CJCP
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2019 Vol.  21 No.  5
Published: 2019-05-25
CLINICAL RESEARCH
EXPERIMENTAL RESEARCH
CASE REPORT
CLINICAL RESEARCH
405 LAN Yang, CHEN Xiao-Juan, ZOU Yao, RUAN Min, ZHU Xiao-Fan
Clinical effect of CCLG-ALL2008 regimen in treatment of children and adolescents aged > 10 years with acute lymphoblastic leukemia

Objective To study the long-term clinical effect of CCLG-ALL2008 regimen in the treatment of children and adolescents, aged > 10 years, with newly diagnosed acute lymphoblastic leukemia (ALL). Methods A retrospective analysis was performed for the clinical data of 150 ALL children and adolescents aged > 10 years who were treated with CCLG-ALL2008 regimen from April 2008 to April 2015. The Kaplan-Meier method was used to analyze overall survival (OS) rate and event-free survival (EFS) rate. Results Among the 150 children and adolescents, there were 87 (58.0%) boys and 63 (42.0%) girls, with a median age of 11 years (range 10-15 years). Of the 150 children and adolescents, 84 (56.0%) had intermediate risk and 66 (44.0%) had high risk; 122 (81.3%) had B-lineage acute lymphoblastic leukemia (B-ALL) and 28 (18.7%) had T-lineage acute lymphoblastic leukemia (T-ALL). The fusion gene test yielded positive results in 51 children and adolescents (34.0%), among whom 16 (31%) had positive BCR-ABL, 11 (22%) had positive TEL-AML1, 8 (16%) had positive E2A-PBX1, and 16 (31%) were positive for other fusion genes. The complete remission rate was 96.0% (144/150) after one course of treatment with CCLG-ALL2008 regimen. The median follow-up time was 52 months (range 3-122 months). The 5-year OS rate was 79.0%±3.5%, and the 5-year EFS rate was 67.3%±4.1%. There were no significant differences in 5-year OS and EFS rates between the children with intermediate or high risk, as well as between the children with B-ALL or T-ALL (P > 0.05). The children and adolescents who achieved complete remission of bone marrow at the end of induction therapy had significantly higher 5-year OS and EFS rates than those who did not achieve complete remission (P < 0.05). Conclusions In ALL children and adolescents aged > 10 years, CCLG-ALL2008 regimen can help to achieve high complete remission rate, 5-year OS rate and 5-year EFS rate. The children and adolescents failing to achieve complete remission at the end of induction therapy tend to have a poor prognosis.
2019 Vol. 21 (5): 405-410 [Abstract] ( 2821 ) [HTML 1KB] [PDF 1251KB] ( 723 )
411 WANG Wen-Peng, GUO Lei, LI Yan, LU Li-Hui, CHANG Ying, ZHOU Bi, ZHOU Min, LI Xuan, GAO Ji-Zhao
Expression and significance of dishevelled proteins in the Wnt pathway in childhood acute lymphoblastic leukemia

Objective To study the significance of dishevelled (DVL) proteins in the Wnt signaling pathway in the pathogenesis and prognosis of childhood acute lymphoblastic leukemia (ALL). Methods A total of 33 children with new-onset ALL were enrolled as the case group. According to the degree of risk, they were divided into 3 groups:low-risk (n=14), intermediate-risk (n=5) and high-risk (n=14). A total of 29 children with immune thrombocytopenia were enrolled as the control group. At diagnosis and on day 33 of induction therapy, 2 mL bone marrow samples were collected from the case and control groups, and qRT-PCR was used to measure the mRNA expression of DVL1, DVL2 and DVL3 in blood cells of bone marrow. Results The mRNA expression of DVL1, DVL2 and DVL3 in the case group in the incipient stage was significantly higher than that in the remission stage and the control group (P < 0.05). Compared with the control group, the case group had a significant increase in the mRNA expression of DVL2 in the remission stage (P < 0.05). The mRNA expression of DVL2 was significantly higher than that of DVL1 and DVL3 in both remission and incipient stages (P < 0.05). The high-and intermediate-risk groups had significantly higher mRNA expression of DVL1 and DVL2 than the low-risk group (P < 0.05). The mRNA expression of DVL2 was significantly higher than that of DVL1 and DVL3 in the low-, intermediate-and high-risk groups (P < 0.05). Conclusions The change in the expression of DVL, especially DVL2, in the Wnt signal pathway has certain significance in the pathogenesis and prognosis of childhood ALL.
2019 Vol. 21 (5): 411-414 [Abstract] ( 2497 ) [HTML 1KB] [PDF 1058KB] ( 710 )
415 WANG Ying-Chao, LI Zhuang-Zhuang, YIN Chu-Yun, JIANG Lan-Jun, WANG Long
Langerhans cell histiocytosis involving the oral and maxillofacial region: an analysis of 12 cases
Objective To study the clinical features of Langerhans cell histiocytosis (LCH) involving the oral and maxillofacial region in children. Methods A retrospective analysis was performed for the clinical data of 12 children with LCH involving the oral and maxillofacial region who were hospitalized and treated from September 2012 to September 2017, including clinical manifestations, pathological features, treatment and prognosis. Results Of the 12 children, 8 (67%) had multiple system involvement and 7 (58%) had the involvement of organs at risk. Bone was the most common affected site (11 children, 92%), among whom 7 children had the involvement of the mandible. Oral soft tissue involvement manifested as gingival ulcer or hyperplasia in 4 children, loose teeth in 5 children, oral mucosal lesions in 2 children, and nodular lesions in 1 child. Pathological examination showed positive CDla in 11 children and positive CD207, CD68, S-100, and LCA in 12 children. Surgery combined with chemotherapy was the major treatment method, and surgical resection alone was performed for focal lesions. After treatment, 11 children were cured or improved and 1 gave up treatment and was lost to follow-up. No recurrence was observed. Conclusions LCH children with oral and maxillofacial involvement often have the involvement of multiple systems and organs at risk, with the mandible as the most common affected site. These children may also have the involvement of gingiva, oral mucosa and teeth. Surgery combined with chemotherapy is the major treatment method, and the patients generally have a good prognosis without recurrence.
2019 Vol. 21 (5): 415-420 [Abstract] ( 3035 ) [HTML 1KB] [PDF 1694KB] ( 800 )
421 ZHANG Min, WEN Zhi-Hong, YANG Cai-Qiong
A clinical follow-up study of children with well-controlled asthma after withdrawal of low-dose inhaled corticosteroids

Objective To study the incidence of acute attacks of asthma and dynamic changes in laboratory markers in children with well-controlled asthma after the withdrawal of low-dose inhaled corticosteroids (ICS), and to provide a basis for optimal long-term control regimens for children with asthma. Methods A total of 63 children with well-controlled asthma were enrolled as subjects. According to their parents' wishes, they were continuously administered with ICS (ICS treatment group; n=35) and without ICS (ICS withdrawal group; n=28). They were followed up for 18 months. The incidence of acute attacks of asthma was evaluated, dynamic monitoring was performed for pulmonary function and fractional exhaled nitric oxide (FeNO), and childhood asthma control test (C-ACT) was performed every three months. Results At 3, 6, 9, and 12 months of follow-up, there was no significant difference in FeNO between the ICS treatment and withdrawal groups (P > 0.05). However, at 15 and 18 months of follow-up, the withdrawal group had a significantly higher level of FeNO than the ICS treatment group (P < 0.05). There was no significant difference in the C-ACT score between the two groups at all time points of follow-up (P > 0.05). At 3, 6, 9, and 12 months of follow-up, there were no significant differences between the two groups in the percentage of forced expiratory volume in 1 second, the ratio of forced expiratory volume in 1 second to forced vital capacity, percentage of predicted maximum mid-expiratory flow (MMEF%), and maximal expiratory flow at 50% of vital capacity (MEF50) (P > 0.05), while at 15 and 18 months of follow-up, the ICS treatment group had significantly higher MMEF% and MEF50 than the withdrawal group (P < 0.05). During follow-up, 3 children (9%) in the ICS treatment group and 8 (29%) in the withdrawal group experienced acute attacks of asthma (P=0.0495). Conclusions Continuous inhalation of low-dose ICS can maintain the stability of pulmonary function and reduce acute attacks of asthma in children with well-controlled asthma.
2019 Vol. 21 (5): 421-425 [Abstract] ( 2370 ) [HTML 1KB] [PDF 1138KB] ( 805 )
426 MU Dong-Qin, PAN Jia-Hua
Correlation of fractional exhaled nitric oxide in the upper and lower airways with the level of asthma control
Objective To study the clinical value of combined measurement of fractional exhaled nitric oxide (FeNO) and nasal fractional exhaled nitric oxide (FnNO) and its correlation with the level of asthma control. Methods A total of 120 children who were diagnosed with asthma from January to June, 2018 and were in the chronic persistent stage were enrolled as subjects. The childhood asthma control test (C-ACT) was performed for all the 120 children. According to the C-ACT score, these children were divided into 4 groups:complete control group with a C-ACT score of > 23, partial control group with a C-ACT score of 20-23, and uncontrolled group with a C-ACT score of ≤ 19 (n=40 each). According to the presence or absence of allergic rhinitis, they were divided into 2 groups:non-rhinitis group with 55 children and rhinitis group with 65 children. A total of 40 children who underwent physical examination during the same period of time were enrolled as the control group. FeNO and FnNO levels were measured for all the 120 children. Results The uncontrolled group had the highest level of FeNO, followed by the partial control group and the complete control group (P < 0.05). The uncontrolled and partial control groups had a significantly higher level of FeNO than the control group (P < 0.05). The uncontrolled and partial control groups had a significantly higher level of FnNO than the complete control and control groups (P < 0.05). The rhinitis group had significantly higher FeNO and FnNO levels than the non-rhinitis group (P < 0.05). Conclusions FeNO can be used to assess the level of asthma control in children, and its combination with FnNO may be useful for the evaluation of the degree of inflammation in the upper and lower airways and provide a basis for the combined treatment of the upper and lower airways.
2019 Vol. 21 (5): 426-430 [Abstract] ( 3266 ) [HTML 1KB] [PDF 1112KB] ( 1115 )
431 LIU Zheng, FU Zhou, DAI Ji-Hong, NIU Chao
Clinical features of children with bronchial asthma complicated by pulmonary fungal infection and risk factors for pulmonary fungal infection
Objective To study the clinical features of children with bronchial asthma complicated by pulmonary fungal infection and the risk factors for pulmonary fungal infection. Methods A retrospective analysis was performed for the clinical data of 150 children with bronchial asthma who were admitted from January 2015 to June 2018. Among these children, 75 had pulmonary fungal infection (fungal infection group) and 75 did not have such infection (control group). The distribution of pathogenic fungi, clinical symptoms/signs and treatment outcome were recorded for the fungal infection group. The multivariate logistic regression analysis was used to investigate the risk factors for pulmonary fungal infection. Results A total of 69 pathogenic fungi were detected in 75 children in the fungal infection group, among which Candida albicans had the highest detection rate of 61%. Major clinical symptoms were cough (93%), persistent high fever (56%), wheezing (49%) and dyspnea (48%). Major signs were dry and moist rales (43%) and moist rales (29%). Parts of children had hepatosplenomegaly. Among the 75 children in the fungal infection group, 39 were markedly improved, 26 were improved, 7 had no response, and 3 experienced aggravation and then died. Age < 3 years, comorbidities of nasosinusitis and/or allergic rhinitis, asthma attacks of > 3 times during hospitalization, intravenous administration of glucocorticoids, non-rational use of antibiotics, mechanical ventilation and prolonged hospital stay were independent risk factors for pulmonary fungal infection in children with asthma (OR=4.865, 3.241, 2.255, 3.725, 3.568, 1.549, 3.808; P < 0.05). Conclusions Pulmonary fungal infection should be considered for asthmatic children with cough, persistent high fever, obvious dry and moist rales and hepatosplenomegaly. The asthmatic children with an age of < 3 years, comorbidities of nasosinusitis and/or allergic rhinitis, asthma attacks of > 3 times during hospitalization, intravenous administration of glucocorticoids, non-rational use of antibiotics, mechanical ventilation or prolonged hospital stay have a higher risk for secondary pulmonary fungal infection.
2019 Vol. 21 (5): 431-435 [Abstract] ( 3042 ) [HTML 1KB] [PDF 1137KB] ( 768 )
436 XU Mao-Zhu, LIU Jing-Yue, FU Zhou
Causes of chronic cough in children in Chongqing, China: an analysis of 202 cases
Objective To investigate the constitution of causes of chronic cough in children. Methods The clinical data of 202 children with chronic cough who were hospitalized in the Affiliated Children's Hospital of Chongqing Medical University from May 2015 to November 2017 were retrospectively analyzed. Results As for the causes of chronic cough in the 202 children, 81 (40.1%) had post-infection cough (PIC), 71 (35.1%) had cough variant asthma (CVA), 43 (21.3%) had upper airway cough syndrome (UACS), 3 (1.5%) had foreign body aspiration, 1 (0.5%) had gastroesophageal reflux cough, 2 (1.0%) had Tourette syndrome, and 1 (0.5%) had congenital respiratory disorders. Of the 202 children, 119 (58.9%) had chronic cough caused by a single factor and 83 (41.1%) had chronic cough caused by multiple factors. There was a significant difference in the constitution of causes of chronic cough among the children with different ages (< 1 year, ≥ 1 year, ≥ 3 years and 6-14 years) or natures of cough (wet cough and dry cough) (P < 0.01). Conclusions The top three causes of chronic cough in children are PIC, CVA and UACS. There are significant differences in the main causes of chronic cough between children with different ages and between those with different natures of cough.
2019 Vol. 21 (5): 436-440 [Abstract] ( 2540 ) [HTML 1KB] [PDF 1172KB] ( 1016 )
441 JIANG Rui-Ju, YIN Qiong-Zhou, XU Ming-Jue, ZHAO Zhi-Mei, DENG Yan, CHE Yan-Chun
Epidemiological characteristics of mumps in mainland China from 2004 to 2018 and key population for prevention and control Hot!

Objective To investigate the epidemiological characteristics of mumps in mainland China from 2004 to 2018, and to provide data for the key population for prevention and control of mumps. Methods The epidemiological characteristics of mumps were analyzed with reference to the data of the cases of mumps reported in the National Scientific Data Sharing Platform for Population and Health and Disease Prevention and Control Bureau of National Health Commission of the People's Republic of China. Descriptive epidemiology was used to analyze the epidemiological characteristics of mumps. Results A total of 4 272 368 cases of mumps were reported in China during 2004-2018, with an average annual reported incidence rate of 21.44/100 000. A single dose of mumps-containing vaccine was added to the national Expanded Program of Immunization in 2008, but the annual incidence rate ranged from 12.84/100 000 to 35.59/100 000. The second dose of measles, mumps and rubella combined attenuated live vaccine was included in the routine immunization in Beijing, Tianjin and Shanghai, and then the average incidence rate of mumps reported in these three regions dropped to about 10/100 000. From 2004 to 2016, the population aged 3-14 years accounted for 81.16% of all patients with mumps. The children aged 6 years had the highest incidence rate of mumps during 2004-2013. Conclusions A single dose of mumps-containing vaccine has no obvious effect on the incidence rate of mumps. Children aged 6 years have the highest incidence rate of mumps. A booster dose of mumps-containing vaccine should be given to preschool children.
2019 Vol. 21 (5): 441-444 [Abstract] ( 3850 ) [HTML 1KB] [PDF 1245KB] ( 820 )
445 ZHOU Hao, LI Chun-Pei, WANG Tian-Qi, LONG Sha-Sha, DU Xiao-Nan, MA Yu, WANG Yi
Autism spectrum disorder-like symptoms in the population with intellectual disability aged 6 to 18 years
Objective To investigate the incidence of autism spectrum disorder (ASD)-like symptoms in the population with intellectual disability (ID). Methods The students with ASD or ID, aged 6-18 years, who studied in a special school in Shanghai from January to June, 2017, as well as the typically developing (TD) population of the same age, who studied in a general school in Shanghai during the same period, were enrolled. Social Responsiveness Scale (SRS) was completed by their parents or other guardians, and the ASD-like symptoms were evaluated. Results A total of 69 subjects with ASD, 74 subjects with ID and 177 TD subjects were enrolled. The ID group had a significantly higher SRS-positive rate than the TD group (47.3% vs 1.7%; P < 0.001) and a significantly lower SRS-positive rate than the ASD group (47.3% vs 87.0%; P < 0.001). The total score of SRS was 114±26 in the ASD group, 80±24 in the ID group and 38±19 in the TD group. The ID group had a significantly higher total score of SRS than the TD group (P < 0.05), and the score on the subscale of social cognition showed the most significant difference between the two groups (Cohen's d=2.00). There were no significant differences in the total score of SRS and the scores on each subscale of SRS between the mild-to-moderate ID and severe-to-extremely severe ID groups (P > 0.05), and there was no significant correlation between SRS score and IQ (P > 0.05). Conclusions The ID population aged 6-18 years has more ASD-like symptoms than the general population, and ASD screening and intervention should be performed for the ID population as early as possible.
2019 Vol. 21 (5): 445-449 [Abstract] ( 2310 ) [HTML 1KB] [PDF 1189KB] ( 663 )
450 CHEN Hui, CHEN Yan-Hui, LIU Ling, WANG Yong
Effects of ketogenic diet on lipid metabolism in children with intractable epilepsy
Objective To study the effects of ketogenic diet (KD) on lipid metabolism in children with intractable epilepsy and the risk of atherosclerosis in children treated with KD assessed by changes in lipid profile. Methods The clinical data of 47 children with intractable epilepsy from 2013 to 2017 were collected. Blood lipid levels including triglycerides (TG), total cholesterol (TC), high-density lipoprotein (HDL) and low-density lipoprotein (LDL), were detected before and 3 months after KD treatment. LDL/HDL ratio, arterial stiffness index (AI), atherogenic index of plasma (AIP) and lipid comprehensive index (LCI) were calculated to assess the risk of atherosclerosis. Results After 3 months of KD treatment, the TG and TC levels were slightly higher than those before treatment, and the HDL levels were slightly lower than those before treatment, but the differences were not statistically significant (P > 0.05). The LDL levels of the children after 3 months of KD treatment were significantly higher than those before treatment (P < 0.05). After 3 months of KD treatment, the LDL/HDL ratio and AI, AIP and LCI levels of the children were increased compared with those before treatment, but only the increase of the LDL/HDL ratio was statistically significant (P < 0.05). Conclusions KD treatment may lead to increase in LDL level and LDL/HDL ratio, suggesting that KD treatment may increase the risk of atherosclerosis.
2019 Vol. 21 (5): 450-453 [Abstract] ( 2855 ) [HTML 1KB] [PDF 1116KB] ( 829 )
454 ZHANG Hong-Qin, HUANG Lu, CHEN Jun, HUANG Wei-Min
Association of peripheral blood pH early after birth with amplitude-integrated electroencephalography findings in neonates with intrauterine distress

Objective To study the association of peripheral blood pH early after birth with amplitude-integrated electroencephalography (aEEG) findings in neonates with intrauterine distress. Methods The clinical data of the neonates with intrauterine distress who were born from January 2017 to May 2018 were collected. According to peripheral blood pH within 30 minutes after birth, these neonates were divided into 3 groups:pH ≤ 7.15, 7.15 7.25, with 30 neonates in each group. aEEG mornitoring was performed within 6 hours after birth, and the abnormal rate of aEEG findings and average cerebral function monitoring (CFM) score were compared between groups. Results The pH ≤ 7.15 group had a significantly higher abnormal rate of aEEG findings than the pH >7.25 group (P < 0.0167) and a significantly lower aEEG CFM score than the 7.15 7.25 groups (P < 0.05). Peripheral blood pH was positively correlated with the CFM score (r=0.26, P < 0.05). Conclusions Peripheral blood pH is significantly associated with aEEG findings in neonates with intrauterine distress. Active measures should be adopted for the prevention and treatment of nervous system injury in neonates with intrauterine distress who have a significant reduction in peripheral blood pH early after birth (pH ≤ 7.15).
2019 Vol. 21 (5): 454-457 [Abstract] ( 2440 ) [HTML 1KB] [PDF 1215KB] ( 643 )
458 ZHONG Qing-Hua, DUAN Jiang, LIANG Kun, ZENG Jie, QI Zhi-Ye, HE Xiang-Ying
A clinical study of growth and metabolism of small for gestational age infants

Objective To study the differences in growth and metabolism between small for gestational age (SGA) infants and appropriate for gestational age (AGA) infants. Methods A total of 1 370 preterm infants were enrolled in this study. According to the association between gestational age and birth weight, they were divided into SGA group with 675 infants and AGA group with 695 infants. The two groups were compared in terms of general conditions, physical growth and blood biochemical parameters. Results The SGA group had a significantly longer length of hospital stay than the AGA group (P < 0.05). Compared with the AGA group, the SGA group had significantly lower body weight, body weight Z score, and body length at discharge and significantly higher incidence rate of extrauterine growth retardation and growth rate of head circumference (P < 0.05). Compared with the AGA group, the SGA group had significantly longer time to full enteral nutrition and duration of parenteral nutrition (P < 0.05). Compared with the AGA group, the SGA group had significantly higher levels of albumin, prealbumin, and serum phosphorus on admission and total bile acid before discharge, as well as a significantly lower albumin level before discharge (P < 0.05). The incidence rates of asphyxia, neonatal respiratory distress syndrome, myocardial damage, feeding intolerance, pneumonia, sepsis, hypoglycemia and hypothyroxinemia in the SGA group were significantly higher than in the AGA group (P < 0.05). Conclusions Compared with AGA infants, SGA infants have significantly delayed physical development during hospitalization and significantly higher incidence rates of extrauterine growth retardation and related complications.
2019 Vol. 21 (5): 458-462 [Abstract] ( 2746 ) [HTML 1KB] [PDF 1291KB] ( 902 )
463 LIU Chao, CHEN Xiao-Yan, WU Wen-Qi, AN Wen-Bin, CHANG Li-Xian, LAN Yang, YI Mei-Hui, CAI Yu-Li, FENG Jing, ZHU Xiao-Fan
Clinical features of Wiskott-Aldrich syndrome: an analysis of 13 cases
Objective To study the clinical features of Wiskott-Aldrich syndrome (WAS) in children. Methods A retrospective analysis was performed for the clinical data of 13 children with WAS. Results All 13 children were boys, with a median age of onset of 3 months (range 1-48 months) and a median age of 24 months (range 1-60 months) at the time of diagnosis. Of the 13 children, only 3 had typical WAS and the remaining 10 children had X-linked thrombocytopenia (XLT). The mean WAS score was 2 (range 1-3), the mean platelet count was 20.5×109/L[range (13-46)×109/L], and the mean platelet volume was 8.1 fl (range 6.7-12.1 fl). Lymphocyte subsets and immunoglobulins were measured for 4 children, among whom 1 (25%) had a reduction in both the percentage of CD3+T cells per lymphocyte and lymphocyte per nuclear cells, 1(25%) had a reduction in CD3-CD56+ NK cells. Among these 4 children, 1 (25%) had an increase in IgG, 2 (50%) had a reduction in IgM, 1 (25%) had a reduction in IgA, and 4 (100%) had an increase in IgE. A total of 14 gene mutations belonging to 13 types were found in 13 children, among which there were 9 missense mutations (65%), 2 splicing mutations (14%), 2 nonsense mutation (14%), and 1 frameshift mutation (7%). The median follow-up time was 39 months (range 3-62 months), and all 13 children survived. Conclusions Children with WAS often have a young age of onset, and most of them are boys. Major clinical features include thrombocytopenia with a reduction in platelet volume. Missense mutation is the main type of gene mutation.
2019 Vol. 21 (5): 463-467 [Abstract] ( 3368 ) [HTML 1KB] [PDF 1350KB] ( 750 )
468 WANG Hui, YAN Yu-Chun, LI Qi, ZHANG Zhen, XIAO Ping, YUAN Xin-Yu, LI Long, JIANG Qian
Clinical and genetic features of Mowat-Wilson syndrome: an analysis of 3 cases
Mowat-Wilson syndrome (MWS) is a rare autosomal dominant genetic disease caused by zinc finger E-box-binding homeobox 2 (ZEB2) gene mutation and has various clinical manifestations including intellectual disability/global developmental delay, unusual facies and multiple congenital malformations. This article reports the clinical features and gene mutations of three children diagnosed with MWS by ZEB2 gene analysis. All three children had Hirschsprung disease and unusual facies. One child died of severe heart failure and pneumonia at the age of 4 months. Global developmental delay was not discovered by her parents due to her young age. The other two children had severe global developmental delay. All three children carried a de novo heterozygous nonsense mutation in the ZEB2 gene, among which c.756C > A (p.Y252X) had not been reported before. Such mutations produced truncated proteins and were highly pathogenic. MWS is presented with strong clinical and genetic heterogeneity. Clinicians should consider the possibility of MWS when a child has unusual facies of MWS, intellectual disability/global developmental delay and multiple congenital malformations. Gene detection helps to make a confirmed diagnosis.
2019 Vol. 21 (5): 468-473 [Abstract] ( 3488 ) [HTML 1KB] [PDF 2597KB] ( 784 )
474 YAN Xiao-Tian, BO Tao, WANG Cheng, LI Fang, BAI Lu
Heart rate variability in neonates with non-benign tachyarrhythmia
Objective To study heart rate variability (HRV) in neonates with non-benign tachyarrhythmia (NNTA) and the role of automatic nervous system (ANS) in NNTA. Methods The neonates who were admitted to the Department of Neonatology, the Second Xiangya Hospital of Central South University, from January 2010 to June 2018 and were diagnosed with NNTA were enrolled as the NNTA group, and the neonates with sinus rhythm or accidental premature beats on ambulatory electrocardiography were enrolled as the control group. Each group was further subdivided into preterm and term subgroups. A retrospective analysis was performed for their clinical data. Results A total of 27 NNTA neonates were enrolled, accounting for 0.28% (27/9 632) of all neonates hospitalized during the same period of time, and 53 neonates were enrolled in the control group. Compared with the preterm and term control subgroups, the preterm NNTA and term NNTA subgroups had a significant increase in the standard deviation of average RR interval (P < 0.05). Conclusions Immature and unbalanced ANS function may play an important role in the development and progression of NNTA.
2019 Vol. 21 (5): 474-478 [Abstract] ( 2741 ) [HTML 1KB] [PDF 1307KB] ( 727 )
479 CONG En-Zhao, WU Yan, CAI Yi-Yun, CHEN Hai-Ying, XU Yi-Feng
Association of suicidal ideation with family environment and psychological resilience in adolescents
Objective To study the association of suicidal ideation with family environment and psychological resilience in adolescents. Methods Cluster sampling was used to perform an investigation among 3 230 junior and senior high school students in Xinxiang of Henan Province, China December 2014. A general social information questionnaire, 11-Item Kutcher Adolescent Depression Scale(KADS-11), Family Environment Scale-Chinese Version (FES-CV) and Connor-Davidson Resilience Scale (CD-RISC; Chinese version) were used for evaluation. A multivariate logistic regression analysis and a case-control study were used to investigate the association of suicidal ideation with family environment and psychological resilience in adolescents. Results A total of 2 960 usable questionnaires were received. Among the 2 960 adolescents, 247 (8.50%) had suicidal ideation (98 boys and 149 girls). The multivariate logistic regression analysis showed that after adjustment for age and sex, single-parent/remarried family was associated with an increased risk of suicidal ideation (OR=2.655). Suicidal ideation in boys was negatively correlated with family cohesion (OR=0.750, P < 0.001) and organization (OR=0.855, P=0.036) and was positively correlated with family conflict (OR=1.159, P=0.017). Suicidal ideation in girls were negatively correlated with family cohesion (OR=0.771, P < 0.001), emotional expression (OR=0.815, P=0.001) and intellectual-cultural orientation (OR=0.915, P=0.037). The adolescents with suicidal ideation had a significantly lower total score of psychological resilience than those without suicidal ideation (P < 0.05). Compared with those without suicidal ideation, the adolescents with suicidal ideation had significantly lower scores on 4 factors of the CD-RISC (ability, tolerance of negative emotions, acceptance of changes and control) (P < 0.05). Conclusions Family cohesion is a protective factor against suicidal ideation in adolescents. Family organization in boys and family emotional expression in girls are associated with a decreased risk of suicidal ideation. Enhanced psychological resilience may help to reduce the incidence of suicide ideation in adolescents.
2019 Vol. 21 (5): 479-484 [Abstract] ( 3926 ) [HTML 1KB] [PDF 1480KB] ( 1017 )
473
No abstract available
2019 Vol. 21 (5): 473-473 [Abstract] ( 1169 ) [HTML 1KB] [PDF 772KB] ( 555 )
490
No abstract available
2019 Vol. 21 (5): 490-490 [Abstract] ( 1097 ) [HTML 1KB] [PDF 785KB] ( 443 )
EXPERIMENTAL RESEARCH
485 XING Peng-Rui, PAN Jin-Yong, ZHANG Hui-Rong
Expression and significance of Shh and Wnt5a genes in Cornelia de Lange syndrome
Objective To study the expression of Shh and Wnt5a genes in the limb buds of NIPBL+/- fetal rats and the association of these two genes with Cornelia de Lange syndrome (CdLS). Methods A total of 72 NIPBL+/- fetal rats were divided into an experimental group and a control group, with 36 rats in each group. The limb buds were collected from 12 fetal rats each on embryonic days 10, 11 and 12 (E10, E11 and E12) respectively. Real-time PCR and Western blot were used to measure the mRNA and protein expression of Shh and Wnt5a. Results The mRNA and protein expression of Shh and Wnt5a was detected in the limb buds on E10, E11 and E12, and the experimental group had significantly lower expression than the control group (P < 0.01). The mRNA and protein expression of Shh and Wnt5a in limb buds was at a low level on E10, followed by an increase on E11 and a reduction on E12, and the expression on E12 was still lower than that on E10 (P < 0.01). Conclusions The mRNA and protein expression of Shh and Wnt5a are consistent. The pathogenesis of CdLS may be associated with the low mRNA and protein expression of Shh and Wnt5a inhibited by the low expression of NIPBL gene.
2019 Vol. 21 (5): 485-490 [Abstract] ( 2191 ) [HTML 1KB] [PDF 1507KB] ( 851 )
491 WANG Yi-Lin, WANG Ling-Zhen, SUN Jian-Dong, LI Xue-Rong, WANG Zhi, SUN Li-Rong
Effect of ultraviolet irradiation on the proliferation of acute promyelocytic leukemia cells under hypoxic conditions and related mechanisms

Objective To study the effect of 280 nm-LED ultraviolet irradiation on the proliferation of acute promyelocytic leukemia (APL) HL-60 cells under hypoxic conditions and related mechanism. Methods HL-60 cells in the logarithmic growth phase were selected and divided into control, hypoxia, ultraviolet and hypoxia+ultraviolet groups. The cells in the hypoxia group were treated with cobalt chloride (with a final concentration of 150 μmol/L), those in the ultraviolet group were irradiated by 280 nm-LED ultraviolet with an energy intensity of 30 J/m2, and those in the hypoxia+ultraviolet group were treated with cobalt chloride and then irradiated by 280 nm-LED ultraviolet. After 48 hours of treatment, the cells were placed under an invert microscope to observe cell morphology. CCK-8 assay was used to measure the inhibition rate of cell proliferation. Annexin V-FITC/PI double staining flow cytometry was used to evaluate cell apoptosis. Quantitative real-time PCR was used to measure the mRNA expression of Bcl-2. Each experiment above was repeated three times independently. Results Compared with the control group, the experimental groups showed shrinkage, decreased brightness, and disordered arrangement of cells, and the number of cells decreased over the time of culture. There were significant differences in the inhibition rate of cell proliferation and cell apoptosis rate among the groups (P < 0.01), and the hypoxia+ultraviolet group showed the strongest inhibition of cell proliferation and induction of cell apoptosis, followed by the ultraviolet group and the hypoxia group. Compared with the control group, the other three groups had a gradual reduction in the mRNA expression of Bcl-2, and the hypoxia+ultraviolet group had a significantly greater reduction than the hypoxia and ultraviolet groups (P < 0.01). Conclusions Both hypoxia and ultraviolet irradiation can inhibit the proliferation of HL-60 cells and induce cell apoptosis, and ultraviolet irradiation has a better effect on proliferation inhibition and cell apoptosis under hypoxic conditions than under normoxic conditions, possibly by downregulating the mRNA expression of Bcl-2.
2019 Vol. 21 (5): 491-496 [Abstract] ( 2095 ) [HTML 1KB] [PDF 1922KB] ( 581 )
CASE REPORT
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