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Vol 16, No 6
Vol 16, No 6 December 2020 ISSN 1708-8569
Review articles
Original articles
Brief reports
Letters to the editor
Management of orthostatic intolerance in children: the state of the art
  Chun-Yan Tao, Hong-Fang Jin, Jun-Bao Du
  [Abstract] [Full Text] [PDF]  
Differential diagnosis of vasovagal syncope and postural tachycardia syndrome in children
  Yuan-Yuan Wang, Jun-Bao Du, Hong-Fang Jin
  [Abstract] [Full Text] [PDF]  
COVID-19 and the young heart: what are we missing?
  Robert Spencer, Nak Hyun Choi, Keriann Potter, Sanghee Suh, Michael Fremed
  [Abstract] [Full Text] [PDF]  
Strategies for children*s hospital in response to COVID-19 pandemic: perspective and practice at a designated pediatric hospital in Shanghai, China
  Xiao-Bo Zhang, Xiao-Jing Hu, Xiao-Wen Zhai, Ying Gu, Lin Yuan, Peng Shi, Xiao-Lin Ge, Gong-Bao Liu, Guo-Mei Shen, Chuan-Qing Wang, Ai-Mei Xia, Mei Zeng, Guo-Ying Huang
  [Abstract] [Full Text] [PDF]  
Say no to drugs: wait and watch strategy for medications used in the neonatal intensive care unit (NICU)
  Hilary Tice, Shabih Manzar, Nitin Walyat, Kelsey Trimble
  [Abstract] [Full Text] [PDF]  
Uveitis in juvenile idiopathic arthritis
  Ross E. Petty, Qi Zheng
  [Abstract] [Full Text] [PDF]  
Review articles:
Macrophage activation syndrome in children with Kawasaki disease: diagnostic and therapeutic approaches
  Seung Beom Han, Soo-Young Lee
Background: Macrophage activation syndrome (MAS) is a rare, life-threatening complication of Kawasaki disease (KD). Early recognition and treatment of MAS are very important, but sometimes it is difficult to distinguish MAS from a severe form of KD.
Data sources: A PubMed search was performed in Clinical Queries using the key terms ※macrophage activation syndrome or secondary hemophagocytic lymphohistiocytosis (HLH)§ and ※Kawasaki disease§.
Results: KD patients with MAS show high intravenous immunoglobulin (IVIG) resistance and coronary complications. Mortality is also as high as MAS in other diseases. Persistent fever greater than 10 days is highly associated with development of MAS in KD. Splenomegaly is observed in more than two-thirds of KD patients with MAS. Thrombocytopenia is often the earliest laboratory finding of MAS. Hyperferritinemia is highly specific and sensitive for detecting MAS in KD; so, ferritin levels should be checked if there are unexplained clinical exacerbations in KD patients. Given the under-recognition of MAS in KD, it is prudent to consider resistant KD as occult/subclinical MAS. Many KD patients with MAS have good outcomes on immune modulators. However, if KD patients fulfill the HLH-2004 diagnostic criteria, they may undergo longer and more intensive treatment than needed.
Conclusions: The possible existence of MAS should be taken into account when a KD patient shows persistent fever, splenomegaly, thrombocytopenia, hyperferritinemia, or IVIG resistance. The under-diagnosis of MAS in patients with KD is an important issue to be addressed. Therapeutically, however, there is a possibility of over-treatment of MAS in patients with KD.
  [Abstract] [Full Text] [PDF]  
Haploinsufficiency of A20 (HA20): updates on the genetics, phenotype, pathogenesis and treatment
  Mei-Ping Yu, Xi-Sheng Xu, Qing Zhou, Natalie Deuitch, Mei-Ping Lu
Background: A20, a protein encoded by the tumor necrosis factor alpha-induced protein 3 gene (TNFAIP3), plays a vital role in the negative regulation of inflammation and immunity. Loss-of-function mutation in TNFAIP3 leads to a new described autoinflammatory disease-haploinsufficiency of A20 (HA20). Since HA20 was first described in 2016, a number of new cases have been described in this literature, however, the disease and its pathogenesis are poorly understood. This review seeks to improve clinical recognition of this disorder, and promote both earlier diagnosis and initiation of targeted therapies to improve patients* outcomes.
Methods: We reviewed 26 papers about A20 and HA20, and we summarized genetic variants and clinical manifestations of a total of 61 reported patients from 26 families identified to have a genetic diagnosis of germline pathogenic variants in TNFAIP3/A20. Additionally, we discussed the pathogenesis and treatment of HA20.
Results: A total of 24 pathogenic variants of A20 had been reported. There was significant clinical heterogeneity, even among those with the same variants in TNFAIP3 . Prior to receiving a molecular diagnosis of HA20, patients had been diagnosed with Behcet*s disease, rheumatoid arthritis, rheumatic fever, juvenile idiopathic arthritis, systemic lupus erythematosus, and even adult-onset Stills* disease. The patients with HA20 that presented with inflammatory signatures in NF-百B signaling were mostly responsive to treatment.
Conclusions: HA20 is a monogenic autoinflammatory disease with highly variable clinical manifestations. This extensive heterogeneity makes it difficult to set a clinical diagnostic criteria, and genetic sequencing is necessary for a definitive diagnosis of HA20.
  [Abstract] [Full Text] [PDF]  
Incidence characteristics of testicular microlithiasis and its association with risk of primary testicular tumors in children: a systematic review and meta-analysis
  Cheng-Jun Yu, Jian-Dong Lu, Jie Zhao, Yi Wei, Tian-Xin Zhao, Tao Lin, Da-Wei He, Sheng-De Wu, Guang-Hui Wei
Background: To systematically evaluate the incidence characteristics of testicular microlithiasis (TM) in children and its association with primary testicular tumors (PTT).
Methods: A systematic review and meta-analysis were conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. A priori protocol was registered in the PROSPERO database (CRD42018111119), and a literature search of all relevant studies published until February 2019 was performed. Prospective, retrospective cohort, or cross-sectional studies containing ultrasonography (US) data on the incidence of TM or the association between TM and PTT were eligible for inclusion.
Results: Of the 102 identified articles, 18 studies involving 58,195 children were included in the final analysis. The overall incidence of TM in children with additional risk factors for PTT was 2.7%. In children, the proportion of left TM in unilateral cases was 55.7%, the frequency of bilateral TM was 69.0%, and proportion of classic TM was 71.8% [95% confidence interval (CI) 62.4每81.1%, P = 0.0, I2 = 0.0%]. About 93.5% of TM remained unchanged, and newly detected PTT rate was very low (4/296) during follow-up. The overall risk ratio of TM in children with a concurrent diagnosis of PTT was 15.46 (95% CI 6.93每34.47, P < 0.00001).
Conclusions: The incidence of TM in children is highly variable. Nonetheless, TM is usually bilateral, of the classic type, and remains stable or unchanged at follow-up. Pediatric patients with TM and contributing factors for PTT have an increased risk for PTT; however, there is no evidence to support mandatory US surveillance of children with TM.
  [Abstract] [Full Text] [PDF]  
Associations between PRF1 Ala91Val polymorphism and risk
  Guang-Hua Zhu, Li-Ping Zhang, Zhi-Gang Li, Ang Wei, Ying Yang, Yu Tian, Hong-Hao Ma, Dong Wang, Xiao-Xi Zhao, Yun-Ze Zhao, Na Li, Wei Liu, Tian-You Wang, Rui Zhang
Background: Perforin (PRF1) gene mutation can cause the onset of hemophagocytic lymphohistiocytosis (HLH). It has reported that PRF1 Ala91Val polymorphism was related with HLH risk. In the meta-analysis, we aim to evaluate the association between PRF1 Ala91Val polymorphism and HLH risk.
Methods: We accomplished a meta-analysis of six published case-control studies including 391 patients with HLH and 975 controls. We evaluated the quality of each study through Newcastle每Ottawa Scale (NOS). Data analysis was performed with Stata software.
Results: In general, all studies were of high quality (NOS score higher than 7). There were statistically significant between the PRF1 Ala91Val polymorphism and HLH risk though the pooled analysis [for Ala/Val vs. Ala/Ala: pooled odds ratio (OR) = 3.22, 95% confidence interval (CI) 1.08每9.56, P = 0.035, random model; for Ala/Val + Val/Val vs. Ala/Ala: pooled OR = 2.96, 95% CI 1.14每7.69, P = 0.025, random model]. Furthermore, sensitivity analysis also revealed a relationship between PRF1 Ala91Val polymorphism and HLH risk (for Ala/Val vs. Ala/Ala: pooled OR = 5.236, 95% CI 2.72每10.08, P < 0.000, I2 = 12.1%, P heterogeneity = 0.332; for Ala/Val + Val/Val vs. Ala/Ala, pooled OR = 4.856, 95% CI 2.66每8.85, P < 0.000, I2 = 5.9%, P heterogeneity = 0.373). Funnel plot and Egger*s test did not indicate obvious published bias (P = 0.841 for Ala/Val vs. Ala/Ala; P = 0.284 for Ala/Val + Val/Val vs. Ala/Ala).
Conclusion: This meta-analysis indicated that PRF1 Ala91Val polymorphism affects the factor for developing HLH and future studies of PRF1 Ala91Val on the onset of HLH will be guaranteed.
  [Abstract] [Full Text] [PDF]  
Original articles:
Prediction of intravenous immunoglobulin resistance in Kawasaki disease in children
  Shu Wu, Ying Liao, Yan Sun, Chun-Yu Zhang, Qing-You Zhang, Hui Yan, Jian-Guang Qi, Xue-Qin Liu, Yong-Hong Chen, Yu-Li Wang, Xue-Ying Li, Hong-Fang Jin, Jun-Bao Du
Background: We aimed to explore predictive measures for intravenous immunoglobulin (IVIG) resistance in children with Kawasaki disease (KD).
Methods: Patients diagnosed with KD were enrolled in this study. Univariate analysis and multiple logistic regression were utilized to analyze the clinical features and laboratory results prior to IVIG-treatment of the two groups. Independent predictors of IVIG resistance were analyzed, and a predictive model for KD children with IVIG resistance was constructed.
Results: A total of 277 children with KD, 180 boys and 97 girls, aged 2-128 (median 23) months, were enrolled in the study. Compared with the IVIG-responsive group, the IVIG-resistant group had higher levels of the peripheral neutrophil count, mean platelet volume, mean platelet volume-to-lymphocyte ratio and C-reactive protein, and total serum bilirubin, but lower levels of peripheral lymphocyte count, serum albumin and serum prealbumin. Age (in months), peripheral neutrophil count, lymphocyte count and mean platelet volume and serum albumin were independent indicators for IVIG resistance by multivariate logistic regression analysis. A logistic regression model and a scoring system were set up, where cut-off values of - 0.46 and 6.5 points yielded sensitivities of 83.9% and 77.4%, and specificities of 74.8% and 61.0%, respectively. The areas under the curve (AUC) were 0.808 in the logistic regression model, and 0.750 in the scoring system.
Conclusion: Our model for predicting IVIG-resistant children with KD, involving age (months), peripheral neutrophil count, lymphocyte count and mean platelet volume and serum albumin prior to IVIG-treatment, is helpful for clinical prediction of children with IVIG-resistant KD.
  [Abstract] [Full Text] [PDF]  
SARS-CoV-2 infection in children requiring hospitalization: the experience of Navarra, Spain
  Laura Moreno-Galarraga, Mar赤a Urretavizcaya-Mart赤nez, Josune Alegr赤a Echauri, Marcos Garc赤a Howard, Eva Ruperez Garc赤a, Sergio Aguilera-Albesa, Valent赤n Alzina de Aguilar, Mercedes Herranz Aguirre
Background: Coronavirus disease 2019 in children, or pediatric COVID-19, initially was described as an acute respiratory syndrome similar to the adult presentation but with less severe manifestations.
Methods: We describe the clinical characteristics, disease presentation, treatments and outcomes of all pediatric cases with COVID-19 admitted to the reference hospitals in Navarra, Spain during the first wave of the COVID-19 outbreak (February-May 2020).
Results: We found a low number of hospitalized cases in infants and children compared to adults with a hospitalization ratio of 1:180. Most of these hospitalized cases did not suffer from severe disease. Over 80% of infections reported household contacts, and the mother was the known-contact in 83% of these cases. 72% of hospitalized cases were previously healthy children. We describe how symptoms in pediatric cases are nonspecific and how COVID-19 can be presented with a wide range and variety of symptoms. Respiratory symptoms are not always present, and severe neurological and cardiac features can occur in previously healthy children.
Conclusion: Epidemiological description and case reports will be key to a better recognition and to adequate treatment of pediatric patients with COVID-19.
  [Abstract] [Full Text] [PDF]  
Brief reports:
Decreased hemoglobin after initial treatment is associated with treatment resistance in Kawasaki disease in Kobayashi risk stratification
  Yukari Atsumi, Hiroshi Sakakibara, Yoshihiko Morikawa, Koichi Miyata, Hiroyuki Yamagishi, Masahiro Misawa, Masaru Miura
Background: Although Kawasaki disease (KD) is known to cause anemia, the relationship between its prognosis and hemoglobin level is less well known.
Methods: We herein performed a secondary analysis of data from a previous study (Post-RAISE). Children aged 6 months to 2 years at the time of KD diagnosis were enrolled. Decreased hemoglobin was defined as a decrease > 10% on post-treatment day 2 compared with the level prior to the initial therapy.
Results: Of 1253 KD cases, treatment resistance was observed in 209 (17%) and was significantly more frequent in the decreased hemoglobin group (37/167 vs. 172/1086, P = 0.041). Multivariable logistic regression analysis revealed that decreased hemoglobin was associated with resistance to the initial treatment (odds ratio 1.53; 95% confidence interval 1.00-2.33; P = 0.048).
Conclusion: Decreased hemoglobin was significantly associated with resistance to initial treatment in patients with KD.
  [Abstract] [Full Text] [PDF]  
Acral lesions in a pediatric population during the COVID-19 pandemic: a case series of 36 patients from a single hospital in Spain
  Pau Ros谷s-Gibert, Javier Gimeno Castillo, Amaia Saenz Aguirre, Francisco Javier De la Torre Gomar, Luc赤a Carnero Gonz芍lez, Zuriñe Martinez de Lagr芍n Alvarez de Arcaya, Ricardo Gonzalez-Perez
Background: To describe the cutaneous acral findings in a pediatric population noticed during this pandemic.
Methods: A retrospective descriptive study was performed collecting data on 36 patients under 14 years old, presenting suspicious acral skin manifestations for coronavirus disease 2019 (COVID-19).
Results: Patients were mostly male (63.8%). The mean age was 11.11 years. 66.67% of patients showed erythematous papules, and 44.44% purpuric macules. Feet were affected in 97.22% of patients and hands in 5.55%. Lesions were asymptomatic in 50% of patients. 30.55% of patients showed extracutaneous findings, preceding skin lesions in 12.62 days. Seven patients underwent specific severe acute coronavirus 2 (SARS-CoV-2) testing; all of these patients tested negative.
Conclusions: The association between these symptoms and SARS-CoV-2 remains unclear. We recommend using these manifestations as a sign of SARS-CoV-2 infection in children. This could lead to the examination of asymptomatic and mildly symptomatic children so that contagions may be avoided.
  [Abstract] [Full Text] [PDF]  
Letters to the editor:
Neonatal pemphigus vulgaris characterized by tensive blisters
  Guang-Wen Yin, Xiao-Yan Li
  [Abstract] [Full Text] [PDF]  
Season of birth influences on breastfeeding incidence and duration
  Claudia W. Strow, Brian K. Strow
  [Abstract] [Full Text] [PDF]  
Continuous positive airway pressure in children with severe pneumonia: a meta-analysis
  Zhi-Li Wang, Yu He, Zheng-Xiu Luo
  [Abstract] [Full Text] [PDF]  
Pediatric adenovirus gastroenteritis disease severity, viral co-infection and regional seasonal trends in Ireland
  Zakaria Barsoum
  [Abstract] [Full Text] [PDF]  
World Journal of Pediatric Surgery
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