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06 January 2022, Volume 37 Issue 1 Previous Issue    Next Issue

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Expert consensus on the diagnosis and treatment of X-linked dominant hypophosphatemic rickets Genetics and Metabolism Group, Child Disease and Health Care Branch of Chinese Maternal and Child Health Association, Department of Genetics and Metabolism, Rare Diseases Branch, Beijing Medical Association, Genetic Diseases Group, Birth Defects Prevention and Control Professional Committee, Chinese Preventive Medical Association 2022, 37(1): 1-6.  DOI: 10.19538/j.ek2022010601 Abstract ( )

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Expert consensus on the diagnosis and treatment of functional dyspepsia with integrated traditional Chinese and western medicine Committee of Collaborative Innovation Platform for Children’s Health of China Association of Chinese Medicine 2022, 37(1): 7-11.  DOI: 10.19538/j.ek2022010602 Abstract ( )

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From Henoch-Schönlein purpura to IgA vasculitis: A new insight of pathogenesis GAO Chun-lin, XIA Zheng-kun 2022, 37(1): 12-16.  DOI: 10.19538/j.ek2022010603 Abstract ( )   Henoch-Schönlein purpura is the most common systemic vasculitis in childhood，which was considered as an allergic disease in the early stage. With the development of immunopathology technique，it is now defined as IgA vasculitis，and when involving the kidney it is called IgA vasculitis nephritis（IgAVN） or Henoch-Schönlein purpura nephritis. There are two hypotheses concerning the pathogenesis of IgAV（N），one is the four-hit hypothesis similar to IgA nephropathy（IgAN），and the other is the novel multi-hit hypothesis centered on vascular inflammation and mucosal immunity. The latter hypothesis holds that IgAVN is a dual disease，which has the Gd-IgA1 abnormalities of IgAN and the vasculitis pathogenesis of IgAV. Here we review the up-to-date studies.

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Diagnosis and treatment of IgA vasculitis in children FU Tong, DU Yue 2022, 37(1): 17-21.  DOI: 10.19538/j.ek2022010604 Abstract ( )   IgA vasculitis（IgAV），belonging to systemic vasculitis，mainly involves small vessels and has a good prognosis and is self-limited. I+has 4 main symptoms of palpable purpura，arthritis/arthralgia，gastrointestinal tract and kidney involvement. There are no specific abnormalities in Endoscopy and kidney biopsy can be used to assist the diagnosis. In termsof different degree and organ of IgAV，multiple therapeutics chedules，mainly including general treatment，anti-inflammatory therapy，glucocorticoid therapy，immunoglobulin therapy and blood purification，can be used. Pediatricians should master the clinical manifestations and characteristics of IgAV and diagnose correctly at early as possible in order to shorten the length of stay in hospitals and reduce the occurrence of complications.

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Monitoring and early biomarkers of kidney injury in children with IgA vasculitis SHEN Qian 2022, 37(1): 21-24.  DOI: 10.19538/j.ek2022010605 Abstract ( )   IgA vasculitis is the most common vasculitis of small vessels in children，about 40%～50% involving kidney，which partly could develop into chronic kidney failure，even end-stage kidney disease，and seriously affects life quality of children and their families. Currently，the golden standard to diagnose and evaluate IgAV kidney damage is kidney biopsy，which is invasive and not easy for dynamic monitoring，and this forces us to find noninvasive and more accessible ways. Recently，with the progress of proteomics and molecular biological research，more attention is paid to urine and blood biomarkers which predict kidney damage in children with IgAV. This article reviews the related research progress.

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Clinicopathological features and prognosis of renal involvement in children with IgA vasculitis QIU Li-ru 2022, 37(1): 24-29.  DOI: 10.19538/j.ek2022010606 Abstract ( )   IgA vasculitis（IgAV） is the most common vasculitis in children，in which long-term prognosis is mostly dependent upon the severity of renal involvement. The risk factors of developing to IgAV nephritis（IgAVN） are age above 10 years at onset，mail，abdominal pain and recurrence of vasculitis. 97% of IgAVN developed renal symptoms within 6 months after purpura presentation. Haematuria，mostly microscopic，company with or without microproteinuria is the most common resentation of IgAVN in children. The pathology of IgAVN is characterized by intrarenal，mesangial or vascular deposits of IgA and mesangial cells and mesangial matrix proliferation，which iasimilar with IgA nephropathy. The important risk factors of poor kidney out come were IgAVN presented with nephrotic syndrome or kidney in sufficiency at onset. However some IgAVN patients who pressent with mild initial symptoms also eventually developed CKD. The patients with the ISKDC class Ⅳ and Ⅴ are more likely to progress to ESRD. Control of nephrotic range proteinuria is beneficial to long-term prognosis. Improved treatment strategies and detection of IgAVN patients at risk of CKD at an early stage are necessary.

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Treatment for renal damage in children with IgA vasculitis WANG Hui, LIANG Ying 2022, 37(1): 29-33.  DOI: 10.19538/j.ek2022010607 Abstract ( )   renal damage is the most serious complication of IgA vasculitis，which is closely related to the course and long-term prognosis for IgA vasculitis. At present，there is still no unified conclusion on the treatment of IgA vasculitis renal involvement at home and abroad. The application of glucocorticoidand immunosuppressants is more active in updated tuidelines. In this artical，the current treatment for IgAVN is reviewed，and it is expected that there will be more affirmative-quality large-sample randomized controlled trials on IgA vasculitis renal involvement in children in the future to provide more evidence-based evidence for clinical diagnosis and treatment.

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Childhood IgA vasculitis with nephritis and IgA nephropathy WANG Fang 2022, 37(1): 33-36.  DOI: 10.19538/j.ek2022010608 Abstract ( )   The first description of IgA vasculitis with nephritis was reported about 160 years earlier than that of IgA nephropathy. The diagnosis of IgA vasculitis with nephritis is typically based on the clinical manifestation，where as IgA nephropathy can only be diagnosed by a kidney biopsy. Some recent studies have demonstrated the similarities in the pathogenesis，genesis，clinical manifestations，pathological characteristics between the two diseases，and treatment，but there are some differences in terms of out come and age at onset，and the fact that not all the patients with IgA vasculitis have renal involvement，and skin manifestations do not occur in patients with IgA nephropathy，which indicates these two entities need to be differentiated. There is insufficient evidence to support the hypothesis that IgA vasculitis with nephritis and IgA nephropathy are two clinical presentations of the same disease.

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Diagnostic value of Expert Consensus on the Diagnosis of Hypertrophic Cardiomyopathy in Chinese Children：A domestic multicenter study Cooperation Group of Precision Diagnosis and Treatment of Cardiomyopathy in Children, the Subspecialty Group of Cardiology, the Society of Pediatrics, Chinese Medical Association, Editorial Board of Chinese Journal of Practical Pediatrics 2022, 37(1): 37-44.  DOI: 10.19538/j.ek2022010609 Abstract ( )   Objective　To compare the diagnostic results and values for hypertrophic cardiomyopathy（HCM） in children between Expert Consensus on the Diagnosis of Hypertrophic Cardiomyopathy in Chinese Children（Served as domestic criteria） and 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy：Executive summary：A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines（Served as AHA/ACC guidelines）. Methods　The clinical data of 466 patients with myocardial hypertrophy who were treated in 32 hospitals during June 2015 to June 2019 were analyzed. The gold standard for diagnosisis the echocardiographic final follow-up results and genetic testing results，so as to compare the sensitivity，specificity and accuracy of the two criteria for the diagnosis of HCM. Results　For the entire group of cases，the most common echocardiographic examination in children with myocardial hypertrophy was interventricular septum and posterior wall hypertrophy（57.08%）,followed by interventricular septum hypertrophy（31.33%）；the third was posterior wall hypertrophy（9.23%），and the least was apical hypertrophy（2.36%）. Genetic testing were performed in 93 children，of which 32 cases were definitely pathogenic variants，27 cases were possibly pathogenic variants，and 34 cases had unkown clinical significance variants. Based on the gold standard，396 cases were diagnosed as HCM and others were excluded；370 cases were diagnosed and 96 cases were excluded according to the domestic consensus；346 cases were diagnosed and 120 cases were diagnosed as non-hypertrophic cardiomyopathy according to the AHA/ACC guidelines. The diagnostic specificity of the domestic consensus and AHA/ACC guidelines for all cases was 100%；the entire group of cases，cases were older than lyear old，and cases without genetic testing，the sensitivity and accuracy of the domestic consensus diagnosis were higher than those of AHA/ACC guidelines，and the Youden index of domestic consensus was higher than that of AHA/ACC guidelines（P＜0.01）；cases were younger than 1 year old，the diagnostic sensitivity and accuracy of AHA/ACC guidelines were slightly higher than those of domestic consensus，but there was no significant difference in Youden index（P＞0.05）. Conclusion　Domestic consensus and AHA/ACC guidelines can accurately diagnose HCM in children，but domestic consensus is more suitable for accurate diagnosis of HCM in children.

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Detection of Helicobacter pylori and host genotypes in children with gastric juice real-time polymerase chain reaction LI Dong-dan, PENG Xian-hui, FANG Yong-li, et al 2022, 37(1): 45-50.  DOI: 10.19538/j.ek2022010610 Abstract ( )   Objective　To investigate the efficiency of gastric juice for detecting H.pylori infection，clarithromycin resistance，and CYP2C19 polymorphism. Methods　The specimens of gastric jjice were collected from all patients while receiving gastroscopy. DNA was extracted from gastric juice and then cagH were amplified by polymerase chain reaction（PCR） for detecting the existence of H.pylori. By PCR restriction fragment length polymorphism（PCR-RFLP），the 23SrRNA of H.pylori and CYP2C19 genotypes of host were examined respectively. During endoscopy examination，biopsy-based specimens were also collected. We compared the results of gastric juice tests with the biopsy-based specimens. Results　The sensitivity of gastric juice real-time PCR for the diagnosis of H.pylori infection was 100%（94/94）. The accuracy was 76.4%（94/123），the sensitivity to detect clarithromycin resistance was 98.9%（90/91），the specificity was 66.7%（2/3），and the positive predictive value was 98.9%（90/91）. The negative predictive value was 66.7%（2/3） and the accuracy was 97.9%（92/94）. The accuracy of detecting host CYP2C19 metabolome was 90.0%（90/100）. Conclusion　Gastric juice real-time polyme rase chain reaction is an effective method to detect Helicobacter pylori infection，clarithromycin sensitivity and host CYP2C19 gene metabolization in children，and it is worthy of promotion in children.

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Correlations between the clinical characteristics and the onset age of childhood-onset systemic lupus erythematosus ZHANG Yu, WANG Li, XU Li, et al 2022, 37(1): 51-54.  DOI: 10.19538/j.ek2022010611 Abstract ( )   Objective　To investigate the correlation between the clinical characteristics and onset age of childhood-onset systemic lupus erythematosus（SLE）. Methods　The clinical data and prognosis of SLE patients in our hospital from January 2011 to October 2016 were analyzed retrospectively，and the correlation between the clinical characteristics and onset age was evaluated by Spearman correlation coefficient analysis. Results　In this study，the average onset age of the 159 SLE children was （11.05±2.73） years old. Among the initial manifestations，fever was negatively correlated with the onset age（r＝-0.164，P＜0.05），leukopenia was positively correlated with the onset age（r＝0.158，P＜0.05），nervous system involvement was positively correlated with the onset age（r＝0.169，P＜0.05），and class Ⅳ lupus nephritis was positively correlated with the onset age（r＝0.314，P＜0.05）. There was no obvious correlation between the onset age and the other initial manifestations，immundogical indexes，the complications after treatment or death. Conclusion　With the increase in onset age，it is more likely to have leukopenia and nervous system involvement，and serious renal damage is ore severe，but the inflammatory response is less. After standardized treatment，the onset age doesn’t determine the prognosis of childhood-onset SLE.

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A retrospective study of 357 colonoscopy in children WANG Yang, SUN Mei 2022, 37(1): 55-58.  DOI: 10.19538/j.ek2022010612 Abstract ( )   Objective　To investigate the clinical value，safety and causes of colonoscopy application in children as well as the distribution of intestinal diseases in different age groups. Methods　The clinial data of patients aged＜15 years who under went their colonoscopy in the endoscopy center at Shengjing Hospital of China Medical University between Jan.1，2018 and Dec.31，2020 were collected and analyzed retrospectively. Results　The terminal ileum in tubation success rate was 94.5%（377/399）. A total of 219 patients（61.3%） had a positive diagnosis under colonoscopy. No serious complications occurred during the procedures in 357 cases. The most frequent diagnosis in 0-3-year group was Crohn’s disease（36.8%）. A total of 46 cases of disease（90.2%） were found in 3-6-year group. The most frequent diagnosis in 3-6-year group was colonic polyps（56.9%）. A total of 155 cases（54%） were found positive diagnosis in the 7-14-year group，and the detection rate of inflammatory bowel disease（IBD） was the highest（19.2%）. Conclusion　Colonoscopy in pediatric patients is a safe and effective procedure with a high diagnostic rate，and has important clinical diagnostic and therapeutic value. Diarrhea and blood in the stool are the main reasons for colonoscopy in children aged 0-6 years. The main reasons for colonoscopy in children aged 7-14 years are abdominal pain and diarrhea. Colorectal polypis the main lesion revealed by colonoscopy in 3-6-year-old group. IBD is the main disease in children aged 0-3 and 7-14.

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Analysis of clinical features of Angiostrongylus cantonensis meningitis in 3 children and the value of metagenomics next-generation sequencing of cerebrospinal fluid in its diagnosis LUO Zhi-qiang, LIAO Jian-xiang 2022, 37(1): 59-62.  DOI: 10.19538/j.ek2022010613 Abstract ( )

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Research progress in magnetic resonance imaging for skeletal muscle of lower limbs in children with muscular dystrophy CHEN Jia-kun, HAN Chun-xi, LI Zhi-yong 2022, 37(1): 63-67.  DOI: 10.19538/j.ek2022010614 Abstract ( )

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Research progress in conversion disorder in children MENG Lin-xue, HU Yue 2022, 37(1): 68-72.  DOI: 10.19538/j.ek2022010615 Abstract ( )

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Latest research progress in regressive autism LI Xue 2022, 37(1): 73-76.  DOI: 10.19538/j.ek2022010616 Abstract ( )

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Report on one case of infantile liver failure syndrome due to NBAS gene mutation JIN Meng, MA Xin, ZHU Dan, et al 2022, 37(1): 77-80.  DOI: 10.19538/j.ek2022010617 Abstract ( )