration and renal replacement therapy are usually preferred as adjunct therapy for sepsis to improve hemodynamics and fluid balance，as well as to remove noxious molecules and inflammatory factors. It is well known that continuous renal replacement therapy（CRRT） is an important adjunct therapy for sepsis-associated kidney injury. Recently， blood purification is widely used to treat septic shock， acute respiratory distress syndrome（ARDS）， severe acute pancreatitis and electrolyte imbalance. Currently， there are four different patterns of blood purification used including continuous veno-venous hemofiltration（CVVH）， high volume hemofiltration（HVHF）， continuous veno-venous hemodiafiltration（CVVHDF） and plasma exchange（PE） in pediatric sepsis. However，there is no standard recommendation for blood purification model and the optimal timing in pediatric sepsis.

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Treatment strategy with antimicrobial agents for pediatric sepsis LIU Chun-feng 2017, 32(6): 439-443.  DOI: 10.19538/j.ek2017060610 Abstract ( )   Severe sepsis remains a major cause of death in children. Timely and appropriate use of antimicrobial agents is associated with lower mortality and continues to be a cornerstone of therapy for patients with sepsis. Initial empirical therapy is mainly relying on epidemiology，age，site of infection，and the place of infection. Once the pathogen is clear，we should treat the patient accordingly. In addition，we should consider the PK/PD and side effects of the agents，pay attention to other therapeutic measures，such as increasing immunity of host and eradication of infection focus. The diagnosis should be reassessed if the patient is not improved.

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Sepsis and anticoagulant therapy ZHU Yi-min，WU Qiong 2017, 32(6): 443-448.  DOI: 10.19538/j.ek2017060611 Abstract ( )   Sepsis is a major problem in the field of critical illness. One of the important reasons for the death of sepsis is the disorder of coagulation function，especially in the case of DIC. Coagulation dysfunction is always in the pathological process of sepsis，and its occurrence and prognosis is an important and independent prognostic factor. The activation of coagulation system and inflammatory response promote each other，and the inhibition of physiological anticoagulant system and fibrinolysis system are the pathophysiological basises of coagulation dysfunction in sepsis. Therefore，the anticoagulant therapy of sepsis has sufficient theoretical basis. However，there is no consistent conclusion about the treatment of sepsis at present. This article will discuss the mechanism of coagulation dysfunction and the research progress of anticoagulation therapy.

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Challenge and strategy of nutrition support in sepsis children TANG Qing-ya，WU Qing-qing 2017, 32(6): 448-451.  DOI: 10.19538/j.ek2017060612 Abstract ( )   Sepsis is one of important reasons for the death of children in intensive care unit. Although anti-infection and active symptomatic treatment are the key to treatment，whether nutrition support is rational and effective or not can also affect  clinical prognosis. Sepsis children are in stress status，so nutrition support needs to meet the demand for energy，protein and other nutrients without increasing the burden of organs. Although intestinal function is damaged because of systemic infection，enteral nutrition（EN） is still the first choice by actively creating conditions. If the EN is not enough，supplemental parenteral nutrition（PN） should be given. In the process of nutrition support，immune nutrients can be added to regulate immune function and attenuate inflammation.

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Testicular adrenal rest tumors（TARTs） in two brothers with nonclassic congenital adrenal hyperplasia due to HSD3B2 gene mutation LIANG Yan，YU Xiao，WEI Hong，et al 2017, 32(6): 452-457.  DOI: 10.19538/j.ek2017060613 Abstract ( )   Objective　Testicular adrenal rest tumors（TARTs） are common in males with congenital adrenal hyperplasia（CAH） due to 21-hydroxylase deficiency and 11-β hydroxylase deficiency. It’s rare to find TARTs in CAH due to 3β-hydroxysteroid dehydrogenase deficiency. We present rare cases of two brothers with nonclassic CAH due to 3β-hydroxysteroid dehydrogenase deficiency，who presented with TARTs. The clinical characteristics and mutation of HSD3B2 gene of the two patients were analyzed. Methods　The clinical data of the two brothers with TARTs in nonclassic CAH were collected. HSD3B2 genes were amplified with polymerase chain reaction and screened for mutations by sequencing. Results　Both of the two brothers were admitted into our hospital presenting bilateral testicular irregular lump with significantly high ACTH，DHEAS level，and increased An，T and 17-OHP level. The HSD3B2 gene analysis revealed that two patients carried C.776 C＞T（p. Thr259Met） and C.674 T＞A（p.Val225Asp） heterozygous missense mutation，which were inherited from parents respectively. The C.674 T＞A（p.Val225Asp） mutation, inherited from mother，was not reported untill now. After treatment with hydrocortisone, TARTs decreased and the markers of adrenal function were improved. Conclusion　The study shows，for the first time，that TARTs in nonclassic CAH in these two brothers is due to 3β-hydroxysteroid dehydrogenase deficiency. A new HSD3B2 gene mutation，C.674 T＞A（p.Val225Asp），is reported here. The presence of TARTs is a known factor for infertility，however，if given early diagnosis and appropriate management，TARTs could be reduced and the fertility will be improved. It is important for pediatricians to screen for TARTs in CAH males.

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Expression profile of long non-coding RNA in Hirschsprung’s disease TIAN Jiao， HAO Li-jun， WANG Ya，et al 2017, 32(6): 458-462.  DOI: 10.19538/j.ek2017060614 Abstract ( )   Objective　To investigate the long non-coding RNAs（lncRNAs） expression profiles in the bowels of patients with Hirschsprung disease（HSCR），and to explore the role of dysregulated  lncRNAs in the pathogenesis of HSCR. Methods　Thirty pairs of tissue samples，including aganglionic segments and ganglionic segments，were obtained from patients with HSCR during operation. Then lncRNA microarrays were used to investigate the expression profiles in 3 pairs of specimens coupled as HSCR tissues compared with matched normal colon tissues. Candidate  genes were selected from these differentially expressed lncRNAs based on arti?cial criterion（raw signal intensity≥100；fold change≥3） and then validated by quantitative real-time polymerase chain reaction（qRT-PCR） in 27 pairs of tissues. Results　A total of 217 lncRNAs in aganglionic colon segment were found to be more than two fold greater than that in ganglionic segment tissues，including 13 up-regulated and 204 down-regulated（P＜0.05）. The expression of two lncRNAs were significantly changed in aganglionic tissues compared with the ganglionic tissues after qRT-PCR validation（P＜0.05），whereas the change of other lncRNAs selected from artificial criterion were without statistical difference（P＞0.05）. Conclusion　Abnormal expression of lncRNAs is found in HSCR aganglionic segments，suggesting that lncRNAs may be involved in the pathogenesis of HSCR.

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Change of FeNO and serum SIgG4，IL-4 and INF-γ levels using SILT in children with mites allergic asthma JI Yan-ni，LI Meng-rong，WEI Hong-ping，et al 2017, 32(6): 463-466.  DOI: 10.19538/j.ek2017060615 Abstract ( )   Objective　To investigate the change of FeNO and serum SIgG4，IL-4 and INF-γ levels before and after SLIT in children with mites allergic asthma. Methods　All 118 cases with mites allergic asthma were randomly divided into two groups：the control group（59 cases） was treated with ladder type inhalation，and the treatment group（59 cases） was administered dust mite drops on the basis of conventional treatment. FeNO and serum SIgG4，IL-4 and INF-γ levels were measured before and after treatment. Results　After treatment,the levels of FeNO and serum IL-4 were decreased in treatment group  than before treatment（P＜0.05），the levels of serum SIgG4、INF-γand the  ratio of INF-γ/IL-4 were increased（P＜0.05）. Compared with the control group，the levels of FeNO and serum IL-4 were decreased in treatment group after treatment（P＜0.05），the levels of serum SIgG4 and INF-γ and the ratio of INF-γ/IL-4 were increased（P＜0.05）. After treatment，the scores of allergic asthma symptom were decreased significantly in treatment group than before treatment（P＜0.05）. Conclusion　SLIT can decrease airway inflamation and correct immune imbalance in children with mites allergic asthma.

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Clinical analysis of 84 children with trachea or bronchus foreign body ZHAI Jia，ZOU Ying-xue，GUO Yong-sheng，et al 2017, 32(6): 467-470.  DOI: 10.19538/j.ek2017060616 Abstract ( )   Objective    To evaluate the clinical characteristics of children with trachea or bronchus foreign body. Methods    The data of 84 children with confirmed diagnosis of trachea or bronchus foreign body was retrospectively analyzed in this study form Jun 2011 to Jun 2016 in Tianjin Children Hospital，including general situation，history of foreign body aspiration，medical history，course of disease，foreign body property，clinical and imaging characteristics. Results    The rate for tracheobronchial foreign body occurring in boys and girls were 2.23∶1 with the main incidence age of 6 months to 3 years old. The incidence rate in countryside was higher than that in city（69.05% vs. 30.95%），especially boys in countryside. Children who are able to provide an accurate history of foreign body aspiration may be helpful in early diagnosis and treatment of trachea or bronchus foreign body（P＜0.05）. The main type of foreign body was food，especially nuts. The proportion of bronchial foreign body which remained in the left or right main bronchial tubes was not different，but the number of cases in right lobe was higher than that in the left. The clinical symptom was different according to the different lesions with block of foreign bodies. The main symptom was cough（98.81%） and breezing（58.33%），with the imaging characteristics of emphysema （55.95%）. Conclusion    In prevention and control of tracheobronchial foreign bodies，boys under the age of 3 should be paid most attention to in rural areas. The children should be reduced contact with nuts food. The guardian must attach more importance to tracheobronchial foreign body. For children with symptom of cough and wheezing weakening breathing sounds on single side by physical examination，emphysema and pulmonary atelectasis on imaging，health providers should pay attention to the history of foreign body aspiration or cough history，and should actively perform bronchoscopy for early diagnosis and treatment.

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Recurrent fever，abnormal liver function and coronary dilation JIN Feng-hua，HU Hui-li，LIU Gang 2017, 32(6): 471-477.  DOI: 10.19538/j.ek2017060617 Abstract ( )

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Electronic bronchoscopy intervention in the auxillary treatment of children with bronchial mucoepidermoid carcinoma：one case report CHEN Meng，TANG Heng，MEI Juan-juan，et al 2017, 32(6): 478-480.  DOI: 10.19538/j.ek2017060618 Abstract ( )