Zhonghua er ke za zhi = Chinese journal of pediatrics最新文献

{"title":"[Clinical characteristics and related factors analysis of adrenal crisis occurred in children with primary nephrotic syndrome].","authors":"N Guan,&nbsp;H J Xiao,&nbsp;B G Su,&nbsp;X H Zhong,&nbsp;F Wang,&nbsp;S N Zhu","doi":"10.3760/cma.j.cn112140-20230509-00323","DOIUrl":"10.3760/cma.j.cn112140-20230509-00323","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the clinical characteristics and related factors of corticosteroid induced adrenal crisis (AC) in children with primary nephrotic syndrome (NS). <b>Methods:</b> Case control study. The case group included 7 children aged 1 to 18 years with NS combined with AC hospitalized in Peking University First Hospital from January 2016 to May 2021 (AC group). According to the ratio of case group: control group 1: 4, 28 children aged 1 to 18 years who were diagnosed with NS without AC during the same period were matched as controls (non-AC group). Clinical data were collected. The clinical characteristics of AC were described. The clinical parameters were compared between the 2 groups by <i>t</i> test, Mann-Whitney <i>U</i> test or Fisher's test. Receiver operating characteristic (ROC) curve was used to analyze the cutoff values of clinical parameters for prediction of AC. <b>Results:</b> The AC group included 4 boys and 3 girls aged 6.9 (4.6, 10.8) years. The non-AC group included 20 boys and 8 girls aged 5.2 (3.3, 8.4) years. All AC events occurred during the relapse of NS with infection. Seven children had gastrointestinal symptoms such as nausea, vomiting and abdominal pain. Six children had poor mental state or impaired consciousness. No significant differences in NS course, corticosteroid treatment course, corticosteroid type, steroid dosage, steroid medication interval, the proportion of gastroenteritis and fever existed between the two groups (all <i>P</i>>0.05). Compared with the non-AC group, the duration from the onset of the relapse of NS until hospitalization in the AC group was significantly shorter (0.2 (0.1, 0.6) <i>vs.</i> 1.0 (0.4, 5.0) month,<i>U</i>=25.50<i>, P</i>=0.005). The 24 h urinary total protein (UTP) level was significantly higher in the AC group (193 (135, 429) <i>vs.</i> 81 (17, 200) mg/kg, <i>U</i>=27.00<i>,P</i>=0.036) than the non-AC group. The serum albumin level in the AC group was significantly lower((13.1±2.1) <i>vs.</i> (24.5±8.7) g/L,<i>t=</i>-6.22,<i>P</i><0.001) than the non-AC group. There were significantly higher total white blood cell counts ((26±9)×10⁹ <i>vs.</i> (11±5)×10⁹/L,<i>t=</i>4.26,<i>P</i>=0.004), percentage of neutrophils (0.71±0.08 <i>vs.</i> 0.60±0.19,<i>t=</i>2.56,<i>P</i>=0.017) and the proportion of children with C reactive protein level≥8 mg/L (3/7 <i>vs.</i> 0,<i>P</i>=0.005) in the AC group than in the non-AC group. ROC curve analysis showed that the cutoff value of 24 h UTP was 122 mg/(kg·d) with a sensitivity of 100.0% and specificity of 70.4%. The cutoff value of serum albumin was 17.0 g/L with a sensitivity of 100.0% and specificity of 82.1%. <b>Conclusions:</b> Gastrointestinal symptoms and poor mental state were prominent manifestations of AC in children with NS. High 24 h UTP level, low serum albumin level, high peripheral white blood cell counts, high neutrophils percentage, and high C-reactive protein level during the early s","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"805-810"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10128835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Research progress on the pathogenesis autosomal recessive polycystic kidney disease].","authors":"H X Li, R C Che, A H Zhang, G X Ding","doi":"10.3760/cma.j.cn112140-20230209-00086","DOIUrl":"10.3760/cma.j.cn112140-20230209-00086","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"848-851"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10130961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Autologous umbilical cord mesenchymal stem cells for treatment of severe skin injury in an extremely low birth weight infant].","authors":"G C Chen, T Yang, Q Zhao, J M Tang, H Chuan, L Lin, H X Gao","doi":"10.3760/cma.j.cn112140-20230324-00206","DOIUrl":"10.3760/cma.j.cn112140-20230324-00206","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"839-841"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10130966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Expert consensus on the diagnosis and treatment of bronchiolitis obliterans in children (2023)].","authors":"","doi":"10.3760/cma.j.cn112140-20230301-00146","DOIUrl":"10.3760/cma.j.cn112140-20230301-00146","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"786-793"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10125563","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Diagnosis and treatment of anti-neutrophil cytoplasmic antibody associated vasculitis in children].","authors":"A H Zhang, C H Zhu","doi":"10.3760/cma.j.cn112140-20230703-00437","DOIUrl":"10.3760/cma.j.cn112140-20230703-00437","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"769-772"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10125559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Recent advances in the diagnosis and treatment of Fabry disease].","authors":"J L Liu, H Xu","doi":"10.3760/cma.j.cn112140-20230314-00179","DOIUrl":"10.3760/cma.j.cn112140-20230314-00179","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"860-862"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10130965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A control study of steroid withdrawal protection strategy after kidney transplantation in children].","authors":"J Y Lu,&nbsp;M Zhang,&nbsp;J A Lin,&nbsp;H R Chen,&nbsp;Y J Li,&nbsp;X Gao,&nbsp;C X Wang,&nbsp;L S Liu,&nbsp;X Liao","doi":"10.3760/cma.j.cn112140-20230212-00097","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230212-00097","url":null,"abstract":"<p><p><b>Objective:</b> To study the influence of steroid withdrawal protection strategy on height growth in pediatric patients after kidney transplantation. <b>Methods:</b> The prospective cohort study enrolled 40 stage 5 chronic kidney disease children receiving kidney transplantation from July 2017 to September 2022 at Guangzhou Women and Children's Medical Center. Based on the primary preoperative disease, patients with immune abnormality-associated glomerular diseases or unknown causes were assigned to the steroid maintenance group, in which patients received steroid tapering within 3 months after surgery to a maintenance dose of 2.5 to 5.0 mg/d. While patients with hereditary kidney disease or congenital urinary malformations were assigned to the steroid withdrawal group, in which patients had steroids tapered off within 3 months. The characteristics of height catch-up growth and clinical data were compared between the 2 groups at baseline, 6, 12, 18 and 24 months after kidney transplantation. T-test, repeated measurement of variance analysis, Mann-Whitney <i>U</i> test, and Fisher exact test were used for the comparison between the 2 groups. <b>Results:</b> Among the 40 children, 17 were males, 23 were females, 25 were in the steroid withdraw group ((7.8±2.8) years old when receiving kidney transplantation) and 15 cases were in the steroid maintenance group ((7.6±3.5) years old when receiving kidney transplantation). The study population was followed up for (26±12) months. The total dose per unit body weight of steroids in the steroid withdrawal group was lower than that in the steroid maintenance group ((0.13±0.06) <i>vs.</i> (0.36±0.19) mg/(kg·d), <i>t</i>=5.83, <i>P</i><0.001). The height catch-up rate (ΔHtSDS) in the first year after kidney transplantation in the steroid withdraw and steroid maintenance groups was 1.0 (0.7, 1.4) and 0.4 (0.1, 1.0), respectively; in the second year, the ΔHtSDS in the steroid withdraw group was significantly higher than that in the steroid maintenance group (1.1 (0.2, 1.7) <i>vs</i>. 0.3 (0, 0.8), <i>U</i>=28.00, <i>P=</i>0.039). The HtSDS in the steroid withdrawal group at the five follow-up time points was -2.5±0.8, -2.0±0.8, -1.5±0.8, -1.3±0.9 and -0.5±0.3, respectively, while in the steroid maintenance was -2.4±1.3, -2.2±1.1, -2.0±1.0, -1.8±1.0 and -1.6±1.0, respectively. There were statistically significant differences in HtSDS at different follow-up time points in both 2 groups (<i>F=</i>19.81, <i>P</i><0.01), but no statistical differences in overall impact between the 2 groups (<i>F=</i>1.13, <i>P=</i>0.204). The steroid treatment was interaction with the increase of follow-up time (<i>F=</i>3.62, <i>P=</i>0.009). At the 24^th month after transplantation, the HtSDS in the steroid withdrawal group was significantly higher than that in the steroid maintenance group (<i>P=</i>0.047). Six patients in the steroid withdrawal group experienced antibody-mediated immune rejection (AMR), while 3 ","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"799-804"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10125566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Diagnosis and treatment of familial adenomatous polyposis in children].","authors":"Y L Hu, W X Chen, Y Jin, H Yang","doi":"10.3760/cma.j.cn112140-20230221-00117","DOIUrl":"10.3760/cma.j.cn112140-20230221-00117","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"856-859"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10125560","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Evidence-based guideline for diagnosis and treatment of pediatric anti-neutrophil cytoplasmic antibody associated glomerulonephritis (2023)].","authors":"","doi":"10.3760/cma.j.cn112140-20230531-00368","DOIUrl":"10.3760/cma.j.cn112140-20230531-00368","url":null,"abstract":"","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"773-785"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10184657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Investigation of extrauterine growth restriction in very preterm infants in Chinese neonatal intensive care units].","authors":"Y Y Lyu,&nbsp;Y Cao,&nbsp;Y X Chen,&nbsp;H Y Wang,&nbsp;L Zhou,&nbsp;Y Wang,&nbsp;Y C Wang,&nbsp;S Y Jiang,&nbsp;K L E E Lee,&nbsp;L Li,&nbsp;J H Sun","doi":"10.3760/cma.j.cn112140-20230609-00388","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230609-00388","url":null,"abstract":"<p><p><b>Objective:</b> To comprehensively assess the current status of extrauterine growth restriction (EUGR) in very preterm infants (VPI) and its associated factors in Chinese neonatal intensive care units (NICU). <b>Methods:</b> In this cohort study, 6 179 preterm infants born at <32 weeks' gestation were included, who were admitted to 57 hospitals in the China Neonatal Network in 2019 and hospitalized for ≥7 days. EUGR was evaluated by a cross-sectional definition (weight at discharge<10^th percentile for postmenstrual age), a longitudinal definition (decline in weight Z score>1 from birth to discharge), and weight growth velocity. The comparison between infants with and without EUGR was conducted by <i>t</i>-test, Mann-Whitney <i>U</i> test or <i>χ</i>² test as appropriate. Multivariable Logistic regression models were used to evaluate associations between EUGR with different definitions and maternal and neonatal factors, clinical practices, and neonatal morbidities. <b>Results:</b> A total of 6 179 VPI were enrolled in the study, with a gestational age of (29.8±1.5) weeks and birth weight of (1 365±304) g; 56.2% (3 474) of them were male. Among them, 48.4% (2 992 VPI) were cross-sectional EUGR and 74.9% (4 628 VPI) were longitudinal EUGR. Z score of weight was (0.13±0.78) at birth and decrease to (-1.35±0.99) at discharge. The weight growth velocity was 10.13 (8.42, 11.66) g/(kg·d). Multivariate Logistic regression analysis showed that among the influential factors that could be intervened after birth, late attainment of full enteral feeds (<i>OR</i>_adjust=1.01, 95%<i>CI</i> 1.01-1.02, <i>P</i><0.001; <i>OR</i>_adjust=1.01, 95%<i>CI</i> 1.01-1.02, <i>P</i><0.001), necrotizing enterocolitis≥Ⅱstage (<i>OR</i>_adjust=2.64, 95%<i>CI</i> 1.60-4.35, <i>P</i><0.001; <i>OR</i>_adjust=1.62, 95%<i>CI</i> 1.10-2.40, <i>P</i><0.001) and patent ductus arteriosus (<i>OR</i>_adjust=1.94, 95%<i>CI</i> 1.50-2.51, <i>P</i><0.001; <i>OR</i>_adjust=1.63, 95%<i>CI</i> 1.29-2.06, <i>P</i><0.001) were all associated with increased risks of both cross-sectional and longitudinal EUGR. In addition, late initiation of enteral feeds (<i>OR</i>_adjust=1.06, 95%<i>CI</i> 1.02-1.09, <i>P</i>=0.020) and respiratory distress syndrome (<i>OR</i>_adjust=1.45, 95%<i>CI</i> 1.24-1.69, <i>P</i><0.001) were all associated with cross-sectional EUGR. Breast milk feeding (<i>OR</i>_adjust=1.33, 95%<i>CI</i> 1.05-1.68, <i>P</i><0.001) was associated with a higher risk of longitudinal EUGR. <b>Conclusions:</b> The incidence of EUGR in VPI in China is high. Some modifiable risk factors provide priorities to improve postnatal growth for VPI. Nutritional management of VPI and the efforts to decrease the incidence of complications are still the focus of clinical management in China.</p>","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 9","pages":"811-819"},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10130957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}