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06 September 2020, Volume 35 Issue 9 Previous Issue    Next Issue

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6th of the recommendations series of experts on clinical practical hot issues of children’s immune related diseases——Advice on the application of cyclophosphamide in the immune diseases in children Pediatric Allergy Immunology Rheumatology Branch of Asia-Pacific Association of Medicine and Bio-Immunology，Editorial Committee of Chinese Journal of Practical Pediatrics 2020, 35(9): 665-672.  DOI: 10.19538/j.ek2020090601 Abstract ( )

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Expert consensus on pain management in children with cerebral palsy Subspecialty Group of Rehabilitation of the Society of Pediatrics of Chinese Medical Association 2020, 35(9): 673-677.  DOI: 10.19538/j.ek2020090602 Abstract ( )

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Paying attention to pulmonary vascular diseases in children LIU Han-min 2020, 35(9): 678-680.  DOI: 10.19538/j.ek2020090603 Abstract ( )   Pulmonary vascular diseases are one of the hottest fields in the researches on vascular diseases，due to the important role of circulation system in individual survival，growth，and development，and also because lung is the unique organ in human body with both systemic and pulmonary circulations. Along with the development of clinical techniques in all subdisciplines of pediatrics，pulmonary vascular diseases in children have obtained attentions  from more and more researchers. This review focuses on the differences in pulmonary vascular disease spectrum between children and adults，the research direction of pulmonary hypertension in children，the mechanism of pulmonary embolism in children，and the vascular abnormality of chronic lung diseases after preterm，with the aim to display the importance of the researches on pulmonary vascular diseases in children and to launch more systematic researches in this field with the integration of multiple disciplines in future.

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Development and anatomicophysiological characteristics of pulmonary vasculature WANG Ya-ru，JIN Hong-fang 2020, 35(9): 681-683.  DOI: 10.19538/j.ek2020090604 Abstract ( )   Pulmonary vascular blood volume accounts for about 9% of the cardiovascular system and plays an important role in maintaining the stability of human respiratory function and cardiac circulation. Congenital developmental abnormalities of pulmonary vasculatures and disorders of anatomical and physiological functions can lead to pulmonary vascular diseases，such as pulmonary hypertension，pulmonary embolism，pulmonary vasculitis，etc. Therefore，understanding the development and anatomical physiology of pulmonary vasculatures is essential to the study of the pathogenesis of pulmonary vascular diseases.

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Congenital pulmonary vascular disease LI Hai-yan*，CHEN Bo，ZHANG Hai-lin 2020, 35(9): 684-688.  DOI: 10.19538/j.ek2020090605 Abstract ( )   Congenital pulmonary vascular disease are a group of diseases，which are caused by deformity of the anatomical morphology and structure of pulmonary arteries，pulmonary veins，and pulmonary vascular plexus during the embryonic development. In recent years，there have been increasing reports of such diseases. However，there is no comprehensive and systematic classification of congenital pulmonary vascular disease，which leads to confusion in the clinical diagnosis and treatment. This article summarizes the classification，clinical manifestations，diagnostic methods and treatment strategies of congenital pulmonary vascular disease.

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Pediatric pulmonary hypertension WANG Wen-di， FAN Wen-wen 2020, 35(9): 688-693.  DOI: 10.19538/j.ek2020090606 Abstract ( )   Pulmonary hypertension can occur at all ages in childhood，which can be caused by many reasons. It is relatively difficult to diagnose pediatric pulmonary hypertension due to its lack of specific clinical manifestations. In some cases，right heart failure and sudden death can occur before diagnosis，so it is with a high fatality rate.  Strengthening the pediatrician’s understanding of this disease is especially important for early diagnosis and treatment and improving prognosis of pulmonary hypertension.

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Pulmonary embolism in children ZHAO De-yu，ZHANG Jia-min 2020, 35(9): 693-699.  DOI: 10.19538/j.ek2020090607 Abstract ( )   The incidence of pulmonary embolism in children is increasing. Different from adults，there is no obvious specificity in clinical manifestations of pulmonary embolism. Hemodynamic instability is rare. It is likely to be misdiagnosed or to fail to diagnose and the mortality is high. Most of the experience in treatment comes from that for adults. Due to the characteristics of children，the experience of treatment is insufficient. Now in order to improve clinicians’ understanding of the disease and reduce mortality，we review the incidence，risk factors，clinical manifestations，diagnosis，treatment，prevention and prognosis of pulmonary embolism in children in order to make early diagnosis and treatment.

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Pulmonary vasculitis in children WEI Qing，NONG Guang-min 2020, 35(9): 699-704.  DOI: 10.19538/j.ek2020090608 Abstract ( )   Pulmonary vasculitis is a group of diseases，which are characterized by the inflammation and destruction of pulmonary vessel walls. Usually，it is one of the pulmonary manifestations of the sysetmic disease，whereas it can also be the isolated pulmonary vascular involvement. In children，it’s rare and caused by a variety of causes. Due to the diversity of affected vessels，pulmonary vasculitis is highly heterogenous in the respiratory manifestations，and may result in pulmonary arterial stenosis，pulmonary arterial aneurysm，pulmonary arterial thrombosis，pulmonary infarction，alveolar hemorrhage，pulmonary arterial hypertension and so on. The aim of therapy is to control the vascular inflammation and manage the pulmonary complications. Usually，glucocorticoid is cornerstone in the treatment of primary pulmonary vasculitis. The combination therapy of glucocorticoid with immunosuppressant is critical to improving the prognosis. Recently，biologics have showed a good effect in the treatment of pulmonary vasculitis.

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Imaging diagnosis of pulmonary vascular diseases in children DUAN Xiao-min，YU Tong 2020, 35(9): 704-711.  DOI: 10.19538/j.ek2020090609 Abstract ( )   Pulmonary vascular diseases are a group of diseases involving all levels of pulmonary arteries，veins and pulmonary capillaries of pulmonary circulation. It has different pathogenic factors and pathological characteristics. There are significant differences between children’s and adults’ pulmonary vascular diseases in terms of pathogenic factors，pathogenesis，pathophysiology，clinical manifestations and prognosis. Pediatric pulmonary vascular diseases include：1. congenital pulmonary vascular malformation，including pulmonary arteriovenous malformation，pulmonary capillary dysplasia，tracheobronchial malformation with pulmonary vascular malformation and vascular lymphangiopathy，etc；2. pulmonary embolism including inflammatory embolism and tumor embolism；3. pulmonary vasculitis；4. pulmonary hypertension；5. pulmonary angiogenic tumor. The common imaging examination methods of pediatric pulmonary vascular diseases are chest X-ray plain film，ultrasonic echocardiography，chest CT enhanced scan，pulmonary angiography，radionuclide ventilation and perfusion lung scan，etc. In this paper，the evaluation of imaging examinations and the key points of imaging diagnosis in the above-mentioned pediatric pulmonary vascular diseases are summarized.

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Causes and characteristics of 637 children with congenital pulmonary vascular disease in the Second Affiliated Hospital of Wenzhou Medical University from 2010 to 2019 SHI Lin-wei*，LYU Fang-fang，LI Hai-yan，et al 2020, 35(9): 712-716.  DOI: 10.19538/j.ek2020090610 Abstract ( )   Objective　 To summarize the classification，diagnosis and clinical characteristics of congenital pulmonary vascular disease from 2010 to 2019.  Methods　The clinical data of 637 children with congenital pulmonary vascular disease from January 2010 to December 2019，who were admitted to the Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University，were retrospectively analyzed and summarized.  Results　A total of 637 children（368 males and 269 females） were included. The diagnosis age ranged from 20 minutes to 13 years，and the median age was 6 month；420 patients were diagnosed at less than 1 year old（65.93%）. Among the 637 children，pulmonary artery abnormality was found in 545 cases，pulmonary vein abnormality in 103 cases，and circulatory traffic abnormality in 14 cases. Among them 25 cases had two kinds of pulmonary vascular abnormalities，and 11 cases had two kinds of pulmonary artery abnormalities. Pulmonary atresia and anomalous pulmonary venous connection were diagnosed at a younger age than pulmonary artery dysplasia，pulmonary stenosis and pulmonary artery sling，and there was statistical difference（χ2＝82.28，P＜0.05）. The clinical manifestations included respiratory tract infection，cyanosis，hemoptysis，etc.，and 197 cases had no obvious clinical symptoms. The primary symptoms were mainly respiratory infection symptoms（32.65%），cardiac murmur（26.69%），cyanosis（20.72%），and B-ultrasound findings on physical examination（4.71%）. Totally 629 children underwent cardiac ultrasound examination with a positive diagnosis rate of 91.73%；179 patients underwent CTA examination with a positive diagnosis rate of 70.47%. The overall positive rate of cardiac ultrasound was higher than that of CTA（χ2＝59.10，P＜0.05）. Operative treatment was performed in 190 cases（29.83%） and interventional treatment in 62 cases（9.73%）. A total of 427 cases（67.03%） were cured or improved，103 cases（16.17%） died or gave up treatment，53 cases（8.32%） were transferred to other hospital and failed to be followed-up. The remaining palliative patients without surgical treatment were followed up.  Conclusion　The 637 cases of congenital pulmonary vascular disease in the Second Affiliated Hospital of Wenzhou Medical University from 2010 to 2019 were mainly pulmonary artery abnormalities，accounting for 85.56%. The top three types were pulmonary stenosis，pulmonary atresia，and anomalous pulmonary venous connection. The clinical manifestations of congenital pulmonary vascular disease were nonspecific. Cardiac ultrasound is sensitive to pulmonary stenosis，pulmonary atresia，and pulmonary artery dysplasia，and CTA can be the first choice for diagnosis of pulmonary artery sling.

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Clinical diagnosis and treatment analysis of congenital pulmonary arteriovenous malformation in children ZHANG Shu-lan，TIAN Man，BAO Yu-ling，et al 2020, 35(9): 717-721.  DOI: 10.19538/j.ek2020090611 Abstract ( )   Objective　To explore the clinical features of congenital pulmonary arteriovenous malformations in children，and evaluate the role of CT angiography（CTA） and digital subtraction angiography（DSA） in the diagnosis. Methods　Retrospective analysis method was used to analyze the clinical features，laboratory tests，imaging data and treatment outcomes of seven cases of congenital pulmonary arteriovenous malformation admitted to Children’s Hospital of Nanjing Medical University. Results　（1）Four cases were pulmonary arteriovenous fistula with the main clinical symptom of cyanosis and clubbed fingers，and were shown to be pulmonary arteriovenous malformations by CT angiography（CTA）. One case received surgical resection of the right lower lung.  Two cases received thoracoscopic ligation surgery for the involved pulmonary arteriovenous fistula. One case underwent occluded embolization surgery for the right lower arteriovenous fistula under DSA. The cyanosis and clubbed fingers were significantly improved after 1-5 years’ clinical follow-up in three cases. One case still stayed in the hospital for treatment. （2）Three cases were bronchial artery-pulmonary arterial fistula with clinical manifestation of hemoptysis. According to the CT angiography（CTA）imaging of two cases，one case was thickened and tortuous right bronchial artery，and the other case had no abnormal bronchial artery. DSA surgery was performed in all three patients. Abnormal bronchial artery branch development and shunt were observed during the surgery. Two cases received occluded embolization treatment for the bronchial artery. One case had hemoptysis again two months after the occluded embolization treatment，and was occluded again. The 3-year follow-up showed no hemoptysis after that. One case was clinically observed due to small shunt and was followed up for about 15 months，which showed nohemoptysis. Conclusion　Children with unexplained hemoptysis，cyanosis and clubbed fingers should be considered as with congenital pulmonary arteriovenous malformations；CTA or digital subtraction angiography is helpful to make a definite diagnosis. Compared with CTA，DSA shows the involved blood vessels and their movement more directly，and helps to judge the range and complexity of the involved vessels.

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A cross-sectional survey and analysis of childhood allergic diseases in two communities of Yangpu District，Shanghai XU Jing*，WANG Xiao-qiu，LI Wen-xiu，et al 2020, 35(9): 722-726.  DOI: 10.19538/j.ek2020090612 Abstract ( )   Objective　To compare the prevalence of childhood allergic diseases in the new and old communities of Yangpu District，Shanghai，analyze the morbidity factors and diseases control level，and improve clinicians’ understanding and diagnosis/treatment of childhood allergic diseases. Methods　In this study，from December 2019 to January 2020，parents of children aged 0-5 in the new and old communities of Yangpu District（the new community is the Xinjiangwan City community；the old community is the Wujiaochang community） were surveyed about allergic diseases. The questionnaire included the core questions of asthma，rhinitis，eczema in International Children’s Asthma and Allergy Research（ISAAC） questionnaire，plus the child’s family background information，indoor and outdoor environment，lifestyle habits and other issues；analyze and study the related data on children’s allergic diseases. Results　In this questionnaire survey，a total of 1247 valid questionnaires were collected from Xinjiangwan City community and Wujiaochang community（590 from Xinjiangwan City community，298 male，292 female；657 from Wujiaochang community，346 male，311 female）. The prevalence of wheezing was：20.4% in Xinjiangwan City community，12.6% in Wujiaochang community，P＜0.001；the prevalence of rhinitis was：40.0% in Xinjiangwan City community，28.0% in Wujiaochang community，P＜0.001；the prevalence of eczema was：17.5% in Xinjiangwan City community，8.5% in Wujiaochang community，P＜0.001；the prevalence of food allergies was：23.3% in Xinjiangwan City community，16.6% in Wujiaochang community，P＝0.004；family history of allergic diseases was：34.2% in Xinjiangwan City community，23.0% in Wujiaochang community，P＜0.001. Conclusion The prevalence of childhood allergic diseases in Xinjiangwan City community is significantly higher than that in Wujiaochang community. External environmental factors and lifestyle changes play an increasingly important role in the development of childhood allergic diseases.

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Changes of plasma endotoxin in children  with craniocerebral injury and its clinical significance CUI Tian-tian，GUAN Wen-he，WANG Li-jie 2020, 35(9): 727-730.  DOI: 10.19538/j.ek2020090613 Abstract ( )   Objective　To study the changes of plasma endotoxin levels in children with craniocerebral injury and its clinical significance. Methods　Twenty-nine children with craniocerebral injury who were hospitalized in PICU in Shengjing Hospital affiliated to China Medical University were enrolled as the case group. Peripheral blood was collected at 1 d，3 d and 5 d after craniocerebral injury，and 10 healthy children were selected as control group. Toxin determination was used to dynamically detect plasma endotoxin levels，and the changes of endotoxin levels at 1 d，3 d and 5 d after craniocerebral injury were compared. At the same time，the data on children’s fever，hospitalization time，feeding mode，surgical history and mechanical ventilation were collected to evaluate the clinical factors affecting plasma endotoxin levels. Results　Endotoxin levels on day 1，day 3 and day 5 after craniocerebral injury were significantly higher than those in the control group（P＜0.05），and endotoxin levels on day 3 and day 5 were higher than that on day 1（P＜0.05）. GCS score was negatively correlated with endotoxin levels（P＜0.05）. The level of endotoxin in the severe head injury group was higher than that in the light and moderate group. The level of endo toxin in the MODS group was higher than that in the non-MODS group. The endotoxin level in the gastrointestinal dysfunction group was higher than that in the non-gastrointestinal dysfunction group. Intestinal feeding and GCS score on admission were independent factors of endotoxemia（P＜0.05）. PCIS score was negatively correlated with endotoxemia，but it was not an independent factor. Conclusion　Endotoxin levels increase significantly within 3 days after craniocerebral injury. The more severe the craniocerebral injury，the higher the endotoxin level；the plasma endotoxin level in children with gastrointestinal dysfunction and MODS increases significantly；enteral feeding on admission can reduce endotoxin levels. The lower the GCS score and PCIS score，the higher the endotoxin level.

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Expression of miRNA in the lung in infants and young children ZOU Xin-yi*，ZHANG Hang-hu，LI Cai-xue，et al 2020, 35(9): 731-735.  DOI: 10.19538/j.ek2020090614 Abstract ( )   Objective　To investigate the effect of miRNA on lung physiology in infants and toddlers by measuring the microRNAs（miRNAs） levels in the bronchoalveolar lavage fluid（BALF） cells. Methods　A total 13 infants and toddlers with airway foreign body removed within 24 hours were enrolled. The BALF cells of 4 children were taken and levels of miRNAs were measured by miRNA microarray and verified by q-PCR. Results　Over 800 miRNAs expression were found among BALF cells. Among the most abundant 100 miRNA，only one pre-miRNA，miR-6800 with 82 bases，was found，and the orthers were mature miRNA. Moreover，the let-7 family was very rich in BALF cells. In the most abundant 100 miRNAs，there were 6 miRNAs of let-7 family，6 miRNAs of miR-17 family，5 of miR-34/449 family，4 of miR-320 family，3 of miR-378 and 2 of miR-146. The miRNA of miR-34/449 family verified by q-PCR showed similar results to miRNA microarray. Conclusion　The data show that there are a large number of miRNA expressions in lung of infants and toddlers，including let-7，miR-17，miR-34/449，miR-146 families，and so on，which suggests that miRNAs participate in not only the normal metabolism of lung cell，but also the maintenance of physiological functions such as immune regulation.

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Research progress in the mechanism of action of respiratory antiviral drugs DING Wei，CAO Ling 2020, 35(9): 736-740.  DOI: 10.19538/j.ek2020090615 Abstract ( )

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Research progress in systemic lupus erythematosus and regulatory T cells in children LU Lin，LU Mei-ping 2020, 35(9): 741-744.  DOI: 10.19538/j.ek2020090616 Abstract ( )