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06 February 2023, Volume 38 Issue 2 Previous Issue   

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Interpretation of ELSO guidelines for pediatric extracorporeal cardiopulmonary resuscitation FENG Qiu-shuang, HONG Xiao-yang 2023, 38(2): 81-84.  DOI: 10.19538/j.ek2023020601 Abstract ( )   Extracorporeal cardiopulmonary resuscitation is the technique of cardiopulmonary resuscitation using extracorporeal membrane oxygenation. To further improve the survival rate of cardiac arrest and improve the long-term neurological outcomes，the Extracorporeal Life Support Group developed pediatric ECPR guidelines in 2021. Detailed guidance is provided on decision and timing of ECPR，team building，and implementation of ECPR. This article interprets the recommended content of the guidelines.

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Strengthening deep research of pediatric extracorporeal membrane oxygenation ZHAO Zhe, FENG Zhi-chun 2023, 38(2): 85-88.  DOI: 10.19538/j.ek2023020602 Abstract ( )   Extracorporeal Membrane Oxygenation（ECMO） has been used more and more widely in rescuing pediatric patients with most severe cardiac and pulmonary failure. However，there are still dozens of problems puzzling the physicians in the clinical practice. With the development of ECMO technology，more and more attention has been paid to the clinical research of pediatric ECMO. This review focuses on the following aspects of pediatric ECMO：research of clinical application of pediatric ECMO，basic research of pediatric ECMO and research about equipment and consumables of ECMO with the aim to present the importance of researches related to pediatric ECMO，push forward the development of the related researches in China，and improve the treatment level in pediatric ECMO.

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Research and application of ventricular assist device with impeller for children HAN Lu, WANG Wei 2023, 38(2): 89-93.  DOI: 10.19538/j.ek2023020603 Abstract ( )   The role of ventricular assist devices with impeller in the treatment and extracorporeal life support of patients with heart failure has been widely recognized. After decades of research，various types of ventricular assist devices for adult have been successfully used in clinical treatment，but the research in the development of similar devices for pediatric patients is relatively rare. Ventricular assist devices for children are not a smaller version of adults，but they have to consider the following characteristis：the difference in body weight is large，the flow range is wide，the absolute flow is small，and it's very likely to have thrombosis. Thus，the development of children’s ventricular assist device is more difficult. This paper introduces the existing blood pump with impeller developed for children.

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Anticoagulation management in pediatric extracorporeal membrane oxygenation YANG Bao-wang, WANG Yi 2023, 38(2): 93-98.  DOI: 10.19538/j.ek2023020604 Abstract ( )   Extracorporeal membrane oxygenation（ECMO） has become a critical and essential technology in adult and pediatric intensive care units and is the ultimate treatment for severe respiratory and/or circulatory failure. Complications related to the coagulation system such as bleeding and thrombosis during ECMO are one of the main factors affecting the mortality of patients. There is no unified standard on the best anticoagulant strategy for ECMO. This paper reviews the research progress of anticoagulation management and monitoring methods during ECMO in children，and discusses how to achieve the best anticoagulation management during ECMO.

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Pediatric extracorporeal membrane oxygenation transport HU Yan, ZHOU Cheng-bin, GUO Yu-xiong 2023, 38(2): 98-103.  DOI: 10.19538/j.ek2023020605 Abstract ( )   Extracorporeal membrane oxygenation （ECMO） transport of critically ill patients is a procedure that consumes a lot of medical resources，is technically difficult，and has high medical risks. With the advancement of technology and the accumulation of experience，ECMO is being used more and more in the treatment of critically ill child patients. Researches and practice have proved that the implementation by experienced ECMO team is helpful to improve the success rate of treatment. A good understanding of the safety，implementation points and adverse events of ECMO transport helps to improve the application of ECMO transport technology in pediatric critical illnesses.

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Artificial placenta：Recent advances and challenges towards clinical translation WANG Gang, LI Qiu-ping, HONG Xiao-yang, et al 2023, 38(2): 103-108.  DOI: 10.19538/j.ek2023020606 Abstract ( )   Artificial placenta technology aims to maintain the fetal physiological state of extremely preterm infants by extracorporeal life supporttechnology，simulate the intrauterine environment，maintain the development of vital organs，avoid the iatrogenic injury of traditional treatment，so as to decrease the mortality and severe morbidity of extremely preterm infants，and improve the long-term quality of life of extremely preterm infants.In recent years，great progress has been made in the field of artificial placenta，and clinical translation is around the corner. This article reviews the research background，recent advances and the challenges in the clinical translation of artificial placenta.

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Continuous renal replacement therapy with the support of extracorporeal membrane oxygenation ZHOU Yi-ping, MIAO Hui-jie, ZHANG Yu-cai 2023, 38(2): 109-113.  DOI: 10.19538/j.ek2023020607 Abstract ( )   Extracorporeal membrane oxygenation （ECMO） is used in salvage treatment for patients with cardiopulmonary failure. Continuous renal replacement therapy （CRRT） is increasingly used to manage the acute kidney injury（AKI） and/or fluid overload （FO），and electrolyte acid-base disorders in patients receiving ECMO support. Multiple factors need to be considered when CRRT is used in combination with ECMO，including the indications，connection mode，when to initiate and terminate CRRT ，complications，anticoagulation，and drug concentration.

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Survey of the for recurrence of functional constipation in pediatric patients and analysis of the risk factors CHENG Wei-wei, CHEN Ke, ZHOU Li, et al 2023, 38(2): 114-118.  DOI: 10.19538/j.ek2023020608 Abstract ( )   Objective　To study the epidemiology and some of the risk factors for recurrence of childhood functional constipation（FC）. Methods　One hundred and twenty-one children with functional constipation were selected，who were admitted to the Digestive Specialist Clinic of Shanghai Children’s Hospital and were clinically cured. We used a questionnaire concerning general demographic characteristics，date of confirmation，date of clinical cure，symptom recurrence after drugs withdrawal，date of first recurrence，exposures of the various categories of risk factors is identified. Results　The rate of recurrence within one year was 58.7%. The risk factors for recurrence of functional constipation in children were analyzed by univariate regression analysis. The significant risk factors for recurrence of functional constipation in children analyzed were age，history of allergy，family history of constipation，picky eating，history of colds in one year，history of antibiotic use and so on. After multivariable Cox regressicn analysis found that the risk factors for recurrence of functional constipation were age（RR=2.093，P=0.029），history of colds in one year（RR=2.226，P=0.037），guardian's education level（RR=2.556，P=0.003），family monthly incom（RR=2.353，P=0.011）. Conclusion　The functional constipation in children is closely related to baseline age，history of colds，guardian's education level，family monthly incom and so on. Early intervention can be carried out based on its risk factors，so as to reduce the possibility of recurrence.

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Clinical study of ulinastatin in neonatal necrotizing enterocolitis based on hemodynamic monitoring MA Le, TANG Xiao-jing, WANG Yi 2023, 38(2): 119-124.  DOI: 10.19538/j.ek2023020609 Abstract ( )   Objective　To investigate the hemodynamic effects of ulinastatin on neonatal necrotizing enterocolitis（NEC） and the effectiveness of clinical treatment. Methods　A reprospective study was adopted. A total of 150 cases of neonatal necrotizing enterocolitis were chosen，who were clinically diagnosed in Xi 'an Children's Hospital from August 2016 to January 2020. 30 cases in the observation group were treated with ulinastatin，and the other 120 cases without ulinastatin were used as the control group. Demographic baseline data of the two groups were collected，the changes in clinical biochemical indexes before and 48 h after treatment were compared between the two groups. The changes in hemodynamic indexes in the observation group at different time points of ulinastatin injection were continuously measured. At the same time，compare the hemodynamic changes at 24h and 48h after medication between the two groups，and compare the difference in hospitalization as well. Results　After 48 hours of treatment，the levels of prothrombin time（PT），partial thrombin time（APTT），D-dimer（D-D），serum creatinine（Scr），serum lactate（Lac），serum precalcitonin（PCT），interleukin-6 （IL-6）and interleukin-8 （IL-8） were all lower than those before treatment，in the observation group（all P<0.05） while APTT，D-dimer，PCT，IL-6 and I L-8 in the control group were higher than those before treatment（all P<0.05）. The PT，APTT，D-dimer，Scr，Lac，PCT，IL-6，IL-8 in the observation group were lower than those in the control group at 48 hours after treatment. After 48 h of treatment，the incidence of blood stool，abdom-inal distension，bowel sound attenuation，abdominal ultrasound and abdominal tablet abnormality in the observation group was all lower than that before treatment and in the control group （all P<0.05）. In the observation group，after 30 minutes in ulinastatin，the changes of hemodynamic indexes were not significant. After 60 minutes of medication，hemodynamics tended to improve and gradually remained stable .The difference in continuous measurement within the group was statistically significant（all P<0.05）. Compared with the control group，the fasting time，length of hospital stay，incidence of surgical operation and mortality were all decreased in the observation group （all P<0.05）. Conclusion　Ulinastatin can improve hemodynamics by reducing the inflammatory response of the body，and reduce the fasting time，hospitalization time，incidence of surgery and mortality of neonatal necrotizing enterocolitis.

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Application of blood metabolites by determination ultra-high performance liquid chromatography-tandem mass spectrometry in screening and monitoring of methylmalonic acidemia GUO Yuan-fang, YAN Yan, LI Gai-jie, et al 2023, 38(2): 125-128.  DOI: 10.19538/j.ek2023020610 Abstract ( )   Objective　To investigate the application value of ultra-performance liquid chromatography-tandem mass spectrometry by second-order screening method in methylmalonic acidemia （MMA） screening and follow-up detection of positive children，and analyze the its correlation with traditional tandem mass spectrometry screening method and gas chromatography-mass spectrometry （GC-MS） in the detection of MMA indicators. Methods　A total of 49，490 neonatal dried blood filter paper samples with abnormal propionyl carnitine（C3） or abnormal ration between C3 acetylcarnitine （C2） were collected for second-order screening. Analyze the changes of the concentration of each index detected by the second order screening， tandem mass spectrometry and urinary organic acid spectrum and the correlation in the children with MMA who were followed up regularly. Results　Among 49490 newborns，500 samples were positive for MMA screening by traditional tandem mass spectrometry，with a positive rate of 1.01%，and 16 samples were confirmed by gene testing. Of the 827 samples that entered the second-stage screening process，31 samples were positive，the screening positive rate was 0.06%，and 16 samples were confirmed by gene testing，which was consistent with the confirmed samples by traditional tandem mass spectrometry. There was a positive correlation between the second order screening and serum homocysteine，traditional tandem mass spectrometry and urine organic acid spectrum in the detection of various indicatiors（P<0.05）. Conclusion　The positive rate of MMA screening by the second-order screening method is significantly lower than that by the traditional tandem mass spectrometry. The combined detection of the second-order screening method is the traditional tandem mass spectrometry and urinary organic acid spectrometry can effectively improve the diagnostic efficiency of MMA screening，and can be used to observe the efficacy in children in order to carry out more targeted treatment.

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High-risk factors for the occurrence and development of severe Kawasaki disease with the increase of C-reactive protein，procalcitonin and the decrease of blood potassium and the evaluation of clinical prediction value BING Zhen, WANG Xian-dong, SHAN Guang-song, et al 2023, 38(2): 129-135.  DOI: 10.19538/j.ek2023020611 Abstract ( )   Objective　To explore the high-risk factors for the occurrence and development of severe Kawasaki disease（SKD） and the evaluation of its clinical prediction value. Methods　The children with Kawasaki disease were retrospectively analyzed，who were diagnosed in the Heart Center of the Women and Children's Hospital Affiliated to Qingdao University from November 2016 to July 2021. They were divided into the SKD group（18 cases） and the ordinary Kawasaki disease（OKD） group（639 cases） according to whether there was serious organ system damage or unstable vital signs. After matching compare the differences between the two groups in clinical manifestations，auxiliary examinations，response to intravenous immunoglobulin，coronary artery lesion，and length of hospital stay. Stepwise logistic regression was used to explore the high-risk factors for SKD，The ROC curves of single factor and joint prediction were drawn. Results　After propensity score matching，there were 18 cases in the SKD group and 36 cases in the OKD group. There were significant differences between the two groups in terms of multiple serous cavity effusions，non-response to intravenous immunoglobulin and length of hospital stay （P<0.05）. There were significant differences in hemoglobin，C-reactive protein，procalcitonin，serum sodium，serum potassium，total bilirubin，direct bilirubin，aspartate aminotransferase，albumin，globulin，atrial natriuretic peptide precursor and proteinuria between the two groups （P<0.05）. Stepwise logistic regression analysis and ROC curve analysis showed that the increase in C-reactive protein and procalcitonin and the decrease of serum potassium were the risk factors and predictive indicators for the occurrence of SKD （P<0.05）. The sensitivity and specificity of the combination of the three methods in predicting the occurrence of SKD were 94.44% and 80.00%，respectively. The prediction threshold was 3.7 mmol/L for serum potassium，104.7 mg/L for C-reactive protein，and 1.11 ng/mL for procalcitonin，respectively. The predictive value of the three indicators combined was higher than that of C-reactive protein or procalcitonin （P<0.05） alone. Conclusion　The clinical manifestations of SKD are diverse. Multiple serous cavity effusions and non-response to intravenous immunoglobulin often occur. The increase in C-reactive protein and procalcitonin and the decrease in blood potassium are the high-risk factors for SKD.

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Pediatric inflammatory bowel disease and small intestinal bacterial overgrowth HUANG Shuai, WANG Yi-lang, WANG Yi-xuan, et al 2023, 38(2): 136-139.  DOI: 10.19538/j.ek2023020612 Abstract ( )   Small intestinal bacterial overgrowth refers to a group of clinical syndromes in which a variety of factors cause an increase in the number of bacteria in the small intestine， resulting in non-specific symptoms such as abdominal distension， abdominal pain and diarrhea. It is also a manifestation of intestinal bacterial disorder.Studies have shown that it is associated with a variety of gastrointestinal diseases in children. Breath test， a simple and easy diagnostic method， is used to detect small intestinal bacterial overgrowth in patients with inflammatory bowel disease， so as to avoid adverse reactions caused by improper use of drugs because of misunderstanding it as disease recurrence. At present， there is a lack of understanding of small intestinal bacterial overgrowth in children with inflammatory bowel disease. This article reviews the pathogenesis， diagnostic methods， clinical significance and the relationship between intestinal bacterial overgrowth and inflammatory bowel disease.

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Research progress of chromosomal microarray analysis for screening fetal congenital heart disease ZHANG Xiao-meng, YE Yu-jiao, WANG Xian-min 2023, 38(2): 140-144.  DOI: 10.19538/j.ek2023020613 Abstract ( )   Congenital heart disease（CHD） is a common birth defect caused by both environmental and genetic factors，of which genetic factors play a major role. G-banding karyotype detection，fluorescence in situ hybridization，and chromosomal microarray analysis（CMA） are common genetic diagnostic techniques. In recent years，CMA has gradually become the first-line method for prenatal diagnosis of fetal CHD. One of the main advantages of CMA is its ability to precisely define unbalanced regions，to definitively diagnose microdeletion/microduplication syndromes，and to detect a large number of copy number variations of unknown clinical significance. When detecting total copy number variation in prenatal samples，copy number variation detection is characterized by high reliability，accuracy and reproducibility. However，in many cases，the impact of copy number variation on cardiac development in patients with CHD remains unclear. This article will focus on chromosomal microarray analysis technology，and review its diagnostic significance and prognostic guidance in fetal CHD.

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Effect of Claudin-7 in the pathogenesis of bronchopulmonary dysplasia in preterm infants BAI Jing-xuan, YUE Dong-mei 2023, 38(2): 145-150.  DOI: 10.19538/j.ek2023020614 Abstract ( )   Bronchopulmonary dysplasia（BPD） is a complication that frequently occurs in preterm infants and is linked to the barrier structure and malfunction of the pulmonary epithelium. Claudins，known as a family of proteins，are one of the major components of tight junctions（TJs） and participate in relating intercellular exchange. Although little is known about the role of Claudin-7 in influencing alveolar barrier function and its regulation mechanism in the lung，there is evidence that expression of Claudin-7 can affect alveolar epithelial permeability and alveolar development，which in turn affects the development of BPD. The expression of Claudin-7 is affected by multiple regulatory factors，and understanding its effect will help to clarify the pathogenesis of BPD，and then open up new options for the diagnosis and the treatment of BPD.

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Congenital disorder of glycosylation-Iy caused bya novel mutation of SSR4 gene：A case report SUN Wen-qiang, ZHU Xue-ping 2023, 38(2): 151-153.  DOI: 10.19538/j.ek2023020615 Abstract ( )   This paper retrospectively analyzes the clinical and follow-up data and genetic test results of a male child with congenital disorder of glycosylation（CDG）-Iy caused by SSR4 mutation，who was admitted to the Children's Hospital of Soochow University on July，2021 due to respiratory distress. The genetic tests showes the presence of a hemizygous variant c.80_96del in the SSR4 gene，which is inherited from the child's mother. Based on the literature，the children with this disease are often complicated with appearance deformities，feeding difficulties，and moderate to severe comprehensive growth retardation. Early genetic testing can help clarify the diagnosis and provide symptomatic interventions to improve the prognosis.

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Membranous nephropathy after allogeneic hematopoietic stem cell transplantation in a child: a case report and literature review WANG Ting-jie, ZHENG Wei-min, FU Rui, et al 2023, 38(2): 154-156.  DOI: 10.19538/j.ek2023020616 Abstract ( )   A child at 6 years and 9 months old with thalassemia was admitted to our hospital in September， 2020 due to more than 2 months of edema and over 1 moth of albuminuria，hematuria at 2 years and 10 months atter hematopoietic stem cell transplantation. After completing examination in our hospital， hypoalbuminemia and massive albuminuria were observed. Renal biopsy pathology indicated stage II membranous nephropathy （MN），which was diagnosed as secondary MN. Complete remission of urinary protein was achieved after treatment with glucocorticoid combined with tacrolimus.

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A case report of infant death caused by holocarboxylase synthetase deficiency CHEN Rui, NING Li-hua 2023, 38(2): 157-160.  DOI: 10.19538/j.ek2023020617 Abstract ( )   The clinical and molecular hereditary of a child died of HLCS deficiency in May， 2020 in the Fourth Hospital of Baotou were retrospectively reviewed. The child，a 2-month-and-29cl old boy，was admitted to hospital due to sputum in the throat for 5 days，convulsions for 1 day and coma for 12 hours. He was poorly responsive and his metabolic acidosis was intractable. Two days later，the patient asked for discharged and died on the day of his returning home. Blood tandem mass spectrometry showed a significant increase in 3-hydroxyisovaleryl carnitine and propionyl carnitine. Urine gas chromatography mass spectrometry showed a significant increase in 3-hydroxypropionic acid，propionylglycine，methylcrotonylglycine and methylcitricacid. Whole exome sequencing showed there was a compound heterozygous mutation of the c.1522C>T （p.R508W）/c.782delG （p.G260V） in the HLCS. The Sanger family verified that the mutations were derived from their parents. The clinical presentations of HLCS deficiency are atypical. In children with severe pneumonia combined with epilepsy and intractable metabolic acidosis，HLCS deficiency should be considered and the diagnosis should be confirmed early by timely MS/MS，GC/MS screening and genetic analysis.