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Table of Content

    15 January 2023 Volume 41 Issue 1
      
    Commentary
    Concept and practice of intrauterine pediatrics
    SUN Kun
    Journal of Clinical Pediatrics. 2023, 41(1):  1-5.  doi:10.12372/jcp.2023.22e1340
    Abstract ( 163 )   HTML ( 11 )   PDF (1238KB) ( 211 )  
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    In recent years, population aging and negative population growth are occurring globally, leading to a series of economic and social problems including a shrinking labor force, slow economic growth, increasing public finance spending and medical burden. Therefore, ensuring healthy early childhood development and improving population quality are fundamental conditions for building a new pattern of population development in China. Since the 1970s, it has gradually become a consensus in the medical field to pay attention to the intrauterine health of the fetus. As an emerging interdisciplinary discipline, intrauterine pediatrics focuses on prevention, screening, diagnosis and treatment of diseases of developmental origin, and realizes the concept of health management from the fertilized egg to the whole life cycle of children and adolescents, emphasizing the whole process and consistency of health management in early life. The rise and development of intrauterine pediatrics will lay the foundation for building a management system for the whole life health care cycle.

    The role of pediatric specialists in multidisciplinary diagnosis and treatment of fetal diseases
    SUN Luming, DUAN Tao
    Journal of Clinical Pediatrics. 2023, 41(1):  6-10.  doi:10.12372/jcp.2023.22e1573
    Abstract ( 140 )   HTML ( 9 )   PDF (1471KB) ( 122 )  
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    With the development of prenatal imaging, molecular genetic diagnosis and intrauterine therapy, fetal medicine has faced new opportunities and challenges. To provide accurate prenatal diagnosis, genetic counseling and prognosis assessment for high-risk fetuses, and to develop a whole-process management plan of antenatal, perinatal and postnatal integration around sick fetuses and pregnant women on the premise of ensuring the safety of pregnant women, can improve the quality of life of children after birth. This paper analyzed two multidisciplinary diagnosis and treatment modes involving pediatric experts, fetal structure and developmental abnormalities, points out the existing problems and puts forward some suggestions to promote the high-quality development of fetal special disease diagnosis and treatment in China.

    Diagnosis and treatment of fetal congenital malformation
    WANG Jun
    Journal of Clinical Pediatrics. 2023, 41(1):  11-17.  doi:10.12372/jcp.2023.22e1345
    Abstract ( 143 )   HTML ( 13 )   PDF (1244KB) ( 212 )  
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    Congenital malformation is one of the main causes of death and congenital disability in infants and children, which seriously affects the survival and life quality of children. With the remarkable development of prenatal diagnostic techniques, such as medical imaging, clinical genetics, and molecular biology, more and more fetal congenital malformations can be screened and diagnosed during pregnancy. Some fetal congenital malformations deteriorate rapidly in utero, and there is a lack of effective treatment after birth, so the prognosis is poor. Intrauterine fetal therapy can prevent the irreversible damage or death caused by the deterioration of congenital malformation and create conditions for postpartum treatment. Accurate diagnosis and evaluation of congenital malformations, effective prevention and early intervention are expected to reduce mortality and improve the prognosis and the quality of life. In this paper, the development of diagnosis and treatment of intrauterine fetal congenital malformations, the concept of clinical diagnosis and treatment of intrauterine fetal structural malformations and the clinical application of common intrauterine fetal structural malformations will be elaborated in order to provide reference for clinical work.

    Intrauterine Pediatrics
    Application of clinical swallowing evaluation in the treatment of long-gap esophageal atresia children
    WANG Junli, WU Wenjie, TANG Chunyan, ZHANG Ning, WANG Jun, LI Fei
    Journal of Clinical Pediatrics. 2023, 41(1):  18-24.  doi:10.12372/jcp.2023.22e1230
    Abstract ( 137 )   HTML ( 5 )   PDF (1307KB) ( 202 )  
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    Objective To analyze the effect of clinical swallow evaluation (CSE) on the complications and growth of children with long gap esophageal atresia (LGEA) within 24 months of age. Methods The clinical data of children who underwent LGEA surgery from January 2016 to January 2022 were retrospectively analyzed. Children with LGEA who had regular CSE follow-up were included in the CSE group and those who did not participate in CSE were included in the control group. The incidence of complications and the growth of children within 24 months were compared between the two groups. Results A total of 50 children with LGEA were included in the analysis. There were 19 children (11 boys and 8 girls) in the CSE group, including 16 cases of type Ⅰ congenital esophageal atresia (2 cases with fistula), 2 cases of type Ⅱ and 1 case of type Ⅲa. Twelve children received prenatal diagnosis. In the control group, there were 31 children (16 boys and 15 girls), including 28 cases of typeⅠand 3 cases of type Ⅱ (2 cases with fistula). Prenatal diagnosis was obtained in 21 cases. The birth weight of the CSE group was lower than that of the control group, the difference distance of the blind end of the esophagus was longer than that of the control group, and the difference was significant (P<0.05). The interval from postoperative to complete oral feeding was 34.0 (22.0-76.0) days in the CSE group and 46.0 (18.0-181.0) days in the control group, and the difference was statistically significant (Z=2.30, P=0.028). Compared with the control group, the CSE group had a lower incidence of lung infection, lower weight-for-age z score (WAZ) and lower length-for-age z score (LAZ) at 6 months. The detection rates of underweight and stunting in the CSE group at 24 months were lower than those in the control group, and the differences were statistically significant (P<0.05). The increment value of LAZ in the CSE group at 24 months was higher than that in the control group, and the difference was statistically significant (P<0.05).Conclusions Regular CSE follow-up in the long-term treatment of children with LGEA can reduce the incidence of lung infection within 24 months of age, and is conducive to early postoperative complete oral feeding and weight and height catch-up.
    Analysis of clinical and prognostic factors of neonatal congenital chylothorax
    HUA Minmin, XIA Lei, HUO Wanying, ZHANG Yanhua, XU Falin
    Journal of Clinical Pediatrics. 2023, 41(1):  25-29.  doi:10.12372/jcp.2023.22e0961
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    Objective To explore the clinical characteristics of neonatal congenital chylothorax (CC) and the high-risk factors affecting its prognosis. Methods The clinical data of children with CC admitted to the neonatal intensive care unit from January 2012 to May 2022 were retrospectively analyzed. Results Sixty children (36 boys and 24 girls) with CC were included, including 31 premature infants and 29 full-term infants. The gestational age was 36.7 (34.7-38.8) weeks, and the birth weight was 3160.0 (2755.0-3645.0) g. There were 52 cases of prenatal diagnosis of pleural effusion, 13 cases of intrauterine treatment, 31 cases of asphyxia at birth, 27 cases of intubation in the delivery room, 8 cases of pleural puncture during resuscitation, 30 cases of bilateral pleural effusion, 19 cases of fetal edema, 29 cases of hypoproteinemia, 14 cases of congenital chylous ascites, and 12 cases of congenital heart disease/chromosomal abnormalities. Compared with the full-term infant group, the preterm infant group had a lower male proportion, lower birth weight, higher rates of intrauterine treatment, fetal edema, asphyxia, intubation in the delivery room, mechanical ventilation, bilateral pleural effusion, albumin <30g/L, congenital chylous ascites and death, and the differences were significant (P<0.05). There were 49 children in the survival group and 11 in the death group. All the children died of respiratory failure within 1 week after birth. Binary logistic regression analysis showed that fetal edema was an independent risk factor for early neonatal death in children with CC (P<0.05). All children were treated conservatively and followed up. Three children were lost to follow-up, 1 relapsed, and the remaining 45 were in good condition. Conclusions Among neonates with CC, preterm infants are more likely to suffer from sphyxia, fetal edema, hypoproteinemia, bilateral pleural effusion, congenital chylous ascites, and the proportion of intubation in the delivery room, mechanical ventilation and death is higher than that of full-term infants. The CC neonates with fetal edema have an increased risk of early death. The long-term prognosis of children treated conservatively is good.
    Follow-up analysis of 13 fetuses with complete atrioventricular conduction block
    OUYANG Jing’e, MIAO Huiyu, QIU Lianghua, WANG Zaiyu, YE Fengling, LIN Lili, CHEN Jiejian
    Journal of Clinical Pediatrics. 2023, 41(1):  30-33.  doi:10.12372/jcp.2023.22e1143
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    Objective To explore the clinical characteristics and prognosis of fetuses with complete atrioventricular conduction block (CAVB). Methods The clinical characteristics, treatment and outcome of 13 fetal CAVB patients diagnosed by the 900TH Hospital of Joint Logistics Support Force from January 2012 to August 2022 were retrospectively analyzed. Results A total of 13 CAVB fetuses were included, of whom 7 were terminated and 6 survived. Among the 7 terminated fetuses, 6 were complicated with decreased cardiac function, 5 had cardiac structural abnormalities, and mothers of 4 fetuses were positive for ANA and SSA antibodies. Among the 6 surviving fetuses, 1 case was complicated with decreased cardiac function, 2 cases had cardiac structural abnormalities, and mothers of 5 cases were positive for ANA and SSA antibodies. One child had a resting ventricular rate of 42-45 beats/min after birth. He was implanted with a permanent pacemaker because of sudden syncope at the age of 5 years. One patient had atrial septal defect with ventricular rate <70 beats /min. He underwent atrial septal defect repair without pacemaker implantation at the age of 2 years. The remaining 4 patients had ventricular rate >52 beats /min without pacemaker implantation.Conclusion If the CAVB fetus with normal cardiac function and normal cardiac structure has no obvious symptoms after birth, most of them have a good prognosis and permanent pacemaker implantation can be deferred.
    General Report
    Efficacy and safety of lactase additive in preterm infants with lactose intolerance: a prospective, multi-center, randomized controlled trial
    ZHA Xinyi, WANG Yiwen, MAO Pengliang, CHEN Mingyan, JIANG Wei, WANG Huawei, HU Xuefeng, SHI Liping, ZHU Xueping, QIAN Jihong
    Journal of Clinical Pediatrics. 2023, 41(1):  34-41.  doi:10.12372/jcp.2023.22e1095
    Abstract ( 245 )   HTML ( 9 )   PDF (1405KB) ( 212 )  
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    Objective To explore the efficacy and safety of lactase additive in improving lactose intolerance in premature infants. Methods Preterm infants with lactose intolerance admitted to Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai First Maternal and Infant Health Hospital, Children's Hospital Affiliated to Zhejiang University School of Medicine and Children's Hospital Affiliated to Soochow University from January 2018 to December 2019, who met the inclusion criteria were included in the study. They were divided into lactase treatment group and control group randomly, with 80 cases in each group. For the patients in the lactase treatment group, 4 drops (180 mg) of lactase were added to each feeding of breast milk or formula milk for premature infants, and Bifidobacterium triple viable powder was orally administered and abdominal massage was used as adjuvant therapy. For patients in the control group, 4 drops (180 mg) of placebo were added to each feeding, and the same probiotics and abdominal massage as the treatment group were given, After 1 and 2 weeks treatment, symptoms of lactose intolerance, weight, fecal pH, fecal reducing sugar and other indicators were compared between the two groups. Results seventy-eight patients in the lactase treatment group and 77 patients in the control group, respectively, completed the whole study. After the first week intervention, the positive rate of reducing sugar in the lactase treatment group was lower than that in the control group (P<0.05). At the end of the second week treatment, the proportion of abdominal distension in the lactase treatment group were lower than those in the control group (P<0.05), and the positive rate of reducing sugar was lower than that in the control group (P<0.05). The increase of feeding amount was also higher than that of the control group (P<0.05). During the course of the study, no adverse reactions to lactase additives or probiotics were found in both groups. Conclusions Lactase supplementation can effectively and safely improve the clinical symptoms caused by lactose intolerance in premature infants.
    Risk factors of hemoptysis volume and postoperative recurrence in children with vascular-related hemoptysis
    XIE Lei, LYU Tiewei
    Journal of Clinical Pediatrics. 2023, 41(1):  42-47.  doi:10.12372/jcp.2023.22e0415
    Abstract ( 168 )   HTML ( 3 )   PDF (1300KB) ( 107 )  
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    Objective To summarize the clinical characteristics of children with vascular-related hemoptysis, in order to find the risk factors of hemoptysis volume and recurrence. Methods The clinical data of children with vascular-related hemoptysis who received interventional therapy from January 2012 to January 2020 were retrospectively analyzed. According to the hemoptysis volume, children were divided into massive hemoptysis group and non-massive hemoptysis group. According to the clinical outcome, they were divided into relapsed group and non-relapsed group. Logistic multivariate regression analysis was used to analyze the factors affecting the hemoptysis volume and the recurrence of postoperative hemoptysis. Results A total of 41 children (19 boys and 22 girls) with vascular-related hemoptysis were included, and the median age was 8.9 (6.9-11.2) years. Chest vascular enhancement CT showed that abnormal blood vessels were located in the right lung in 32 cases, mainly in the lower lobe of the right lung. Abnormal blood vessels were found in both lungs in 3 cases, in the left lung in 1 case, and no abnormal blood vessels were found in 5 cases. Binary logistic regression analysis showed that large abnormal blood vessel diameter and Mycoplasma pneumoniae (MP) infection were independent risk factors for hemoptysis (P<0.05), while increased abnormal blood vessels were independent risk factors for the recurrence of hemoptysis (P<0.05). Conclusions The high risk factors affecting the hemoptysis volume in children with vascular-related hemoptysis are abnormal vascular diameter and MP infection. Interventional occlusion of abnormal blood vessels is a safe and effective treatment method, and the number of abnormal blood vessels is an important predictor of postoperative recurrence.
    Analysis of the clinical characteristics of epilepsy associated with CHD2 gene variation
    FENG Fan, CHEN Fang, SUN Suzhen, LI Xin, LIU Xuefang, ZHAO Tong
    Journal of Clinical Pediatrics. 2023, 41(1):  48-53.  doi:10.12372/jcp.2023.22e0007
    Abstract ( 306 )   HTML ( 6 )   PDF (4324KB) ( 118 )  
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    Objective To analyze the clinical features of epilepsy in children with CHD2 gene variation. Methods The clinical characteristics and treatment efficacy of 7 children with epilepsy related to CHD2 gene variation were analyzed and summarized. Results The median onset age of seizure in 7 children was 3 years and 2 months old. There were two or more seizure types during the course of the disease in 5 children, and the main seizure was generalized tonic-clonic seizure. Seven children had varying degrees of motor, intellectual and language retardation, and one had a tendency to autism. Abnormal discharge was detected by interictal electroencephalogram in 5 children, and clinical episodes were observed in 4 children. There were no specific changes in skull MRI. More than 2 antiepileptic drugs were needed in 4 children, and the number of seizures decreased after treatment. One patient was not sensitive to antiepileptic drugs and was finally treated with ketogenic diet. At present, the child did not have seizures. The age of the last follow-up was 2 to 7 years old, and seizures were controlled for more than half a year in 3 children. Valproate and levetiracetam are effective drugs for the treatment of CHD2 variation-related epilepsy.Conclusions Children with epilepsy related to CHD2 gene variation have early onset age and various seizure types. Most of the children have refractory epilepsy and poor prognosis, so early intervention should be given.
    Hemophagocytic syndrome caused by visceral leishmaniasis in children:a report of four cases and literature review
    LI Wanyi, WANG Yongjun, LIU Donghai, DONG Xuemei, WANG Wenyuan, HAN Qian
    Journal of Clinical Pediatrics. 2023, 41(1):  54-59.  doi:10.12372/jcp.2023.22e0096
    Abstract ( 175 )   HTML ( 5 )   PDF (1495KB) ( 90 )  
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    Objective To analyze the clinical data of children with visceral leishmaniasis (VL) presenting as hemophagocytic syndrome (HPS) in order to improve the clinical diagnosis and treatment level. Methods The clinical data of 4 children with HPS secondary to VL hospitalized from March 2020 to March 2021 were retrospectively analyzed, and were compared with the literature data. Results All the 4 patients showed persistent fever, splenomegaly and cytopenia. Combined with relevant examinations, the patients were diagnosed with HPS. Considering the epidemiological factors, the examination of VL was performed, and the results showed that rK39 test was positive in 4 cases, and Leishmanias donovani was detected by bone marrow smear in 3 cases. Sodium stibogluconate treatment in the four children was successful. A review of the literature on VL-HLH in the past 5 years showed that compared with primary HLH, children with VL-HLH had higher ferritin and increased erythrocyte sedimentation rate. Compared with VL, VL-HLH may be more prone to pancytopenia, hypertriglyceridemia, hyperserum ferriminemia, and lower absolute monocyte counts. Amphotericin B may be more efficient and safer than sodium stibogluconate for the treatment of VL-HLH.Conclusions For children with a history of staying in the epidemic area, the possibility of VL should be considered in the comprehensive analysis of the primary etiology of HPS, and rK39 test and bone marrow smear examination should be performed at the same time. Early and accurate treatment of the cause can avoid aggravation of the disease and the toxic side effects caused by chemotherapy.
    17q12 microdeletion syndrome: a report of three cases and literature review
    XU Yongli, YANG Jing, ZHOU Lanqi, ZHOU Jianhua
    Journal of Clinical Pediatrics. 2023, 41(1):  60-65.  doi:10.12372/jcp.2023.22e0461
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    Objective To summarize the clinical characteristics of chromosomal 17q12 microdeletion syndrome in children in order to improve the understanding of the disease. Methods The clinical data of 3 children with chromosomal 17q12 microdeletion syndrome admitted from October 2014 to October 2021 were retrospectively analyzed. Genome-wide chromosomal copy number variation was detected by second-generation sequencing and the relevant literature was reviewed. Results Large deletions (1.89Mb, 1.4Mb and 1.8Mb, respectively) were found on chromosome 17q12 of 3 children (2 boys and 1 girl), and the deletions were all de novo variations. All three patients had renal cysts, hyperuricemia and elevated alkaline phosphatase. Two patients had unilateral renal dysplasia and proteinuria. Two patients had hypomagnesemia, 2 had hypercholesterolemia, 2 had diabetes mellitus, and 1 had elevated liver enzymes. Conclusions Chromosome 17q12 microdeletion syndrome is a rare genetic disorder affecting multiple organ systems, mainly manifesting as renal cysts and dysplasia, as well as metabolic and endocrine abnormalities such as diabetes, hyperuricemia, and hypercholesterolemia.
    Literature Review
    Immune genetics of coronary artery injury pathogenesis in Kawasaki disease
    Reviewer: QIU Jiayun, Reviser: ZHOU Guoping
    Journal of Clinical Pediatrics. 2023, 41(1):  66-72.  doi:10.12372/jcp.2023.21e1773
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    Kawasaki disease (KD) is a kind of common diseases in children mainly characterized by systemic vasculitis. The most serious complication of KD is cardiovascular disease involving coronary arteries. Even after treatment, some children still have sequelae such as coronary aneurysms. KD has become one of the most common causes of acquired heart disease in children. At present, the pathogenesis of KD coronary artery injury is not clear. From the perspective of immunogenetics, the latest research results and progress of coronary artery injury mechanism of KD are reviewed in the article.

    Continuing Medical Education
    The classification and diagnose approach on childhood glomerulonephritis
    ZHANG Hongwen
    Journal of Clinical Pediatrics. 2023, 41(1):  73-76.  doi:10.12372/jcp.2023.21e1455
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    Glomerulonephritis is one of the most common renal diseases in children. There are still some confusions and doubts on the definition, classification (etiology, course and pathology) and diagnosis of childhood glomerulonephritis, especially the etiology and pathological classification, which are often confused with nephrotic syndrome. In this paper, the classification of childhood glomerulonephritis is expounded, and it is especially suggested that the diagnostic procedure of children glomerulonephritis should follow the symptoms, course of disease, etiology, primary disease, pathology and renal function diagnosis, which can be used for reference and discussion by clinicians.