Table of Content

15 October 2024 Volume 42 Issue 10

  

Standard·Protocol·Guideline

Interpretation of Expert Consensus on Standardization for Pediatric Nutrition Outclinic

PAN Liya, HONG Li

Journal of Clinical Pediatrics. 2024, 42(10):  827-832.  doi:10.12372/jcp.2024.24e0964

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Expert Review

The current status and prospects of fetal cardiac intervention for congenital heart diseases

CHEN Sun

Journal of Clinical Pediatrics. 2024, 42(10):  833-836.  doi:10.12372/jcp.2024.24e0967

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Fetal cardiac intervention (FCI) has evolved since its inception in 1991. With the improvement in technical success rates, reduction in complications, and enhancement of fetal outcomes, this technique has gradually gained recognition and promotion. The establishment of the International Fetal Cardiac Intervention Registry has facilitated international exchange and technological development in this field, with over 400 clinical cases reported worldwide. In China, several medical institutions in Guangdong, Shanghai, Qingdao, Chongqing, and other regions have initiated in-utero cardiac intervention treatments, although the number of cases remains limited. This article summarized the types of fetal in-utero cardiac interventions, the procedural process, surgical complications, indications for treatment, and treatment outcomes, while also highlighting current challenges and future prospects of the technology.

The current status and progress of diagnosis and treatment of glycogen storage cardiomyopathy

FU Lijun, QIAO Yuhui

Journal of Clinical Pediatrics. 2024, 42(10):  837-842.  doi:10.12372/jcp.2024.24e0922

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Glycogen storage cardiomyopathy is a class of cardiomyopathies caused by the excessive accumulation of glycogen in myocardial cells due to defects in glycogen metabolism. The most common types include glycogen storage disease type Ⅱ (Pompe disease), Danon disease, and PRKAG2 cardiac syndrome. These conditions are important etiology of hypertrophic cardiomyopathy in children. The main symptoms of glycogen storage cardiomyopathy include myocardial hypertrophy, arrhythmias, and heart failure. In severe cases, it can lead to early death. Early recognition and diagnosis, along with appropriate intervention, have the potential to improve symptoms of cardiomyopathy and enhance the quality of life for patients. Therefore, increasing the awareness of glycogen storage cardiomyopathies among clinical physicians is of significant importance.

Original Article

Risk factors of cerebral oxygen desaturation events after cardiopulmonary bypass in children with congenital heart disease under 1 year old

WANG Siyuan, CHEN Yu, SUN Menglian, HE Xiaomin, HUANG Jianhu, SHEN Nanping

Journal of Clinical Pediatrics. 2024, 42(10):  843-848.  doi:10.12372/jcp.2024.24e0252

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Objective To investigate the incidence of cerebral oxygen desaturation events (CDEs) and its related risk factors in infants under 1 year old with congenital heart disease (CHD) after surgery. Methods The clinical data of infants under 1 year old with CHD who underwent cardiopulmonary bypass surgery between January and June 2023 were retrospectively analyzed. According to whether CDEs occurred after surgery, the patients were divided into CDEs group and non-CDEs group. The clinical characteristics of the two groups were compared, and the influencing factors related to CDEs occurrence were analyzed. Results Among 397 infants who underwent CHD surgery, 67 infants (16.9%) developed CDEs after surgery, including 39 boys and 28 girls, with a median age of 4.0 (3.1-5.6) months. There were 330 patients (83.1%) without CDEs, 177 boys and 153 girls, with a median age of 4.5 (3.2-7.7) months. Binary logistic regression analysis showed that higher postoperative pulse oxygen saturation (SpO₂) and higher intraoperative hematocrit (Hct) were independent protective factors for CDEs, while prolonged cardiopulmonary bypass time and moderate to deep hypothermic circulatory arrest were independent risk factors for CDEs (P<0.05). ROC curve analysis showed that postoperative SpO₂, intraoperative Hct, and cardiopulmonary bypass time all had certain predictive value for the occurrence of CDEs (AUC>0.6, P<0.05). Conclusions The incidence of postoperative CDEs in CHD infants under 1 year old is higher. Postoperative SpO₂, intraoperative Hct, duration of cardiopulmonary bypass and cardiopulmonary bypass regimen may predict the incidence of postoperative CDEs in CHD patients in this age group.

Ultrasonic classification diagnosis of Kawasaki disease complicated with coronary aneurysms based on deep learning

DANG Yan, ZHANG Jing, GAO Yan, HUANG Guoying, MA Xiaojing

Journal of Clinical Pediatrics. 2024, 42(10):  849-856.  doi:10.12372/jcp.2024.23e0717

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Objective To investigate the feasibility of ultrasonic classification diagnosis based on deep learning for Kawasaki disease complicated with coronary aneurysms. Methods Echocardiography images of Kawasaki patients diagnosed in the Pediatric Hospital of Fudan University were collected. The parasternal short axis sections were selected and two-dimensional images without color Doppler imaging were included. In addition, the cases with missing images, poor image quality and imperfect images were eliminated. The collected dataset contains 664 images of coronary aneurysms and 664 images of normal coronary arteries. The collected images are used for classification diagnosis based on four classical deep neural networks including AlexNet, LeNet, VggNet19 and ResNet18. The dataset was divided into a training set of 1000 images, a validation set of 164 images, and a test set of 164 images, accounting for approximately 80%, 10%, and 10% of the total image count, respectively. Results The AlexNet based deep neural network has the best results for ultrasonic image classification of Kawasaki disease complicated with coronary aneurysms and the diagnostic accuracy can reach 98%. In addition, this depth model has obvious advantages in the parameter number and training efficiency. Conclusion The ultrasonic classification diagnosis of Kawasaki disease complicated with coronary aneurysms based on deep convolutional neural network is feasible.

Clinical characteristics and follow-up of catecholaminergic polymorphic ventricular tachycardia associated with RYR2 gene variation in children

WANG Yefeng, ZENG Min, XIAO Yunbin, WANG Xiang, CHEN Zhi

Journal of Clinical Pediatrics. 2024, 42(10):  857-862.  doi:10.12372/jcp.2024.23e0983

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Objective To investigate the clinical manifestations, treatment and follow-up of RYR2 gene variation-related catecholaminergic polymorphic ventricular tachycardia (CPVT). Methods The clinical data of CPVT children admitted from January 2017 to January 2023 were retrospectively analyzed, and the course of treatment and follow-up results were summarized. Results A total of 6 children (4 boys and 2 girls) with CPVT were admitted, and the mean age of the patients was (3.5±0.5) years old when the first symptoms appeared. The median time from first symptom onset to diagnosis was 1.5 (0.1-5.9) years. The most common clinical manifestation was syncope, with exercise and emotions being the main triggers. All 6 children had de novo missense mutations in the RYR2 gene identified through whole-exome sequencing. In dynamic electrocardiography, atrial arrhythmias and sinoatrial node dysfunction were commonly observed in younger children. Four patients underwent exercise stress testing, with 2 experiencing bidirectional ventricular premature contractions and 2 experiencing bidirectional ventricular premature contractions and polymorphic ventricular tachycardia. Initial treatment involved oral propranolol or metoprolol. If arrhythmias persisted, flecainide or propafenone was added as adjunctive therapy. Two patients received permanent cardiac pacemaker treatment (VVI). The mean follow-up time was (24.3±3.7) months, and all patients survived. During the follow-up period, 3 children had occasional syncope, 1 had intermittent palpitation, and 2 had no discomfort. Conclusions CPVT associated with RYR2 gene variations in children can present with various clinical manifestations. Atrial arrhythmias combined with sinoatrial node dysfunction are commonly observed in younger children. The combination of pharmacological therapy and cardiac pacemaker treatment yields favorable treatment outcomes.

Study on the influence factors for hemodynamically significant patent ductus arteriosus in the early life after birth of very preterm and extremely preterm infants

WANG Shanshan, ZHAO Pu, HUANG Liping, HU Xuefeng, LI Jing

Journal of Clinical Pediatrics. 2024, 42(10):  863-867.  doi:10.12372/jcp.2024.23e1120

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Objective This study aimed to identify influence factors of hemodynamically significant patent ductus arteriosus (hsPDA) in very preterm and extremely preterm infants during the early postnatal period. Methods A retrospective analysis of 172 very preterm and extremely preterm infants were conducted from November 2016 to March 2022. Infants were categorized into a PDA closure group (n=83) and a PDA persistence group (n=89), based on the PDA diameter approximately two weeks after birth. Clinical data, including obstetric factors and the infants' conditions at birth, treatment, and complications, were analyzed using univariate and logistic regression methods. Results Univariate analysis indicated that hsPDA was correlated with PDA size, gestational age, birth weight, 1-minute Apgar score, natural conception, prenatal dexamethasone, prenatal magnesium sulfate, maternal gestational diabetes, and premature rupture of membranes (P < 0.05). Logistic regression identified natural conception, prenatal dexamethasone, and magnesium sulfate were helpful for PDA closure, while a large PDA diameter, low birth weight, and maternal gestational diabetes were significant risk factors for hsPDA persistence. Conclusion Clinical predictors can significantly aid in the early recognition of infants at high risk for hsPDA, guiding timely treatment strategies.

Analysis of drug resistance and clinical characteristics of children with drug-resistant tuberculosis in Chongqing

LONG Xiaoru, YANG Ruling, ZHANG Zhenzhen, XU Hongmei, ZHAO Ruiqiu, ZHENG Gaihuan

Journal of Clinical Pediatrics. 2024, 42(10):  868-875.  doi:10.12372/jcp.2024.24e0778

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Objective Our study was to analyze the drug resistance spectrum, drug resistance sites, and clinical characteristics of children with drug-resistant tuberculosis (DR-TB) in Chongqing. Methods A total of 296 Mycobacterium tuberculosis (MTB) strains were isolated from pediatric patients of Children's Hospital of Chongqing Medical University from January 2014 to December 2022. These isolates were tested for anti-TB drugs with phenotypic drug susceptibility test (DST), and their potential genotypic resistance mutations were identified with whole genome sequencing (WGS). The clinical characteristics of DR-TB patients were analyzed retrospectively. Results According to phenotypic DST, 17.2% (51/296) of the strains were DR-TB, and no new gene mutations associated with drug resistance were detected by WGS. There were 240 patients in the drug-sensitive group and 51 in the drug-resistant group. Compared with the drug-sensitive group, the proportion of female, retreatment, severe tuberculosis, pulmonary tuberculosis combined with extrapulmonary tuberculosis, second-line drug use, treatment failure or death was higher, but the BCG vaccination rate was lower in the drug-resistant group, and the differences were statistically significant (P<0.05). A total of 288 children completed chest CT examination. Compared with the drug-sensitive group, the drug-resistant group involved a higher proportion of both lungs, involved more lobes, more likely to involve the lower right lobe, and had a higher percentage of necrosis, and the differences were all statistically significant (P<0.05). Compared with the drug-sensitive group, the proportion of positive acid-fast staining smear ≥1 times was higher, and the positive rate of T-SPOT.TB was lower in the drug-resistant group, with statistical significance (P<0.05). Conclusions The proportion of DR-TB in children in Chongqing is relatively high, and the gene mutations associated with drug resistance are similar to those in adults. Female, retreatment, and no BCG vaccination may be associated with DR-TB. Children with DR-TB have a wider range of lung involvement, are more likely to experience lung necrosis, and have a higher risk of lung lesions spreading to extra-pulmonary organs and progressing to severe disease, with higher rates of treatment failure or death. The overall positive rate of pathogen detection was higher in DR-TB children, but the positive rate of immunological examination was lower, suggesting that the children may have immune suppression.

Ponderal growth and survival post-chemotherapy of optic pathway glioma in children with diencephalon syndrome

LIU Jingjing, WU Wanshui, DU Shuxu, SUN Yanling, GONG Xiaojun, ZHANG Jin, LI Miao, SUN Liming

Journal of Clinical Pediatrics. 2024, 42(10):  876-880.  doi:10.12372/jcp.2024.23e0869

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Objective To investigate the ponderal growth and survival post-chemotherapy of optic pathway glioma (OPG) in children with diencephalon syndrome (DS). Methods Six OPG patients with DS admitted from May 2018 to December 2020 were analyzed retrospectively. The median age of children was 13.5 (3 - 43) months. The median weight Pre-chemotherapy of children was 6.9(6-8) kg. None of the children gained weight prior to the OPG diagnosis, and all exhibited cachexia and extreme emaciation upon admission. The weight-for-age Z score (WAZ) was below -2 in all six patients pre-chemotherapy. The weight changes were dynamically monitored during chemotherapy, and WAZ was assessed at 1, 3, 6, 9 and 12 months post-chemotherapy. Repeated measures analysis of variance was used to compare the WAZ and survival at different time points after chemotherapy. Results The median follow-up time was 46(29-59) months. Visual acuity did not significantly improve in two children with anopsia after surgery or chemo-radiotherapy. Nystagmus, present in four patients at disease onset, resolved post-chemotherapy and one child had central precocity. After chemotherapy, one patient was lost to follow-up, and three experienced tumor progression, which was managed with maintenance chemotherapy to stabilize the disease. There were no fatalities.The weight of all children increased significantly compared with that prechemotherapy, and WAZ increased significantly with the extension of chemotherapy time (P<0.05). Conclusion In children with DS with OPG, postoperative chemotherapy can improve ocular symptoms and promote weight gain.

Clinical efficacy and prognosis analysis of 60 high-risk neuroblastoma with children in a single center

TANG Wei, CHEN Kailan, NIE Yingming, WU Bin, WU Sha, FANG Zijian, LI Hui

Journal of Clinical Pediatrics. 2024, 42(10):  881-887.  doi:10.12372/jcp.2024.23e0511

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Objective To analyze the clinical characteristics and prognosis factors of high-risk neuroblastoma (NB) in children. Methods A retrospective analysis of clinical data collected from children with high-risk NB from May 2017 to May 2022. Follow-up was conducted until February 1,2023. Prognosis-related factors were analyzed by comparing different clinical characteristics, treatment regimens, and survival status. Results Sixty high-risk NB children were included in this study, consisting of 36 males (60.0%) and 24 females (40.0%). The median age at diagnosis was 45 (8-103) months, median follow-up time was 39 (2-65) months, median time to disease progression/recurrence was 18 (2-62) months; and the median survival time was 41 months.. The 1-year, 2-year and 3-year overall survival (OS) rate were 76.4%, 65.6% and 54.8% respectively. The event-free survival (EFS) rate at 1 year, 2 years, and 3 years were 63.0%, 41.9% and 31.6% respectively. As of February 1, 2023, there were 34 survivors (56.7%) and 26 deaths (43.3%), with 17 of the deaths (65.3%) due to recurrence. Univariate survival analysis showed that pathological type, clinical stage,surgical status at initial diagnosis, bone marrow metastasis status, MYCN amplification, anemia status, NSE level, LDH level, and intracranial metastasis at relapse were adverse prognosis risk for high-risk NB (P<0.05). Cox multivariate analysis showed MYCN amplification, and bone marrow metastases at initial diagnosis were independent prognostic factors for high-risk NB (P=0.017). Conclusion The 3-year OS rate of high-risk NB children was 54.8%, High-risk neuroblastoma has a poor overall prognosis, with recurrence being the main cause of death. MYCN amplification is an independent adverse prognostic factor for high-risk NB.

Clinical analysis of CD19/CD22 CAR-T cell therapy for MLL gene rearrangement-positive refractory/relapsed childhood acute B-lineage lymphoblastic leukemia

YANG Liu, SU Meng, ZHANG Jing, AN Kang, CAI Jiaoyang, QIAN Juan, TANG Yanjing, LI Benshang

Journal of Clinical Pediatrics. 2024, 42(10):  888-894.  doi:10.12372/jcp.2024.24e0711

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Objective To analyze the efficacy and safety of dual-targeted CD19/CD22 chimeric antigen receptor T-cells (CAR-T) in the treatment of refractory/relapsed B-lineage acute lymphoblastic leukemia (B-ALL) in children with MLL gene rearrangement (MLL-r). Methods The clinical data of children with MLL-r positive R/R B-ALL treated with dual-targeted CD19/CD22 CAR-T therapy between October 2019 and November 2021 were retrospectively analyzed. Results A total of 37 children (24 boys and 13 girls) with MLL-r positive R/R B-ALL were included and the median age was 1.2 (0.5-2.6) years at diagnosis, of whom 17 (45.9%) had infantile leukemia. At a median time of 9 (7-13) days after CAR-T cell infusion, 37 patients achieved a complete response rate of 100%. With a median follow-up of 28.2 (11.3 to 30.9) months, the 3-year overall survival rate was 67.6% (95% CI: 52.5 to 82.7%), and the 3-year event-free survival rate was 59.5% (95% CI: 43.6 to 75.4%).Twenty-eight patients (75.7%) underwent allogeneic hematopoietic stem cell transplantation after CAR-T cell therapy, and the median time between CAR-T infusion and transplantation was 83 (61 to 92) days. The 2-year OS for children who received consolidation grafts was 75.0% (95% CI: 58.9 to 91.1%), compared to 44.4% (95% CI 11.9 to 76.9%) for those who did not receive grafts. The difference between the two groups was not statistically significant (P=0.068). A total of 13 patients (35.1%) relapsed, and the median time from cell infusion to recurrence was 156 (86 to 202) days. Among them, 4 cases had double-positive recurrence of CD19 and CD22, 2 cases had double-negative recurrence of CD19 and CD22, 4 cases had CD19-negative recurrence, 1 case had myeloid transformation, and the other 2 cases were unclear. Cytokine release syndrome occurred in 97.3% (36/37) of patients in this study, with 29.7% (11/37) achieved grades 3 to 4. Immune effector cell-associated neurotoxicity syndrome was observed in 5 (13.5%) patients. There were no deaths due to CAR-T comorbidities.Conclusions CD19/CD22 CAR-T cell therapy is effective in inducing rapid remission in MLL-r R/R childhood B-lineage acute lymphoblastic leukemia with tolerable side effects.

Literature Review

Clinical manifestations and progress in diagnosis and treatment of digestive system involvement in children with juvenile systemic lupus erythematosus

JIN Tong, LIU Chang, ZHAO Xue

Journal of Clinical Pediatrics. 2024, 42(10):  895-901.  doi:10.12372/jcp.2024.23e0969

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Digestive system involvement is common in juvenile systemic lupus erythematosus (JSLE). Gastrointestinal involvement occurs in 15% to 60% of children with JSLE, and 2% to 30% of gastrointestinal symptoms are directly attributable to JSLE. The main pathological changes of JSLE are inflammation and vasculitis, and the symptoms are not specific. Clinically, it can be manifested as lupus mesenteric vasculitis, hepatitis and pancreatitis, so early recognition is of great significance. For children with JSLE who present with digestive system symptoms, the cause should be identified based on objective laboratory findings and treated accordingly. Once it is clear that the digestive system is caused by JSLE itself, adequate glucocorticoids should be used intravenously at an early stage, and pulse therapy should be given if necessary. This paper reviews the progress of etiology, pathology, clinical manifestations, diagnosis, differential diagnosis and treatment of JSLE digestive system involvement, and provides suggestions for further improving the diagnosis and treatment norms applicable to JSLE digestive system involvement.

Expert Comment

Diagnosis and treatment of infectious endocarditis in children

CHENG Zhenli, ZHANG Zhenzhen

Journal of Clinical Pediatrics. 2024, 42(10):  902-906.  doi:10.12372/jcp.2024.24e0887

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Infective endocarditis (IE) is an infection originating from the endocardium and/or heart valves. Although the overall incidence of IE in children is relatively low, it carries a high mortality once happened. Timely identification and prompt treatment are crucial to avert adverse clinical outcomes. In recent issue, Prof. Cao et al. reported a case of IE caused by methicillin-resistant Staphylococcus aureus after congenital heart disease operation (J Clin Pediatr Vol.42 No.8 Aug. 2024), which underscored the complexities and challenges involved in diagnosing and managing IE in pediatric patients. Herein, we will further discuss the clinical features, antibiotic options, and optimal timing of surgical intervention for pediatric IE, aiming to enhance clinicians' understanding of this condition.