CJCP
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2022 Vol.  24 No.  6
Published: 2022-06-28
STANDARD·PROTOCOL·GUIDELINE
EXPERT LECTURE
CLINICAL RESEARCH
CASE ANALYSIS
REVIEW
STANDARD·PROTOCOL·GUIDELINE
597 Shaanxi Province Diagnosis and Treatment Center of Kawasaki Disease/Children's Hospital of Shaanxi ProvincialPeople's Hospital, Children's Hospital of Shanghai Jiao Tong University, Beijing Children's Hospital of Capital MedicalUniversity, Shengjing Hospital of China Medical University, Affiliated Hospital of Yan'an University, Expert Committeeof Advanced Training for Pediatrician, China Maternal and Children's Health Association, General Pediatric Group ofPediatrician Branch of Chinese Medical Doctor Association, Pediatric International Exchange and Cooperation Center,Shanghai Cooperation Organization Hospital Cooperation Alliance, Editorial Board of Chinese Journal ofContemporary Pediatrics
Pediatric expert consensus on the application of aspirin in Kawasaki disease
Kawasaki disease (KD) is one of the common acquired heart diseases in children aged <5 years and is an acute systemic vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for the prevention of coronary artery lesion in acute KD; however, there are still controversies over the role and optimal dose of aspirin. The consensus was formulated based on the latest research findings of KD treatment in China and overseas and comprehensive discussion of pediatric experts in China and put forward recommendations on the dose, usage, and course of aspirin treatment in the first-line treatment of KD.
2022 Vol. 24 (6): 597-603 [Abstract] ( 2953 ) [HTML 1KB] [PDF 629KB] ( 1163 )
EXPERT LECTURE
604 YAO Kai-Hu, MENG Qing-Hong, YU Dan
The investigation on the acute, severe hepatitis of unknown origin in children Hot!
In April 2022, the United Kingdom notified the World Health Organization (WHO) of an unexpected increase in acute hepatitis of unknown origin in children. Subsequent investigations have found more than 400 cases in more than 20 countries and regions around the world. Although the potential role of adenovirus type 41 in the pathogenesis of these cases is one hypothesis, it is probably not the only pathogenic factor, and other infectious and non-infectious causes cannot be completely ruled out. For hepatitis caused by non-hepatitis A, B, C, D and E viruses, there is a lack of systematic monitoring and research, and many unknowns still exist. According to the current etiology speculation and epidemiological characteristics of adenovirus in China, cases of acute hepatitis with unknown origin may be found in China in the future. There is also a risk of imported cases. This article systematically sorts out the reports and studies on child acute hepatitis of unknown origin, hoping to attract the attention of pediatric clinicians in China, raise awareness and vigilance, and calmly prepare for possible abnormal situations.
2022 Vol. 24 (6): 604-613 [Abstract] ( 2050 ) [HTML 1KB] [PDF 655KB] ( 671 )
604 YAO Kai-Hu, MENG Qing-Hong, YU Dan
The investigation on the acute, severe hepatitis of unknown origin in children Hot!
In April 2022, the United Kingdom notified the World Health Organization (WHO) of an unexpected increase in acute hepatitis of unknown origin in children. Subsequent investigations have found more than 400 cases in more than 20 countries and regions around the world. Although the potential role of adenovirus type 41 in the pathogenesis of these cases is one hypothesis, it is probably not the only pathogenic factor, and other infectious and non-infectious causes cannot be completely ruled out. For hepatitis caused by non-hepatitis A, B, C, D and E viruses, there is a lack of systematic monitoring and research, and many unknowns still exist. According to the current etiology speculation and epidemiological characteristics of adenovirus in China, cases of acute hepatitis with unknown origin may be found in China in the future. There is also a risk of imported cases. This article systematically sorts out the reports and studies on child acute hepatitis of unknown origin, hoping to attract the attention of pediatric clinicians in China, raise awareness and vigilance, and calmly prepare for possible abnormal situations.
2022 Vol. 24 (6): 604-613 [Abstract] ( 753 ) [HTML 1KB] [PDF 1219KB] ( 494 )
614 MA Jing, ZHOU Wen-Hao
Genetic characteristics of microtia-associated syndromes in neonates
Microtia is the second most common maxillofacial birth defect in neonates and has an prevalence rate of 3.06/10 000 in China, and 20%-60% of microtia cases is associated with a certain type of syndrome. This article elaborates on the clinical phenotypes and genetic characteristics of three microtia-associated syndromes (MASs) with high prevalence, high incidence rate of ear deformity, and definite genetic etiology, i.e., oculo-auriculo-vertebral spectrum, branchio-oto-renal spectrum disorder, and Treacher-Collins syndrome, and summarizes another three common MASs, so as to provide a reference for the genetic diagnosis of neonatal MAS.
2022 Vol. 24 (6): 614-619 [Abstract] ( 1801 ) [HTML 1KB] [PDF 566KB] ( 782 )
CLINICAL RESEARCH
620 YAO De-Zhen, WANG Li-Jie
Monitoring of gastrointestinal dysfunction by near-infrared spectroscopy in children with sepsis: a prospective study
Objective To investigate the incidence and mortality of gastrointestinal dysfunction in children with sepsis, the application of near-infrared spectroscopy (NIRS) in monitoring mesenteric regional tissue oxygen saturation (rSO2), and the association between rSO2 and gastrointestinal dysfunction. Methods In this prospective study, 79 children with sepsis in the pediatric intensive care unit (sepsis group) and 40 children who underwent physical examination in the Department of Child Healthcare (healthy control group) from January to December, 2021 were enrolled as subjects. The related medical data were collected, including general information on admission and at discharge, treatment during hospitalization, and laboratory examination results. NIRS was used to measure mesenteric rSO2. Clinical characteristics were compared between the patients with and without gastrointestinal dysfunction. Results For the 79 children with sepsis, the incidence rate of gastrointestinal dysfunction was 49% (39/79), and the mortality rate of the children with gastrointestinal dysfunction was 26% (10/39). The children with gastrointestinal dysfunction had a longer duration of mechanical ventilation and a higher 28-day mortality rate (P<0.05). The children with gastrointestinal dysfunction had a significantly lower median rSO2 (64%) than the children without gastrointestinal dysfunction (72%) and the healthy control group (78%) (P<0.05). Conclusions There are high incidence and mortality rates of gastrointestinal dysfunction in children with sepsis, and the reduction in rSO2 may be associated with the development of gastrointestinal dysfunction.
2022 Vol. 24 (6): 620-625 [Abstract] ( 1810 ) [HTML 1KB] [PDF 549KB] ( 566 )
626 LUO You-You, FANG You-Hong, YU Jin-Dan, XU Luo-Jia, SUN Ming-Fang, CHENG Qi, CHEN Jie
Effectiveness of induction therapy with exclusive enteral nutrition in pediatric Crohn's disease
Objective To evaluate the effectiveness of induction therapy with exclusive enteral nutrition (EEN) in pediatric Crohn's disease (CD). Methods A retrospective analysis was performed on the medical data of 62 children with CD who received EEN in Children's Hospital, Zhejiang University School of Medicine, from March 2013 to August 2021. The medical data included general information and height, weight, Pediatric Crohn's Disease Activity Index (PCDAI), Crohn's Disease Endoscopic Index of Severity, C-reactive protein, erythrocyte sedimentation rate, and serum albumin level before treatment and after 8 weeks of treatment. The changes in the above indicators were compared before and after treatment. Results Among the 62 children with CD, there were 39 boys (63%) and 23 girls (37%), with a mean age of (11.9±3.0) years at diagnosis. Among the 55 children who completed EEN treatment for at least 8 weeks, 48 (87%) achieved clinical remission at week 8. PCDAI at week 8 was significantly lower than that before treatment (P<0.001). Except for 17 children with involvement of the small intestine alone and 3 children with involvement of the colon who did not receive colonoscopy reexamination, the remaining 35 children with involvement of the colon received colonoscopy reexamination after the 8-week EEN treatment. Of the 35 children, 29 (83%) achieved mucosal healing. As for the 48 children who achieved clinical remission at week 8, there were significant improvements in height-for-age Z-score and body mass index-for-age Z-score at week 8 (P<0.01). As for the 7 children who did not achieve clinical remission at week 8, there were no significant changes in height-for-age Z-score and body mass index-for-age Z-score at week 8 (P>0.05). Conclusions The 8-week EEN treatment has a good effect on clinical remission and mucosal healing in children with CD. For the children with CD achieving clinical remission, EEN can improve their height and body mass index.
2022 Vol. 24 (6): 626-630 [Abstract] ( 1593 ) [HTML 1KB] [PDF 528KB] ( 640 )
631 WANG Yu-Fei, LIANG Guang-Li, WANG Wei, QU Cheng-Bin, LI Chun-Yin, WANG Qing-Xin
')" href="#"> Application of "kindergarten effect" in radiotherapy for children with tumor aged 3-5 years
Objective To study the clinical application effect of "kindergarten effect" in radiotherapy for children with tumor based on the psychology of preschool children aged 3-5 years. Methods A total of 30 children, aged 3-5 years, who were admitted to the Department of Radiotherapy, Tianjin Medical University Cancer Institute and Hospital, from January 2020 to August 2021 were enrolled in this prospective study. The children were randomly divided into a control group and a test group, with 15 children in each group. The children in the test group were treated in "kindergarten mode", i.e., all children were treated together at a specified time and left together after all children completed treatment. Those in the control group were treated alternately with adult patients according to the treatment time based on the type of radiotherapy fixation device. The treatment compliance was evaluated for both groups, and the two groups were compared in terms of the setup errors in the superior-inferior (SI), left-right (LR), and anterior-posterior (AP) directions. Results Compared with the control group, the test group showed a significantly shorter time for finishing the treatment (P<0.05) and a significantly lower proportion of children with treatment interruption (P<0.05). Compared with the control group, the test group showed smaller mean errors in the SI, LR and AP directions after image-guided radiotherapy, with significant differences in the mean errors in the SI and LR directions (P<0.05). Conclusions With the application of the "kindergarten effect", most children can actively cooperate in radiotherapy, and it can also improve the accuracy and repeatability of positioning and help to achieve the desired treatment outcome.
2022 Vol. 24 (6): 631-634 [Abstract] ( 1426 ) [HTML 1KB] [PDF 517KB] ( 482 )
635 WANG Zi-Qi, MENG Yan, DOU Ying, GUAN Xian-Min, ZHANG Lu-Ying, YU Jie
Clinical effect of allogeneic hematopoietic stem cell transplantation in children with hyper-IgM syndrome
Objective To evaluate the clinical effect of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with hyper-IgM syndrome (HIGM). Methods A retrospective analysis was performed on the medical data of 17 children with HIGM who received allo-HSCT. The Kaplan Meier method was used for the survival analysis of the children with HIGM after allo-HSCT. Results After allo-HSCT, 16 children were diagnosed with sepsis; 14 tested positive for virus within 100 days after allo-HSCT, among whom 11 were positive for Epstein-Barr virus, 7 were positive for cytomegalovirus, and 2 were positive for JC virus; 9 children were found to have invasive fungal disease. There were 6 children with acute graft-versus-host disease and 3 children with chronic graft-versus-host disease. The median follow-up time was about 2 years, and 3 children died in the early stage after allo-HSCT. The children had an overall survival (OS) rate of 82.35%, an event-free survival (EFS) rate of 70.59%, and a disease-free survival (DFS) rate of 76.47%. The univariate analysis showed that the children receiving HLA-matched allo-HSCT had a significantly higher EFS rate than those receiving HLA-mismatched allo-HSCT (P=0.019) and that the children receiving HLA-matched unrelated allo-HSCT had significantly higher OS, EFS, and DFS rates than those receiving HLA-mismatched unrelated allo-HSCT (P<0.05). Compared with the children with fungal infection after allo-HSCT, the children without fungal infection had significantly higher EFS rate (P=0.02) and DFS rate (P=0.04). Conclusions Allo-HSCT is an effective treatment method for children with HIGM. HLA-matched allo-HSCT and active prevention and treatment of fungal infection and opportunistic infection may help to improve the prognosis of such children.
2022 Vol. 24 (6): 635-642 [Abstract] ( 1789 ) [HTML 1KB] [PDF 871KB] ( 675 )
643 HU Xiao-Shan, ZHANG Min, JIANG Cheng-Yao, YU Zhang-Bin, GU Nan
Influence of the coronavirus disease 2019 pandemic on maternal breastfeeding for very low birth weight infants
Objective To investigate the changes in the rate and volume of mother's own milk (MOM) feeding for very low birth weight infants (VLBWIs) hospitalized during the prevention and control of the coronavirus disease 2019 (COVID-19) pandemic. Methods A retrospective analysis was performed on the medical data of the VLBWIs with a gestational age of <32 weeks who were born and admitted to the Neonatal Intensive Care Unit of Nanjing Maternal and Child Health Hospital from January 2019 to December 2020. The changes in the rate and volume of MOM feeding for VLBWIs during hospitalization were examined. Results A total of 301 VLBWIs were enrolled. According to the timing of COVID-19 outbreak, these infants were divided into a pre-CIVID-19 group with 205 VLBWIs and a post-COVID-19 group with 96 VLBWIs. Compared with the pre-CIVID-19 group, the post-COVID-19 group had a significantly lower rate of MOM feeding within 28 days after birth and during hospitalization (P<0.05), a significantly lower volume of MOM feeding within 0-7 days, 0-14 days, and 0-28 days after birth (P<0.05), and significantly higher incidence rates of moderate-to-severe bronchopulmonary dysplasia and feeding intolerance (P<0.05). Conclusions The COVID-19 pandemic has a significant impact on MOM feeding for VLBWIs, and there are significant reductions in the rate and volume of MOM feeding for VLBWIs within 28 days after birth, as well as a significant reduction in the rate of MOM feeding during hospitalization.
2022 Vol. 24 (6): 643-647 [Abstract] ( 1708 ) [HTML 1KB] [PDF 516KB] ( 583 )
648 WANG Lin, ZHAO Xiao-Peng, LIU Hui-Juan, DENG Li, LIANG Hong, DUAN Si-Qin, YANG Yi-Hui, ZHANG Hua-Yan
Evidence-based standardized nutrition protocol can shorten the time to full enteral feeding in very preterm/very low birth weight infants
Objective To investigate whether evidence-based standardized nutrition protocol can facilitate the establishment of full enteral nutrition and its effect on short-term clinical outcomes in very preterm/very low birth weight infants. Methods A retrospective analysis was performed on the medical data of 312 preterm infants with a gestational age of ≤32 weeks or a birth weight of <1 500 g. The standardized nutrition protocol for preterm infants was implemented in May 2020; 160 infants who were treated from May 1, 2019 to April 30, 2020 were enrolled as the control group, and 152 infants who were treated from June 1, 2020 to May 31, 2021 were enrolled as the test group. The two groups were compared in terms of the time to full enteral feeding, the time to the start of enteral feeding, duration of parenteral nutrition, the time to recovery to birth weight, the duration of central venous catheterization, and the incidence rates of common complications in preterm infants. Results Compared with the control group, the test group had significantly shorter time to full enteral feeding, time to the start of enteral feeding, duration of parenteral nutrition, and duration of central venous catheterization and a significantly lower incidence rate of catheter-related bloodstream infection (P<0.05). There were no significant differences between the two groups in the mortality rate and the incidence rate of common complications in preterm infants including grade II-III necrotizing enterocolitis (P>0.05). Conclusions Implementation of the standardized nutrition protocol can facilitate the establishment of full enteral feeding, shorten the duration of parenteral nutrition, and reduce catheter-related bloodstream infection in very preterm/very low birth weight infants, without increasing the risk of necrotizing enterocolitis.
2022 Vol. 24 (6): 648-653 [Abstract] ( 2063 ) [HTML 1KB] [PDF 565KB] ( 699 )
654 JUE Zhen-Zhen, SONG Juan, ZHOU Zhu-Ye, LI Wen-Dong, YUE Yu-Yang, XU Fa-Lin
Establishment of a predictive nomogram model for predicting the death of very preterm infants during hospitalization
Objective To establish a nomogram model for predicting the risk of death of very preterm infants during hospitalization. Methods A retrospective analysis was performed on the medical data of 1 714 very preterm infants who were admitted to the Department of Neonatology, the Third Affiliated Hospital of Zhengzhou University, from January 2015 to December 2019. These infants were randomly divided into a training cohort (1 179 infants) and a validation cohort (535 infants) at a ratio of 7∶3. The logistic regression analysis was used to screen out independent predictive factors and establish a nomogram model, and the feasibility of the nomogram model was assessed by the validation set. The area under the receiver operating characteristic curve (AUC), calibration curve, and decision curve analysis (DCA) were used to assess the discriminatory ability, accuracy, and clinical applicability of the model. Results Among the 1 714 very preterm infants, 260 died and 1 454 survived during hospitalization. By the multivariate logistic regression analysis of the training set, 8 variables including gestational age <28 weeks, birth weight <1 000 g, severe asphyxia, severe intraventricular hemorrhage (IVH), grade III-IV respiratory distress syndrome (RDS), and sepsis, cesarean section, and use of prenatal glucocorticoids were selected and a nomogram model for predicting the risk of death during hospitalization was established. In the training cohort, the nomogram model had an AUC of 0.790 (95%CI: 0.751-0.828) in predicting the death of very preterm infants during hospitalization, while in the validation cohort, it had an AUC of 0.808 (95%CI: 0.754-0.861). The Hosmer-Lemeshow goodness-of-fit test showed a good fit (P>0.05). DCA results showed a high net benefit of clinical intervention in very preterm infants when the threshold probability was 10%-60% for the training cohort and 10%-70% for the validation cohort. Conclusions A nomogram model for predicting the risk of death during hospitalization has been established and validated in very preterm infants, which can help clinicians predict the probability of death during hospitalization in these infants.
2022 Vol. 24 (6): 654-661 [Abstract] ( 2064 ) [HTML 1KB] [PDF 821KB] ( 884 )
662 HE Xiao-Guang, LI Jin-Feng, XU Feng-Dan, XIE Hao-Qiang, HUANG Tian-Li
Clinical features of severe meconium aspiration syndrome (MAS) and early predicting factors for severe MAS in neonates with meconium-stained amniotic fluid
Objective To study the clinical features of severe meconium aspiration syndrome (MAS) and early predicting factors for the development of severe MAS in neonates with meconium-stained amniotic fluid (MSAF). Methods A total of 295 neonates who were hospitalized due to Ⅲ° MSAF from January 2018 to December 2019 were enrolled as subjects. The neonates were classified to a non-MAS group (n=199), a mild/moderate MAS group (n=77), and a severe MAS group (n=19). A retrospective analysis was performed for general clinical data, blood gas parameters, infection indicators, and perinatal clinical data of the mother. The respiratory support regimens after birth were compared among the three groups. The receiver operating characteristic (ROC) curve and multivariate logistic regression analysis were used to investigate predicting factors for the development of severe MAS in neonates with MSAF. Results Among the 295 neonates with MSAF, 32.5% (96/295) experienced MAS, among whom 20% (19/96) had severe MAS. Compared with the mild/moderate MAS group and the non-MAS group, the severe MAS group had a significantly lower 5-minute Apgar score (P<0.05) and a significantly higher blood lactate level in the umbilical artery (P<0.05). Compared with the non-MAS group, the severe MAS group had a significantly higher level of interleukin-6 (IL-6) in peripheral blood at 1 hour after birth (P<0.017). In the severe MAS group, 79% (15/19) of the neonates were born inactive, among whom 13 underwent meconium suctioning, and 100% of the neonates started to receive mechanical ventilation within 24 hours. Peripheral blood IL-6 >39.02 pg/mL and white blood cell count (WBC) >30.345×109/L at 1 hour after birth were early predicting indicators for severe MAS in neonates with MSAF (P<0.05). Conclusions Meconium suctioning cannot completely prevent the onset of severe MAS in neonates with MSAF. The neonates with severe MAS may develop severe respiratory distress and require mechanical ventilation early after birth. Close monitoring of blood lactate in the umbilical artery and peripheral blood IL-6 and WBC at 1 hour after birth may help with early prediction of the development and severity of MAS.
2022 Vol. 24 (6): 662-668 [Abstract] ( 2087 ) [HTML 1KB] [PDF 650KB] ( 641 )
669 WANG Zeng-Qin, GAO Yan, DONG Xiao-Yue, WANG Huai-Yan, LU Hong-Yan, CHEN Xiao-Qing, XUE Mei, ZHANG Jia, WU Ming-Fu, WAN Jun, WU Xin-Ping, PAN Zhao-Jun, DENG Xiao-Yi, HAN Shu-Ping, QIAO Yu, YANG Li, YU Zhang-Bin
Preadmission follow-up condition of neonates hospitalized due to severe hyperbilirubinemia after discharge from the department of obstetrics and influencing factors for follow-up compliance: a multicenter investigation
Objective To investigate the preadmission follow-up condition of neonates hospitalized due to severe hyperbilirubinemia after discharge from the department of obstetrics and the influencing factors for follow-up compliance. Methods A multicenter retrospective case-control study was performed for the cases from the multicenter clinical database of 12 units in the Quality Improvement Clinical Research Cooperative Group of Neonatal Severe Hyperbilirubinemia in Jiangsu Province of China from January 2019 to April 2021. According to whether the follow-up of neonatal jaundice was conducted on time after discharge from the department of obstetrics, the neonates were divided into two groups: good follow-up compliance and poor follow-up compliance. The multivariate logistic regression model was used to identify the influencing factors for follow-up compliance of the neonates before admission. Results A total of 545 neonates with severe hyperbilirubinemia were included in the study, with 156 neonates (28.6%) in the good follow-up compliance group and 389 (71.4%) in the poor follow-up compliance group. The multivariate logistic regression analysis showed that low gestational age at birth, ≥10% reduction in body weight on admission compared with birth weight, history of phototherapy of siblings, history of exchange transfusion of siblings, Rh(-) blood type of the mother, a higher educational level of the mother, the use of WeChat official account by medical staff to remind of follow-up before discharge from the department of obstetrics, and the method of telephone notification to remind of follow-up after discharge were associated with the increase in follow-up compliance (P<0.05). Conclusions Poor follow-up compliance is observed for the neonates with severe hyperbilirubinemia after discharge from the department of obstetrics, which suggests that it is necessary to further strengthen the education of jaundice to parents before discharge and improve the awareness of jaundice follow-up. It is recommended to remind parents to follow up on time by phone or WeChat official account.
2022 Vol. 24 (6): 669-674 [Abstract] ( 1708 ) [HTML 1KB] [PDF 545KB] ( 576 )
675 TONG Ping, HUANG Fu-Rong, XU Jun, WU Zi-Qi, HU Xing, LING Ming, WANG Die, WU Bu-Fei, YANG Du-Jiao, ZHANG Ai-Min
Metabolomic changes of neonatal sepsis: an exploratory clinical study
Objective To study the metabolic mechanism of neonatal sepsis at different stages by analyzing the metabolic pathways involving the serum metabolites with significant differences in neonates with sepsis at different time points after admission. Methods A total of 20 neonates with sepsis who were hospitalized in the Department of Neonatology, Hunan Provincial People's Hospital, from January 1, 2019 to January 1, 2020 were enrolled as the sepsis group. Venous blood samples were collected on days 1, 4, and 7 after admission. Ten healthy neonates who underwent physical examination during the same period were enrolled as the control group. Ultra-performance liquid chromatography-quadrupole time-of-flight mass spectrometry was used for the metabonomic analysis of serum samples to investigate the change in metabolomics in neonates with sepsis at different time points. Results On day 1 after admission, the differentially expressed serum metabolites between the sepsis and control groups were mainly involved in the biosynthesis of terpenoid skeleton. For the sepsis group, the differentially expressed serum metabolites between days 1 and 4 after admission were mainly involved in pyruvate metabolism, and those between days 4 and 7 after admission were mainly involved in the metabolism of cysteine and methionine. The differentially expressed serum metabolites between days 1 and 7 after admission were mainly involved in ascorbic acid metabolism. Conclusions The metabolic mechanism of serum metabolites varies at different stages in neonates with sepsis and is mainly associated with terpenoid skeleton biosynthesis, pyruvate metabolism, cysteine/methionine metabolism, and ascorbic acid metabolism.
2022 Vol. 24 (6): 675-680 [Abstract] ( 1930 ) [HTML 1KB] [PDF 760KB] ( 686 )
681 HU Hui-Min, CHEN Xiao-Zheng, ZHANG Yong-Lan, DU Zhong-Dong
Association of liver damage with coronary artery lesion and no response to intravenous immunoglobulin in the acute stage of Kawasaki disease
Objective To summarize the clinical features of liver damage in children in the acute stage of Kawasaki disease (KD), and to investigate the clinical value of liver damage in predicting coronary artery lesion and no response to intravenous immunoglobulin (IVIG) in children with KD. Methods The medical data were collected from 925 children who were diagnosed with KD for the first time in Beijing Children's Hospital from January 1, 2016 to December 31, 2017. According to the presence or absence of abnormal alanine aminotransferase (ALT) level on admission, the children were divided into a liver damage group (n=284) and a non-liver damage group (n=641). A logistic regression analysis was used to investigate the clinical value of the indicators including liver damage in predicting coronary artery lesion and no response to IVIG in children with KD. Results Compared with the non-liver damage group, the liver damage group had a significantly earlier admission time and significantly higher serum levels of inflammatory indicators (P<0.05). The liver damage group had a significantly higher incidence rate of coronary artery lesion on admission than the non-liver damage group (P=0.034). After initial IVIG therapy, the liver damage group had a significantly higher proportion of children with no response to IVIG than the non-liver damage group (P<0.001). In children with KD, coronary artery lesion was associated with the reduction in the hemoglobin level and the increases in platelet count, C-reactive protein, and ALT (P<0.05), and no response to IVIG was associated with limb changes, the reduction in the hemoglobin level, the increases in platelet count, C-reactive protein, and ALT, and coronary artery lesion (P<0.05). Conclusions Compared with those without liver damage, the children in the early stage of KD with liver damage tend to develop clinical symptoms early and have higher levels of inflammatory indicators, and they are more likely to have coronary artery lesion and show no response to IVIG treatment.
2022 Vol. 24 (6): 681-686 [Abstract] ( 1740 ) [HTML 1KB] [PDF 537KB] ( 622 )
687 WANG Xue-Mei, NURIDDIN Hailigulli, LIU Yu, MAIMAITI Gulibaha, YAN Mei
Expression of thyroglobulin antibody and thyroid peroxidase antibody in children with immune thrombocytopenia
Objective To examine the expression of serum thyroglobulin antibody (TGAb) and thyroid peroxidase antibody (TPOAb) in children with immune thrombocytopenia (ITP). Methods A total of 120 children with ITP who were admitted from October 2019 to October 2021 were enrolled as the ITP group. A total of 60 children without ITP were enrolled as the non-ITP group. According to the clinical classification of ITP, the children in the ITP group were further divided into a newly diagnosed ITP group, a persistent ITP group, and a chronic ITP group. The clinical data were compared between the ITP group and the non-ITP group and between the children with different clinical classifications of ITP. The expression levels of serum TGAb and TPOAb in children with ITP were measured and their association with the clinical classification of ITP was analyzed. Results Compared with the non-ITP group, the ITP group had significantly lower levels of CD3+, CD4+, and platelet count (PLT) and significantly higher levels of CD8+, TGAb, and TPOAb (P<0.05). The children with chronic ITP had significantly lower levels of CD3+, CD4+, and PLT and significantly higher levels of CD8+, TGAb, and TPOAb than those with newly diagnosed ITP or persistent ITP (P<0.05). The logistic regression analysis showed that CD3+, CD4+, CD8+, TGAb, and TPOAb were the influencing factors for chronic ITP (P<0.05). A decision curve was plotted, and the results showed that TGAb combined with TPOAb within the high-risk threshold range of 0.0-1.0 had a net benefit rate of >0 in evaluating the clinical classification of ITP in children. Conclusions TGAb and TPOAb are abnormally expressed in children with ITP and are associated with the clinical classification of ITP in children.
2022 Vol. 24 (6): 687-692 [Abstract] ( 1881 ) [HTML 1KB] [PDF 648KB] ( 546 )
693 LIU Yan, WANG Yuan-Yuan, CHENG Yan, TAN Xiao-Yan, YANG Chun
Growth and development of children and related influencing factors: a cross-sectional study of the families with children aged 0-6 years in Jiangsu Province
Objective To investigate the growth and development of children aged 0-6 years in Jiangsu Province of China and the effects of family factors on the children's growth and development. Methods The stratified random sampling method was used to select 2 004 families with children aged 0-6 years of Jiangsu Province. A questionnaire survey on nutritional knowledge, attitude, and behavior was performed for the main caregivers. The Z-score method was used to evaluate nutritional status. The ordinal polytomous logistic regression analysis model was used to investigate the influencing factors for nutritional status. Results A total of 2 290 questionnaires were distributed, among which 2 004 valid questionnaires were collected (87.51%). The mean age of the children was (28±14) months, and there were 1 105 boys (55.14%) and 899 girls (44.86%). The primary caregivers were mostly parents (1 867 families, 93.16%), and the caregivers of 1 466 families (73.15%) had a university/college degree. Most families had an annual income of 50 000-100 000 yuan/year or 100 000-200 000 yuan/year, accounting for 32.88% and 34.23%, respectively. The results of Z-score showed the growth retardation rates of 1.45% (16/1 105) in boys and 1.78% (16/899) in girls, the underweight rates of 0.54% (6/1 105) in boys and 0.67% (6/899) in girls, the emaciation rates of 1.36% (15/1 105) in boys and 2.00% (18/899) in girls, and the obesity rates of 8.78% (97/1 105) in boys and 10.12% (91/899) in girls, respectively. The multivariate logistic regression analysis showed that a higher annual family income and a higher nutrition knowledge level of caregiver were beneficial to the growth and development of children (P<0.05). Conclusions Children aged 0-6 years in Jiangsu Province have a good status of growth and development but a relatively high obesity rate, and therefore, the monitoring of obesity should be taken seriously. Family economic status and the level of nutritional knowledge of caregivers have a significant impact on children's growth and development.
2022 Vol. 24 (6): 693-698 [Abstract] ( 1468 ) [HTML 1KB] [PDF 561KB] ( 776 )
CASE ANALYSIS
699 TIAN Mao-Qiang, CHEN Xiao-Xi, LI Lei, LANG Chang-Hui, LI Juan, CHEN Jing, YU Xiao-Hua, SHU Xiao-Mei
Progressive psychomotor regression for 2.5 years in a boy aged 5 years
A boy, aged 5 years, attended the hospital due to progressive psychomotor regression for 2.5 years. Motor function regression was the main manifestation in the early stage, and brain MRI and whole-exome sequencing (WES) of the family showed no abnormalities. After the age of 4 years and 9 months, the boy developed cognitive function regression, and brain MRI showed cerebellar atrophy. The reanalysis of WES results revealed a compound heterozygous mutation, [NM_000520, c.784C>T(p.His262Tyr]), c.1412C>T(p.Pro471Leu)], in the HEXA gene. The enzyme activity detection showed a significant reduction in the level of β-hexosaminidase encoded by this gene. The boy was diagnosed with juvenile Tay-Sachs disease (TSD). TSD has strong clinical heterogeneity, and cerebellar atrophy may be an important clue for the diagnosis of juvenile TSD. The reanalysis of genetic data when appropriate based on disease evolution may improve the positive rate of WES.
2022 Vol. 24 (6): 699-704 [Abstract] ( 1347 ) [HTML 1KB] [PDF 1973KB] ( 630 )
705 DENG Liang-Ji, XIONG Jie, ZHONG Li-Li, LIN Xiao-Juan, XIAO Xu-Ping, MAO Zhi-Qun
')" href="#"> "Massive hemoptysis" and shock after fever and cough
A boy, aged 11 years, was admitted due to intermittent fever for 15 days, cough for 10 days, and "hemoptysis" for 7 days. The boy had fever and cough with left neck pain 15 days ago, and antibiotic treatment was effective. During the course of disease, the boy developed massive "hemoptysis" which caused shock. Fiberoptic bronchoscopy revealed a left pyriform sinus fistula with continuous bleeding. In combination with neck and vascular imaging examination results, the boy was diagnosed with internal jugular vein injury and thrombosis due to congenital pyriform sinus fistula infection and neck abscess. The boy was improved after treatment with temperature-controlled radiofrequency ablation for the closure of pyriform sinus fistula, and no recurrence was observed during the follow-up for one year and six months. No reports of massive hemorrhage and shock due to pyriform sinus fistula infection were found in the searched literature, and this article summarizes the clinical features, diagnosis, and treatment of this boy, so as to provide a reference for the early diagnosis of such disease and the prevention and treatment of its complications.
2022 Vol. 24 (6): 705-710 [Abstract] ( 1468 ) [HTML 1KB] [PDF 810KB] ( 568 )
REVIEW
711 HUANG Zhe-Lan, ZHOU Wen-Hao
Research advances in the clinical genetics of leukodystrophy in children
Leukodystrophy (LD) is a group of genetic heterogeneous diseases characterized by primary abnormalities in glial cells and myelin sheath, and it is a common nervous system disease in children and has significant genotype-phenotype correlation. In recent years, the improvement in high-throughput sequencing has changed the diagnostic and therapeutic mode of LD, and elaborative phenotype analysis, such as the collection of natural history and multimodal neuroimaging evaluation during development, also provides important information for subsequent genetic diagnosis. This article reviews LD from the perspective of clinical genetics, in order to improve the awareness of this disease among pediatricians in China.
2022 Vol. 24 (6): 711-716 [Abstract] ( 2015 ) [HTML 1KB] [PDF 522KB] ( 900 )
717
2022 Vol. 24 (6): 717-717 [Abstract] ( 384 ) [HTML 1KB] [PDF 952KB] ( 410 )
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