CJCP
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2021 Vol.  23 No.  8
Published: 2021-08-10

STANDARD·PROTOCOL·GUIDELINE
CLINICAL RESEARCH
EXPERIMENTAL RESEARCH
REVIEW
STANDARD·PROTOCOL·GUIDELINE
761 Yan-Mei CHANG, Xin-Zhu LIN, Rong ZHANG, Xi-Hong LIU, Xiao-Mei TONG, Ping-Yang CHEN, Zhi-Chun FENG
Expert consensus on clinical management of metabolic bone disease of prematurity (2021) Hot!
Metabolic bone disease of prematurity (MBDP) is a systemic bone disease with a reduction in bone mineral content due to disorder of calcium and phosphorus metabolism. There is still a lack of in-depth research and systematic understanding of MBDP in China, and there are many irregularities in clinical management of this disease. Based on relevant studies in China and overseas, Grading of Recommendations Assessment, Development and Evaluation was used to develop the expert consensus on the clinical management of MBDP, which provides recommendations from the following five aspects: high-risk factors, screening/diagnosis, prevention, treatment, and post-discharge follow-up of MBDP, so as to provide relevant practitioners with recommendations on the clinical management of MBDP to reduce the incidence rate of MBDP and improve its short- and long-term prognosis.
2021 Vol. 23 (8): 761-772 [Abstract] ( 4068 ) [HTML 1KB] [PDF 1202KB] ( 2151 )
761 Yan-Mei CHANG, Xin-Zhu LIN, Rong ZHANG, Xi-Hong LIU, Xiao-Mei TONG, Ping-Yang CH
Expert consensus on clinical management of metabolic bone disease of prematurity (2021)(English Translation) Hot!

Metabolic bone disease of prematurity (MBDP) is a systemic bone disease with a reduction in bone mineral content due to disorder of calcium and phosphorus metabolism. There is still a lack of in-depth research and systematic understanding of MBDP in China, and there are many irregularities in clinical management of this disease. Based on relevant studies in China and overseas, Grading of Recommendations Assessment, Development and Evaluation was used to develop the expert consensus on the clinical management of MBDP, which provides recommendations from the following five aspects: high-risk factors, screening/diagnosis, prevention, treatment, and post-discharge follow-up of MBDP, so as to provide relevant practitioners with recommendations on the clinical management of MBDP to reduce the incidence rate of MBDP and improve its short- and long-term prognosis.

2021 Vol. 23 (8): 761-772 [Abstract] ( 886 ) [HTML 166KB] [PDF 1202KB] ( 617 )
CLINICAL RESEARCH
773 ZHAO Yan-Jun, CHEN Qian, HUANG Li-Su, LIU Han, ZHANG Jun
Association between cesarean section and sensory integration dysfunction in preschool children: a prospective cohort study
Objective To study the association between cesarean section and sensory integration dysfunction (SID) in preschool children through a prospective cohort study. Methods Based on the multicenter mother-infant cohort established by the Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine and the International Peace Maternity and Child Health Hospital Affiliated to Shanghai Jiao Tong University School of Medicine in 2012, the sensory integration functions (three dimensions: vestibular balance, tactile defensiveness, and proprioception) of 392 preschool children were evaluated by the Chinese Children Sensory Integration Capacity Development Rating Scale in 2017. Births by cesarean section were the exposure factors, and the children born by vaginal delivery were enrolled as controls. A multivariable logistic regression analysis was used to evaluate the association of cesarean section with each dimension of SID. Results The prevalence rate of SID was 21.9% (86/392) among the preschool children, and the prevalence rates of vestibular balance disorder, tactile over-responsivity, and proprioceptive disorder were 5.9% (23/392), 5.4% (21/392), and 15.1% (59/392) respectively. After adjustment for the confounding factors including maternal age at delivery and maternal educational level and child birth situation, the cesarean section group had a significant increase in the risk of proprioceptive disorder (RR=4.16, 95%CI: 1.41-12.30, P<0.05). The stratified analysis based on sex showed that the boys born by cesarean section had a significantly higher risk of proprioceptive disorder than those born by vaginal delivery (RR=5.75, 95%CI: 1.26-26.40, P<0.05). Conclusions Cesarean section can significantly increase the risk of proprioceptive disorder in preschool children, especially in boys.
2021 Vol. 23 (8): 773-778 [Abstract] ( 2461 ) [HTML 1KB] [PDF 526KB] ( 799 )
779 LIU Gui-Hua, OU Ping, HUANG Long-Sheng, XIE Na-Mei, LIN Jin-Ling, HE Ying-Shuang, HU Rong-Fang
Effects of parent-child painting and creative crafting therapy on preschool children with autism spectrum disorder and their mothers: a prospective randomized controlled trial
Objective To study the effects of parent-child painting and creative crafting therapy on the core symptoms of preschool children with mild-to-moderate autism spectrum disorder (ASD) and the parenting stress and hope level of their mothers. Methods A total of 56 preschool children with mild-to-moderate ASD and their mothers were divided into an experimental group and a control group using the block randomization method, with 28 pairs in each group. The subjects in the control group received an applied behavior analytic intervention and those in the experimental group received parent-child painting and creative crafting therapy in addition to the intervention in the control group. The intervention time was 20 weeks for both groups. Autism Behavior Checklist (ABC), Social Responsiveness Scale (SRS), Parenting Stress Index-Short Form (PSI-SF), and Herth Hope Index (HHI) were used to evaluate the core symptoms of children and the parenting stress and hope level of their mothers before and after 20 weeks of intervention. Results Forty-nine child-mother pairs completed the study (25 pairs in the intervention group and 24 pairs in the control group). The children in the experimental group had significantly lower scores of social interaction, language, social communication, and social motivation and total scores of ABC and SRS compared with those in the control group (P<0.05). The mothers in the experimental group had significantly lower scores of parental distress and parent-child dysfunctional interaction and total score of PSI-SF (P<0.05) and significantly higher total score of HHI and scores of each dimension compared with those in the control group (P<0.05). Conclusions The combination of applied behavior analytic intervention with parent-child painting and creative crafting therapy can more effectively improve the core symptoms and social interaction of preschool children with mild-to-moderate ASD, reduce the parenting stress of mothers and improve their hope level.
2021 Vol. 23 (8): 779-785 [Abstract] ( 2689 ) [HTML 1KB] [PDF 582KB] ( 948 )
786 YUAN Jin-Jing, WU De, WANG Wen-Wen, DUAN Jun, XU Xiao-Yan, TANG Jiu-Lai
A prospective randomized controlled study on mouse nerve growth factor in the treatment of global developmental delay in children
Objective To study the clinical effect of mouse nerve growth factor (mNGF) in the treatment of children with global developmental delay (GDD). Methods A prospective clinical trial was conducted in 60 children with GDD who were treated in the First Affiliated Hospital of Anhui Medical University between July 2016 and July 2017. These children were randomly divided into two groups: conventional rehabilitation treatment and mNGF treatment group (n=30 each). The children in the conventional rehabilitation treatment group were given neurodevelopmental therapy, and those in the mNGF treatment group were given mNGF treatment in addition to the treatment in the control group. The evaluation results of the Gesell Developmental Scale were compared between the two groups before and after treatment. Results Before treatment and after 1.5 months of treatment, there was no significant difference in the developmental quotient (DQ) of each functional area of the Gesell Developmental Scale between the mNGF treatment and conventional rehabilitation treatment groups (P>0.05). After 3 months of treatment, the mNGF treatment group had significantly higher DQs of gross motor, fine motor, and personal-social interaction than the conventional rehabilitation treatment group (P?0.05). The incidence rate of transient injection site pain after injection of mNGF was 7% (2/30), and there was no epilepsy or other serious adverse reactions. Conclusions In children with GDD, routine rehabilitation training combined with mNGF therapy can significantly improve their cognitive, motor, and social abilities.
2021 Vol. 23 (8): 786-790 [Abstract] ( 2563 ) [HTML 1KB] [PDF 692KB] ( 737 )
791 FANG Hai-Bo, WANG Rong, CHU Lin-Na, FENG Yan-Fang, BAI Rong-Rong, GUO Feng-Tong
Cognitive impairment in children with benign childhood epilepsy with centrotemporal spikes and attention deficit hyperactivity disorder: a prospective study
Objective To study the difference in cognitive impairment between the children with benign childhood epilepsy with centrotemporal spikes (BECT) and attention deficit hyperactivity disorder (ADHD) and those with BECT or ADHD alone. Methods A prospective study was performed on 80 children with BECT and ADHD, 91 children with BECT, and 70 children with ADHD , who were diagnosed with the diseases for the first time. Seventy children of the same age who underwent physical examination were enrolled as the healthy control group. Event-related potential P300, Wechsler Intelligence Scale for Children, and integrated visual and auditory continuous performance test were used to measure and compare each index between groups. Results Compared with the healthy control group, the BECT+ADHD group, the BECT group, and the ADHD group had a significantly prolonged P300 latency, a significant reduction in the amplitude of P300, and significant reductions in the scores of verbal comprehension index (VCI), perceptual reasoning index (PRI), working memory index (WMI), processing speed index (PSI), full scale intelligence quotient (FSIQ), auditory response control quotient (ARCQ), visual response control quotient, full response control quotient (FRCQ), auditory attention quotient (AAQ), visual attention quotient, and full attention quotient (P<0.05). Compared with the BECT group, the BECT+ADHD group had a significantly prolonged P300 latency, a significant reduction in the amplitude of P300, and significant reductions in the scores of VCI, PRI, WMI, PSI, FSIQ, and FRCQ (P<0.05). Compared with the ADHD group, the BECT+ADHD group had a significantly prolonged P300 latency, a significant reduction in the amplitude of P300, and significant reductions in the scores of VCI, PRI, FSIQ, ARCQ, FRCQ, and AAQ (P<0.05). Conclusions Compared with the children with BECT or ADHD alone, the children with both BECT and ADHD have basically the same fields of cognitive impairment but a higher degree of cognitive impairment in some fields.
2021 Vol. 23 (8): 791-796 [Abstract] ( 2203 ) [HTML 1KB] [PDF 566KB] ( 759 )
797 FU Qiang, SHI Ming-Fang, CHEN Ying
Clinical effect of alfacalcidol in children with Henoch-Sch?nlein purpura: a prospective randomized controlled trial
Objective To study the effects of alfacalcidol on serum 25-(OH)D3 level, cellular immune function, and inflammatory factors in children with Henoch-Sch?nlein purpura (HSP). Methods A total of 200 children with HSP were prospectively enrolled from June 2018 to June 2020. According to the random number table method, they were divided into an observation group and a control group (n=100 each). The control group was treated with vitamin C, rutin tablets, dipyridamole, cimetidine, calcium supplements, and glucocorticoids. In addition to the treatment for the control group, the observation group received alfacalcidol capsules (0.25 μg/d) orally before bed for 4 weeks. The two groups were compared in terms of the level of 25-(OH)D3, the percentages of T lymphocyte subsets (CD3+, CD4+, and CD8+) and NK cells, and the levels of inflammatory factors, interleukin-6 (IL-6), interleukin-17 (IL-17), interleukin-21 (IL-21), and tumor necrosis factor-α (TNF-α), before treatment and after 4 weeks of treatment. The children were followed up for 6 months to determine the recurrence rate and the incidence of renal damage. Results After treatment, the observation group showed a significantly higher serum 25-(OH)D3 level, significantly higher percentages of CD3+T cells, CD4+T cells, and NK cells, and significantly lower levels of IL-6, IL-17, IL-21, and TNF-α compared with the control group (P<0.05). After 6 months of follow-up, the recurrence rate and the incidence of renal damage in the observation group were significantly lower than those in the control group (P<0.05). Conclusions Alfacalcidol can increase the serum 25-(OH)D3 level, improve cellular immune function, decrease inflammatory factor levels, and reduce recurrence and renal damage in children with HSP.
2021 Vol. 23 (8): 797-801 [Abstract] ( 2552 ) [HTML 1KB] [PDF 565KB] ( 1140 )
802 LI Min, TANG Yu, ZHAO Er-Yao, CHEN Chao-Hui, DONG Li-Li
Relationship between MTHFR gene polymorphism and susceptibility to bronchial asthma and glucocorticoid efficacy in children
Objective To study the association of methylenetetrahydrofolate reductase (MTHFR) gene polymorphism with susceptibility to bronchial asthma and glucocorticoid (GC) efficacy in children. Methods A total of 173 children with bronchial asthma who were hospitalized between June 2018 and December 2020 were selected as the observation group. The children received aerosol inhalation of GC for three consecutive months. A total of 178 healthy children who underwent physical examination during the same period were selected as the control group. PCR was used to detect the genotypes of the MTHFR C677T for the two groups. The differences in genotype distribution between the two groups were analyzed. Children with different genotypes in the observation group were compared in terms of immunoglobulin E (IgE), interleukin-8 (IL-8), leukotriene B4 (LTB4), lung function, and clinical outcome before and after treatment. Results TT genotype and T allele were significantly more frequent in the observation group than in the control group (P<0.001). TT/CT genotypes and T allele were independent risk factors for bronchial asthma (OR=6.615 and 7.055 respectively; P<0.001). After GC treatment, the children with CC, CT or TT genotypes experienced significantly decreased levels of IgE, IL-8, and LTB4 and significantly increased forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and FEV1/FVC ratio (P<0.001). The children with TT genotype showed significantly lower levels of IL-8 and LTB4 than those with CC genotype, a significantly lower level of LTB4 than those with CT genotype, significantly higher FVC than those with CT genotype, and a significantly higher FEV1/FVC ratio than those with CC genotype (P<0.05). The children with TT genotype had better GC efficacy compared with those with CC genotype (P<0.05). TT genotype was an independent factor for good GC efficacy (OR=2.111, P=0.018). Conclusions MTHFR gene polymorphism is associated with asthma susceptibility and GC efficacy in children. Children carrying TT/CT genotypes have a higher risk of developing asthma, and those with TT genotype are more sensitive to GC treatment.
2021 Vol. 23 (8): 802-808 [Abstract] ( 1900 ) [HTML 1KB] [PDF 645KB] ( 609 )
809 LI Bing-Hui, ZHAO Chang-Liang, CAO Shun-Li, GENG Hong-Li, LI Jing-Jing, ZHU Min, NIU Shi-Ping
Effect of electrode temperature on measurements of transcutaneous carbon dioxide partial pressure and oxygen partial pressure in very low birth weight infants
Objective To evaluate the accuracy and safety of measurements of transcutaneous carbon dioxide partial pressure (TcPCO2) and transcutaneous oxygen partial pressure (TcPO2) at electrode temperatures lower than the value used in clinical practice in very low birth weight infants. Methods A total of 45 very low birth weight infants were enrolled. TcPCO2 and TcPO2 measurements were performed in these infants. Two transcutaneous monitors were placed simultaneously for each subject. One electrode was set and maintained at 42℃ used in clinical practice for neonates (control group), and the other was successively set at 38℃, 39℃, 40°C, and 41℃ (experimental group). The paired t-test was used to compare the measurement results between the groups. A Pearson correlation analysis was used to analyze the correlation between the measurement results of the experimental group and control group, and between the measurement results of experimental group and arterial blood gas parameters. Results There was no significant difference in TcPCO2 between each experimental subgroup (38-41℃) and the control group. TcPCO2 in each experimental subgroup (38-41℃) was strongly positively correlated with TcPCO2 in the control group (r>0.9, P<0.05) and arterial carbon dioxide partial pressure (r>0.8, P<0.05). There were significant differences in TcPO2 between each experimental subgroup (38-41℃) and the control group (P<0.05), but TcPO2 in each experimental subgroup (38-41℃) was positively correlated with TcPO2 in the control group (r=0.493-0.574, P<0.05) and arterial oxygen partial pressure (r=0.324-0.399, P<0.05). No skin injury occurred during transcutaneous measurements at all electrode temperatures. Conclusions Lower electrode temperatures (38-41℃) can accurately measure blood carbon dioxide partial pressure in very low birth weight infants, and thus can be used to replace the electrode temperature of 42°C. Transcutaneous measurements at the lower electrode temperatures may be helpful for understanding the changing trend of blood oxygen partial pressure.
2021 Vol. 23 (8): 809-813 [Abstract] ( 1605 ) [HTML 1KB] [PDF 515KB] ( 563 )
814 WU Xin-Ping, GU Chuan-Li, HAN Shu-Ping, DENG Xiao-Yi, CHEN Xiao-Qing, WANG Huai-Yan, LI Shuang-Shuang, WANG Jun, ZHOU Qin, HOU Wei-Wei, GAO Yan, HAN Liang-Rong, LIU Hong-Jie, YU Zhang-Bin, WANG Zeng-Qin, LI Na, LI Hai-Xin, ZHOU Jin-Jun, CHEN Shan-Shan, JIANG Shan-Yu, LU Xing-Xing, PAN Zhao-Jun, CHEN Xiao-Hui
A multicenter retrospective study on survival rate and complications of very preterm infants
Objective To study the survival rate and the incidence of complications of very preterm infants and the factors influencing the survival rate and the incidence of complications. Methods The medical data of the very preterm infants with a gestational age of <32 weeks and who were admitted to the Department of Neonatology in 11 hospitals of Jiangsu Province in China from January 2018 to December 2019 were retrospectively reviewed. Their survival rate and the incidence of serious complications were analyzed. A multivariate logistic regression analysis was used to evaluate the risk factors for death and serious complications in very preterm infants. Results A total of 2 339 very preterm infants were enrolled, among whom 2 010 (85.93%) survived and 1 507 (64.43%) survived without serious complications. The groups with a gestational age of 22-25+6 weeks, 26-26+6 weeks, 27-27+6 weeks, 28-28+6 weeks, 29-29+6 weeks, 30-30+6 weeks, and 31-31+6 weeks had a survival rate of 32.5%, 60.6%, 68.0%, 82.9%, 90.1%, 92.3%, and 94.8% respectively. The survival rate tended to increase with the gestational age (P<0.05) and the survival rate without serious complications in each gestational age group was 7.5%, 18.1%, 34.5%, 52.2%, 66.7%, 75.7%, and 81.8% respectively, suggesting that the survival rate without serious complications increased with the gestational age (P<0.05). The multivariate logistic regression analysis showed that high gestational age, high birth weight, and prenatal use of glucocorticoids were protective factors against death in very preterm infants (P<0.05), and 1-minute Apgar score ≤3 was a risk factor for death in very preterm infants (P<0.05); high gestational age and high birth weight were protective factors against serious complications in very preterm infants who survived (P<0.05), while 5-minute Apgar score ≤3 and maternal chorioamnionitis were risk factors for serious complications in very preterm infants who survived (P<0.05). Conclusions The survival rate is closely associated with gestational age in very preterm infants. A low 1-minute Apgar score (≤3) may increase the risk of death in very preterm infants, while high gestational age, high birth weight, and prenatal use of glucocorticoids are associated with the reduced risk of death. A low 5-minute Apgar score (≤3) and maternal chorioamnionitis may increase the risk of serious complications in these infants, while high gestational age and high birth weight may reduce the risk of serious complications.
2021 Vol. 23 (8): 814-820 [Abstract] ( 3783 ) [HTML 1KB] [PDF 597KB] ( 924 )
821 HUANG Xun-Bin, ZHONG Xiao, LIU Ting, CHENG Guo-Qiang, QIU Hui-Xian
Value of near-infrared spectroscopy in monitoring intestinal tissue oxygen saturation in preterm infants with hemodynamically significant patent ductus arteriosus: a prospective research
Objective To study the change in regional oxygen saturation (rSO2) of intestinal tissue in preterm infants with hemodynamically significant patent ductus arteriosus (hsPDA) by near-infrared spectroscopy, and the clinical significance of the change in intestinal oxygen level in preterm infants with hsPDA. Methods The preterm infants with patent ductus arteriosus (PDA) who had gestational age <32 weeks and/or birth weight <1 500 g were prospectively enrolled, who were admitted to the Department of Neonatology, Shenzhen Longgang Central Hospital from October 2017 to October 2020.According to the diagnostic criteria for hsPDA, the preterm infants with patent ductus arteriosus (PDA) were divided into two groups: hsPDA and non-hsPDA. According to closure of the ductus arteriosus after oral administration of ibuprofen, the preterm infants in the hsPDA group were subdivided into two groups: hsPDA closure and hsPDA non-closure. Hemodynamic parameters were measured at diagnosis of PDA and after treatment, and the level of intestinal tissue rSO2 was monitored continuously to analyze its change. Results A total of 241 preterm infants with PDA were enrolled, with 55 infants (22.8%) in the hsPDA group and 186 infants (77.2%) in the non-hsPDA group. There were 36 infants (65%) in the hsPDA closure group and 19 infants (35%) in the hsPDA non-closure group. Compared with the non-hsPDA group, the hsPDA group had a significantly higher left atrial diameter/aortic root diameter ratio and significantly lower left ventricular ejection fraction and fractional shortening (P<0.05). At each time point within 6 hours after diagnosis (1, 2, 4, and 6 hours), the hsPDA group had significantly lower intestinal tissue rSO2 than the non-hsPDA group (P<0.05), and intestinal tissue rSO2 gradually decreased over time in the hsPDA group (P<0.05), with the lowest level of 0.448±0.014 at 6 hours. Compared with the hsPDA non-closure group, the hsPDA closure group had a significantly lower left atrial diameter/aortic root diameter ratio and significantly higher left ventricular ejection fraction and fractional shortening (P<0.05). At each time point within 48-96 hours after treatment (48, 72, and 96 hours), the hsPDA closure group had significantly higher intestinal tissue rSO2 than the hsPDA non-closure group (P<0.05), and intestinal tissue rSO2 gradually increased since 24 hours after treatment in the hsPDA closure group (P<0.05), with the highest level of 0.578±0.031 at 96 hours. Conclusions hsPDA has an impact on intestinal tissue oxygenation in preterm infants, and continuous monitoring of intestinal tissue rSO2 by near-infrared spectroscopy can help to guide the clinical management of hsPDA in preterm infants.
2021 Vol. 23 (8): 821-827 [Abstract] ( 2912 ) [HTML 1KB] [PDF 565KB] ( 931 )
828 ZHANG Ru-Xin, ZHANG Xuan, ZHANG Bi-Li, LIU Zhu-Feng, LIN Shu-Xiang
Expression of adipokines in children with primary nephrotic syndrome and its association with hyperlipidemia
Objective To study the expression of adipokines in children with primary nephrotic syndrome (PNS) before and after treatment and its correlation with blood lipids, as well as the role of adipokines in PNS children with hyperlipidemia. Methods A total of 90 children who were diagnosed with incipient PNS or recurrence of PNS after corticosteroid withdrawal for more than 6 months were enrolled as subjects. Thirty children who underwent physical examination were enrolled as the control group. Venous blood samples were collected from the children in the control group and the children with PNS before corticosteroid therapy (active stage) and after urinary protein clearance following 4 weeks of corticosteroid therapy (remission stage). ELISA was used to measure the levels of adipokines. An automatic biochemical analyzer was used to measure blood lipid levels. Results Compared with the control group, the children with PNS had a significantly lower level of omentin-1 in both active and remission stages, and their level of omentin-1 in the active stage was significantly lower than that in the remission stage (P<0.001). For the children with PNS, the level of chemerin in the active stage was significantly higher than that in the remission stage, and the children with PNS in the active stage had a significantly higher level of chemerin than the control group (P<0.001). For the children with PNS, atherogenic index of plasma, atherogenic coefficient (AC), castelli risk index-1 (CRI-1), castelli risk index-2 (CRI-2), and non-high-density lipoprotein in the active stage were significantly higher than those in the remission stage (P<0.001), and these indices in the children with PNS in the active stage were significantly higher than those in the control group (P<0.001). The children with PNS in the remission stage had significantly higher atherogenic index of plasma, AC, CRI-1, and non-high-density lipoprotein than the control group (P<0.001). Compared with the control group, the children with PNS in the remission stage had significantly higher serum levels of total cholesterol, triglyceride, high-density lipoprotein, low-density lipoprotein, apolipoprotein B, and apolipoprotein A (P<0.01). In the children with PNS, the ratio of omentin-1 before and after corticosteroid therapy was positively correlated with that of high-density lipoprotein, 24-hour urinary protein excretion, and high-density lipoprotein/apolipoprotein A before and after treatment, and it was negatively correlated with the ratio of AC and CRI-1 before and after treatment (P<0.05). The PNS children with low omentin-1 levels in the active stage had significantly higher levels of CRI-1, CRI-2, AC, and apolipoprotein B/apolipoprotein A ratio than those with high omentin-1 levels (P<0.05). Conclusions Omentin-1 may be associated with disease activity, dyslipidemia, and proteinuria in children with PNS. Blood lipid ratios may be more effective than traditional blood lipid parameters in monitoring early cardiovascular risk in children with PNS.
2021 Vol. 23 (8): 828-834 [Abstract] ( 1748 ) [HTML 1KB] [PDF 558KB] ( 693 )
835 GAO Hui-Qin, GUAN Xian-Min, WEN Xian-Hao, SHEN Ya-Li, GUO Yu-Xia, DOU Ying, MENG Yan, YU Jie
Clinical features and prognosis of children with acute leukemias of ambiguous lineage under different diagnostic criteria
Objective To study the clinical features and prognosis of children with acute leukemias of ambiguous lineage (ALAL) under different diagnostic criteria. Methods A retrospective analysis was performed on the medical data of 39 children with ALAL who were diagnosed and treated from December 2015 to December 2019. Among the 39 children, 34 received treatment. According to the diagnostic criteria for ALAL by World Health Organization and European Group for the Immunological Characterization of Leukemias, the 39 children were divided into two groups: ALAL group (n=28) and myeloid expression group (n=11). The clinical features, treatment, and prognosis were compared between the two groups. Results The 34 children receiving treatment had a 3-year event-free survival (EFS) rate of 75%±9% and an overall survival rate of 88%±6%. The children treated with acute myeloid leukemia (AML) protocol had a 3-year EFS rate of 33%±27%, those treated with acute lymphoblastic leukemia (ALL) protocol had a 3-year EFS rate of 78%±10%, and those who had no remission after induction with AML protocol and then received ALL protocol had a 3-year EFS rate of 100%±0% (P<0.05). The children with negative minimal residual disease (MRD) after induction therapy had a significantly higher 3-year EFS rate than those with positive MRD (96%±4% vs 38%±28%, P<0.05). Positive ETV6-RUNX1 was observed in the myeloid expression group, and positive BCR-ABL1, positive MLL-r, and hyperleukocytosis (white blood cell count ≥50×109/L) were observed in the ALAL group. There was no significant difference in the 3-year EFS rate between the myeloid expression and ALAL groups (100%±0% vs 66%±11%, P>0.05). Conclusions ALL protocol has a better clinical effect than AML protocol in children with ALAL, and positive MRD after induction therapy suggests poor prognosis. Hyperleukocytosis and adverse genetic changes are not observed in children with myeloid expression, and such children tend to have a good prognosis, suggesting that we should be cautious to take it as ALAL in diagnosis and treatment.
2021 Vol. 23 (8): 835-840 [Abstract] ( 1917 ) [HTML 1KB] [PDF 596KB] ( 640 )
841 LI Dong-Ming, HE Sheng
Genotypes of thalassemia in children: an analysis of 30 417 cases
Objective To investigate the distribution of genotypes of thalassemia in children in Guangxi, China. Methods A total of 30 417 children with positive results of thalassemia screening in the Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region from January 2011 to December 2019 were enrolled. Single-tube multiplex PCR, agarose gel electrophoresis, and reverse dot blot hybridization technique were used for the detection of common α- and β-thalassemia genes. Gap-PCR or gene sequence analysis was performed for 2 703 children suspected of rare thalassemia. Results Among the 30 417 children with positive results of thalassemia screening, 23 214 (76.32%) were diagnosed with thalassemia, and the detection rates of α-thalassemia, β-thalassemia, and α-thalassemia with β-thalassemia were 47.77%, 23.75%, and 4.80% respectively. A total of 13 types of α-thalassemia alleles (18 480 alleles in total) were detected, mainly --SEA (54.98%), including seven rare alleles, i.e., --THAI (0.43%), HKαα (0.02%), -α30 (0.01%), -α1.0 (0.01%), -α2.4 (0.01%), -α21.9 (0.01%), and HBA2:C272-279 del (0.01%). A total of 17 types of β-thalassemia alleles (9 168 alleles in total) were detected, mainly CD41-42 (47.79%), followed by CD17 (25.53%), including three rare alleles, i.e., IVS-II-5 (0.02%), IVS-I-2 (0.01%), and Gγ(Aγδβ)0 (0.01%). A total of 37 genotypes were detected in 14 531 children with α-thalassemia, among which the most common 6 genotypes were --SEA/αα (52.20%), -α3.7/αα (13.24%), αCSα/αα (7.52%), -α4.2 (6.06%), --SEA/-α3.7 (5.91%), and αWSα/αα (3.41%), accounting for 88.34%. A total of 49 genotypes were detected in 7 223 children with β-thalassemia, among which the most common 6 genotypes were CD41-42/βN (45.81%), CD17/βN (24.30%), IVS-II-654/βN (7.49%), -28/βN (5.62%), CD71-72/βN (4.42%), and CD26/βN (3.94%), accounting for 91.13%. A total of 137 genotypes were detected in 1 460 children with both α- and β-thalassemia, mainly --SEA/αα combined with CD41-42/βN (14.17%) and CD17/βN (8.35%). A total of 2 050 children were diagnosed with hemoglobin H disease (α0+), among whom 134 had β-thalassemia heterozygote and 12 had Bart hydrops fetalis syndrome (--SEA/--SEA); 355 children were diagnosed with β-thalassemia double heterozygote, and 128 were diagnosed with β-thalassemia homozygote, including 93 children with α-thalassemia. Conclusions There are diverse gene mutations and rich genotypes of thalassemia among children in Guangxi, and α-thalassemia is more common, with --SEA/αα as the major genotype. There is a high proportion of children with both α- and β-thalassemia, and there are relatively high incidence rates of β-thalassemia double heterozygote and homozygote (intermedia and major).
2021 Vol. 23 (8): 841-847 [Abstract] ( 1995 ) [HTML 1KB] [PDF 557KB] ( 694 )
EXPERIMENTAL RESEARCH
848 YI Yan-Jun, RAN Xiao, XIANG Jing, LI Xin-Yang, JIANG Li, CHEN Heng-Sheng, HU Yue
Effect of gap junction blockers on hippocampal ripple energy expression in rats with status epilepticus
Objective To study the effect of gap junction blockers, quinine (QUIN) and carbenoxolone (CBX), on hippocampal ripple energy expression in rats with status epilepticus (SE). Methods A total of 24 rats were randomly divided into four groups: model, QUIN, valproic acid (VPA), and CBX (n=6 each). A rat model of SE induced by lithium-pilocarpine (PILO) was prepared. The QUIN, VPA, and CBX groups were given intraperitoneal injection of QUIN (50 mg/kg), VPA by gavage (200 mg/kg), and intraperitoneal injection of CBX (50 mg/kg) respectively, at 3 days before PILO injection. Electroencephalography was used to analyze the change in hippocampal ripple energy before and after modeling, as well as before and after chloral hydrate injection to control seizures. Results Ripple expression was observed in the hippocampal CA1, CA3, and dentate gyrus regions of normal rats. After 10 minutes of PILO injection, all groups had a gradual increase in mean ripple energy expression compared with 1 day before modeling, with the highest expression level before chloral hydrate injection in the model, VPA and CBX groups (P<0.05). The QUIN group had the highest expression level of mean ripple energy 60 minutes after PILO injection. The mean ripple energy returned to normal levels in the three intervention groups immediately after chloral hydrate injection, while in the model group, the mean ripple energy returned to normal levels 1 hour after chloral hydrate injection. The mean ripple energy remained normal till to day 3 after SE in the four groups. The changing trend of maximum ripple energy was similar to that of mean ripple energy. Conclusions The change in ripple energy can be used as a quantitative indicator for early warning of seizures, while it cannot predict seizures in the interictal period. Gap junction blockers can reduce ripple energy during seizures.
2021 Vol. 23 (8): 848-853 [Abstract] ( 1650 ) [HTML 1KB] [PDF 856KB] ( 562 )
REVIEW
854 GAO Yang-Yang, CHEN Xiao-Juan, LUO Rong-Mu
Research advances on haploidentical hematopoietic stem cell transplantation in the treatment of severe aplastic anemia in children
Haploidentical hematopoietic stem cell transplantation is a recommended alternative therapy for children with severe aplastic anemia who lack a human leukocyte antigen (HLA)-identical sibling donor and do not respond well to immunosuppressive therapy; however, due to non-identical HLA, the patients may have donor-specific anti-HLA antibody, which may lead to a relatively high incidence rate of poor graft function. Compared with HLA-identical transplantation, conditioning regimen for haploidentical transplantation still needs to be explored. This article reviews the detection and treatment of donor-specific anti-HLA antibody, the selection of conditioning regimen, and the mechanism and treatment of poor graft function in haploidentical hematopoietic stem cell transplantation.
2021 Vol. 23 (8): 854-859 [Abstract] ( 2832 ) [HTML 1KB] [PDF 550KB] ( 923 )
860 SUN Jing, CHEN Dan, MAO Jian
Research advances in neonatal cerebral sinovenous thrombosis
Neonatal cerebral sinovenous thrombosis (CSVT) is a cerebrovascular disease with a seriously underestimated incidence rate. Due to a lack of specific clinical manifestations and the low sensitivity of conventional imaging examinations, it has long been considered a rare disease in neonates. In recent years, the development of magnetic resonance technology has improved the diagnostic rate of CSVT. This article reviews the research advances in intracranial venous anatomy of neonates and clinical manifestations, imaging features, treatment, and prognosis of CSVT and deep venous thrombosis, in order to improve the understanding and to make correct diagnosis and treatment of neonatal CSVT.
2021 Vol. 23 (8): 860-866 [Abstract] ( 2131 ) [HTML 1KB] [PDF 923KB] ( 888 )
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