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Vol 17, No 6
Vol 17, No 6 December 2021 ISSN 1708-8569
Review article
Original articles
Clinical image
Letters to the editor
Challenges and suggestions for precise diagnosis and treatment of Wilson*s disease
  Yi Dong, Zhi-Ying Wu
  [Abstract] [Full Text] [PDF]  
Everything that is not given is lost
  Javier Arredondo Montero, Carlos Bardaj赤 Pascual
  [Abstract] [Full Text] [PDF]  
Review article:
Application of topical gentamicin〞a new era in the treatment of genodermatosis
  Shan Wang, Zhou Yang, Ying Liu, Mu-Tong Zhao, Juan Zhao, Huan Zhang, Zong-Yang Liu, Xiao-Ling Wang, Lin Ma, Yong-Hong Yang
Background: The clinical use of gentamicin always lies in its antimicrobial activity in the past as an aminoglycoside antibiotic. However, in the past decade, there were considerable interests in therapeutic approaches in treating hereditary diseases. Some of the genodermatosis is caused by nonsense mutations that create premature termination codons and lead to the production of truncated or non-functional proteins. Gentamicin could induce readthrough of nonsense mutations and enable the synthesis of full-length proteins. We focus on previous publications on topical application of gentamicin and review its utility in genetic skin diseases.
Data sources: We search the MEDLINE through PubMed, EMBASE databases, and the Clinical Trials Registry Platform from January 1960 to July 2020 using the key search terms "gentamicin, topical gentamicin, genodermatosis, genetic skin diseases".
Results: The application of gentamicin in genodermatosis yielded promising results, both in vivo and in vitro, including Nagashima-type palmoplantar keratosis, epidermolysis bullosa, Hailey-Hailey disease, hereditary hypotrichosis simplex of the scalp, etc. CONCLUSIONS: Topical gentamicin is a potential treatment option for genodermatosis caused by nonsense mutation.
Conclusions: Topical gentamicin is a potential treatment option for genodermatosis caused by nonsense mutation.
  [Abstract] [Full Text] [PDF]  
Original articles:
Consensus statement on the epidemiology, diagnosis, prevention, and management of cow's milk protein allergy in the Middle East: a modified Delphi-based study
  Moustafa A El-Hodhod, Mortada H F El-Shabrawi, Ahmed AlBadi, Ahmed Hussein, Ali Almehaidib, Basil Nasrallah, Ebtsam Mohammed AlBassam, Hala El Feghali, Hasan M Isa, Khaled Al Saraf, Maroun Sokhn, Mehdi Adeli, Najwa Mohammed Mousa Al-Sawi, Pierre Hage, Suleiman Al-Hammadi
Background: This study aimed to develop an expert consensus regarding the epidemiology, diagnosis, and management of cow's milk protein allergy (CMPA) in the Middle East.
Methods: A three-step modified Delphi method was utilized to develop the consensus. Fifteen specialized pediatricians participated in the development of this consensus. Each statement was considered a consensus if it achieved an agreement level of ≡ 80%.
Results: The experts agreed that the double-blind placebo-controlled oral challenge test (OCT) should be performed for 2-4 weeks using an amino acid formula (AAF) in formula-fed infants or children with suspected CMPA. Formula-fed infants with confirmed CMPA should be offered a therapeutic formula. The panel stated that an extensively hydrolyzed formula (eHF) is indicated in the absence of red flag signs. At the same time, the AAF is offered for infants with red flag signs, such as severe anaphylactic reactions. The panel agreed that infants on an eHF with resolved symptoms within 2-4 weeks should continue the eHF with particular attention to the growth and nutritional status. On the other hand, an AAF should be considered for infants with persistent symptoms; the AAF should be continued if the symptoms resolve within 2-4 weeks, with particular attention to the growth and nutritional status. In cases with no symptomatic improvements after the introduction of an AAF, other measures should be followed. The panel developed a management algorithm, which achieved an agreement level of 90.9%.
Conclusion: This consensus document combined the best available evidence and clinical experience to optimize the management of CMPA in the Middle East.
  [Abstract] [Full Text] [PDF]  
Surges of hospital-based rhinovirus infection during the 2020 coronavirus disease-19 (COVID-19) pandemic in Beijing, China
  Ruo-Xi Zhang, Dong-Mei Chen, Yuan Qian, Yu Sun, Ru-Nan Zhu, Fang Wang, Ya-Xin Ding, Qi Guo, Yu-Tong Zhou, Dong Qu, Ling Cao, Chun-Mei Zhu, Lin-Qing Zhao
Background: A series of public health preventive measures has been widely implemented in Beijing to control the coronavirus disease-19 (COVID-19) pandemic since January 2020. An evaluation of the effects of these preventive measures on the spread of other respiratory viruses is necessary.
Methods: Respiratory specimens collected from children with acute respiratory infections were tested by NxTAG™ respiratory pathogen panel assays during January 2017 and December 2020. Specimens characterized as rhinoviruses (RVs) were sequenced to identify the RV species and types. Then, the epidemiology results of respiratory pathogens in 2020 were compared with those from 2017 to 2019 using SPSS statistics 22.0.
Results: The positive rates of adenovirus (ADV), influenza virus (flu), RVs, and respiratory syncytial virus (RSV) dropped abruptly by 86.31%, 94.67%, 94.59%, and 92.17%, respectively, from February to May 2020, compared with the average level in the same period during 2017-2019. Positive rates of RVs then steeply increased from June 2020 (13.77%), to an apex (37.25%) in August 2020, significantly higher than the average rates (22.51%) in August 2017-2019 (P = 0.005). The increase, especially in group ≡ 3 years, was accompanied by the reopening of schools and kindergartens after the 23rd and 24th week of 2020 in Beijing.
Conclusion: Whereas the abrupt drop in viral pathogen positive rates from February to May 2020 revealed the remarkable effects of the COVID-19 preventive measures, the sharp increase in positive rates of RVs from the 23rd week of 2020 might be explained by the reopening of schools and kindergartens in Beijing.
  [Abstract] [Full Text] [PDF]  
Muscle strength and its association with cardiometabolic variables in adolescents: does the expression of muscle strength values matter?
  Tiago Rodrigues de Lima, Xuemei Sui, Luiz Rodrigo Augustemak de Lima, Diego Augusto Santos Silva
Background: We investigate the association between different muscle strength (MS) indices with cardiometabolic variables in adolescents.
Methods: Cross-sectional study comprising 351 adolescents (male 44.4%, age 16.6 ㊣ 1.0 years) from Brazil. MS was assessed by handgrip strength and analyzed in five different ways: absolute MS and MS normalized for body weight, body mass index (BMI), height, and fat mass, respectively. Cardiometabolic variables investigated as outcomes were systolic and diastolic blood pressure (DBP), waist circumference (WC), high-sensitive C-reactive protein (hs-CRP), lipid and glucose metabolism markers. Multiple linear regression models adjusted for confounding factors were used.
Results: Absolute MS and/or MS normalized for height was directly associated with WC [up to 32.8 cm, standard error (SE) = 4.7] and DBP (up to 8.8 mmHg, SE = 0.8), and inversely associated with high-density lipoprotein cholesterol (up to -8.0 mg/dL, SE = 14.1). MS normalized for body weight, BMI or fat mass was inversely associated with WC (up to -17.5 cm, SE = 2.2). According to sex, MS normalized for fat mass was inversely associated with triglycerides (male: 0.02 times lower, SE = 0.01; female: 0.05 times lower, SE = 0.01) and homeostatic model assessment for insulin resistance (male: 0.02 times lower, SE = 0.01; female: 0.06 times lower, SE = 0.01), and inversely associated with hs-CRP only among male (0.03 times lower, SE = 0.01).
Conclusion: When normalized for body weight, BMI or fat mass, MS was superior to absolute MS or MS normalized for height in representing adequately cardiometabolic variables among adolescents.
  [Abstract] [Full Text] [PDF]  
Comparison of clinical outcomes between unrelated single umbilical cord blood and ※ex-vivo§ T-cell depleted haploidentical transplantation in children with hematological malignancies
  Carmen G車mez Santos, Marta Gonz芍lez Vicent, Blanca Molina, Natalia Deltoro, Blanca Herrero, Julia Ruiz, Antonio P谷rez Mart赤nez, Miguel A. Diaz
Background: Over the last two decades, umbilical cord blood (UCB) and haploidentical transplantation (HaploHSCT) have emerged as alternative sources of hematopoietic stem cell for allogeneic transplantation. There are few retrospective studies and no prospective studies comparing both types of alternative transplantation in pediatric patients.
Results: We analyzed the data of 134 children with hematological malignancies who received a hematopoietic stem cell transplantation from a single umbilical cord blood (UCB) (n = 42) or an "ex-vivo" T-cell depleted transplant from a haploidentical-related donor (HaploHSCT) (n = 92) between 1996 and 2014. Hematological recovery was faster after HaploHSCT than the UCB transplant group (median times to neutrophil and platelet recovery: 13 vs. 16 days, 10 vs. 57 days, respectively) (P < 0.001). The HaploHSCT group had a significantly early immune reconstitution based on NK and CD8+ T cells compared with the UCB group. However, after the first year post-transplantation, HaploHSCT had a lower number of CD4+ T and B lymphocytes compared with the UCB transplant recipients. The cumulative incidence of TRM was 29 ㊣ 8% in the HaploHSCT group versus 40 ㊣ 5% in the UCB group. Relapse incidence was 21 ㊣ 7% in the HaploHSCT group and 19 ㊣ 8% in the UCB group. Probability of DFS was 58 ㊣ 8% in the HaploHSCT group versus 40 ㊣ 9% in the UCB group (P = 0.051).
Conclusions: TCD haploidentical transplant is associated with advantages in terms of engraftment and early immune reconstitution kinetics. TCD haploidentical transplant was associated with lower incidence of infectious and non-infectious complications, especially in the early phases of the transplant compared with UCB transplant recipients. However, there are no advantages in transplant outcomes compared with UCB transplant.
  [Abstract] [Full Text] [PDF]  
Treatment of pediatric mild persistent asthma with low dose budesonide inhalation suspension vs. montelukast in China
  Zhi Min Chen, De Yu Zhao, Li Xiang, Jian Guo Hong
Background: There are limited studies comparing budesonide inhalation suspension (BIS) with montelukast in real-world settings where treatment adherence and persistency may be suboptimal. This real-world study aims to investigate the control effectiveness of montelukast or BIS as a monotherapy in Chinese children with mild asthma.
Methods: Data were derived from a retrospective questionnaire-based analysis of 2‒14-year-old children with mild persistent asthma, who received either 500 µg of BIS (n = 153) or 4‒5 mg of montelukast (n = 240) once daily. The indicators of asthma control, the Asthma Control Test (ACT)/Childhood ACT (C-ACT) score, and the asthma-related medical costs were assessed. The differences between the two groups were compared using an unpaired t-test (normally distributed), Mann每Whitney U test (non-normally distributed) or chi-squared test (categorical variables).
Results: Medication compliance in the past 3-month period was better in the montelukast group than in the BIS group (P = 0.042). The montelukast group exhibited better asthma control in the past 4-week period, including lower percentages of asthmatic children with symptoms more than twice a week (P = 0.021), had night waking or night coughing (P = 0.022), or required reliever medication more than twice a week (P<0.001). The montelukast group had a lower percentage of children with an ACT/C-ACT score≒19 (P=0.015). Caregivers reported a signifcantly better exercise tolerance in the children who received montelukast vs. BIS in the past 12 months (P><0.001). Signifcantly higher medical expenditures attributable to asthma in the past 12 months were observed in the BIS group vs. montelukast group (P> < 0.001). The montelukast group had a lower percentage of children with an ACT/C-ACT score ≒ 19 (P = 0.015). Caregivers reported a significantly better exercise tolerance in the children who received montelukast vs. BIS in the past 12 months (P<0.001). Signifcantly higher medical expenditures attributable to asthma in the past 12 months were observed in the BIS group vs. montelukast group (P> < 0.001).
Conclusion: Both treatments provided acceptable overall asthma control in children with mild persistent asthma; however, more reliever medication and more medical expenditures attributable to asthma were needed for BIS vs. montelukast in real-world settings, where factors such as compliance were also taken into account.
  [Abstract] [Full Text] [PDF]  
Haploidentical hematopoietic stem cell transplantation for pediatric patients with chronic active Epstein每Barr virus infection: a retrospective analysis of a single center
  Yan Hui Luo, Jun Yang, Ang Wei, Guang Hua Zhu, Bin Wang, Rui Zhang, Chen Guang Jia, Yan Yan, Kai Wang, Sidan Li, Xuan Zhou, Mao Quan Qin, Tian You Wang
Background: This study aimed to evaluate the feasibility and clinical effect of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for the treatment of pediatric patients with chronic active Epstein每Barr virus infection (CAEBV).
Methods: Children with CAEBV who did not have matched donors and underwent haplo-HSCT in Beijing Children*s Hospital, Capital Medical University, from October 2016 to June 2020 were analyzed retrospectively. Data relating to the clinical manifestations, engraftment, and prognosis of the children were extracted from medical records.
Results: Twenty-five patients, including 16 males and 9 females, with an onset age of 5.0 ㊣ 2.6 years and a transplantation age of 6.9 ㊣ 2.9 years, were enrolled in this study. The mean time from diagnosis to transplantation was 3.8 (2.0每40.2) months. The mean observation time was 19.0 ㊣ 12.0 months. Three patients received the reduced intensity conditioning regimen, and the remaining patients all received the modified myeloablative conditioning regimen. By the end of the follow-up, 23 patients were characterized by disease-free survival (DFS), 22 were characterized by event-free survival (EFS), and two died. One of the patients died of thrombotic microangiopathy (TMA), and another died of graft versus host disease (GVHD); this patient discontinued the treatment for economic reasons. The 3-year overall survival (OS) rate was estimated to be 92.0% ㊣ 5.4%, and the 3-year EFS rate was estimated to be 87.4% ㊣ 6.8%. All active patients survived after HSCT event-free. Acute GVHD degrees 1每3 were observed in ten patients (40.0%), and degree IV was observed in six (24.0%), who were all cured except for one patient. Chronic GVHD was observed in nine (36.0%), and most of these cases were mild. The incidence of TMA and veno-occlusive disease (VOD) was 28.0% and 4.0%.
Conclusions: Haploidentical hematopoietic stem cell transplantation is safe and effective in the treatment of pediatric CAEBV and can be used as an alternative therapy without matched donors or emergency transplantation. Patients with active disease before HSCT also benefited from haplo-HSCT. Haplo-HSCT requires careful monitoring for complications, such as GVHD and TMA. Early detection of TMA and timely treatment can reduce mortality and can improve the survival rate.
  [Abstract] [Full Text] [PDF]  
Vestibular function of pediatric patients with sudden sensorineural hearing loss: based on vertigo symptom and vestibular function testing
  Yan-Hong Li, Bing Liu, Yang Yang, Min Chen, Wei Liu, Jian-Bo Shao, Xiao Zhang, Jie Zhang, Xin Ni
Background: This study aimed to evaluate the relationship between vestibular and auditory functions in pediatric patients with sudden sensorineural hearing loss (SSHL).
Methods: A total of 30 pediatric patients experiencing unilateral SSHL between 5.9 and 13.0 years of age were enrolled in this study. Pure tone audiometry was evaluated prior to treatment initiation and again after one month of treatment. Prior to treatment initiation, vertigo symptoms were noted; then several vestibular function tests were conducted including caloric testing, ocular vestibular evoked myogenic potentials (o-VEMPs) and cervical vestibular evoked myogenic potentials (c-VEMPs). Associations between pre and post-treatment vestibular function and hearing threshold levels were analyzed.
Results: Initial hearing thresholds of children with vertigo were higher than thresholds of children without vertigo (92 dB vs. 79 dB, P = 0.033), while initial hearing thresholds of children with abnormal caloric test findings were higher than thresholds of children with normal caloric test findings (93 dB vs. 67 dB, P = 0.014). Cutoff values of hearing thresholds for those with vertigo symptoms and abnormal vestibular test results were 86.000- and 89.583-dB HL, respectively. Regarding prognosis, children with vertigo exhibited lower recovery rates than children without vertigo (33% vs. 75%, P = 0.025); recovery rates of children with abnormal caloric test results were lower than the overall recovery rate (25% vs. 73%, respectively, P = 0.039).
Conclusion: Assessments of vertigo symptoms and vestibular function are useful tools in predicting pediatric SSHL patient disease severity and prognosis.
  [Abstract] [Full Text] [PDF]  
Evaluation of a new frequency每volume chart for children with primary monosymptomatic nocturnal enuresis: a prospective, comparative study
  Jing-Jing Wang, Dong-Jie Wang, Hai-Dong Fu, De-Xuan Wang, Qing Yang, Xiao-Jie Peng, Rui Fu, Huan-Dan Yang, Rui-Feng Zhang, Xiao-Wen Wang, Jiang-Wei Luan, Fei Zhao, Qiu-Xia Chen, Fang-Fang Liang, Lu Cao, Jian-Jiang Zhang, Ying-Jie Li, Yang Dong, Hui-Mei Huang, Ying Bao, Xiao-Yu Shen, Wei Zhou, Guang-Hai Cao, Cui-Hua Liu, Yan-Yan Jin, Mei-Lin Ma, Li Jiang, Qian Shen, Hong Xu, Jian-Hua Mao
Background: To improve compliance with voiding diaries in children with primary monosymptomatic nocturnal enuresis (PMNE), a new modified 3-day weekend frequency每volume chart (FVC) was designed, and the compliance and validity of this modified FVC was evaluated by comparing with the International Children's Continence Society (ICCS) recommended voiding diary.
Methods: A total of 1200 patients with PMNE were enrolled in the study from 13 centers in China and were randomly assigned to record this modified FVC or the ICCS-recommended voiding diary. The primary outcome measure was the compliance, assessed by comparing the completing index and the quality score of diaries between two groups. The secondary outcome measure was the validity, evaluated by comparing the constituent of subtypes, micturition parameters and response rate to desmopressin.
Results: Among the 1200 participants enrolled in the study, 447 patients completed the ICCS-recommended voiding diary and 469 completed the modified diary. The diurnal completing index and the quality score of the modified FVC group were better than those of the ICCS group. In addition, there was no significant difference between these two groups in the subtype classification, or in the response rate to desmopressin.
Conclusions: The modified FVC could be applied to obtain the voiding characteristics of children with PMNE as the ICCS-recommended voiding diary does and offers a reasonable and better choice for children with PMNE from the unselected population in the future.
  [Abstract] [Full Text] [PDF]  
FMR1 allele frequencies in 51,000 newborns: a large-scale population study in China
  Jin-Yu Zhang, Ding-Wen Wu, Ru-Lai Yang, Lin Zhu, Meng-Yi Jiang, Wen-Jun Wang, Xue-Kun Li, Xiao-Ling Jiang, Fan Tong, Qiang Shu
Background: Fragile X syndrome (FXS), caused by CGG-repeat expansion in FMR1 promoter, is one of the most common causes of mental retardation. Individuals with full mutation and premutation alleles have a high risk of psychophysiological disorder and of having affected off spring. Frequencies of FMR1 alleles in general newborns have been reported in Caucasians but have not been investigated in the large-scale population in the mainland of China.
Methods: The sizes of FMR1 CGG-repeats were analyzed in 51,661 newborns (28,114 males and 23,547 females) and also in a cohort of 33 children diagnosed with developmental delay using GC-rich polymerase chain reaction (PCR) and triple repeat primed PCR.
Results: The frequency of CGG repeats > 100 was 1/9371 in males and 1/5887 in females, and the frequency of CGG repeats > 54 was 1/1561 in males and 1/1624 in females. FMR1 full mutation and premutation were identified in 27.27% of children who had Ages and Stages Questionnaire scores less than two standard deviations from the cutoff value.
Conclusions: Our study revealed the prevalence of FXS in China and improved the sample databases of FXS, suggesting that the prevalence of FXS in Chinese is higher than estimated previously and that FXS screening can be advised to high-risk families.
  [Abstract] [Full Text] [PDF]  
AST-to-ALT ratio and coronary artery lesions among patients with Kawasaki disease
  Lei Cao, Yun-Jia Tang, Miao Gang, Jin Ma, Wei-Guo Qian, Qiu-Qin Xu, Hai-Tao Lv
Background: The aim of this study was to explore the associations between the aspartate aminotransferase-to-alanine aminotransferase ratio (AST/ALT) and coronary artery lesions (CALs) among patients with Kawasaki disease (KD).
Methods: Medical records of KD patients presenting to a single center between January 2019 and December 2020 were retrospectively collected and analyzed. Univariate, multivariable-adjusted analyses, subgroup analyses, restricted cubic spline test, and fitted curves were used to evaluate the associations between AST/ALT and CALs.
Results: A total of 831 patients were enrolled, of which 201 (24.2%) had CALs on admission and 21 (2.5%) developed CALs de novo after intravenous immunoglobulin (IVIG). Multivariable-adjusted analyses models revealed that a lower AST/ALT was associated with an increased risk of CALs on admission when AST/ALT was a continuous variable (P = 0.007) and when it was a categorical variable (P for trend = 0.004). Each unit increase in AST/ALT was associated with a 22% lower risk of CALs on admission (odds ratio = 0.78, 95% confidence interval 0.65-0.94). A negative linear relationship was noted between AST/ALT and the risk of CALs on admission in both observed and fitted models. However, such associations were not observed in AST/ALT and CALs de novo after IVIG. None of the variables significantly modified the association between AST/ALT and CALs on admission and CALs de novo after IVIG (P > 0.05).
Conclusion: Our findings suggested that AST/ALT was a risk factor of CALs, but was not associated with progressive CALs.
  [Abstract] [Full Text] [PDF]  
Clinical image:
Infant eczema or insect dermatitis? Dermoscopy solved the mystery
  Xiao-Xi Xu, Lu Zheng, Yu-Ping Ran
  [Abstract] [Full Text] [PDF]  
Letters to the editor:
Circulating immunocompetent cell profiles during oral cyclosporine therapy for immunoglobulin-resistant Kawasaki disease
  Seigo Okada, Yuji Ohnishi, Takashi Furuta, Yasuo Suzuki, Akiko Kawakami-Miyake, Chie Matsuguma, Takako Waniishi, Hiroki Yasudo, Shunji Hasegawa
  [Abstract] [Full Text] [PDF]  
Importance of excluded duplicates reporting in a systematic review
  Hong-Xi Zhang, Jiang-Hui Cai
  [Abstract] [Full Text] [PDF]  
World Journal of Pediatric Surgery
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