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":null,"pages":null},"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,&nbsp;R C Che,&nbsp;A H Zhang,&nbsp;G X Ding","doi":"10.3760/cma.j.cn112140-20230209-00086","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230209-00086","url":null,"abstract":"常染色体隐性遗传多囊肾病（ARPKD）是累及肾脏和肝脏的罕见纤毛病之一，也是儿童慢性肾脏病（CKD）的常见原因之一。尚缺乏针对ARPKD的特异性治疗方法，故了解其机制至关重要。信号通路异常激活、调节因子和细胞因子的异常分泌、细胞外基质重塑、细胞极性和机械性质的改变等相关机制逐渐被认识。尽管ARPKD的遗传基础是明确的，但对ARPKD的变异基因及其表达蛋白的确切功能和囊肿形成的关键分子机制的认识仍不足，相关信号通路的具体机制也不完全清楚。为了加强对ARPKD的认识，寻找临床上特异的治疗药物，对该病的致病机制进行综述。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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,&nbsp;T Yang,&nbsp;Q Zhao,&nbsp;J M Tang,&nbsp;H Chuan,&nbsp;L Lin,&nbsp;H X Gao","doi":"10.3760/cma.j.cn112140-20230324-00206","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230324-00206","url":null,"abstract":"患儿　女，30分钟龄，因呼吸急促入院。2日龄肺出血继发弥散性血管内凝血，微血栓导致右手前臂循环障碍，皮肤出现青紫，早期依据新生儿医源性皮肤损伤的管理原则进行皮肤护理，皮损恢复欠佳，手背及前臂皮肤逐渐出现红肿、坏死、溃烂，创面久不愈合，37日龄接受皮下注射自体脐带间充质干细胞［2×105个/（kg·次），隔日1次，共2次］，皮肤创面逐渐愈合，形成少量瘢痕，患儿住院111 d出院，右手经功能训练后恢复良好。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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":"[Diagnosis and treatment of anti-neutrophil cytoplasmic antibody associated vasculitis in children].","authors":"A H Zhang,&nbsp;C H Zhu","doi":"10.3760/cma.j.cn112140-20230703-00437","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230703-00437","url":null,"abstract":"抗中性粒细胞胞质抗体相关性血管炎（AAV）是一类以小血管炎症和坏死、寡免疫复合物沉积为特征的系统性血管炎，临床罕见，预后差。AAV的诊断主要依赖于临床表现和ANCA检查，临床诊断时有延误。因此，提高AAV的早期诊断率，有助于早期治疗，改善预后。儿童AAV主要目标是尽早诊断，及时诱导缓解，维持治疗以减少疾病复发。激素、免疫抑制剂、生物制剂是治疗AAV的重要药物。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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":"[Expert consensus on the diagnosis and treatment of bronchiolitis obliterans in children (2023)].","authors":"","doi":"10.3760/cma.j.cn112140-20230301-00146","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230301-00146","url":null,"abstract":"2012年我国制定的“儿童闭塞性细支气管炎的诊断和治疗建议”，为临床医生认识闭塞性细支气管炎（BO）的病因和临床诊疗提供了很大帮助，现由中华医学会儿科学分会呼吸学组、中国医师协会呼吸医师分会儿科呼吸工作委员会、中国医药教育协会儿科专业委员会、中华儿科杂志编辑委员会共同发起“儿童闭塞性细支气管炎的诊断和治疗专家共识（2023）”，对2012年建议进行更新。进一步规范儿童BO的诊断、治疗及预防，提高儿科医生对该病的认知度，落实有效防治措施。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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":"[Recent advances in the diagnosis and treatment of Fabry disease].","authors":"J L Liu,&nbsp;H Xu","doi":"10.3760/cma.j.cn112140-20230314-00179","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230314-00179","url":null,"abstract":"法布雷病是一种罕见的 X 连锁遗传溶酶体贮积症，是由于 GLA 基因变异引起 α 半乳糖苷酶 A（α-Gal A）活性异常，从而代谢底物在多脏器贮积，导致多系统受累。儿童期早筛、早诊、早治是关键。法布雷病需多学科综合管理，本文着重介绍儿童法布雷病症状、诊断和鉴别诊断、治疗、筛查和遗传咨询进展的相关知识，有助于儿科医师提高对本病的认知和防治水平。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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":"[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":"https://doi.org/10.3760/cma.j.cn112140-20230531-00368","url":null,"abstract":"抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）是一类发病原因不明，以小血管炎症和纤维素样坏死为主要病理改变、累及全身的自身免疫性疾病。AAV患儿肾脏受累导致ANCA相关性肾炎（AAGN），引起肾功能持续性恶化，是儿童终末期肾病的重要原发疾病。为规范儿童AAGN的诊断与治疗，改善AAGN预后，中华医学会儿科学分会肾脏学组、中华儿科杂志编辑委员会联合发起制订“中国儿童抗中性粒细胞胞质抗体相关性肾炎诊断与治疗临床实践指南（2023）”，为临床医务工作者对儿童AAGN的诊断、治疗等重要问题提供规范的指导。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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":"[Diagnosis and treatment of familial adenomatous polyposis in children].","authors":"Y L Hu,&nbsp;W X Chen,&nbsp;Y Jin,&nbsp;H Yang","doi":"10.3760/cma.j.cn112140-20230221-00117","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230221-00117","url":null,"abstract":"儿童家族性腺瘤性息肉病（FAP）是一种常染色体显性遗传病，主要由腺瘤性息肉病（APC）基因突变引起，多数患儿有家族史。FAP患儿的结直肠中常出现成百上千个腺瘤，发展为腺癌的风险较高。内镜检查和基因检测有利于早期诊断和干预。FAP复发率高且无有效根治方法。现就儿童FAP的诊断和治疗进行综述。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":null,"pages":null},"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":"[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":null,"pages":null},"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":"[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":null,"pages":null},"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}