Journal of Clinical Pediatrics

Diagnosis and treatment strategy of childhood-onset systemic lupus erythematosus

ZHOU Wei

Journal of Clinical Pediatrics. 2022, 40(10): 721-725. doi:10.12372/jcp.2022.22e0849

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Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune disease characterized by multiple organ involvement, a large number of autoantibodies and low complement. The affected organs are prone to irreversible damage, which threatens the lives of children. This article introduces the clinical characteristics of cSLE, severe lupus and monogenic lupus, and summarizes the diagnosis methods, disease activity assessment, treat-to-target, treatment principles and treatment schemes for different organ involvement of cSLE, and puts forward the treatment strategy of combination of traditional treatment and biological drugs, so as to better target the pathogenesis of cSLE, fully treat and control disease activity as soon as possible, reduce disease recurrence and side effects of traditional drugs, and provide reference for the continuous improvement of diagnosis and treatment of cSLE.

Pulmonary manifestation associated with rheumatic diseases in children

CUI Jieyuan

Journal of Clinical Pediatrics. 2022, 40(10): 726-732. doi:10.12372/jcp.2022.22e0923

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Childhood rheumatic diseases are a group of autoimmune diseases mainly manifested by multi-system damage. Pulmonary involvement is rare in pediatric rheumatic diseases. The symptoms of pulmonary involvement are often insidious, and there may be no obvious clinical manifestations or only mild symptoms in the early stage, leading to the diagnostic difficulties, which is one of the main factors affecting the mortality of children with rheumatic diseases. Therefore, understanding the specific manifestations of pulmonary involvement in different children with rheumatic diseases, early screening, early identification and early intervention for high-risk children can better improve the prognosis and life quality of children.

The application of anti-neutrophil cytoplasmic antibodies tests in pediatric diseases

KANG Yulin

Journal of Clinical Pediatrics. 2022, 40(10): 733-738. doi:10.12372/jcp.2022.22e0962

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Anti-neutrophil cytoplasmic antibodies (ANCA) are a class of autoantibodies that target the cytoplasmic components of neutrophils and monocytes. ANCA includes cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA) and atypical ANCA (a-ANCA). The target antigens of c-ANCA and p-ANCA are proteinase 3 and myeloperoxidase, respectively. At present, the ANCA detection assay mainly includes indirect immunofluorescence, enzyme-linked immunosorbent assay, chemiluminescent immunoassays and fluorescent-enzyme immunoassays. ANCA associated vasculitis (AAV) is a necrotizing vasculitis mainly involving small and medium sized arteries with pauci-immune deposits. The incidence of AAV is low in children, but the onset of the disease is urgent, the progression is rapid, the clinical manifestations are diverse, and the disease is easy to relapse after drug withdrawal. ANCA has become an important marker for the diagnosis of granulomatous vasculitis, microscopic vasculitis and eosinophilic granulomatous vasculitis. ANCA detection is helpful for the early diagnosis and treatment of AAV, thus improving the prognosis of the disease.

Clinical significance of perinuclear anti-neutrophil cytoplasmic antibody in children with systemic lupus erythematosus

HE Ping, SHEN Jia, XU Dan, WANG Ziyan

Journal of Clinical Pediatrics. 2022, 40(10): 739-744. doi:10.12372/jcp.2022.21e1211

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Objective To investigate the clinical significance of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) in children with systemic lupus erythematosus. Methods The clinical data of SLE children treated from January 2016 to July 2021 were retrospectively analyzed. The autoantibody related indexes, immune function, renal function and renal pathology of pANCA positive and negative children were analyzed. Results A total of 191 SLE children (160 girls and 31 boys) were enrolled, and the average age was (12.0±3.5) years. There were 81 children in pANCA positive group, among whom 26 were formaldehyde-resistant pANCA positive and 55 were formaldehyde-sensitive pANCA positive. Anti-myeloperoxidase (MPO) antibody was positive in 29 children, accounting for 35.8% of pANCA positive children. Compared with pANCA negative group, children in pANCA positive group had higher ESR, lower Hb, and higher positive rates of antinuclear antibody, anti-double-stranded DNA (dsDNA) antibody, anti-Sjögren's-syndrome-related antigen A (SSA) autoantibody, anticentromere protein B (CENP-B) antibody, anti-histone antibody, anti-ribosome ribonucleoprotein (rRNP) antibody, anti-nucleosome antibody and anti-Ro-52 antibody. The differences were statistically significant (P<0.05). Compared with pANCA negative group, pANCA positive group had lower levels of complement C3 and C4, higher levels of urinary creatinine (UCr), urinary immunoglobulin G/UCr, α1-microglobulin/UCr, β1-microglobulin/UCr and urinary microalbumin/UCr, and higher proportion of urinary occult blood and urinary protein, and the differences were statistically significant (P<0.05). A total of 112 children with SLE underwent renal biopsy. Compared with the pANCA negative group, the proportion of mesangial hyperplasia and the deposition proportion of IgG, IgM, IgA, C1q and C3 in glomerular mesangium and capillary loop was higher in the pANCA positive group, and the differences were statistically significant (P<0.05). Conclusions In children with SLE, pANCA positive children are more likely to have renal injury and more severe symptoms.

Clinical effect of belizumab on 17 children with systemic lupus erythematosus

GAO Yutong, HE Xiaoliang, CHEN Denghuan, HANG Shouwei, CHEN Yuqing

Journal of Clinical Pediatrics. 2022, 40(10): 745-749. doi:10.12372/jcp.2022.22e0328

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Objective To investigate the efficacy and safety of belizumab in the treatment of children with systemic lupus erythematosus (SLE). Methods The clinical data of SLE children who completed 28-week treatment with beliumab from January 1, 2020 to December 31, 2021 were retrospectively analyzed. Results A total of 17 children (5 boys and 12 girls) were enrolled, and the average age was (12.1±2.3) years. The median disease duration from onset to targeted drug therapy was 5.0 (1.0-22.0) months. Compared with the pre-treatment group, the proportion of rash, fever, lupus nephritis and blood system damage decreased, the CD3⁺ and CD4⁺ cell counts increased, the CD19⁺ cell counts decreased, the positive rate of anti-ds-DNA decreased, and the dosage of glucocorticoids decreased in the post-treatment group, and the differences were statistically significant (P<0.05). There was significant difference in the systemic lupus erythematosus disease activity index (SLEDAI-2000) at different time points (weeks 0, 2, 4, 8, 12, 16, 20, 24 and 28) after beliumab treatment (P<0.01), and the score showed a downward trend. Among the 17 SLE children, 5 (29.4%) achieved lupus low disease activity state (LLDAS) and 3 (17.6%) achieved clinical remission by the end of observation (week 28). There was no complication of serious infection and no allergic reaction to belizumab. Conclusions Beliumab combined with traditional drugs in the treatment of SLE may help children more easily achieve LLDAS and clinical remission without severe adverse effects.

Clinical analysis of fifteen patients with systemic juvenile idiopathic arthritis associated lung disease

QIU Lingzhi, ZHANG Yayuan, MA Le, FAN Zhidan, HUANG Na, YU Haiguo

Journal of Clinical Pediatrics. 2022, 40(10): 750-754. doi:10.12372/jcp.2022.22e0104

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Objective To investigate the clinical features, imaging manifestations, treatment response and prognosis of children with systemic juvenile idiopathic arthritis associated lung disease (sJIA-LD). Methods The clinical data of 15 children with sJIA-LD were retrospectively analyzed, and the clinical characteristics, laboratory parameters, imaging changes and treatment prognosis were summarized. Results There were 7 boys and 8 girls in current study and the average age was 7 years. The common clinical symptoms were fever (15 cases), joint swelling and pain (12 cases), cough (12 cases), rash (11 cases), lymph node enlargement (9 cases), serositis (8 cases), hepatosplenomegaly (8 cases), shortness of breath (7 cases), chest pain (2 cases) and abdominal pain (1 case). Seven patients were complicated with macrophage activation syndrome (MAS). All the 15 children underwent chest high-resolution CT examination, and the results showed strip or patchy shadow in 15 children, consolidation shadow in 3 children, mesh shadow in 2 children, nodular shadow, calcification shadow and atelectasis in 1 child each. The commonest lesion site was the left lower lobe of the lung (13/15). The imaging findings revealed 9 cases of organizing pneumonia, 5 cases of non-specific interstitial pneumonia, and 1 case of usual interstitial pneumonia. All the 13 patients were treated with non-steroid anti-inflammatory drug and glucocorticoids. In the diagnosis of sJIA-LD, 8 cases were treated with slow-acting antirheumatic drugs, including 3 cases of cyclosporine, 4 cases of methotrexate and 1 case of cyclophosphamide. Eight children were treated with tocilizumab. The condition of 12 children improved, and chest high-resolution CT showed improvement. One patient had lung disease progression and eventually died. Conclusion The early clinical symptoms of sJIA-LD are mild but the mortality is high. The high-resolution CT is helpful for early diagnosis.

Application value of spontaneous breathing trial in the extubation of premature infants

LI Zhe, ZHU Xiaobo, XUE Jiang

Journal of Clinical Pediatrics. 2022, 40(10): 755-759. doi:10.12372/jcp.2022.21e1563

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Objective To investigate the clinical application value of spontaneous breathing trial (SBT) in the extubation of premature infants requiring invasive mechanical ventilation. Methods A total of 117 preterm infants requiring invasive mechanical ventilation were randomly divided into two groups. The children in SBT group (n=57) were extubated after SBT, and the children in clinical judgment group (n=60) were extubated based on the clinical experience. The reintubation rate within 72 hours after weaning (failure rate of weaning), mechanical ventilation duration and total hospital stay were compared between the two groups. Results The median gestational age and birth weight of 117 children (60 boys and 57 girls) were 27.7 (26.3-30.1) weeks and 1000.0 (825.0-1300.0) g. In SBT group, 57 children underwent 113 SBT. The failure rate of weaning in the SBT group was significantly lower than that in the clinical judgment group (P<0.05). There were no significant differences in mechanical ventilation duration and total hospital stay between the two groups (P>0.05). Conclusions SBT can effectively reduce the failure rate of extubation in preterm infants without increasing the duration of mechanical ventilation, and it may be used to predict successful extubation in preterm infants.

Clinical analysis of neonates affected by maternal chronic myeloid leukemia

DING Jing, XIAO Yihan, XUE Yujuan, FU Jie, LIU Jie, QIN Jiong, ZENG Chaomei

Journal of Clinical Pediatrics. 2022, 40(10): 760-764. doi:10.12372/jcp.2022.22e0782

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Objective To retrospectively analyze the influence of chronic myeloid leukemia (CML) on neonates. Methods From January 2010 to January 2022, 41 pregnant women with CML and 36 neonates delivered by them in Peking University People's Hospital were enrolled in this study. Thirty-six neonates born by age-matched healthy pregnant women during the same period were used as controls. The effect of chronic myelogenous leukemia on neonates was analyzed. Results The survival rate of neonates with CML mothers was 100%. Compared with neonates with healthy mothers, the incidence of prematurity (22.2% vs 5.6%, P<0.05), low birth weight (19.4% vs 0, P<0.05) and referral to neonatal ward (30.6% vs 5.6%, P<0.05) was higher in the neonates with CML mothers. Neonates whose mothers were exposed to the drug during pregnancy were compared with neonates whose mothers were not exposed, and there were no differences in the incidence of congenital malformation (4.8% vs 6.7%, P>0.05), prematurity (23.8% vs 20%, P>0.05), small for gestational age (14.3% vs 6.7%, P>0.05) and perinatal asphyxia (28.6% vs 13.3%, P>0.05) between the two groups. The average length of hospital stay was (9.6±4.5) days in 11 neonates with CML mothers who were transferred to neonatal ward. One of them suffered from thrombocytopenia, but none suffered from leukopenia or anemia. Biochemical examination showed abnormal elevation of ALT in 1 child and AST in 2 children. Conclusion When mothers with CML receive appropriate treatment, the vast majority of their newborns have a good prognosis.

Clinical characteristics and influencing factors of SARS-CoV-2 Omicron variant infection in children

GUI Mingzhu, LIU Yingli, DING Afeng, XIE Xiaotian

Journal of Clinical Pediatrics. 2022, 40(10): 765-769. doi:10.12372/jcp.2022.22e0848

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Objective To explore the clinical characteristics and related factors of SARS-CoV-2 Omicron variant infection in children. Methods The clinical manifestations, laboratory tests, pulmonary CT imaging, nucleic acid detection, clinical typing, vaccination and therapeutic efficacy of 94 children with Omicron infection were analyzed and summarized. Results A total of 94 children were enrolled, including 51 boys (54.3%) and 43 girls, aged from 9 days to 14 years (mean, 4.5±3.3 years), 33 of whom had been vaccinated. All children had a history of close contact with Omicron infected patients. Asymptomatic and mild cases accounted for 76.6%. The mean value of peripheral blood absolute lymphocyte count in pneumonia group was 2.66×10⁹/L, which was significantly lower than that (4.18×10⁹/L) in asymptomatic/mild group (P=0.024). Compared with non-vaccinated group, the proportion of asymptomatic and mild cases was significantly higher (90.9% vs 68.9%, P=0.013), the number of children with fever was lower (42.4% vs 70.5%, P=0.008), and the average length of hospital stay was significantly shorter (P=0.026) in vaccinated group. The C_t values of ORF1ab and N gene in vaccinated group were significantly higher than those in non-vaccinated group (P<0.05). Individualized syndrome differentiation and treatment was carried out by adding or subtracting classic prescription of traditional Chinese medicine decoction and empiric antibiotics (cephalosporins or macrolides) were administered in combination. All patients recovered, and the average hospital stay was (8.5±3.8) days. One severe case was controlled in time by comprehensive therapy including active anti-secondary infection, anticoagulant drugs and short course glucocorticoids. Conclusions Children are generally susceptible to the Omicron strains, and vaccination helps to improve specific immunity. Therefore, strengthening isolation and vaccination are effective measures to prevent Omicron infection in children.

Expert consensus on the treatment and rehabilitation management of chronic airway inflammation-related diseases in children

Journal of Clinical Pediatrics. 2022, 40(10): 770-781. doi:10.12372/jcp.2022.22e1089

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Complex regional pain syndrome in children: a case report

HUANG Hua, DING Fei, XU Xuemei, JIN Yanliang

Journal of Clinical Pediatrics. 2022, 40(10): 782-786. doi:10.12372/jcp.2022.21e1184

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Complex regional pain syndrome (CRPS) is an amplified motor and sensory pain syndrome, which is relatively rare in children. A 9.9-year-old boy presented with abdominal pain at the beginning, and then he was admitted to our hospital because of sever limb pain. After admission, the examination was completed to exclude infection, tumor, connective tissue diseases and other factors. According to the patient’s obvious paroxysm pain in the peripheral limbs, significant decrease of the skin temperature during the pain and clammy of skin, CRPS was considered. Sertraline and other oral medication were prescribed. The pain symptoms were improved after the medication. This case is the first reported in China. Mastering the clinical characteristics and standardized management of CRPS plays an important role in relieving pain, restoring limb function and avoiding long-term dysfunction.

Application of biological agents in idiopathic nephrotic syndrome

WANG Qianhui, LIU Fei, FU Haidong, MAO Jianhua

Journal of Clinical Pediatrics. 2022, 40(10): 787-794. doi:10.12372/jcp.2022.21e1440

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Idiopathic nephrotic syndrome (INS) is the commonest glomerular disease in children. Refractory nephrotic syndromes such as frequent relapses, steroid dependence and steroid resistance are still the current difficulties in clinical INS treatment. In recent years, it has been reported that rituximab (RTX) and other biological agents have been used in the treatment of refractory nephrotic syndrome and have achieved good results. This article reviews the application progress of RTX and other biological agents in INS.

SARS-CoV-2 Omicron variant infection in children: clinical manifestations and biological basis

XU Yanwen, WANG Qun, XIANG Linjuan, HUANG Lisu

Journal of Clinical Pediatrics. 2022, 40(10): 795-800. doi:10.12372/jcp.2022.22e0783

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With the continuous evolution of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), many variants have emerged. A new variant, Omicron, has become the dominant Covid strain globally. The emergence of Omicron not only led to a significant increase in the number of cases in children, but also brought some special symptoms to pediatric patients. For example, Omicron mainly affects the upper respiratory tract, with more acute laryngitis and less pneumonia. These clinical features have biological basis, such as high variation of viral spike protein, increased affinity with host receptors, transition of invasion pathway of host cells, and weakened syncytial formation ability, which lead to significant changes in infectivity, transmissibility, clinical phenotype, pathogenicity and immune evasion capabilities of Omicron. And this brings about the difference in prevention, treatment and prognosis. This article reviews the clinical manifestations and biological basis of Omicron infection in children.

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