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06 April 2018, Volume 33 Issue 4 Previous Issue    Next Issue

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Emphasis on children with visual impairment related to neurological diseases ZOU Li-ping 2018, 33(4): 241-245.  DOI: 10.19538/j.ek2018040601 Abstract ( )   When children develop neurological conditions associated with ocular symptoms，we need to know that they are different from those of adults. Children are often likely to have congenital and neoplastic diseases more than other types of diseases. It is easier and more important to recover and establish visual function in children as soon as possible if found and diagnosed early and quickly. This article is mainly about the diseases related to the damaged eye incoming system in children，such as optic neuritis and optic neuromyelitis spectrum disorders，hereditary optic neuropathy（Leber disease，Kjer optic neuropathy，Wolfram syndrome），ONH，and so on. Because optic and nervous systems are still developing in children，and children can not accurately describe their symptoms，often there is a delay in diagnosis and the loss of vision. Therefore，in clinical practice early detection and early diagnosis and treatment of neurological disorders related to visual impairment in children are needed in order to minimize visual acuity or permanent blindness in children.

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Progress in diagnosis and treatment of neuromyelitis optica spectrum disorders in children TENG Zi-teng，WANG Hua 2018, 33(4): 246-251.  DOI: 10.19538/j.ek2018040602 Abstract ( )   At present，the people are still in the understanding and research stage of the pathogenesis，clinical manifestation，diagnosis，treatment and prognosis of children with Neuromyelitis spectrum disorder（NMOSD）. In many aspects of the disease，there is still some controversy and no consensus. However, the prognosis of NMOSD is poor，and severe cases can cause permanent irreversible damage to the central nervous system. Therefore，early identification，diagnosis and treatment of the disease have important clinical and social significance for children’s prognosis and long-term quality of life.

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Ophthalmological manifestations and genetic characteristics in mitochondrial disease REN Chang-hong，FANG Fang 2018, 33(4): 252-257.  DOI: 10.19538/j.ek2018040603 Abstract ( )   Mitochondrial diseases is one of the most common genetic metabolic diseases in childhood，which involve multiple systems，typically affecting high-energy-demand systems. Ophthalmological findings in mitochondrial disease are very common，and sometimes are the first and only manifestations. This paper introduces the ophthalmologic symptoms，genetic characteristics and therapy in several common mitochondrial syndromes.

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Classification and diagnosis of neuronal ceroid lipofuscinoses ZHANG Jing，ZHANG Yue-hua 2018, 33(4): 257-261.  DOI: 10.19538/j.ek2018040604 Abstract ( )   Neuronal ceroid lipofuscinoses（NCL） is a rare neurodegenerative disease. The clinical manifestations of this disease are complex，mainly including multiple types of seizures，myoclonus，cognitive and motor delay or regressions；visual loss is common. Brain imaging indicates cerebral cortex and cerebellar atrophy. The clinical classification of NCL includes congenital，infantile，late-infantile，juvenile and adult types. Combined with the age of onset and genotype，the latest classification includes 14 types（CLN1-CLN14）. The genotype and phenotype of the disease are not fully matched. The approach to diagnosis should consider clinical features and age of onset. Related enzyme testing，genetic detection，skin and muscular pathological examination should be done for diagnosis. No effective treatment has been found so far，and the current treatment is mainly symptomatic.

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Several common inflammatory diseases affecting visual function in children PU Wei，ZHAO Dai-xin 2018, 33(4): 261-264.  DOI: 10.19538/j.ek2018040605 Abstract ( )   Orbital cellulitis，Vogt-Koyanagi-Harada syndrome，Behcet disease and neuromyelitis optica are common inflammatory diseases in children which may cause severe damage to visual function. Clinical manifestation，diagnosis and treatment are discussed in this article. In addition to detailed clinical data，making correct diagnosis and giving proper treatment according to relative criteria and panel are also very important to the healthy development of children’s visual function.

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Diagnosis and examination of visual impairment in children CUI Li-hong，YAO Cong 2018, 33(4): 265-267.  DOI: 10.19538/j.ek2018040606 Abstract ( )   Visual impairment in children is considered to be an area of priority because of its long term of disability and its influence on development. Early diagnosis of visual impairment in children is very important；visual defects should be discovered as soon as possible and timely treatment should be given in order to obtain maximum vision. It is difficult for children，especially infants，to cooperate in the complicated vision examinations. Therefore，the diagnosis and examination of children’s visual impairment should be based on the age and development phase. The diagnosis and examination of children’s visual impairment can be made by objective methods. The population of visually impaired children includes a substantial proportion of children with additional systemic disorders or damage. Paediatricians and other paediatric professionals play a key role in early detection and multidisciplinary management.

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Efficacy of rituximab therapy for relapsed opsoclonus myoclonus syndrome CHEN Hui*，TIAN Xiao-juan，ZHANG Yue-hua，et al 2018, 33(4): 268-271.  DOI: 10.19538/j.ek2018040607 Abstract ( )   Objective　To summarize the efficacy of rituximab therapy for the relapsed opsoclonus myoclonus syndrome （OMS）. Methods　Analyze the clinical data collected from 5 patients with the relapsed OMS treated with rituximab between 2012 and 2015 at Peking University First Hospital. Results　The onset age ranged from 13 months to 20 months （mean age 16.8 months）. The course of the disease ranged from 10 months to 31 months on admission（average duration 16.8 months）. Before the rituximab therapy，all of them received hormone alone or hormone combined with high dose immunoglobulin therapy，but the disease relapsed due to a poor efficacy. Five patients were treated with rituximab. The follow-up time was 14 months to 46 months. The clinical symptoms of 4 patients were controlled. In 1 case the disease relapsed during follow-up，but the clinical symptoms were improved. There were no severe side effects during medication period or follow-up stage. Conclusion　Our data have shown that rituximab tends to be a safe and effective treatment for the replapsed OMS. An RCT with larger samples is necessary in the future.

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Study on the establishment of prediction model of dynamic eye tack system for children with autism spectrum disorder SUN Bin-bin，HE Hui-jing， JIANG Wen， et al 2018, 33(4): 272-275.  DOI: 10.19538/j.ek2018040608 Abstract ( )   Objective　To understand the characteristics of eye tracking in children with autism spectrum disorder（ASD）， to provide objective indicators for early diagnosis of ASD，and to establish a diagnostic predictive model which is suitable for clinical diagnosis. Methods　The subjects of the study were 38 children with ASD（22.03±4.97 months old） and 35 children （21.38±4.36 months old） with developmental normal children（TD group）. Visual stimulation is a dynamic video of geometries and social figures，for 2 minutes with Eyelink1000 plus eye tack instrument. The ASD group and the TD group indexes were analyzed by ndependent-samples T-test and Chi-square Test，showed that the factors affecting the difference was statistically significant the regression analysis of the two Logistic classification，based on the children with ASD eye tacking prediction model and dynamic scene video scoring system. The receiver operating haracteristic curve（ROC curve） and the area under the curve（AUC） model is used to predict the accuracy of self display of children with ASD. Results　73 children were analyzed with single factor and Logistic regression analysis，we selected 4 significant predictive indexes of children with ASD：（DGI）fixation time，and（DGI） numbers of fixations，the percentage of fixation time on DGI times，and the numbers of fixations on DSI. Regression equation In（P/1-P）＝β0+β1X1+β3X3+β5X5+β6X6＝13.03+1.01X1+0.25X3-98.72X5-0.55X6， which β0＝13.03 is a constant term，βi is regression coefficient. The self built model predicts AUC for children with ASD was 0.713. Conclusion　Self built model is of great value in predicting children with ASD.

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Analysis of clinical features and related factors of epilepsy in children with attention deficit hyperactivity disorder WANG Long-fei*，HU Chun-hui，WANG Hua 2018, 33(4): 276-280.  DOI: 10.19538/j.ek2018040609 Abstract ( )   Objective　To analyse clinical features of attention deficit hperactivity disorder（ADHD） and its relationships with factors related to epilepsy. Methods　The clinical data of 180 children diagnosed with epilepsy in Pediatric Neurology Department of Shengjing Hospital between September 2015 and February 2016 were obtained and analyzed. 125 healthy children in the same district served as control. Results　The morbidity of attention deficit hyperactivity disorder in children with epilepsy was 28.89%. The morbidity was 7.2% in the controls. The difference was statistically significant （P＜0.05）. Among the risk factors of epilepsy， the gender，seizure times and a single oral AEDS had no effect on the morbidity of ADHD. The morbidity was connected with an earlier onset age，GTCS，an unrecovered EEG and multiple AEDS of epilepsy. The difference was statistically significant （P＜0.05）. The children with epilepsy had a higher score by the evaluation of Conners Scale. The behavior，study and the impulsive-hyperactive behavior problems were more obvious among epilepsy children with attention deficit hyperactivity disorder（P＜0.05）. Conclusion　The incidence of ADHD is high in children with epilepsy，and the initial onset age of epilepsy，the unrecovered EEG and various antiepileptic drugs are closely related with the prevalence of ADHD in children with epilepsy. In children with epilepsy and ADHD，the behavior problems，learning problems and hyperactivity-impulsive aspects are more significant.

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Application of modified pediatric malnutrition risk screening tool in hospitalized children SHENG Jin-ye*，MAO Xiao-meng，LU Li-na，et al 2018, 33(4): 281-285.  DOI: 10.19538/j.ek2018040610 Abstract ( )   Objective　To use the modified pediatric malnutrition risk screening tool to investigate the prevalence of malnutrition in hospitalized children and assess the clinical effectiveness of the screening tool；to provide the basis for nutritional assessment and reasonable nutrition support. Methods　Choose hospitalized children in Xinhua Hospital Affiliated to Shanghai Jiao Tong University of Medicine from August 2014 to September 2015 as the investigation subjects. The modified pediatric malnutrition risk screening tool was used to further explore the relationship between results of nutritional risk screening and the clinical outcome. Results　The screening results showed that the total incidence of malnutrition in 2632 hospitalized children was 53.2%. The younger the hospitalized children were，the higher the incidence of malnutrition risk was（χ2＝59.89，P＜0.05）. There was statistical difference among children with different diseases in incidence of malnutrition risk（χ2＝425.12，P＜0.05），and the incidence of malnutrition was higher in the alimentary system disease group（70.1%） and hematological malignancies group（86.7%） than in other disease groups. In the absence of nutritional support，the length of hospital stay of children with malnutrition was significantly longer（Z＝-9.293，P＜0.05），and there was a significant difference in the outcome of the disease（χ2＝4.937，P＜0.05）；the incidence of complications during hospitalization had a rising trend，but the difference was not statistically significant（χ2＝2.203，P＞0.05）. Conclusion　The results suggest that the modified pediatric malnutrition risk screening tool can effectively screen out the risk of malnutrition in hospitalized children， with better clinical predictability.

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Clinical analysis of 38 children with hypokalemic renal tubular diseases KANG Yu-lin，FENG Dan，ZHENG Wei-hua，et al 2018, 33(4): 286-290.  DOI: 10.19538/j.ek2018040611 Abstract ( )   Objective　To analyze the clinical features and the results of genetic diagnosis in children with hypokalemic renal tubular diseases. Methods　The clinical data of 38 patients with hypokalemic renal tubular diseases were analyzed retrospectively，who were treated in Children’s Hospital Affiliated to Shanghai Jiao Tong University from Jan. 2010 to Jan. 2016. Results　Totally 38 patients with hypokalemic renal tubular diseases were enrolled in this study. There were 18 cases of renal tubular acidosis（RTA） including 17 cases of typeⅠRTA and 1 case of typeⅡ RTA. There were 11 cases of Bartter syndrome，5 cases of Gitelman syndrome and 4 cases of Fanconi syndrome. The common clinical manifestations of hypokalemic renal tubular diseases included myasthenia，nausea，vomiting，polydipsia，polyurine and growth retardation. One case of Fanconi syndrome progressed to chronic Kidney disease（phase Ⅲ），while the other children had normal renal function. Glomerular proteinuria was found in 1，1 and 3 children with Bartter syndrome，Gitelman syndrome and Fanconi syndrome，respectively. Additionally，1 case with Fanconi syndrome has tubular proteinuria. However，urinary trace proteins associated with glomerular and tubular injury commonly elevated in these hypokalemic renal tubular diseases. Genetic analysis showed a new potential heterozygous mutations of ATPV0A4 in typeⅠRTA and three heterozygous mutations of SLC12A3 in Gitelman syndrome. Conclusion　The clinical symptoms vary in patients and are featured mainly by myasthenia，nausea，vomiting，polydipsia，polyurine and growth retardation. Glomerular and tubular injuries are commonly found in hypokalemic renal tubular diseases. Moreover，genetic diagnosis may be helpful in diagnosis，treatment and genetic counseling.

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Efficiency of eradication therapy for Helicobacter pylori infection in children CHEN Yi，YU Jin-dan，ZHAO Hong，et al 2018, 33(4): 291-296.  DOI: 10.19538/j.ek2018040612 Abstract ( )   Objective　To understand the clinical eradication therapy for Helicobacter pylori infection in children and analyze the causes. Methods　H.pylori eradication therapy for 10 or 14 days was accepted by 301 cases of children，who were diagnosed with H.pyori infection，in the Children’s Hospital of Zhejiang University School of Medicine from January，2012 to October，2015.  Finally，the eradication rate of H.pylori was judged. Results　In initial treatment，the eradication rate of OAC program（omeprazole，amoxicillin and clarithromycin） was 42.3%，the eradication rate of non-OAC program was 56.7%，and there were statistically significant differences between the two groups（P＜0.05）. The eradication rate of bismuth-based regimens was 50.0%，the eradication rate of standard triple therapy was 41.2%，and there were no statistically significant differences between the two groups（P＞0.05）. In the rescue treatment，the eradication rate of bismuth-based quadruple therapy was 58.3%，the eradication rate of triple therapy with furaxone was 55.0%，and there were no statistically significant differences between the two groups（P＞0.05）. Totally 119 strains of H.pylori were not resistant to amoxicillin，while the resistance rate to clarithromycin and metronidazole was 27.7% and 69.3%，respectively. Conclusion　In this study，the clinical eradication rate in children with Hp infection fails to achieve the goal eradication rate of 80%. Drug sensitivity test is still the first choice for treatment of Helicobacter pylori infection in children.

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Characteristics of cold executive functions in children with attention deficit hyperactivity disorder CHENG Qiu-ying*，YANG Bin-rang，ZENG Hong-wu，et al 2018, 33(4): 297-301.  DOI: 10.19538/j.ek2018040613 Abstract ( )   Objective　To explore the characteristics of cold executive function（response inhibition and working memory） in children with attention deficit hyperactivity disorder（ADHD）. Methods　Ninety-two boys with ADHD and twenty-one healthy control subjects received assessment of inhibition functions（go/no-go task and Stroop color-word test） and working memory functions（Corsi block recall task，visual spatial N-back，digits span test and letter-number sequencing）. These children with ADHD were compared with healthy control subjects by t-test or Mann-Whitney rank. Then they were compared with healthy controls after controlling for FIQ by using analysis of covariance（ANCOVA）. Results　There were significant differences in all task between ADHD groups and healthy controls（P＜0.05）. The differences of each test showed that children with ADHD performed worse than healthy controls in go /no-go，Stroop color-word test，Corsi block recall task，visual spatial N-back，digits span test and letter-number sequencing（P＜0.05）. Conclusion　There are significant deficiencies in cold executive function in children with ADHD.

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Clinical analysis of brucellosis in 128 children SUN Mei-yan，QI Qi-ge 2018, 33(4): 302-305.  DOI: 10.19538/j.ek2018040614 Abstract ( )

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Macrophage activation syndrome：A critical illness of chronic rheumatic diseases CHEN Xiang-yuan，ZENG Hua-song，ZENG Ping 2018, 33(4): 306-308.  DOI: 10.19538/j.ek2018040615 Abstract ( )

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Research progress in alternating hemiplegia in children LI Shu-pin，ZHANG Yue-hua 2018, 33(4): 309-314.  DOI: 10.19538/j.ek2018040616 Abstract ( )

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Research progress in the clinical significance and detection methods of bilirubin HAN Mei-lin，GONG Zhen-hua 2018, 33(4): 315-318.  DOI: 10.19538/j.ek2018040617 Abstract ( )

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Case report of Guillain-Barre syndrome spectrum diseases in a child with chronic lymphocytic thyroiditis YU Shu-jie，WANG Chun-yu，QI Ying，et al 2018, 33(4): 319-320.  DOI: 10.19538/j.ek2018040618 Abstract ( )