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05 May 2018, Volume 33 Issue 5 Previous Issue    Next Issue

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Milestone of drug therapy in preterm infants as means of respiratory support DU Li-zhong 2018, 33(5): 321-323.  DOI: 10.19538/j.ek2018050601 Abstract ( )   With the increasing number of survival of very low or extremely low birth weight infants, respiratory insufficiency has been a significant problem in Neonatal Intensive Care Unit. There have been greatly improved in the means of respiratory support for those infants. Among the means of respiratory support, drug therapy has been playing an important role. The paper introduces brief history and major contributions of drug therapy in facilitating modern respiratory support for preterm infants.

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Application of neurally adjusted ventilatory assist technology in ventilatory support for premature infants CHEN Zheng，DU Li-zhong 2018, 33(5): 324-327.  DOI: 10.19538/j.ek2018050602 Abstract ( )   With the development of NICU treatment strategies，the survival rate of premature infants has been significantly improved. The treatment concept in ventilatory support is progressing，which is the most essential rescue therapy for preterm. Neurally adjusted ventilatory assist（NAVA） is a novel approach of mechanical ventilation，which is based on the electrical activity of the diaphragm（EAdi） to control ventilation，synchronizing the respiratory trigger and assisted ventilation as much as possible. At present，the NAVA technology has been gradually carried out both at home and abroad. The paper reviews principle and method of NAVA，analyzes clinical applications and the research status，and provides guidance for clinical practice.

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Evaluation on postnatal glucocorticoids in preventing or treating bronchopulmonary dysplasia of preterm infants YANG Chuan-zhong 2018, 33(5): 327-333.  DOI: 10.19538/j.ek2018050603 Abstract ( )   Postnatal glucocorticoid is an effective therapy for preventing or treating bronchopulmonary dysplasia（BPD） among extremely preterm infants，but the side-effect profile limits its use and makes it an unacceptable option for many patients. Early low-dose hydrocortisone therapy may be beneficial to premature infants with intrauterine chorioamnionitis，but concerns remain about possible adverse effects such as gastrointestinal perforation. Late（after the first week of life） postnatal steroids may have a better benefit-to-harm ratio than early steroids. Postnatal dexamethasone（DEX） in 2-3 weeks after birth should mainly be reserved for infants who cannot be weaned from mechanical ventilation and the dose and duration should be kept to a minimum. However，early inhalation of budesonide or intratracheal instillation of budesonide using surfactant as a vehicle can reduce the incidence of BPD，but its safety needs further evaluation. Future studies are required to identify the preferred type，dose and timing of therapy that will provide maximal benefit with minimal side effects.

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Clinical application of noninvasive high-frequency ventilation in preterm neonates SHI Yuan*，ZHU Xing-wang 2018, 33(5): 333-337.  DOI: 10.19538/j.ek2018050604 Abstract ( )   Non-invasive high-frequency ventilation（NIHFV） is a newer form of non-invasive ventilation（NIV） which uses nasopharyngeal tubes，short nasal prongs or masks instead of endotracheal tube to deliver high frequency ventilation. NIHFV offers the advantages of invasive high frequency ventilation（no need for synchronisation，high CO2 removal，less volume/barotrauma） and nasal CPAP（non-invasive interface，increase in functional residual capacity allowing oxygenation to improve）. Clinical pilot studies show NIHFV can be used as a rescue therapy after failure of other NIV modes to help prevent or delay intubation. However，the data is limited. Further multi-center，prospective，randomized，controlled trials are needed.

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Less invasive surfactant administration（LISA）/minimally invasive surfactant treatment（MIST） and prognosis of respiratory diseases in premature ZHU Jia-jun 2018, 33(5): 338-341.  DOI: 10.19538/j.ek2018050605 Abstract ( )   Less invasive surfactant administration（LISA）/ minimally invasive surfactant treatment（MIST） is a novel technique of surfactant replacement for neonatal respiratory distress syndrome. With LISA/MIST technique，surfactant is injected into the trachea through a thin tube and the distribution of surfactant mainly depends on infant’s spontaneous breath. LISA/MIST technique causes less injury to tracheal mucosa and less exposure to positive ventilation in the infants. Therefore，LISA/MIST technique is considered one of the strategies of lung protection in premature infants. Recently meta-analysis has showed LISA/MIST results in less mechanical ventilation time in premature infants with RDS and reduces the composite incidence of death or BPD at corrected gestational age of 36 weeks.  However，the procedure of LISA/MIST varies from site to site.  Well-designed multicenter clinical researches of LISA/MIST by experienced professionals with standardized procedure are highly expected before a widespread implementation of the technique.

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Controversial on target range of oxygen saturation in preterm infants CHEN Chao 2018, 33(5): 341-345.  DOI: 10.19538/j.ek2018050606 Abstract ( )   Oxygen therapy plays an important role in the management of preterm infants，but inappropriate oxygen therapy will cause adverse neurodevelopmental outcome in preterm infants. Monitoring of oxygen saturation is essential in oxygen therapy. However， optimal range of oxygen saturation for preterm infants still remain controversial. In order to solve the clinical dilemma，many prospective multi-center randomized trials about optimal range of oxygen saturation for preterm infants has been completed. Lower target of oxygen saturation（85-89%） may result in a lower incidence of retinopathy of prematurity but higher rate of death than higher oxygen saturation target in preterm infants.

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Caffeine therapy in preterm infants and its effect on their outcomes YANG Xiao-yan，MU De-zhi 2018, 33(5): 346-348.  DOI: 10.19538/j.ek2018050607 Abstract ( )   As one of the xanthine derivates，caffeine could inhibit adenosine receptors in vivo non-specifically，which would lead to a series of pharmacological effects. Caffeine is the choice of treatment for apnea of prematurity（AOP）. Besides，various studies show its protection for both nervous system and respiratory system. Early caffeine administration could bring significant and long-lasting benefits for preterm infants. However，further researches are still needed to discuss the probable mechanisms.

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Analysis of UGT1A1 gene mutation in Uygur neonates with unconjugated hyperbilirubinemia Nuerya Rejiafu*，ZHANG Hui，SU Ya-jie，et al 2018, 33(5): 349-352.  DOI: 10.19538/j.ek2018050608 Abstract ( )   Objective　To analyze the mutation of uridine diphosphate glucuronosyltransterase 1A1，UGT1A1 gene in Uygur neonates with unconjugated hyperbilirubinemia. Methods　Select the neonates with unconjugated hyperbilirubinemia in People’s Hospital of Xinjiang Uygur Autonomous Region during 2013-2016. The genomic DNA of peripheral blood was extracted. Amplify the coding sequence and promoter region of UGT1A1 gene，which were sequenced to identify gene mutations. A total 34 cases were enrolled as hyperbilirubinemia group and 11 cases of physiological jaundice were selected as control group. Results　Seven mutations were detected，and they were 71Arg（G71R），Val674Gly（V225G） at exon 1，Pro1091Leu（P364L），Asp1195Asn（D399N）at exon 3，Pro1352Leu（P451L），Tyr486Asp（Y486D）at exon 5，respectively. TATAA cassette had transcription mutation of insertion TA. The highest mutation frequency occurred in G71R and insertion TA into TATAA cassette. However，there was no statistical difference in frequency of mutation between the hyperbilirubinemia group and the control group（χ2＝1.681，P＝0.195；χ2＝0.214，P＝0.643）；there was no statistical difference in G71R mutation frequency between Han and Uygur control group（χ2＝0.253，P＝0.615），but there was statistically significant difference in insertion TA into TATAA cassette between Han and Uygur group（χ2＝4.675，P＝0.031）. Conclusion　There are several types of mutations detected in Xinjiang Uygur newborns，and Uygur neonates have a significantly higher mutation frequency of TA into TATAA cassette than the local Han Chinese newborns.

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Metoprolol and midodrine for treatment of vasovagal syncope：A meta-analysis DONG Tong，NIU Shao-min，LIU Ya-hong，et al 2018, 33(5): 353-359.  DOI: 10.19538/j.ek2018050609 Abstract ( )   Objective　To compare the effect of metoprolol with that of midodrine in the treatment of vasovagal syncope（VVS） using a systematic review. Methods　The databases of PubMed，Cochrane Library，ScienceDirect，CBM，CNKI and Wanfang were searched by computer. The search time was from the construction of databases to Nov. 2017. According to the Cochrane systematic review，we screened the data on the effects of metoprolol and/or midodrine on VVS；both English and Chinese randomized controlled trials（RCTs） were screened. Meta-analysis was performed using RevMan 5.3 software. Efficacy of metoprolol group and/or midodrine group was compared. Results　The symptom improvement rate：4 RCTs showed that metoprolol group was superior to the basic treatment group（P＜0.01），4 RCTs suggested that the midodrine group was better than the basic treatment group（P＜0.01）， and 2 RCTs showed there was no significant difference between metoprolol group and midodrine group（P＝0.63）；HUTT negative rate：6 RCTs showed metoprolol group was superior to the basic treatment group（P＜0.01），3 RCTs indicated that the efficacy of midodrine group was better than the basic treatment group（P＜0.01），and 3 RCTs indicated there was no significant difference between metoprolol group and midodrine group（P＝0.05），the recurrence syncope rate：3 RCTs showed that metoprolol group was better than the basic treatment group（P＜0.01），4 RCTs indicated that the effect of midodrine group was better than that of the basic treatment group（P＜0.01），and 2 RCTs showed that there was no significant difference between the metoprolol group and midodrine group（P＝0.51）；adverse reaction rate：3 RCTs showed there was no significant difference between the metoprolol group and midodrine groups（P＝0.14）. Conclusion　Both metoprolol and midodrine can improve the clinical symptoms of patients with VVS，increase the number of negative HUTT test after treatment，and reduce the number of recurrence of syncope. However，there is no significant difference in efficacy indicators.Therefore，large-scale，multicenter RCTs are still needed to investigate the efficacy of drug treatment for VVS.

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Clinical features of late-onset ornithine transcarbamylase deficiency and their OTC gene mutations：An analysis of 8 cases WANG Hai-jun，WANG Qi，LI Dong-xiao，et al 2018, 33(5): 360-364.  DOI: 10.19538/j.ek2018050610 Abstract ( )   Objective　To improve the ability of rapid identification and diagnosis of late-onset ornithine transcarbamylase（OTC） deficiency by summarizing the clinical and mutations features of eight cases. Methods　The clinical manifestation，laboratory examination，blood amino acids，urinary organic acids and gene analysis of 8 patients with OTC deficiency treated in Children’s Hospital Affiliated to Zhengzhou University from Jan.2014 to Dec.2017 were studied. The points of diagnosis and treatment for OTC deficiency were summarized. Results　Eight children（3 boys and 5 girls） first visited the emergency department of our hospital at the age from 10 months to 6 years and 10 months due to vomiting，loss of appetite，conscious disturbance and convulsions. Liver dysfunction，significantly elevated blood ammonia，decreased blood citrulline，and elevated urine orotic acid and uracil were found by laboratory tests. Different mutations in their OTC gene were identified from these patients. Five mutations，c.118C＞T，c.482A＞G，c.898delT，c.275G＞A and c.803T＞C，were reported. c.67C＞T，c.416T＞G and c.898dupT were novel mutations. After hemofiltration and other active symptomatic treatment，4 patients were improved，while the other 4 patients who discontinued treatment died soon. Conclusion　OTC deficiency is a severe urea cycle disorder with significant individual differences. The patients are usually lack of specific clinical manifestations. For the patients with vomiting of unknown reasons and disturbance of consciousness，blood ammonia test is the key to the diagnosis. Metabolic studies and genetic studies are important to confirm the diagnosis. Early treatment can improve the prognosis.

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Clinical efficacy of Huaiqihuang granule in the treatment of children with bronchial asthma and its effects on the level of cytokines Th1, Th2 and PON1 JIA Chun-mei，WANG Dong-mei，NING Li-hua，et al 2018, 33(5): 365-369.  DOI: 10.19538/j.ek2018050611 Abstract ( )   Objective　To observe the clinical efficacy of Huaiqihuang in the treatment of children with bronchial asthma and its effects on the level of cytokines Th1，Th2 and PON1. Methods　A total of 180 children with bronchial asthma admitted between June 2014 and June 2015 in the Fourth Hospital of Baotou were randomized into the observation group（96 cases of GINA regimen treatment combined with Huaiqihuang）and the GINA regimen treatment group（84 cases），healthy children were enrolled as control group（50 cases）. Times of respiratory tract infection occurrence, asthmatic attacks, application of emergency medicine, hospitalization due to asthmatic and the levels of IL-4，IL-12，IL-13，INF-γ and PON1 were observed before and after treatment. Results　There were significant differences about the times of upper respiratory tract infections, bronchitis and pneumonia, asthma attacks, application of emergency medicine and hospitalization due to asthmatic（P＜0.05）. There were significant differences about the changes of IL-4, IL-12, IL-13, INF-γ and PON1 before and after treatment（P＜0.01）. Conclusion　The treatment of bronchial asthma in children with Huaiqihuang granule can reduce the incidence of respiratory infections and the number of asthmatic attacks and improve clinical effect. The Huaiqihuang granule alleviating asthma symptoms may by decreasing the levels of Th2 cytokines（IL-4，IL-13）and also by increasing the levels of Th1 cytokines（IL-12, IFN-γ）and PON1.

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Cyclic neutropenia：A report of 1 case and literature review CHEN Si-si，YANG Zhi-liang，SUN Gui-lian 2018, 33(5): 370-373.  DOI: 10.19538/j.ek2018050612 Abstract ( )   Objective　To strengthen the understanding of cyclic neutropenia （CN） and improve the level of diagnosis and treatment. Methods　One case of recurrent fever with oral ulcer in a CN child who remained undiagnosed for five years was admitted to Department of Pediatrics，the First Affiliated Hospital of China Medical University on October 8th， 2015. The clinical manifestations of CN, examination in children and treatment options were summarized，and literatures were reviewed. Results　The patient had a history of recurrent regular infections at a 20-day intervals for five years. During the onset，the child had painful mouth ulcer，fever，bacterial infections and neutropenia. Bone marrow puncture showed granulocyte proliferation decreased, and blood neutrophil counts were normal during the interval of two attacks；ELANE heterozygous gene mutation was in line with CN. Conclusion　If clinical features and neutrophil numbers show a periodic change，bone marrow puncture should be done and ELANE gene should be detected. In cases of repeated low concentration of neutrophils in blood，the detection of ELANE gene mutation is useful for diagnosis of CN.

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Phenotype and genotype of Prader-Willi syndrome：An analysis of 14 neonates LIU Shu，OUYANG Hai-mei，CHEN Nuan，et al 2018, 33(5): 374-376.  DOI: 10.19538/j.ek2018050613 Abstract ( )

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Clinical value of fNIRS-EEG in children with epilepsy ZHU Xiao-han，YANG Jian 2018, 33(5): 377-380.  DOI: 10.19538/j.ek2018050614 Abstract ( )

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Research progress in the application of amplitude integration of electroencephalogram in preterm infants MA Qian，MIAO Jing-kun，HUA Yuan-yuan，et al 2018, 33(5): 377-380.  DOI: 10.19538/j.ek2018050615 Abstract ( )

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Diagnosis and treatment advances of eosinophilic cystitis in children SONG Yan-jun，ZHANG Xin，MA Qing-shan 2018, 33(5): 386-390.  DOI: 10.19538/j.ek2018050616 Abstract ( )

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Progress in the research on stool metabolites and intestinal micro-ecology KONG Wen-wen，WANG Zhao-xia 2018, 33(5): 391-395.  DOI: 10.19538/j.ek2018050617 Abstract ( )

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Multicentric-type and mix-type Castleman’s disease：A report of 1 case CHEN Jian-cong，WU Xi-ping 2018, 33(5): 396-398.  DOI: 10.19538/j.ek2018050618 Abstract ( )

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Maple syrup urine disease in the neonates of different gestational age：A report of 3 cases Nasiman Nijiati，LIU Xian-feng，YAN Chao-ying 2018, 33(5): 399-400.  DOI: 10.19538/j.ek2018050619 Abstract ( )