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06 November 2021, Volume 36 Issue 11 Previous Issue    Next Issue

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Accelerating the coordinated development of sub-specialties such as allergy, immunology and rheumatology of pediatric clinical immunology in China ZENG Hua- song 2021, 36(11): 801-803.  DOI: 10.19538/j.ek2021110601 Abstract ( )   Pediatric clinical immunology is an important branch of pediatric medicine in China. It covers pediatric allergic diseases, immunodeficiency diseases, rheumatic immune diseases, inflammatory diseases, vaccination consulting, peripheral organ transplantation immunity, critical diseases and system and organ immunity, infection and immunity,and mucosal immunology, etc. The interdisciplinary frontier discipline involving multiple sub-disciplines of pediatrics has a long history. In recent years, it has been valued and has developed rapidly in China. However, how to promote the coordinated development of various sub-specialties is an important task we are facing at present. This review is about the history, development, and how to speed up the coordinated development of pediatric clinical immunology and sub-specialties in China.

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Application of pharmacogenomics in the treatment of children with rheumatic diseases XU Xiao- lin, WANG Xiao-ling, MAO Hua-wei 2021, 36(11): 804-811.  DOI: 10.19538/j.ek2021110602 Abstract ( )   Rheumatoid immune diseases in children are a kind of chronic disease with a wide population，long disease course and complex pathogenesis，involving multiple organs and systems. Glucocorticoids，non- steroidal anti-inflammatory drugs，immunosuppressants，and biological agents are widely used in the treatment of such diseases. But significant differences in efficacy and adverse reactions are demonstrated in the whole population. In clinical practice，in order to achieve precise treatment for individual patient，the gene polymorphism and the features of growth and development must be considered in addition to the disease status and the pharmacokinetics of the drug. Pharmacogenomics provides a theoretical evidence for the individualized drug treatment for rheumatic diseases in children.

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Mechanisms of autoimmunity in patients with primary immunodeficiency disease and targeted therapies WU Jun-feng, ZHAO Xiao-dong, AN Yun-fei 2021, 36(11): 811-816.  DOI: 10.19538/j.ek2021110603 Abstract ( )   Primary immunodeficiency disease（PID）is a group of disorders in which the number of immune cells is abnormal and/or the function of immune cells is impaired primarily by mutations in a single gene. Nearly 1/4 of PID patients suffered from autoimmune diseases. The clinical manifestations and mechanisms of PID autoimmunity are diverse，so the treatment is challenging. PID with autoimmunity is a negative prognostic factor. This paper summarized the clinical manifestations，pathogenesis，and treatment progress of autoimmunity in PID，aiming to improve the understanding of this disease，which contributes to early diagnosis and treatment，prevents the related terminal organ injury，and improves patients’ outcome.

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Appropriate application of intravenous immunoglobulin in pediatric rheumatic diseases TANG Hong-xia, YIN Wei 2021, 36(11): 816-821.  DOI: 10.19538/j.ek2021110604 Abstract ( )   The clinical manifestations of pediatric rheumatic diseases are various，and the exact pathogenesis is still unknown. However，many studies have proved that these diseases are autoimmune diseases mediated by the activation of the immune system. Currently there are no precise treatments for these diseases. In addition to glucocorticoids，immunosuppressants and biological agents，immunoglobulin has become an increasingly important therapeutic option as a versatile immunomodulator and anti-inflammatory agent，especially for poor response to the first line treatment of rheumatic diseases in children. For the majority of pediatric rheumatic diseases，intravenous immunoglobulin is used “off label” on the basis of double-blind randomized trials or case reports. It is very important to establish appropriate and standardized therapy of intravenous immunoglobulin in pediatric rheumatic diseases.

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Progress and suggestions in the treatment of macrophage activation syndrome associated with rheumatic diseases in children GUO Li, LU Mei-ping 2021, 36(11): 822-828.  DOI: 10.19538/j.ek2021110605 Abstract ( )   Macrophage activation syndrome（MAS） is a severe，potentially life-threatening complication of rheumatic diseases. Early identification and intensive treatment are the keys to successful treatment. However，because of the lack of effective clinical controlled trial studies，there is no unified treatment options for MAS at present. In this paper，by reviewing a large number of domestic and foreign literatures，we recommend that high-dose glucocorticoid（GC） be used as the initial treatment for MAS. Continuing GC monotherapy for more than 2 weeks in patients with persistent high fever or MAS status is inappropriate. Combination therapy with cyclosporin A（CsA） and/or etoposide（VP16） is the first choice，and anakinra（abroad） is recommended as first-line drugs for MAS. Tocilizumab or ruxolitinib may be effective for refractory fever in the patients.

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Key points and advances in the treatment of systemic juvenile idiopathic arthritis LI Xiao-qing, GENG Ling-ling 2021, 36(11): 828-832.  DOI: 10.19538/j.ek2021110606 Abstract ( )   Systemic juvenile idiopathic arthritis（sJIA），in which fever is the prominent manifestation and which is accompanied with or without arthritis and rash，has different clinical phenotype. Until now，there are great differences in treatment of sJIA，inclucling due to lack of clinical evidence-based guidelines in the whole world. This review summarized the treatment points of sJIA，induding both traditional drugs（non-steroidal anti- inflammatory drugs，glucocorticoids，ameliorative antirheumatic drugs） and emerging biologics，and analysed some recommendations on the treatment of sJIA at home and abroad in recent years. In addition，we discussed the problems of drug withdrawal and long-term management of sJIA，aiming to better understand the concept of treat-to-target（T2T） in order to standardize the treatment strategy and improve the clinical prognosis of sJIA.

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New advances in immunotherapy for allergic diseases in children BIAN Sai-nan, WANG Zi-xi, GUAN Kai 2021, 36(11): 833-838.  DOI: 10.19538/j.ek2021110607 Abstract ( )   Allergen- specific immunotherapy（AIT） has effect on both innate and adaptive immune responses. New approaches of AIT for allergic rhinoconjunctivitis and asthma are intralymphatic immunotherapy（ILIT） and epicutaneous immunotherapy（EPIT）. The effectiveness and standard protocols need to be further studied. Generation of hypoallergenic recombinant allergen derivatives and immunogenic peptides，and new adjuvants are the investigating goals in the future. Food oral immunotherapy（OIT） has some effects on patients with food allergy. However，adverse effect especially anaphylaxis is still the problem needing to be solved. Biologics have effects in patients who are not responsive to traditional drugs. AIT in combination with biologics is a novel approach for patients with severe asthma or atopic dermatitis.

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Advances of slow- acting antirheumatic drug for juvenile dermatomyositis LIU Li, SUN Yan- ru, HU Jian 2021, 36(11): 839-842.  DOI: 10.19538/j.ek2021110608 Abstract ( )   Juvenile dermatomyositis is one of the rare rheumatic diseases in children. The common treatment is hormone combined with slow-acting drugs. It is difficult to choose slow-acting drugs，swith drugs and design course of treatment because of the low incidence and lack of large randomized controlled trials. The author summarizes the common slow-acting antirheumatic drugs used in the treatment of juvenile dermatomyositis，and introduces the mechanism of action，dosage，adverse reactions and indications of these drugs，in order to help doctors to select drugs.

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Advances in chronic disease management and Treatment of cSLE and LN JIANG Xin-hui, SHAO Xiao- shan 2021, 36(11): 842-848.  DOI: 10.19538/j.ek2021110609 Abstract ( )   Childhood- onset systemic lupus erythematosus（cSLE） refers to SLE that develops before the age of 18 years. It involves multiple organs and systems throughout the body. Up to 60% of these children will develop Lupus Nephritis（LN），with 19% of whom progresses to end-stage renal disease. Infection and end-stage renal disease are the main causes of death in cSLE of developing countries. As both the disease itself and the treatment affect the physical and psychological growth and development of children，a well-conducted chronic disease management is extremely important for the disease control and long-term survival of children with cSLE and LN. This article summarised the experience in SLE chronic disease management at home and abroad in recent years，and reviewed chronic disease management and treatment progress of cSLE and LN ，with the aim of improving the attainment rate and reducing the occurrence of disease relapse and complications in order to benefit the children.

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Application of Th2 inhibitor in childhood allergic and immune diseases XU Xue- feng, CHEN Xiang- yuan, ZENG Hua-song, et al 2021, 36(11): 849-853.  DOI: 10.19538/j.ek2021110610 Abstract ( )   Based on the role of Th2 cytokines in allergic diseases，Th2 inhibitors have been used in the treatment of childhood asthma and allergic rhinitis. Suplatast tosilate is a new selective Th2 cytokine inhibitor that can selectively inhibit Th cells，reduce the production of inflammatory mediators，and improve the symptoms of allergic diseases. Both the Japanese guidelines for children bronchial asthma and the guidelines for allergic rhinitis recommended the use of suplatast tosilate. Suplatast tosilate is the first Th2 inhibitor approved for marketing in China. The 2020 Chinese guidelines for adult bronchial asthma also recommended that suplatast tosilate be used to treat bronchial asthma，but its application in China is still in the initial stage. This article reviews the applications of suplatast tosilate in children with allergic and immune diseases，aiming to promote its appropriate use in Chinese children.

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JAK inhibitors as small molecule targeted agents in the treatment of children with rheumatic and immune diseases HAO Sheng, HUANG Wen-yan, ZENG Hua-song 2021, 36(11): 853-857.  DOI: 10.19538/j.ek2021110611 Abstract ( )   JAK inhibitors are small molecule targeted agents that inhibit signal transduction pathways associated with inflammatory factors to get anti-inflammatory effects. Currently，many kinds of JAK inhibitors have been used in the treatment of diseases in each system， including rheumatic and immune diseases. As novel targeted synthetic drugs，JAK inhibitors have been used for adults with rheumatoid arthritis，ankylosing spondylitis，ulcerative colitis，dermatomyositis，vasculitis，and other rheumatic disease. However，there is still insufficient clinical evidence in children with rheumatic and immune diseases. JAK inhibitors for juvenile idiopathic arthritis have entered phase 3 clinical trials，and some JAK inhibitors have been reported to be used in the treatment of juvenile dermatomyositis，systemic lupus erythematosus，multiple arteritis. JAK inhibitors are also targeted agents for some autoinflammatory diseases. Therefore，JAK inhibitors have a great prospect in the treatment of rheumatic and immune diseases in children.

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Efficacy and safety analysis of belimumab for 28 weeks in real-world Chinese children with systemic lupus erythematosus：A retrospective multicenter pilot study Multi-center Research Collaboration Group, Pediatric Allergy Immunology Rheumatology Branchthe Asia-Pacific Association of Medicine and Bio-Immunology（PAIRB-APAMBI）, Editorial Committee of Chinese Journal of Practical Pediatrics 2021, 36(11): 858-868.  DOI: 10.19538/j.ek2021110612 Abstract ( )   Objective　To evaluate the efficacy and safety of 28-week use of belimumab in Chinese children with systemic lupus erythematosus（SLE）. Methods　The study was undertaken by a questionnaire completed in 37 Grade 3A hospitals’ Rheumatology and immunology department in China. To evaluate the efficacy，safety and use of Belimumab in the diagnosis of systemic Lupus Erythematosus of children with the age of 5-18 years during September，2019 to February， 2021. At the same time，patients with SLE who had similar baseline of SLEDAI score and were treated with traditional immunosuppressive drug at the same hospital were selected as a control cohort. Result　The average course of disease was 25.00±27.97 months in 169 children who were treated with belimumab. After 28 weeks of treatment，SLEDAI score，ANA and dsDNA positive rates were significantly decreased in the belimumab-treated group and the traditional treatment group，the difference being with no statistical significance. At the observation end point（28w），the amount of oral hormone was significantly decreased in both groups after treatment（P<0.05）；after 28 weeks of treatment，50% of the children in the belimumab group still had kidney injury of varying degrees，but significantly improved compared with before treatment（69.2% vs. 50%，P<0.05），while the children in the conventional treatment group had no significant improvement compared with before treatment（56.3% vs. 43.4%，P>0.05）. The proportion of patients at the endpoint of 28w reaching the status of LLDAS or remission in the belimumab group was 40.5%，of whom 9% were in clinical remission，while the proportion of patients in the conventional group was only 8.8% whe reached the status of LLDAS，of whom 3.5% were in clinical remission，and the proportion of qualified treatment was lower than that of the belimumab treatment group（Logistic regression analysis，P<0.001）. A lower number of infections occurred in the belimumab group than in the conventional treatment group. Conclusion　Targeted therapy of systemic lupus erythematosus in children with belimumab can reduce the dosage of hormone and traditional immunosuppressive drug. It is more likely to achieve LLDAS and clinical remission and belimumab could be the biologic DMARDs. The incidence of adverse events，such as infections，is low with the use of belimumab，and its effects on lupus nephritis are better than conventional drugs.

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Clinical analysis of peritoneal dialysis in the treatment of acute kidney injury in extremely low birth weight infants CAO Guang-na, ZHANG Jing, PIAO Mei-hua, et al 2021, 36(11): 869-873.  DOI: 10.19538/j.ek2021110613 Abstract ( )   Objective　To summarize the problems，causes and treatment of acute kidney injury（AKI） in extremely low birth weight infants（ELBWI） treated by peritoneal dialysis（PD），so as to improve the treatment ability of ELBWI with AKI. Methods　From May 2011 to December 2019，7 cases of ELBWI with acute kidney injury were treated by peritoneal dialysis in the Neonatal Intensive Care Unit（NICU），Peking University Third Hospital. The clinical data were analyzed retrospectively. Results　Among the 7 cases，the gestational age was（25-28）weeks，the birth weight was 780-990 g，and the duration of continuous dialysis was 2-18 d. There were 3 cases of catheter leakage and 2 cases of peritonitis. Two cases were cured，3 cases were improved，and 2 cases died of hyperkalemia and arrhythmia. The clinical complications of edema，increased serum creatinine，increased urea nitrogen，and potassium were improved in 5 cases after PD. Conclusion　Peritoneal dialysis is a safe，effective and feasible therapy for ELBWI with AKI，which increases the survival rate of ELBWI.

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Application of medical elastic mesh cap and ear print material in the prevention and treatment of noninvasive orthopedic complications of congenital auricle malformation YANG Jing-jing, GUO Ling, REN Ying-xia, et al 2021, 36(11): 874-876.  DOI: 10.19538/j.ek2021110614 Abstract ( )

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Diagnosis and treatment of functional dyspepsia in children WAN Jun, LU Xiao-ying, ZHANG Xiao-mei 2021, 36(11): 877-880.  DOI: 10.19538/j.ek2021110615 Abstract ( )