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06 August 2017, Volume 32 Issue 8 Previous Issue    Next Issue

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Diagnosis and treatment of Kawasaki disease: A long way to go YAN Hui，DU Jun-bao 2017, 32(8): 561-564.  DOI: 10.19538/j.ek2017080601 Abstract ( )   Kawasaki disease is an acute self-limited vasculitis. Timely and accurate diagnosis and treatment can significantly reduce the incidence of coronary artery dilatation or coronary artery aneurysm. However，there are difficulties in the diagnosis and treatment of Kawasaki disease for the time being. We need to make efforts to further improve the accuracy of diagnosis，early predict and identify the high risk population of refractory Kawasaki disease and coronary aneurysm，and optimize the initial treatment of stratified patients according to the coronary risk they are facing. We should constantly promote the experience of long-term follow-up management of Kawasaki disease so as to improve the level of diagnosis and treatment.

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Update in the epidemiologic study of Kawasaki disease DU Zhong-dong*，CHEN Xiao-zheng 2017, 32(8): 565-569.  DOI: 10.19538/j.ek2017080602 Abstract ( )   Kawasaki disease is a systemic vasculitis that mainly affects children younger than 5 years old. Since its first report over 40 years ago in Japan，Kawasaki disease has been diagnosed in more than 60 countries in the world until now and it has also been recognized as a leading cause of acquired heart disease in children in developed countries. Variation in study methods，clinically-based diagnosis and proportion of incomplete cases may lead to variations and limitations of the results，under-reporting or over-reporting the true incidence. However，epidemiological studies have revealed genetic aspect and environmental aspect of etiology，including a heightened incidence in people of Asian descent，higher relative risk of the disease，marked seasonality and age distribution，etc. The true incidence of the disease，the increased awareness and access to special medical services may all contribute to the rising incidence of the disease in most countries over the years. This paper sought to review systemically the epidemiologic features of KD in the world.

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Diagnosis of Kawasaki disease ZHANG Li 2017, 32(8): 569-572.  DOI: 10.19538/j.ek2017080603 Abstract ( )   Typical diagnostic criteria for Kawasaki disease（KD） include：（1）the fever more than 5 days and invalid treatment with antibiotics；（2）changes in distal extremities, including erythema and edema of the hands and feet at acute phase and periungual desquamation of the fingers and toes at subacute phase；（3）polymorphous eruption；（4）bilateral bulbar conjunctival injection without exudate；（5）erythema of the lips, fissured lips and strawberry tongue；（6）cervical lymphadenopathy. The diagnostic criteria for classical KD include fever as essential condition accompanied by at least 5 of 6 items. It is important to note that there may be other diseases with similar performance，because of the non-specific diagnosis criteria for Kawasaki disease.

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Clinical features of incomplete Kawasaki disease HUA Wang，GONG Fang-qi 2017, 32(8): 572-575.  DOI: 10.19538/j.ek2017080604 Abstract ( )   In recent years，the understanding of KD has been deepened. Children with complete KD were timely diagnosed， but there was still a challenge for patients with incomplete KD. Full recognition of clinical features of incomplete KD would enable doctors to avoid delayed diagnosis and treatment， and reduce the incidence of coronary artery complications. Patients with 4 or fewer main criteria were considered with incomplete KD， and those with insufficient criteria and other atypical organ involved were considered with atypical KD. The main criteria for KD were in a clinically dynamic evolution， and some patients with incomplete KD present new main criteria and eventually meet the diagnostic criteria for complete KD. Early recognition of clinical features of patients with potential turning into complete KD was useful to improve the prognosis of children with KD.

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Risk factors and prediction of coronary artery lesions of Kawasaki disease SUN Le，YU Xian-yi 2017, 32(8): 575-579.  DOI: 10.19538/j.ek2017080605 Abstract ( )   The coronary artery lesions（CAL），especially coronary artery aneurysm，is the most serious complication of Kawasaki disease（KD）. In order to predict the possible risk and reduce the incidence of CAL，the articale reviewed the relationship between some clinical indexes and the incidence of CAL，such as age and gender，duration of fever，C-reactive protein，NT-proBNP，peripheral blood leukocyte count，erythrocyte sedimentation rate，serum albumin level，application time and dose of IVIG and recurrent KD. It also introduced the application of Harada score and Kobayashi score to assess the risk of CAL. Some new potential biomarkers for the diagnosis of KD with CAL were also introduced.

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Management and follow-up of cardiovascular sequelae in Kawasaki disease LIU Fang， HUANG Guo-ying 2017, 32(8): 579-584.  DOI: 10.19538/j.ek2017080606 Abstract ( )   Coronary artery lesion（CAL） is the most important complication of Kawasaki disease，and has become the leading cause of acquired heart disease in childhood in developed countries. This paper describes the diagnosis and evaluation of CAL in children with Kawasaki disease，and the management and follow-up of CAL. It focuses on how to use echocardiography to detect the coronary arteries in children with Kawasaki disease and how to evaluate the severity of CAL，the timing and role of coronary artery angiography，the natural history of CAL，and the progress of acute and long-term management of CAL in children with Kawasaki disease.

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Intravenous immunoglobulin resistance in Kawasaki disease：Clinical judgement，prediction and management CHEN Yong-bing， JIN Hong-fang 2017, 32(8): 584-588.  DOI: 10.19538/j.ek2017080607 Abstract ( )   Kawasaki disease is a common acute systemic vasculitis in childhood. Among those untreated， around 25% patients may develop coronary artery lesions. It has become the most common reason for acquired heart disease in children in developed countries. The standard therapy for Kawasaki disease is intravenous immunoglobulin，which can significantly reduce prevalence of coronary artery lesions. If patients have persistence or reoccurrence of fever after treatment，which is recognized as intravenous immunoglobulin resistance，they have an increased risk of developing coronary artery abnormalities. Therefore，it is necessary to timely recognize those who might be resistant to intravenous immunoglobulin in order to administrate intensified initial treatment or rescue therapies. The article summarized common predictors of intravenous immunoglobulin resistance，including patients’ characteristics，clinical and laboratory indicators，such as age，gender，timing for treatment，routine blood test，blood biochemical assays，etc. Rescue therapies for unresponsive patients include additional intravenous immunoglobulin，steroids，infliximab，cyclosporine，etc.

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Indication and application of anticoagulation therapy in Kawasaki disease SHI Lin，LIN Yao，DENG Yan-jun，et al 2017, 32(8): 588-591.  DOI: 10.19538/j.ek2017080608 Abstract ( )   The abnormal coagulation function mediated by vasculitis associated with Kawasaki disease and abnormal hemodynamics in coronary artery lesions will increase the risk of thrombosis in coronary artery，so anticoagulation therapy in Kawasaki disease is very important. The indications is based on the risk stratification of coronary artery. All children with Kawasaki disease should accept antiplatelet therapy. Patients with risk level Ⅲ should accept more than two kinds of antiplatelet drugs，patients with risk level Ⅳ and above should take aspirin and warfarin together，and patients with thrombosis and myocardial infarction should be given thrombolysis therapy early. Anticoagulation treatment should last at least 3 months. If the coronary artery persists，long term anticoagulant therapy should be performed.

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Application of the electronic data capture system in clinical research of Kawasaki disease HUANG Min 2017, 32(8): 592-594.  DOI: 10.19538/j.ek2017080609 Abstract ( )   Kawasaki disease is a kind of systemic vascular inflammatory disease which often occurs in children under the age of five， and the etiology is still unknown. According to the epidemiological survey， the incidence of Kawasaki disease in Japan is the highest， followed by South Korea and China. The incidence in Asian populations is higher than in non-Asian populations. Many countries have  studied the national wide epidemiological survey of Kawasaki disease， but China is still in lack of this kind of multicenter survey data about Kawasaki disease. Electronic Data Capture System can collect and manage the clinical and laboratory database of Kawasaki disease very quickly and accurately. If this system can be promoted to the whole country of China， it will can be helpful to run a multicenter study of Kawasaki disease， in order to provide evidence for making KD diagnosis，treatment plan and evaluation of the risk factors in Chinese children.

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Clinical characteristics and typing of orthostatic hypertension in children HE Yu-wei*，LI Hong-xia，ZHAO Juan，et al 2017, 32(8): 595-600.  DOI: 10.19538/j.ek20170806010 Abstract ( )   Objective　To investigate the incidence and clinical characteristics of orthostatic hypertension（OHT）in children. Methods　Totally 1348 children with syncope，dizziness，etc，were collected in Peking University First Hospital between 2009 and 2015，24 of whom were diagnosed with OHT. Demographic characteristics，the occurrence frequency of various clinical manifestations and its inducing factors，course of disease and the changes of the blood pressure at the lying position were analysed. Results　（1）Among 1348 children，OHT was in 24 （male 14，female 10）. The case proportions of those at or under 12 years old to those beyound 12 years old and the ratio of male to female showed no significant differences.（2）There was no difference between those at or younger than 12 years old and those older than 12 years old（χ2＝0.411，P＝0.521），or the ratio of males to females in sOHT and dOHT. （3）The most common clinical manifestations were syncope，dizziness，chest tightness，and so on. Syncope and chest tightness mostly appeared in dOHT children. The incidence of dizeiness in sOHT and dOHT was similar. In children，common inducements were sudden postural changes（from supine to upright and standing position）（41.7%），persistent standing（37.5%），or persistent seating（37.5%）. Persistent standing and persistent seating were the main cause of sOHT. Sudden postural changes were the main cause of dOHT. Conclusion　There is no age or gender difference in the incidence and typing of OHT in children. Syncope，dizziness and chest tightness are the main clinical manifestations. Persistent standing and persistent seating are the main cause of sOHT. Sudden postural changes are the main cause of dOHT.

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Research on the preventive effect of oral captopril on the pulmonary arterial hypertension associated with ventricular septal defect WANG Xian-min，LU Ya-heng，JIN Mei，et al 2017, 32(8): 601-605.  DOI: 10.19538/j.ek20170806011 Abstract ( )   Objective　To explore the preventive effect of regular doses of captopril on the pulmonary arterial hypertension associated with ventricular septal defect（VSD） and its mechanism. Methods　Forty cases of children with VSD in accordance with the inclusive criteria，who were hospitalized in Chengdu Women and Children’s Central Hospital from July 2013 to July 2015 ，were collected and randomly divided into blank control group（n＝20）and captopril intervention group（n＝20）. Plasma MMP-9 and TIMP-1 were examined by using ELISA，and PASP，the Qp/Qs and right ventricular Tei index were measured by cardiac color Doppler ultrasound at 0 week，1 week，4 weeks，8 weeks，and 12 weeks respectively；side effects in captopril intervention group were also observed. Results　PASP，the Qp/Qs，right ventricular Tei index，plasma MMP-9 and TIMP-1 level in blank control group were increased over time；the same indexes in captopril intervention group were decreased，the changing trend of two groups had statistically significant difference（all P＜0.05）. The  indexes were of no difference between the two groups at 0 week（all P＞0.05）；PASP and right ventricular Tei index in captopril intervention group were lower than blank control group at 1 week，4 weeks，8 weeks，and 12 weeks（all P＜0.05）；the Qp/Qs plasma，MMP - 9 and TIMP - 1 level in captopril intervention group were lower at 4 weeks，8 weeks，and 12 weeks（all P＜0.05）. No side effects were found in captopril intervention group during follow-up. Conclusion　Oral regular doses of captopril in the VSD children undergoing elective surgery may reduce pulmonary vascular remodeling and prevent pulmonary hypertension  in order to achieve the best age at surgery；one of the mechanisms might be improving  indexes of PASP，right ventricular Tei index and the Qp/Qs by reducing the plasma MMP 9 and TIMP - 1 level.

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Relationship between gene polymorphism of β1 adrenergic receptor and outflow tract ventricular premature LI Qi-rui，YUAN Yue，LIN Li，et al 2017, 32(8): 606-610.  DOI: 10.19538/j.ek20170806012 Abstract ( )   Objective　To investigate the relationship between β1 adrenergic receptor（β1-AR）gene polymorphism and outflow tract ventricular premature（OTVP）in Han Chinese children. Methods　A case-control study was performed. A total of 151 children with OTVP were recruited，who were diagnosed in Beijing Children’s Hospital from January 2014 to December 2015，and 150 healthy children served as controls. Peripheral blood samples were collected from all subjects and the genotype of β1-AR were determined by PCR，followed by direct sequencing. The frequency distribution of genotypes was compared between the OTVP and control groups. The association between polymorphism of β1-AR and OTVP was analyzed. Results　The frequencies of β1-AR genotypes Arg/Arg，Arg/Gly and Gly/Gly were 50.5%，38.5% and 11% in all children. The allelic frequencies of Arg389 and Gly389 were 69.8% and 30.2%. The allelic frequencies of Arg389 in female chidren were higher than in male（P＝0.005）.There were significant differences in the genotype and allele frequencies of β1-AR gene Arg389Gly between the OTVP and controls （P＝0.012，P＝0.002），and Arg389 allele was a risk factor （OR＝1.733，95%CI：1.218～2.466）. No differences of genotype were observed between different genders in children with OTVP（P＝0.088），but there were significant differences in the genotype between loads of ventricular premature and nonsustained ventricular tachycardia（P＝0.029，P＝0.019）. Conclusion　The gene polymorphism of Arg389Gly in β1-AR might be associated with the OTVP in Han Chinese children and Arg389 allele gene is a risk factor. The genotype of Arg/Arg might be a genetic susceptibility factor of high-level loads of ventricular premature and nonsustained ventricular tachycardia in children with OTVP.

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Risk factors for neonatal necrotizing enterocolitis: An analysis of 76 cases HOU A-na，LI Xue，FU Jian-hua 2017, 32(8): 611-614.  DOI: 10.19538/j.ek20170806013 Abstract ( )   Objective　To analyze the influence of perinatal period factors，feeding strategies，probiotics usage，complications and mechanical ventilation before the onset of the disease on neonatal necrotizing enterocolitis（NEC），in order to explore the risk factors for NEC. Methods　Totally 76 infants with NEC were classified as the case group，who were hospitalized in the First Neonatal Department of Shengjing Hospital of China Medical University from January 2011 to January 2016；80 infants without NEC who were admitted at the same time were randomly selected as the control group. A retrospective analysis was performed on the clinical data of these patients，including general conditions of newborns，perinatal period factors，feeding strategies，probiotics usage，complications and mechanical ventilation before the development of NEC. Single and multiple factor analysis was performed on 19 items using the SPSS analysis software. Results　Single factor analysis revealed that the incidence of asphyxia，sepsis，hypoglycemia and mechanical ventilation was higher，while the incidence of oral probiotics usage was lower in the case group than the control group，with statistically significant difference（P＜0.05）. According to the logistic multivariate regression analysis，sepsis（OR＝6.531），hypoglycemia（OR＝2.785） and mechanical ventilation（OR＝3.448） were risk factors of NEC，while oral probiotics usage was protective factor of NEC（OR＝0.417）. Conclusion　Sepsis，hypoglycemia and mechanical ventilation are risk factors of NEC，and oral probiotics usage is protective factor of NEC. We should pay attention to the prevention and intervention of NEC by reasonably analyzing these factors，in order to improve the prognosis of these children.

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Kimura’s disease complicated with nephrotic syndrome in children:An analysis of 3 cases and literature revie ZHANG Xiao-ge*，LI Zhi-juan，CHEN Guo-qiang，et al 2017, 32(8): 615-618.  DOI: 10.19538/j.ek20170806014 Abstract ( )   Objective　To improve the level of diagnosis and treatment of children with Kimura’s disease complicated with nephrotic syndrome. Methods　In order to understand the current pathogenesis，diagnosis and treatment of children with Kimura’s disease complicated with nephrotic syndrome，we reviewed 3 cases of  Kimura’s disease in our department from 2011 to 2015，and studied the related literature at home and abroad at the same time. Results　Kimura’s disease is a rare chronic immune inflammatory disease which mainly damaged lymph nodes，soft tissue and salivary glands. It mostly occurs in Asian people between the age of 28 and 32，especially male. At present，the cause of the disease and its pathogenesis is still unclear. Histopathology is the only way to diagnose Kimura’s disease. As the golden standard of kidney disease diagnosis，renal biopsy is a big help for the diagnosis and treatment of renal damage of Kimura’s disease. Current treatments mainly depend on medication，and generally hormone combined immune inhibitors are used. Conclusion　Kimura’s disease is a chronic disease，which is a benign lesion but easy to recur. There usually is no malignant change.

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Clinical study of treatment for childhood acute myeloid leukemia by NOPHO AML  2004 protocol combined with hematopoietic stem cell transplantation HUANG Zhi*，FENG Xiao-qin，ZHANG Yu-ming，et al 2017, 32(8): 619-622.  DOI: 10.19538/j.ek2017080615 Abstract ( )   Objective　To analyze the effectiveness and the practicability of Nordic Society for Pediatric Hematology and Oncology（NOPHO） AML 2004 protocol combined with hematopoietic stem cell transplantation（HSCT） in treating childhood acute myeloid leukemia（AML） in China. Methods　The clinical data of 53 children（aged under 14） with AML（not including acute promyelocytic leukemia） treated with NOPHO AML 2004 protocol（some children followed by HSCT） between Dec. 2009 and Dec. 2015 was retrospectively analyzed. Survival was evaluated by Kaplan Meier analysis and Log-Rank test. Results　Complete remission（CR） rate reached 86.8%. Totally 36 patients（67.9%） finished 6 courses of chemotherapy；14 patients（26.4%）underwent HSCT after 1-3 courses of consolidation chemotherapy；3 patients gave up treatment after 1-3 courses of chemotherapy. The proleptic overall survival（pOS） at 5 years and leukemia-free survival（pLFS） was 72% and 56% respectively during a follow-up period of 12 to 82 months（median 29 months）. For standard-risk patients，the pLFS at 5 years reached 58%， while high-risk patients had 46%（P＝0.779）. The pLFS at 5 years in patients who only underwent chemotherapy and those with additional hematopoietic stem cell transplantation was 48% and 77% respectively（P＝0.1268）. The pLFS in patients with good response，intermediate response and poor response on day 15 bone marrow examination was 75%，29% and 53% respectively（P＝0.0071）. Conclusion　NOPHO AML 2004 protocol is effective and feasible for Chinese patients with childhood AML. The evaluation of blast in bone marrow on day 15 can be used as an important prognostic indicator.

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Diagnostic value of umbilical cord blood procalcitonin in neonatal early-onset sepsis MIAO Li，LI Juan 2017, 32(8): 623-626.  DOI: 10.19538/j.ek20170806016 Abstract ( )

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Research progress of gene polymorphisms related to Henoch-Schönlein purpura DU Fan-fan，JIANG Hong 2017, 32(8): 627-630.  DOI: 10.19538/j.ek20170806017 Abstract ( )

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Present situation of the research in neuroprotection of neonatal hypoxic-ischemic encephalopathy LI Fang，LIU Hua，XIAO Dong-jie，et al 2017, 32(8): 631-635.  DOI: 10.19538/j.ek20170806018 Abstract ( )

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Application progress of echocardiography in evaluating myocardial and left ventricular function of patients with Kawasaki disease WANG Hai-yong，TONG Ming-hui 2017, 32(8): 636-638.  DOI: 10.19538/j.ek20170806019 Abstract ( )

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One case report of kala-azar combined with hemophagocytic syndrome YI Dong-ling 2017, 32(8): 639-640.  DOI: 10.19538/j.ek20170806020 Abstract ( )