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06 January 2021, Volume 36 Issue 1 Previous Issue    Next Issue

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Precise diagnosis and treatment of juvenile idiopathic arthritis LI Cai-feng， LI Shi-peng 2021, 36(1): 1-5.  DOI: 10.19538/j.ek2021010601 Abstract ( )   Juvenile idiopathic arthritis（JIA）is a group of highly heterogeneous childhood chronic rheumatic diseases whose etiology and mechanism are not defined. In addition to joint involvement，the disease may also involve the eyes and systemic organs. Due to the high degree of heterogeneity of the disease，a reasonable classification standard is particularly important for precise diagnosis and treatment of the disease and clinical research. For this reason，the International Rheumatology Organization continuously updates the JIA classification standard. With the in-depth research on the pathogenesis of JIA，new biological agents continue to come out，providing us with better choices and more precise diagnosis and treatment of diseases. This article reviews the current diagnosis and treatment of JIA.

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Systemic juvenile idiopathic arthritis accompanied with pulmonary interstitial disease DENG Jiang-hong，LI Cai-feng 2021, 36(1): 6-9.  DOI: 10.19538/j.ek2021010602 Abstract ( )   Systemic juvenile idiopathic arthritis is one of the most common rheumatic diseases in children. Interstitial lung disease is characterized by interstitial and distal air gap remodeling，which leads to abnormal gas exchange. In recent years，there are increasing reports on systemic juvenile idiopathic arthritis combined with interstitial lung disease at home and abroad. The paper summarizes the recent reports on sJIA with interstitial lung disease，and summarizes the clinical manifestations，imaging manifestations，diagnosis as well as treatment for pulmonary interstitial disease in children，in order to have a deeper understanding of this kind of disease，and contribute to the early diagnosis and treatment of pulmonary interstitial disease.

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Advances in diagnosis and treatment of juvenile idiopathic arthritis related to enthesitis and spondyloarthropathies CAO Lan-fang，XU Jin-ping，XUE Hai-yan 2021, 36(1): 9-16.  DOI: 10.19538/j.ek2021010603 Abstract ( )   Juvenile spondyloarthritis（JSpA）is an important group of articular rheumatism in children under 16 years old，which is associated with human leucocyte antigen B27. It is often misdiagnosed with its diverse clinical manifestations of less involvement of the axial skeletons but more hips and peripheral entheses in children than in adults. The rheumatism  associations international（ILAR） published the definition and classification standard（ILAR standard） of juvenile idiopathic arthritis（JIA） in 2004，which covered almost all childhood chronic arthritis. Among them，the sixth category enthesitis-related arthritis（ERA） contains part of JSpA，resulting in some cases of clinical confusion between JSpA and ERA，of which peripheral arthritis associated with attachment points and/or aponeurotic attachment point inflammation were both diagnosed as ERA. Due to this confusion，the international organization for trials in childhood rheumatism（PRINTO） developed the latest classification criteria in 2018，and proposed the classification criteria of ERA/SpA，to distinguish it from other subtypes of JIA. This article describes the latest diagnosis and treatment progress in ERA/SpA-related JIA，which aims to enhance clinicians’ understanding，diagnosis and treatment level of ERA/SpA related JIA and thus improve the prognosis.

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Update on the pathogenesis of systemic juvenile idiopathic arthritis CHEN Tong-xin，ZHANG Chen-xing 2021, 36(1): 16-19.  DOI: 10.19538/j.ek2021010604 Abstract ( )   Juvenile idiopathic arthritis（JIA） is a chronic systemic disease in children with disease onset prior to age 16 and is featured by chronic arthritis，accompanied by other tissue and organ damage，with exclusion of arthritis caused by other diseases. Compared with other types， systemic JIA（sJIA） is characterized by unique clinical features such as remittent fever， skin rash，serous effusions， hepatomegaly， splenomegaly， lymphadenopathy and multiple organ damage. Some children are also at risk of life-threating complications including macrophage activation syndrome（MAS）. In addition，sJIA is also featured by its laboratory parameters，for instance， significantly elevated levels of inflammatory markers［C reactive protein（CRP），erythrocyte sedimentation rate（ESR）］， significantly elevated levels of peripheral blood white cells and neutrophils as well as absence of autoantibodies. Therefore， it is generally believed that the pathogenesis of sJIA is different from other subsets and sJIA represents a distinct category of disease. Nevertheless，the pathogenesis of sJIA still remains elusive. It is a unique category of arthritis， similar to autoinflammatory diseases， which is probably due to disturbed innate immune system induced by certain infection in genetically susceptible individuals.

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Application of biological agents in JIA ZHOU Zhi-xuan，SU Gai-xiu 2021, 36(1): 19-23.  DOI: 10.19538/j.ek2021010605 Abstract ( )   In the past 20 years，the treatment for juvenile idiopathic arthritis（JIA） has been improving constantly. In order to make full use of target therapy in JIA，we need to clarify the clinical application of biological agents. JIA is a heterogeneous disease with different immunological pathogenesis and different target sites. Many biological agents can be used in treating JIA，including TNF-α inhibitors，IL-1 antagonists and IL-6 antagonists which are acting on cytokines，abatacept acting on T lymphocytes，rituximab acting on B lymphocytes，and cellular targets of small-molecule protein kinase inhibitors. In the article，we will introduce the application of these drugs in treating JIA respectively based on the current treatment strategy both in China and abroad.

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Diagnosis and treatment of systemic juvenile idiopathic arthritis combined with macrophage activation syndrome LU Mei-ping， WU Jian-qiang 2021, 36(1): 23-29.  DOI: 10.19538/j.ek2021010606 Abstract ( )   Macrophage activation syndrome（MAS），a hemophagocytic lymphohistiocytosis（HLH） secondary to rheumatic immune diseases，is a life-threatening complication in children. The most common rheumatic disease associated with MAS is systemic juvenile idiopathic arthritis（sJIA-MAS） and death rate is high. Early diagnosis and timely intervention for sJIA-MAS is critical to improve the prognosis. Although there are similar clinical and laboratory features，classification criteria has a little difference between sJIA-MAS and HLH because of their different basic diseases. Glucocorticoids and cyclosporin A are the first line medications for sJIA-MAS. However，the treatment still remains a major challenge. Biologic agents as targeted medicines are gradually proposed as therapeutic options for refractory MAS，or even as first line medicines. Here we will focus on the clinical and laboratory features，biomarkers，classification criteria and treatment advances of sJIA-MAS.

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Diagnosis and treatment of uveitis related to juvenile idiopathic arthritis SUN Li 2021, 36(1): 29-32.  DOI: 10.19538/j.ek2021010607 Abstract ( )   Juvenile idiopathic arthritis-associated uveitis（JIA-U）is a common extra-articular complication of JIA. Anterior uveitis is common，i.e.iridocyclitis，with hidden symptoms and risk of blindness. In order to maintain the best visual acuity and eye health of JIA children，pediatric rheumatologists should strengthen the education of patients，communicate with ophthalmologists closely，and make efforts in early screening，regular monitoring and appropriate treatment.（1）Screening：according to the risk assessment of different individuals with JIA，the frequency of ophthalmic screening was determined. （2）Monitoring：it is suggested that regular ophthalmic monitoring should be carried out for JIA children with uveitis，and the interval should be based on the results of eye examination and treatment plan. （3）Treatment：A. Topical treatment：glucocorticoids should be used as initial treatment to control inflammation； B. Systemic therapy：methotrexate and monoclonal antibody tumor necrosis factor antagonists are recommended for systemic treatment of uveitis. For patients with local treatment failure or dependence，it is recommended to add traditional and/or biological agents DMARDs in time to control the condition of uveitis children at risk of vision loss as soon as possible to avoid blindness.

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Effect of whole body vibration on gait speed and gross motor function of pediatric patients with spastic cerebral palsy：A study of 20 cases YAN Shan-zhong*，JIN Juan，CHEN Shi-dong，et al 2021, 36(1): 33-37.  DOI: 10.19538/j.ek2021010608 Abstract ( )   Objective　To explore the effect of whole body vibration on gait speed and gross motor function of pediatric patients with spastic cerebral palsy. Methods　Forty pediatric patients with spastic cerebral palsy were recruited from the Out-patient Department of Rehabilitation，Shanghai General Hospital between January 2019 and December 2019，who were randomly distributed to experimental group under whole body vibration combined with conventional rehabilitation，and control group with conventional rehabilitation alone，20 patients in each group.The assessments were done before and after 12-week intervention via score on Dimension D（standing） and Dimension E（walking，running，jumping） in GMFM-88，the score on stationary，locomotion，object manipulation in PDMS-2，and 10-Meter Walking Test，in order to analyze the improvement in the condition of gait and gross motor function. Results　The level of GMFCS，score on Dimension D（standing）and Dimension E（walking，running，jumping） in GMFM-88，stationary，locomotion and object manipulation in PDMS-2，and 10-Meter Walking Test were of no significant difference before intervention between the two groups（P＞0.05）. After treatment，scores on Dimension D（standing） and Dimension E（walking，running，jumping） in GMFM-88 were improved in both groups and the experiment group had greater improvement with significant difference compared with control group（P＜0.01）；the same was with the score on stationary and locomotion in PDMS-2（P＜0.01） and 10MWT（P＜0.01）. The score on object manipulation in PDMS-2 were of no statistical difference in each of or between the two groups（P＞0.05）. Conclusion　Whole body vibration combined with conventional rehabilitation can promote the gait and motor function improvement in pediatric patients with spastic cerebral palsy.

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Intestinal pseudo-obstruction in children with systemic lupus erythematosus：A clinical study of 8 cases TANG Xiao-yan，LI Ji，GOU Li-juan，et al 2021, 36(1): 38-41.  DOI: 10.19538/j.ek2021010609 Abstract ( )   Objective　To investigate the clinical characteristics and treatments of systemic lupus erythematosus（SLE） with intestinal pseudo-obstruction（IPO） in children. Methods　We retrospectively analyzed the clinical data，summarizing the characteristics of the disease，the diagnosis，the treatments and prognosis of 8 cases which were diagnosed as SLE with IPO under16 years old at Peking Union Medical College Hospital from January 2010 to December 2018. Results　All of 8 cases，2 males and 6 females，were included in the study. The mean age of onset was （13.6±1.7） years old（11-15 years）. All of 8 children had abdominal distension，nausea，vomiting，and abdominal pain，and 1 case had diarrhea. Gas-liquid plane can be found in conventional plain abdominal X-ray in 8 children. Six patients had dilated small bowel loops，while 2 with dilated large bowel. All children had elevated anti-nuclear antibody titers. Anti-RNP antibodies were positive in 4 cases，3 positive in anti-SSA/Ro52. All IPO cases were in the active phase of SLE. In 4 cases，IPO was the initial manifestation of SLE，and one patient received abdominal surgery for being suspected as acute abdominal at the local hospital. Four children had hydronephresis and ureterectasis at the same time. All cases received glucocorticoid and cyclophosphamide with good response，and no death was reported. Conclusion　IPO is a rare but severe complication of SLE. It is very important to make timely diagnosis，and the combination treatment with prednisone and cyclophosphamide is very important to better prognosis. Early diagnosis can reduce unnecessary surgery.

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Clinical analysis of intestinal lymphangiectasia in 18 children WANG Hong-li，GENG Lan-lan，CHEN Pei-yu，et al 2021, 36(1): 42-46.  DOI: 10.19538/j.ek2021010610 Abstract ( )   Objective　To investigate the clinical manifestations，diagnosis，treatment and prognosis of intestinal lymphangiectasia（IL） in order to improve the levels of diagnosis and treatment of IL in children. Methods　The clinical data，laboratory examinations，gastroenteroscopic findings，histopathological examinations and other results of 18 children with IL were analyzed retrospectively，who were diagnosed in the Department of Gastroenterology of Guangzhou Women and Children’s Medical Center from January 2010 to December 2019. Compare the related indicators between the children with onset age within 1 year old and those over 1 year old. Results　Among 18 children with IL，there were 8 boys and 10 girls；there were 13（72.2%） children with the onset age younger than 1 year old，while 5（27.8%） children with onset over 1 year old；the main clinical manifestations of IL were edema（17，94.4%），abdominal distention（16，88.8%），diarrhea（15，83.8%），ascites（16，88.8%） and being complicated with infection（14，77.7%）. There was no significant difference between the two groups in gender，lymphocyte absolute count，serum albumin，globulin，IgG or positive histopathology（P＞0.0 5）. However，there was significant difference between the two groups in terms of infection rate（χ2＝4.11，P＜0.05）. One case（5.6%） was lost in follow-up，and 15 cases（83.3%） had good prognosis. The diet rich in medium-chain triglyceride（MCT） was effective. Two cases needed supplementation with the serum albumin irregularly. Conclusion　The main clinical manifestations of IL are edema，abdominal distention and diarrhea，which is likely to be combined with ascites and infection；the younger the onset age，the more susceptible to be infected and the higher the risk of critical illness. The diet rich in MCT is effective and should be in long-term maintenance；a small number of children need irregular supplementation with the serum albumin. Long term follow-up is needed for future prognosis.

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Clinical analysis of Henoch-Schonlein purpura in children with abdominal symptoms as the first manifestation LI Jing*，GAO Cheng-long，WU Jie 2021, 36(1): 47-52.  DOI: 10.19538/j.ek2021010611 Abstract ( )   Objective　To investigate the clinical features of Henoch-Schonlein purpura（HSP） with abdominal symptoms as the first manifestation，and to explore the value of gastroscopy and abdominal CT in its early diagnosis. Methods  　169 hospitalized children with HSP with abdominal pain as the first manifestation were selected as the observation group. 142 patients with rash as the first symptom were selected as the control group. The clinical data were analyzed. Results　The observation group had a lower incidence of prodromal infection and/or positive etiology（P＜0.05），33 cases showed no rash（19.53%）. More children in observation group experienced vomiting（P＜0.05）. The white blood cell count，CRP，platelet count，D-dimer and fecal occult blood positive rate were higher in observation group（P＜0.05），but Albumin，IgA and C3 were lower（P＜0.05）. There was no significant difference in gastrointestinal bleeding，procalcitonin，specific allergen positivity rate，immunoglobulin E，urine protein and erythrocyte elevation（P＞0.05）. There was no difference in positive detection rate between gastroscopy and abdominal CT（P＞0.05）. There was no difference in hormone sensitivity and the incidence of purpura nephritis between two groups（P＞0.05），but recurrence rate was higher in observation group（P＜0.05）. Conclusion　Children with HSP with abdominal pain as the first manifestation are more prone to vomiting，and some children may not have rashes. Their white blood cell count，CRP，platelet count，D-dimer level，positive rate of fecal occult blood，the incidence of hypoalbuminemia and recurrence were higher. There was no significant difference in the positive detection rate of gastroscopy and abdominal CT.

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Value of next-generation sequencing of cerebrospinal fluid in precise diagnosis of tuberculous meningitis：An analysis of 5 cases QIAN Qiao-qiao，LIU Ting，SUN Dan，et al 2021, 36(1): 53-56.  DOI: 10.19538/j.ek2021010612 Abstract ( )

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Sjogren-Larsson syndrome caused by homozygous mutation of ALDH3A2 gene：A report of 1 case and literature review XI Xiao，YANG Jun-feng，LI He-ping，et al 2021, 36(1): 57-59.  DOI: 10.19538/j.ek2021010613 Abstract ( )

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Clinical phenotype, pathology and gene diagnostic analysis of a family with hepatic fibrinogen storage disease by FGG gene mutation ZHANG Hui，OUYANG Wen-xian，YUAN Yuan-hong，et al 2021, 36(1): 60-63.  DOI: 10.19538/j.ek2021010614 Abstract ( )

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Research progress on antibiotic resistance of Helicobacter pylori and management strategies in children REN Xiao-zhou，LI Zhong-yue 2021, 36(1): 64-69.  DOI: 10.19538/j.ek2021010615 Abstract ( )

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Research progress in the relationship between brain-gut peptide and functional dyspepsia in children WANG Dong-wei，WU Jie 2021, 36(1): 70-74.  DOI: 10.19538/j.ek2021010616 Abstract ( )

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Refeeding syndrome in a child with malnutrition with chronic diarrhea and hypophosphatemia as the main manifestations：A report of 1 case CHEN Yan，BAI Hai-tao 2021, 36(1): 75-76.  DOI: 10.19538/j.ek2021010617 Abstract ( )

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Overlapping syndrome of MOG-ab disease and anti-NMDARe：A report of 3 cases YANG Chuan，LI Xiu-juan，ZHONG Min，et al 2021, 36(1): 77-80.  DOI: 10.19538/j.ek2021010618 Abstract ( )