Table of Content

15 November 2015 Volume 33 Issue 11

  

Understanding clinico-radiologic syndromes, carrying out deeper studies　

ZOU Liping

. 2015, 33(11):  921.  doi:10.3969 j.issn.1000-3606.2015.11.001

Abstract ( 363 )   PDF (1049KB) ( 436 )  

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The diagnosis of clinico-radiologic syndromes is based on a set of characteristics of comprehensive evaluation of the factors such as cranial imaging and clinical manifestations. In this article, we focus on the three clinico-radiologic disorders (posterior reversible encephalopath syndrome, clinically mild encephalitis/encephalopathy with a reversible splenial lesion of corpus callosum, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids). In the present issue of the Journal, the cases has been reported. Although magnetic resonance is a revolution in medical imaging, but for doctors, imaging technology will never replace the detailed clinical history and physical examination. Their combination will improve to study the causes and pathogenesis of disorders. Early diagnosis and early treatment will avoid or reduce neurological sequelae of patients.

Clinical analysis of reversible splenial lesion syndrome in children

　ZHANG Xinying, SUN Wenxiu, GAO Yuxing, MA Aihua, WEN Zhaochun, XI Jiashui, WANG Xueyu

. 2015, 33(11):  924.  doi:10.3969 j.issn.1000-3606.2015.11.002

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　Objective　To explore the clinical and imaging features of reversible splenial lesion syndrome (RESLES) in children. Methods　The clinical and imaging data of 11 children with RESLES admitted from December. 2011 to May 2015 were retrospectively analyzed. Results　A total of 11 children, 7 males and 4 females with an average age of (6.24±1.41) years (8 months to 11 years old), were found 8 RESLES type-1 and 3 RESLES type-2. The common causes were convulsion and infection. Excep the manifestations of primary diseases, disturbance of consciousness was the prominent clinical manifestations, other neurological manifestations were mild or absent. Cranial magnetic resonance image (MRI) showed that the splenium of corpus callosum with isolated oval lesions in 8 cases with RESLES type-1, and the lesions involved the whole corpus callosum, periventricular and centrum ovale white matter in 3 cases with RESLES type-2. All the lesions showed low or equal signal on T1 weighed images, high signal on T2 weighed images, FLAIR images, and diffusion weighed images, low signal on apparent diffusion coefficient. The original lesions were completely disappeared on the follow-up MRI (interval of 4-30 days). Follow-up of 2 months to 3 years and 6 months, the cranial MRI were normal. Conclusions　The etiology of RESLES in children is complex, no specific clinical manifestations. The diagnosis mainly depends on the cranial MRI, especially the diffusion weighted images, showing the symmetry lesions on splenium of corpus callosum or with bilateral white matter. The prognosis of EESLES is good. Excessive treatment should be avoided.

Clinico-radiological characteristics of children with mild encephalitis/encephalopathy with a reversible splenial lesion

　SUN Dan, WANG Fang, LIU Zhisheng, HU Jiasheng

. 2015, 33(11):  929.  doi:10.3969 j.issn.1000-3606.2015.11.003

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Objectives　To investigate the clinico-radiological characteristics of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) in children. Methods　The clinical data from 8 children with MERS were retrospectively reviewed. Results　The clinical manifestations of 8 children were 7 cases with serizures, 5 cases with disturbance consciousness. The clinical symptoms disappeared within one week. The underlying diseases were mainly infections in respiratory and digestive tracts. Serum sodium levels <135 mEq/L were observed in 3 cases. According to imaging, 7 children were diagnosed MERS I with lesion only involving the splenium of the corpus callosum, one child was diagnosed MERS II with lesion extended from the splenium of the corpus callosum to the white matter of semi-oval. The lesion had completely disappeared in all children at follow-up MRI exams between 6 days and 1 month after the initial MRI. Conclusion　MERS exhibits acute mild encephalitis/encephalopathy symptoms, which is a clinico-radiological syndrome with good prognosis.

A report of one pediatric patient of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and literature review

XIE Meng, ZOU Liping, MENG Yan, MA Lin

. 2015, 33(11):  933.  doi:10.3969 j.issn.1000-3606.2015.11.004

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Objective　To discuss the clinical and imaging features of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Methods　Clinical characteristics, imaging features, treatment, prognosis, and follow up data from one pediatric patient diagnosed CLIPPERS were analyzed retrospectively. Results　 Female, 12 years old, presented with limb weakness, gait ataxia, and right visual field defect. Neurological signs mainly presented with oculomotor palsies, nystagmus, the left facial nerve palsy and cerebellar ataxia. Brain magnetic resonance imaging (MRI) showed appearance with punctate and curvilinear gadolinium enhancement 'peppering' the pons, medulla oblongata, bilateral arm, and the left side of the midbrain. After treatment with steroids, the clinical and imaging manifestations were rapidly improved. Conclusions　Pediatric patient of CLIPPERS may have more atypical presentations. The diagnosis of CLIPPERS mainly depends on imaging and treatment response.

Clinical characteristics of anti N-methy-D-aspartate receptor encephalitis in children and a literature review

　FANG Qiong, CHEN Lang, CHEN Qiaobing, ZHONG Rirong, CHEN Hui, CHEN Jie, YANG Fang

. 2015, 33(11):  937.  doi:10.3969 j.issn.1000-3606.2015.11.005

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Objectives　To investigate the clinical characteristics of children with anti N-methy-D-aspartate receptor (NMDAR) encephalitis. Methods　The clinical data from 6 children with anti-NMDAR encephalitis were retrospectively analyzed. The related literatures of anti-NMDAR encephalitis were reviewed. Results　The age of 6 children with anti-NMDAR encephalitis were from 2 to 10 years old, 5 were males and 1 was female. Three children had prodromal symptoms. All 6 children had mental and behavior abnormalities and convulsions, 2 cases of ataxia, 2 cases of extrapyramidal symptoms, 2 cases of orofacial dyskinesia, and 3 cases of dysautonomia. There was no abnormality of routine examination of cerebrospinal fluid in all 6 children. The oligoclonal protein antibody in serum and cerebrospinal fluid were all positive in 5 children, and negative in serum and positive in cerebrospinal fluid in one child. The anti-NMDAR in serum and cerebrospinal fluid were positive in all 6 children. Four children had nonspecific abnormal brain MRI. EEG showed diffuse slow wave, and laced with epileptic form discharges in all 6 children. Non-convulsive status epilepticus was monitored in one child. Tumors were not detected in all 6 children. After diagnosis, one child was lost follow up, 5 children were treated by methylprednisolone and gamma globulin, and 4 children received antiepileptic treatment. After 1 year follow-up, three children were with no obvious complications, and 2 children had complications. Conclusions　Children with anti-NMDAR encephalitis would be hinted by the clinical manifestations such as mental and behavioral abnormalities, seizures, dyskinesia, dysautonomia, etc. Detecting the NMDAR antibody in the serum and cerebrospinal fluid may help the diagnosis.

Evaluating the quality of life of children with epilepsy

　LI Xingguang

. 2015, 33(11):  942.  doi:10.3969 j.issn.1000-3606.2015.11.006

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Objective　To investigate the quality of life in children with epilepsy. Methods　Pediatric quality of life inventory measurement models generic core scale (PedsQL) 4.0 was used to evaluate the quality of life in hospitalized children with epilepsy in past three years. At the same time, the healthy children were selected as controls. Results　A total of 152 children with epilepsy had been enrolled, 92 boys and 60 girls, with an average age of (6.79 ± 2.78) years, Another 160 healthy children were selected, 95 boys and 65 girls, with an average age of (7.25±2.45) years. The Cronbach internal consistency coefficient of PedsQL 4.0 was 0.87. The factor analysis presented four dimensions, including physical function, social function, emotional function and school performance, consisting with the original created scale. The score of the quality of life in children with epilepsy was (57.24±4.31), significantly lower than that in healthy children (89.74±9.31) (t=2.71, P<0.05). Conclusions　PedsQL 4.0 can be used to evaluate the quality of life in Chinese children with epilepsy. The quality of life of children with epilepsy is poor.

Clinical analysis of 124 cases of hepatolenticular degeneration in children

CHU Anzhen,HOU Ling,LIANG Yan,XU Sanqing, LUO Xiaoping

. 2015, 33(11):  946.  doi:10.3969 j.issn.1000-3606.2015.11.007

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Objectives　To analyze the clinical characteristics of children with hepatolenticular degeneration. Methods　 The clinical features, laboratory and imaging data of 124 children with hepatolenticular degeneration diagnosed in the past thirteen years were retrospectively analyzed. Results　The mean onset age was (9.6±2.7）years and the mean age at diagnosis was (10.1±2.7) years. Clinical classification shows 75 cases (60.5%) of hepatic type, 19 cases (15.3%) of neurological type, 20 cases (16.1%) of mixed type, and 10 cases (8.1%) of others types. The onset age of hepatic type was significantly younger than that of the rest three types (P<0.05). The positive rate of corneal Kayser-Fleischer rings was 82.1% (92/112). The abnormality rate of ceruloplasmin was 98.4% (122/124). Among 115 children had testing of 24 h urine copper, the abnormality rate was 96.5% (112/115). The positive family history accounted for 39.5% (49/124). Conclusions　The clinical characteristics of children with hepatolenticular degeneration are diverse, which have different symptoms at onset, also changed with ages. The screening test should be applied in suspected children for early diagnosis and management.

Correlations among child abuse, personality, and defense style in middle school students

WU Guoping, ZHANG Yijie, PU Jianwen, LI Zhongcai

. 2015, 33(11):  950.  doi:10.3969 j.issn.1000-3606.2015.11.008

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Objectives　To explore the correlations among child abuse, personality, and defense style in middle school students. Methods　The study adopted systematic stratified sampling questionnaire survey. A total of 1115 middle school students from grade 1 to 3 in 3 middle schools in Kunming were assessed by the Screen Questionnaire of Child Abuse (SQCA). Among them, 212 students were assessed as child abuse (CA group). Another 212 students were selected as control group from non-abuse students, who were matched in sex, age, grade and class with CA group. Children from two groups were tested by the Childhood Trauma Questionnaire (CTQ), Eysenck Personality Questionnaire (EPQ) and Defense Style Questionnaire (DSQ). Results 　Compared with control group, all scores of each factor of CTQ were higher in CA group (P<0.05), the scores of psychoticism and neuroticism of EPQ were higher in CA group (P<0.05), the score of lie was lower in CA group (P<0.05), and the score of immature defense style of DSQ was higher in CA group (P<0.05). In correlated analysis, in CA group, the emotional abuse of CTQ was positively correlated with psychoticism and neuroticism of EPQ and negatively correlated with lie of EPQ, the physical abuse of CTQ was positively correlated with psychoticism and negatively correlated with lie of EPQ, the sexual abuse of CTQ was positively correlated with psychoticism and negatively correlated with lie of EPQ, and the emotional neglect was positively correlated with psychoticism and neuroticism and negatively correlated with lie of EPQ. There were no correlations between the factors of SQCA and the factors of DSQ. Conclusions　The personality of middle school students with childhood abuse were different from non-abuse students, who used more immature defense style. Personality characteristics of middle school students had some correlations with childhood abuse. There was no correlation between defense style and childhood abuse.

Gastric intramucosal pH values in assessment of gastric mucosal lesions in infants and toddlers with severe pneumonia

WANG Hailei,MA Na

. 2015, 33(11):  955.  doi:10.3969 j.issn.1000-3606.2015.11.009

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Objectives　To evaluate the application of gastric intramucosal pH values in assessment of the gastric mucosal lesions in infants and toddlers with severe pneumonia. Methods　One hundred and twenty (120) infants and toddlers with severe pneumonia were recruited. All subjects were treated with pressure support - synchronized intermittent mandatory ventilation according to the condition. The blood gas analysis and gastric carbon dioxide partial pressure (PgCO2) detection were performed at 15 minutes before ventilation and l h, 6 h, 12 h, 18 h, 24 h after ventilation, and the gastric intramucosal pH were calculated. Results　With prolonged ventilation, the gastric intramucosal pH, PaO2 and PaO2/fraction of inspired oxygen (FiO2) were significantly increased, while the (PgCO2-PaCO2) and blood lactic acid were significantly decreased. The (PgCO2-PaCO2), PaO2, PaO2/FiO2, and blood lactic acid began to return normal at 18 h after ventilation, and close to normal at 24 h after ventilation. The gastric intramucosal pH was still significantly lower than normal at 24 h after ventilation, and return to normal at 36 h after ventilation. There were negative correlations between gastric intramucosal pH with (PgCO2-PaCO2) and blood lactic acid (r=-0.30, -0.23, P<0.05), and positive correlations between gastric intramucosal pH with PaO2 and PaO2/FiO2 (r=0.26, 0.16, P<0.05). Conclusion　Gastric intramucosal pH can be served as a sensitive and accurate indicator for gastric mucosal lesions of severe pneumonia in infants and toddlers who need mechanical ventilation.

Clinical features and treatment of refractory Mycoplasma pneumoniae pneumonia in children

SHAO Xinhuan, LI Qianqian, XIANG Zhiwei, LUO Yanfeng, AN Yuqing

. 2015, 33(11):  958.  doi:10.3969 j.issn.1000-3606.2015.11.010

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Objective　To analyze the clinical features and treatment of refractory Mycoplasma pneumoniae pneumonia (RMPP). Method　Retrospective analysis was performed on the clinical data of 193 children with MPP. According to the reaction to the initial administration of erythromycin 20-30 mg/(kg•d) for 7 days, the subjects were divided into general group and refractory group. The clinical manifestations, laboratory examination and imaging characteristics of two groups were compared. The critical values of each independent factors related with RMPP had been detected. Results　In 193 subjects, 35 (18.1%) subjects had no response to erythromycin treatment were recruited into the refractory group, while another 158 cases with responseinto the general group. Between two groups, the duration of fever, percentage of neutrophils (N), C-reactive protein (CRP), serum ferritin (SF), lactic dehydrogenase (LDH), creatine kinase isoenzyme (CK-MB) were statistically significant (P<0.01). In general group, 70 subjects (44.3%) showed the large consolidation in single lobe of lung, 80 subjects (50.6%) showed multiple cloudiness shadow in unilateral or bilateral lung fields, and 8 subjects (5.1%) showed bilateral pulmonary bronchitis. In refractory group, 31 subjects (88.6%) showed the large consolidation in single lobe of lung, 4 cases (11.4%) showed bilateral pulmonary bronchitis. The proporttion of large consolidation in lung was significantly dirrerent between two groups (χ2=22.51,P<0.05) . In refractory group, the scope of consolidation in lung expanded and over the two-thirds of lung after 7 days. Calculated by the area under the ROC curves, the CRP, SF, LDH, and N were the prediction indicators of erythromycin treatment with no response. The cut off value was 51 mg/L, 258 g/L, 353 IU/L, and 0.71 respectively. Conclusion　RMPP is suggested when the MPP children has no response to erythromycin treatment, persistent fever. CRP ≥ 51 mg/L, SF ≥ 258 g/L, LDH ≥ 353 IU/L, N ≥ 71, and chest radiograph shows more than two-thirds of lung dense consolidation. The anti-inflammatory therapy of methylprednisolone should be considered.

A report of one case of systemic lupus erythematosus with chorea as the main manifestation

ZHANG Guoli, LIU Zhuoqiong, XING Mengnan, XU Xiangping

. 2015, 33(11):  962.  doi:10.3969 j.issn.1000-3606.2015.11.011

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Objectives　To analyze the clinical characteristics, diagnosis and treatment of systemic lupus erythematosus (SLE) with chorea as the main manifestation. Methods　The clinical manifestations, laboratory examinations and treatment of one pediatric patient with main presentation as chorea diagnosed with SLE in the year of 2014 were studied. The related literatures were reviewed. Result　A 14 years old female, was admitted with the main complaint of choreatic movements. The physical examination and laboratory tests indicated multi-system involvements (anemia, proteinuria, skin and mucous membrane damage and chorea). Combined with history of hemolytic anemia, positive antinuclear, anti-doublestranded (ds) DNA and anti-cardiolipin (aCL) antibodies, and multiple lesions in magnetic resonance imaging (MRI) scan, the diagnosis of SLE and NPSLE was confirmed. After the treatment of gamma globulin, methylprednisolone, and Aripiprazole, the symptoms were relieved. Conclusion　 SLE should be considered as a possible cause of chorea.

Analysis of 37 cases of neonatal paroxysmal supraventricular tachycardia

XU Baogan, YUAN Lin, TIAN Fang, CHEN Chao, SHI Wenjing

. 2015, 33(11):  966.  doi:10.3969 j.issn.1000-3606.2015.11.012

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Objective　To study the clinical feature, treatment and outcome of newborns with paroxysmal supraventricular tachycardia (PSVT). Methods　A retrospective study of the clinical data from 37 cases collected from January 2005 to December 2014 was performed. Based on whether there was a ventricle pre-excitation performance of the electrocardiogram, the cases were divided into concealed pathway group and Wolff–Parkinson–White syndrome (WPW) group, with further comparasion of the clinical feature, treatment and prognosis between two groups. Results　In 37 cases of newborn PSVT, 28 cases were concealed pathway and 9 cases were WPW syndrome. The gender, gestational age, birth weight, onset age,  fetal original, heart failure, and treatment between the two groups were of no significant differences. The average onset heart rate was significantly different between the two groups (P=0.004). The length of hospital stay and recurrent rate were of no significant differences between the two groups (P>0.05). Conclusion　PSVT is the most important arrhythmia in newborns. There are no obvious differences between the concealed pathway group and WPW syndrome group regarding to the clinical feature, treatment and the prognosis. The heart rate was faster in the WPW syndrome group than that in the concealed pathway group.

Analysis of effect and satisfaction of clinical pathway managment on pediatric Kawasaki disease

LIU Chonghai, KANG Qian, XIE Xuemei, PI Guanghuan

. 2015, 33(11):  971.  doi:10.3969 j.issn.1000-3606.2015.11.013

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Objective　To analyze the effect and satisfaction of clinical pathway for managing children with Kawasaki disease (KD). Methods　Data were collected from 224 children hospitalized with KD from January 2009 to December 2013. According to the implementation of clinical pathway management, all patients were divided into non-pathway management group (n=94) and pathway management group (n=130). Length of stay, antibiotics usage, total cost of hospitalization, treatment cost, medication cost, examination cost, and laboratory testing cost were compared between the two groups. Satisfaction of clinical pathway management was surveyed. Results　The average lengths of stay and percentages of antibiotics usage between the two groups were of no significant difference (P>0.05). The total cost of hospitalization and laboratory testing cost in clinical pathway group were (9597±2199) yuan and (1904±421) yuan, significant higher than those in non-pathway group [(8014±3330) yuan and (1168±409) yuan]. The therapy cost and medication cost between the two groups were of no significant difference (P>0.05). The questionnaire survey showed that application of clinical pathway did not increase the satisfaction of parents. However, the satisfaction of medical staffs was low because the increased workload, especially the pediatricians. Conclusions　Clinical pathway management has some advantages, but should be further improved in accordance with national conditions and regional situation.

Abnormal expression of miR-125a-5P in medulloblastoma and its role as tumor suppressor

CHU Dongmei, LIU Xiaozhi, YAN Yanping, GAO Xinjing, LIU Cuiping, LI Yanxia, JIANG Zhongmin, YAO Ling

. 2015, 33(11):  974.  doi:10.3969 j.issn.1000-3606.2015.11.014

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　Objectives　To study the expression characteristics of miR-125a-5P in medulloblastoma, and its role as tumor suppressor. Methods　The miR-125a-5p level was detected by stem loop Real-time RT-PCR in 58 cases of medulloblastoma tissues and 2 medulloblastoma cell lines, D341 and Daoy cells. miR-125a-5P oligonucleotide was transfected to D341 cells, then Ki-67 expression was detected by immunofluorescence assay, cell migration was tested by transwell assay, and tumorigenicity was determined by clone formation assay. The inhibitory effect of miR-125a-5p on medulloblastoma cells in vivo was detected in tumor bearing mice model. Results　The relative expression level of miR-125a-5P in medulloblastoma tissues was significantly higher than that of normal children's brain tissue (t=17.545, P<0.01), while the expression level of miR-125a-5P in 2 medulloblastoma cells were significantly lower than other control cells (F=9.437, P<0.01). The expression level of miR-125a-5P was significantly higher than that of the neural stem cells, D341 and Daoy cells, and the difference was statistically significant (P<0.05). Compared with the control group and the non-sense group, the ability of proliferation, migration and clone of cells in miR-125a-5p group obviously declined (P <0.05), as well as the tumor volume in mice (P<0.05). Conclusions　The expression of miR-125a-5P in medulloblastoma was reduced or absent, and was supposedly a tumor suppressor gene.

Clinical analysis of eight cases of essential thrombocythemia in children

LIANG Hui

. 2015, 33(11):  979.  doi:10.3969 j.issn.1000-3606.2015.11.015

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Objective　To explore the diagnosis and treatment of essential thrombocythemia in children. Methods　The clinical data from 8 children with essential thrombocythemia were retrospectively analyzed. Results　From January 2010 to June 2015, there were 8 children diagnosed essential thrombocythemia in clinic. One child was only 10 months old. Among them, 6 cases had JAK2V617F mutation, 2 cases had no mutation. All children were treated with hydroxyurea or interferon-α, and achieve remission. Conclusion　Essential thrombocythemia is a rare disease in childhood with a growing trend. More children with essential thrombocythemia should be enrolled for comparison of clinical manifestations and prognosis between JAK2V617F mutation positive and negative cases.

Advances in pulmonary artery sling in children

ZHOU Gan

. 2015, 33(11):  982.  doi:10.3969 j.issn.1000-3606.2015.11.016

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　Pulmonary artery sling (PAS) is a rare congenital cardiovascular disease and usually associated with airway and cardiovascular anomalies. Infants may present with recurrent stridor, infections and dyspnea, and older children may present with dysphagia. Asymptomatic cases may also be found. PAS is easy to be misdiagnosed as other breathing disorders. Imaging examination plays an important role in the diagnosis of PAS. Diagnosis mainly depends on finding relationships between the abnormal left pulmonary artery and the airway by CT and three-dimensional reconstruction. Early diagnosis and treatment is the key point. The traditional treatment for PAS is the left pulmonary artery reconstruction and narrow tracheoplasty. It is still controversial in the management of PAS associated with trachea stenosis. This article reviews the recent progress of definition, classification, clinical manifestation, auxiliary examination, diagnosis, and management of PAS.

Expression and significance of transcription factor hand in congenital heart diseases

MA Jianying

. 2015, 33(11):  986.  doi:10.3969 j.issn.1000-3606.2015.11.017

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　Heart is made from different mesodermal tissue cells under gene regulation through precise and complex processes, so the role of genes involved in the regulation is critical. Current known congenital heart disease-related transcription factor genes include BHLH, NKX2.5, GATA, and TBX. In recent years, foreign literature has reported hand gene, one of the BHLH family, was related to congenital heart diseases. This article briefly introduced hand gene and describes its mechanism in congenital heart diseases.

Advances in the etiology of childhood acute pancreatitis

WANG Zhihua

. 2015, 33(11):  990.  doi:10.3969 j.issn.1000-3606.2015.11.018

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　The childhood acute pancreatitis usually has a good prognosis, but it may be life-threatening in a few serious cases. Therefore, when children have acute abdominal pain, the pancreatitis should always be paid attention to, and appropriate treatments should be started promptly. However, the etiologies of childhood acute pancreatitis are not entirely consistent with adults, and the children cannot clearly articulate their histories of the diseases, the missed or delayed diagnosis often occurs. It is necessary to investigate the etiologies, so that the appropriate treatments will be administered. This paper will review the etiologies of childhood acute pancreatitis.

Progress of continuous electroencephalogram monitoring in the pediatric intensive care unit

ZHU Ling

. 2015, 33(11):  994.  doi:10.3969 j.issn.1000-3606.2015.11.019

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The degree of neurological damage and prognosis in critically ill children couple with social, family, ethical and other concerns. Continuous electroencephalogram monitoring (cEEG) as the most commonly used cerebral function monitoring helps early diagnosis and prevents the secondary brain injury, offers dynamic assessment of efficacy and prognosis. This article reviews the recent progress of application of cEEG monitoring in critically ill children.