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非酒精性脂肪肝病(non-alcoholic fatty liver disease,NAFLD)是一种肝组织学改变与酒精性肝病相似但无过量饮酒史的临床综合征,主要包括单纯性脂肪浸润、非酒精性脂肪肝炎(nonalcoholic steatohepatitis,NASH)和脂肪性肝硬化三种病理类型。其发病机制包括以胰岛素抵抗为主的一次打击和以氧化应激/脂质过氧化损伤为主的二次     

The association between non-alcoholic fatty liver disease and insulin resistance in 20 obese children and adolescents

AIM: To investigate whether there are correlations between non-alcoholic fatty liver disease (NAFLD) and insulin resistance in obese children. For the first time, we present clinical data of 20 obese children with NAFLD, including an oral glucose tolerance test. METHODS: Twenty obese children were diagnosed as having NAFLD by abdominal ultrasonography. Serum levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST) and gamma-glutamyl transferase (gamma-GT) were reported. Insulin sensitivity was evaluated by oral glucose tolerance test, oral glucose insulin sensitivity (OGIS) and homeostasis is model assessment (HOMA) index. All parameters were compared to 20 obese age- and sex-matched patients without NAFLD. RESULTS: With 81% the prevalence of insulin resistance according to HOMA or OGIS criteria was high in the NAFLD-patients compared to the other group with 63%. Statistically significant differences between both groups were found for mean serum ALT levels, mean glucose levels after 30, 60 and 90 minutes and mean insulin levels after 60 minutes of the glucose tolerance test. CONCLUSION: The high prevalence of insulin resistance we found in children with NAFLD confirms the suggestion that there may be an association between insulin resistance and NAFLD in obese children and indicates that markers of insulin sensitivity could be useful screening parameters for NAFLD.     

Surgical treatment of Crohn disease in children and adolescents; how conservative can the paediatrician be?

Thirty-eight children (21 male, 17 female, age 3–18 years), treated for Crohn disease in two Dutch university centres, were retrospectively studied in order to evaluate the results of conservative treatment and to find out in what way surgical treatment in this age group may have differed from treating adults with this disease. Both groups had an equal distribution of age and sex. Diarrhoea with discharge of blood and mucus, abdominal pain, nausea/vomiting, weight loss, fever and general discomfort were the most frequent presenting symptoms. Twenty-three children (60%) showed signs of malabsorption; 4 children (10%) had growth retardation. In 27 children (70%), 63 surgical procedures were performed (2.4 operations per child). There was no surgical mortality. Most operations were performed for ileocolitis and colon-only localizations needed most re-operations. Of the surgical procedures performed, 55% were excisional procedures. Already 3 years after the onset of symptoms, 50% of all children had had their first resection, whereas in adults, 50% of the patients undergo surgery 8 years after disease onset. Eight children were treated with split ileostomy. In only one of these children, operated for non-toxic colitis and severe steroid-dependent growth retardation, could the colon eventually be saved. The time between the onset of symptoms and the first operation seems to be shorter in children compared to adults. Severe malabsorption and growth retardation are additional specific indications for surgery for Crohn disease in childhood. The latter combined with non-toxic colitis, may perhaps be the only indication left for performing split ileostomy in Crohn disease.     

Levels of lipoprotein(a) and plasma lipids in Spanish children aged from 4 to 18 years

Increased plasma lipoprotein(a)-Lp(a)-levels are linked to a high risk of cardiovascular disease unrelated to other lipoproteins. It seems that Lp(a) values in childhood remain unaltered up to adulthood. In a randomly chosen population of 1970 children, aged from 4 to 18 years and living in a Spanish community, the following serum parameters were studied: total cholesterol, total triglycerides, Lp(a), high-density lipoprotein cholesterol and low-density lipoprotein cholesterol. Mean Lp(a) serum values were 15.0 ± 14.7mg dl^-1. No differences were seen between either sex in the first years of childhood. Of the studied children, 15.1% presented Lp(a) concentrations above 30 mg dl^-1. A correlation between Lp(a) and total cholesterol concentrations, which disappeared when low-density lipoprotein cholesterol concentrations were corrected according to cholesterol present in Lp(a), was observed.     

儿童青少年腰围身高比与非酒精性脂肪肝病关系的回顾性分析

背景：肥胖是导致儿童青少年人群非酒精性脂肪肝病（NAFLD）发生的重要原因之一,腰围身高比（WHtR）是反映内脏脂肪和评价儿童青少年心血管代谢风险的简单而准确的体格测量指标,但WHtR 与NAFLD的关系研究十分有限。 目的：分析儿童青少年WHtR与NAFLD的关系。 设计：常规体检数据的回顾性分析。 方法：以所有参加上海市闵行区2014至2020年住校学生健康体检的学生为研究对象,将血清ALT水平高于一般人群性别和年龄别第97.5百分位数水平定义为疑似NAFLD（简称NAFLD）。基于全国数据提示心血管代谢风险聚集的WHtR作为切点值,以男孩和女孩WHtR分别≥0.481和≥0.456定义为WHtR升高;以非条件二分类Logistic回归模型,校正年龄、性别等协变量后,分析WHtR升高与NAFLD的关系。通过计算AUC、敏感度、特异度、阳性预测值和阴性预测值,评价WHtR升高对NAFLD的区分效果。 主要结局指标：WHtR与NAFLD的关联性。 结果：与WHtR正常组相比,NAFLD患病率在 WHtR升高人群中显著升高（16.2% vs 2.3%, P<0.001）,且随着年龄的增长呈现上升趋势。在WHtR升高人群中,男孩NAFLD患病率高于女孩（21.6% vs 11.0%,P<0.001）,而在WHtR正常人群中男孩和女孩的NAFLD患病率接近（2.3% vs 2.2%, P=0.71）。WHtR升高人群NAFLD的发生风险增加 71%,校正的OR =1.71,95% CI：1.26~2.31,P=0.001。分层分析结果显示WHtR升高分别能增加男孩77%（OR=1.77,95% CI：1.19~2.63,P=0.005）和女孩69% （OR=1.69,95% CI：1.05~2.71,P=0.005）的NAFLD发生风险 。WHtR升高区分NAFLD的AUC为0.73（95% CI：0.71~0.76）,敏感度63.2%、特异度83.4%、阳性预测值16.8%和阴性预测值97.7%。 结论：儿童青少年WHtR升高与NAFLD的发生独立相关;学校和社区等基层医疗保健机构要重点关注WHtR升高的人群,除了血压、糖脂代谢异常以外,还需特别关注NAFLD的患病情况。     

非酒精性脂肪肝病儿童、青少年的肝内脂肪定量评价

目的：量化评估非酒精性脂肪肝病(NAFLD)儿童、青少年肝内脂肪的临床意义。方法：对93例肥胖儿童、青少年进行形体学参数、肝功能、血脂、血糖及胰岛素释放试验和肝脏B超等检查,采用氢质子磁共振波谱定量测定肝内脂肪（IHF）含量,并按传统诊断标准将其分为单纯性肥胖组（31例）、非酒精性脂肪肝病-1组（NAFLD-1,33例）（B超示脂肪肝,但不伴有肝损）和NAFLD-2组（B超示脂肪肝,且伴有肝损,29例）。20例健康儿童、青少年作为正常对照组。观察IHF含量在组间的变化情况,分析IHF含量与各临床生化指标的相关关系以及探寻IHF量化改变的影响因素。结果：正常对照组、单纯性肥胖组、NAFLD-1组和NAFLD-2组IHF含量分别为0.80%（0.4%～1.0%）、2.9%（1.7%～4.3%）、14.0%（7.2%～17.5%）、18.8%（14.0%~29.1%）,组间两两比较差异均有统计学意义（P<0.05）。单因素相关分析表明IHF含量与腰围、臀围、腰臀比、体重指数、收缩压、舒张压、丙氨酸氨基转移酶、门冬氨酸氨基转移酶、γ-谷氨酰转肽酶、空腹甘油三酯、低密度脂蛋白、口服葡萄糖耐量试验（OGTT）2 h血糖、空腹胰岛素、2 h胰岛素和胰岛素抵抗指数呈显著正相关,与高密度脂蛋白呈显著负相关。进一步的多元线性回归分析发现增加的腰围、降低的高密度脂蛋白水平和增高的OGTT 2 h血糖是IHF含量升高的独立危险因素。结论：IHF量化改变非常敏感,早期已增高于单纯性肥胖儿童、青少年,并随NAFLD的发生、发展进程而显著升高。量化的IHF与诸多临床生化指标相关,其中腰围、高密度脂蛋白和OGTT 2 h血糖是IHF含量的独立影响因素。     

江西地区23例18岁以下儿童2019冠状病毒病临床分析

目的 研究18岁以下儿童2019冠状病毒病（COVID-19）的临床特征。方法 回顾性分析江西地区2020年1月21日至2020年2月29日确诊的23例儿童COVID-19的临床资料（患儿年龄为3个月至17岁8个月）。结果 23例COVID-19患儿中，17例为家庭聚集性发病；无症状感染3例（13%），轻型6例（26%），普通型14例（61%）。发热16例（70%），咳嗽11例（48%），发热伴咳嗽8例（35%），肺部湿啰音8例（35%）。从发病或首次新型冠状病毒SARS-CoV-2核酸阳性至核酸转阴时间为6~24 d（中位数12 d）。3例白细胞总数降低，2例淋巴细胞下降，2例C反应蛋白增高，2例D-二聚体增高。12例肺部CT异常，其中肺内片状磨玻璃影9例。23例均应用了抗病毒治疗，均治愈出院。结论 18岁以下儿童COVID-19多为家庭聚集性发病，临床表现、实验室检查无特异性，症状较轻，预后较好。流行病学史在18岁以下儿童COVID-19诊断中非常重要。     

上海市闵行区儿童青少年非酒精性脂肪肝病7年患病率横断面调查

背景：儿童非酒精性脂肪肝病（NAFLD）导致成年后心血管病发病风险明显增加,而目前我国儿童青少年一般人群NAFLD患病率研究十分有限,长期变化趋势亦不明确。目的：描述儿童青少年NAFLD患病流行趋势。设计：横断面调查。方法：以上海市闵行区2014至2020年参加健康体检的住校学生为调查对象。将ALT水平高于一般人群性别和年龄别P97.5水平定义为疑似NAFLD,依此估计疑似NAFLD患病率（简称患病率）。根据全国标准分别以BMI和腰围定义一般性肥胖和腹型肥胖。通过计算平均年度变化百分比（AAPC）及其95%CI行描述NAFLD患病率逐年变化幅度,趋势性检验Logistic回归分析,并校正BMI和腰围。主要结局指标：NAFLD患病率。结果：NAFLD总体患病率为5.1%,从2014年至2020年上升了2.5倍（2.1%到7.4%）,NAFLD患病率逐年上升（Ptrend<0.001）,AAPC为0.9%（95%CI：0.1%~1.7%）,且男孩高于女孩（6.3% vs 3.7%）。在体重正常人群中NAFLD患病率为1.9%,7年中呈逐年上升趋势（Ptrend<0.001）。调整BMI和腰围后,NAFLD患病率逐年上升的趋势仍显著（Ptrend<0.001）。结论：上海儿童青少年人群NAFLD患病率呈现每年上升近1.0%的趋势,调整BMI和腰围因素后,NAFLD患病率逐年上升的趋势仍然存在。提示除肥胖外,儿童人群防控NAFLD需进一步关注其他可干预危险因素的作用。     

Impaired intention‐to‐treat survival after listing for liver transplantation in children with biliary atresia compared to other chronic liver diseases: 20 years’ experience from the Nordic countries

Biliary atresia (BA) is the most common indication for LT in children. We investigated whether this diagnosis per se, compared to other chronic liver diseases (OCLD), had an influence on patient survival. Data from 421 Scandinavian children, 194 with BA and 227 with OCLD, listed for LT between 1990 and 2010 were analyzed. The intention‐to‐treat survival and influencing risk factors were studied. Patients with BA had higher risk of death after listing than patients with OCLD. The youngest (<1 year) and smallest (<10 kg) children with the highest bilirubin (>510 μmol/L), highest INR (>1.6), and highest PELD score (>20) listed during 1990s had the worst outcome. Given the same PELD score, patients with BA had higher risk of death than patients with OCLD. For adolescents, low weight/BMI was the only prognostic marker. Impaired intention‐to‐treat survival in patients with BA was mainly explained by more advanced liver disease in younger ages and higher proportion of young children in the BA group rather than diagnosis per se. PELD score predicted death, but seemed to underestimate the severity of liver disease in patients with BA. Poor nutritional status and severe cholestasis had negative impact on survival, supporting the “sickest children first” allocation policy and correction of malnutrition before surgery.     

Lung function and bronchial hyper-reactivity from 11 to 18 years in children with bronchiolitis in infancy

Background

Various trajectories for lung function and bronchial hyper-reactivity (BHR) from early childhood to adulthood are described, including puberty as a period with excessive lung growth. Bronchiolitis in infancy may be associated with increased risk of developing chronic obstructive pulmonary disease, but the development of respiratory patterns during puberty is poorly characterized for these children. We aimed to study the development and trajectories of lung function and BHR from 11 to 18 years of age in children hospitalized for bronchiolitis in infancy.

Methods

Infants hospitalized for bronchiolitis at the University Hospitals in Stavanger and Bergen, Norway, during 1997-1998, and an age-matched control group, were included in a longitudinal follow-up study and examined at 11 and 18 years of age with spirometry and methacholine provocation test (MPT). The MPT data were managed as dose-response slope (DRS) in the statistical analyses. Changes in lung function and DRS from 11 to 18 years of age were analyzed by generalized estimating equations, including interaction terms.

Results

z-scores for forced vital capacity (FVC), forced expiratory volume in first second (FEV₁), FEV₁/FVC ratio, and DRS were not different from 11 to 18 years of age in both the post-bronchiolitis and the control group. The trajectories from 11 to 18 years did not differ between the two groups. BHR at age 11 was independently associated with asthma at age 18.

Conclusion

Children hospitalized for bronchiolitis had stable predicted lung function and BHR from 11 to 18 years of age. The lung function trajectories were not different from controls.

     

Combined liver and pancreas transplantation in two children with cystic fibrosis—First experience in Australia and New Zealand

We report the first two pediatric patients with CF who underwent successful combined liver‐pancreas transplantation in Australia and New Zealand for CF liver disease and CF‐related diabetes mellitus.     

Evaluation of nonalcoholic fatty liver disease using magnetic resonance in obese children and adolescents

Objective

To determine the frequency of nonalcoholic fatty liver disease using nuclear magnetic resonance as a noninvasive method.

无创肝纤维化评估在儿童肝豆状核变性进展期肝纤维化诊断价值

目的 探讨临床生化指标、瞬时弹性成像技术及无创肝纤维化评分在肝豆状核变性(WD)儿童进展期肝纤维化诊断中的价值。方法 收集2018年10月至2021年7月22例WD患儿的临床资料。患儿均行肝脏穿刺,采用瞬时弹性成像技术检测肝脏硬度值(LSM)。根据病理检查结果将患儿分为进展期肝纤维化(S3和S4期)和非进展期肝纤维化(S0、S1和S2期)两组,计算3种无创肝纤维化评分(Sheth、APRI和FIB-4指数),比较两组间临床生化指标、无创肝纤维化评分和LSM的差异。采用受试者工作特征(ROC)曲线分析各指标对进展期肝纤维化的诊断价值。结果 22例WD患儿中,男12例、女10例,平均年龄(8.6±2.7)岁;非进展期肝纤维化为13例(59.1%),进展期肝纤维化9例(40.9%)。与非进展期肝纤维化组相比,进展期肝纤维化组中白蛋白、天冬氨酸氨基转移酶(AST)和血小板(PLT)水平较低,凝血酶原时间(PT)和国际标准化比值(INR)水平较高,LSM、Sheth和FIB-4指数较高,差异均有统计学意义(P均<0.05)。ROC曲线分析提示,LSM(AUC=1.0)对进展期肝纤维化的诊断...     

Cross-calibration of multi-frequency bioelectrical impedance analysis with eight-point tactile electrodes and dual-energy X-ray absorptiometry for assessment of body composition in healthy children aged 6–18 years

Background:  In diagnosis and treatment of obesity, body composition analysis including percent body fat (%BF) is useful in the clinical setting. Because bioelectrical impedance analysis (BIA) could be used quickly, easily and was non-invasive in clinical setting, the purpose of the present study was to evaluate the usefulness of multi-frequency BIA with eight-point tactile electrodes (MF-BIA8; InBody 720, Biospace) compared with dual-energy X-ray absorptiometry (DXA) in healthy children and adolescents.
Methods:  A total of 166 children and adolescents under 18 (male, n  = 86; female, n  = 80) were recruited. Height, weight, body mass index (BMI) and Tanner stage were measured for each subject. The body composition such as fat-free mass (FFM), fat mass (FM), and %BF was measured on BIA and DXA and compared.
Results:  On linear regression analysis, DXA FFM = 1.006(BIA FFM) + 0.554, R ² = 0.99 and the standard error of the estimate (SEE) was 1.16 kg; DXA FM = 0.971(BIA FM) – 0.596, R ² = 0.93; SEE, 1.34 kg; and DXA %BF = 0.940(BIA %BF) – 1.026, R ² = 0.858; SEE, 3.03%. Limit of agreement in FFM, FM, and %BF was 0.7 ± 2.3 kg, −0.9 ± 2.9 kg and –2.2 ± 6.1%, respectively.
Conclusions:  Although the %BF was not interchangeable with DXA, MF-BIA8 (InBody 720; Biospace) could be used to measure body composition of children and adolescents in the clinical field because of its high precision.