新生儿单纯肠穿孔临床分析

目的 分析新生儿单纯肠穿孔的发病机制、临床特点、外科治疗以及预后.方法 对1990年1月至2005年12月本院新生儿外科收治的18例单纯肠穿孔(ⅡP)以及16例坏死性小肠结肠炎(NEC)肠穿孔进行回顾性对比研究.结果 与NEC穿孔相比,ⅡP常发生于胎龄较大、出生体重较高的新生儿.ⅡP平均发病年龄为(20.0±17.8)d,晚于NEC组[(7.8±2.6)d,P＜0.05],发病形式较缓,从发病至肠穿孔平均时间为(5.0±4.1)d[NEC组(2.6±2.0)d,P＜0.05].50%病例穿孔前合并腹泻、肠炎或其他炎性疾病的病史,穿孔以回盲部(60%)及横结肠脾曲(22%)为多见.其病死率为16%(NEC组56%,P＜0.05),预后明显好于NEC穿孔组.结论 新生儿单纯肠穿孔为区别于NEC穿孔的独立疾病,有不同发病机制和治疗预后.     

新生儿肠穿孔101例临床分析

目的分析新生儿肠穿孔的临床特点,为改善新生儿肠穿孔的预后提供理论依据。方法回顾性分析2000年1月至2014年6月入住新生儿重症监护病房的101例新生儿肠穿孔患儿的临床资料。结果新生儿肠穿孔的主要病因是新生儿坏死性小肠结肠炎(NEC,41例,40.6%),其次为特发性肠穿孔(17例,16.8%)、先天性巨结肠(10例,9.9%)。特发性肠穿孔组患儿平均出生体重和平均胎龄明显高于NEC组(P0.05);NEC组致病菌以肠球菌为主,特发性肠穿孔组以革兰阴性菌为主,两组病原菌分布不同(P0.05)。Logistic多元回归分析显示,酸中毒、多部位肠穿孔、穿孔至手术时间较长是新生儿死亡的独立危险因素。结论新生儿肠穿孔病因多样,以NEC为主;NEC所致肠穿孔与特发性肠穿孔具有不同的致病菌,两者可能是相互独立的疾病;早期诊断、尽早手术是挽救新生儿肠穿孔患儿生命的主要措施。     

新生儿胃肠穿孔80例临床特点及预后分析

目的分析新生儿胃肠穿孔的病因、临床特征、治疗及预后。方法回顾性分析2004年1月至2015年12月复旦大学附属儿科医院新生儿科收治的80例新生儿胃肠穿孔患儿的临床资料,根据出生胎龄分为早产儿组与足月儿组,比较两组间的病因、临床表现、治疗及预后。结果 80例新生儿胃肠穿孔中,早产儿62例,足月儿18例。两组病因均以坏死性小肠结肠炎(necrotizing enterocolitis,NEC)为主,临床均以腹胀为主要表现,早产儿发生反应差、休克、弥散性血管内凝血明显多于足月儿(P0.05)。早产儿组平均发病日龄9(1.75,20)d,足月儿组平均发病日龄4.5(1,7.75)d。62例手术治疗,其中胃穿孔8例,肠穿孔54例,18例因未手术穿孔部位不明确。死亡32例,病死率40%,早产儿死亡26例(病死率41.9%),足月儿死亡6例(病死率33.3%)。结论新生儿胃肠穿孔是新生儿期的严重疾病,病死率高。早期诊断、积极治疗和尽早外科干预可能提高患儿的存活率,显著改善患儿的预后。     

新生儿坏死性小肠结肠炎肠穿孔与肠未穿孔患儿术后结局的对比研究CSCD

目的对比新生儿坏死性小肠结肠炎(necrotizing enterocolitis,NEC)肠穿孔与肠未穿孔患儿手术治疗后转归情况,为NEC手术时机的选择及术后治疗提供参考。方法回顾性分析2009年8月至2019年8月中国人民解放军总医院第七医学中心儿科医学部新生儿外科收治的237例经手术治疗的NEC患儿临床资料。按照是否发生肠穿孔分为肠未穿孔组(172例)与肠穿孔组(65例),收集两组患儿术中所见坏死肠管长度、手术后实施肠内及肠外营养时间、呼吸机使用时间、NICU入住时间、术后并发症以及预后情况。结果NEC肠未穿孔组172例中,治愈124例(124/172,72.1%),死亡48例(48/172,27.9%);肠穿孔组65例中,治愈48例(48/65,73.8%),死亡17例(17/65,26.2%)。两组术中所见坏死肠管长度以及术后肠外营养时间、肠内营养时间、呼吸机使用时间、NICU入住时间及术后并发症比较,差异均有统计学意义(P<0.05)。Bell分期为ⅢA与ⅢB期的患儿病死率比较,差异有统计学意义(χ^(2)=4.731,P=0.030)。结论NEC肠未穿孔的患儿可能存在更多肠管坏死,术后并发症多,康复时间长。建议对于NEC肠未穿孔患儿,可根据患儿临床实际情况探讨更合适的手术指征。     

中药方剂“肠粘连方”治疗新生儿肠道疾病的效果评价

目的评价中药方剂"肠粘连方"在新生儿肠道疾病的外科及保守治疗中的作用,并讨论此方剂治疗新生儿肠道疾病的作用机制。方法回顾性分析2012年1月1日至2017年1月1日由华中科技大学同济医学院附属同济医院收治的41例新生儿肠道疾病患儿。其中30例行腹部手术后出现肠梗阻(手术组),14例为中药组,除予以禁食、胃肠减压等常规治疗外,加服中药方剂"肠粘连方"治疗,余16例予以常规治疗,为对照组; 11例为坏死性小肠结肠炎(未手术组),入院时均出现呕吐、腹胀,喂养后反复便血、炎症指标上升,但无手术指征,其中5例加服中药治疗,为中药组,6例予常规治疗,为对照组。分析患儿口服中药后,肠梗阻症状及消化道功能恢复情况。结果手术组:中药组、对照组肠梗阻后至全量喂养时间分别为(10. 6±0. 7) d和(12. 8±0. 6) d,中药组明显短于对照组(t=2. 23,P=0. 03);中药组及对照组二次手术率分别为7. 1%(1/14)和25%(4/16),病死率分别为7. 1%(1/14)和18. 8%(3/16),差异均无统计学意见(P 0. 05)。未手术组:中药组患儿经反复禁食、抗炎治疗无效,停止给予或降级给予抗生素后,炎症指标上升,治疗期间均未出现肠穿孔、大量便血或腹膜炎体征,加服中药方剂后症状均逐渐好转,大便隐血均转为阴性,炎症指标恢复正常,服用中药后全量喂养时间为(17. 4±2. 5) d;对照组中2例经常规治疗好转出院,全量喂养时间为(29. 5±0. 5) d,2例出现肠穿孔予手术治疗,其余2例死亡。结论中药方剂"肠粘连方"不仅对新生儿术后粘连性肠梗阻具有显著疗效,而且对新生儿早期NEC也是安全有效的。     

新生儿坏死性小肠结肠炎与自发性肠穿孔临床对比分析

目的分析新生儿坏死性小肠结肠炎(NEC)与自发性肠穿孔(SIP)的临床特征。方法回顾分析1996年5月至2016年8月收治的NEC发生肠道穿孔以及同期收治的SIP患儿临床资料,比较两组患儿围生期相关指标,主要合并症或并发症,治疗结局等。结果共纳入101例肠穿孔患儿,NEC组70例、SIP组31例。两组患儿的性别比、生产方式、胎龄、出生体质量、窒息、宫内窘迫、早产和低体质量比例,以及发病日龄、住院天数方面的差异均无统计学意义(P0.05)。NEC组患儿病死率高于SIP组,合并或并发低蛋白血症以及败血症的比例均高于SIP组,差异均有统计学意义(P0.05)。NEC组最常见穿孔部位为大肠,其次为小肠;SIP组最常见穿孔部位为小肠,其次为大肠;NEC组与SIP组患儿穿孔部位及穿孔数量比较,差异均无统计学意义(P0.05)。结论与SIP患儿相比,NEC患儿更容易罹患败血症及低蛋白血症,病死率也更高。     

换血疗法在MODS新生猪的实验研究

目的探讨应用换血疗法治疗新生猪多器官功能障碍综合征(MODS)的效果及临床意义。方法对8只MODS新生猪进行了换血治疗,观察换血前后外周血血常规、血生化、动脉血气及血清细胞因子等指标的变化。对照组8只MODS新生猪不做换血治疗,观察两组的病死率。结果换血后新生猪红细胞水平明显升高[(4·14±0·70vs4·45±0·49)×1012/L,P<0·05];白细胞水平明显下降[(19·81±3·14vs12·02±2·84)×109/L,P<0·01];血小板水平变化无显著性意义(P>0·05)。动脉血氧分压升高(45·75±13·07vs50·38±10·16)mmHg(P<0·05),二氧化碳分压降低(40·91±6·80vs35·71±7·52)mmHg(P<0·05)。换血后血丙氨酸氨基转移酶、天冬氨酸氨基转移酶、肌酐、尿素氮及乳酸水平均下降(P<0·05);磷酸肌酸激酶同工酶、C-反应蛋白下降(P<0·01);肿瘤坏死因子-α、白细胞介素-1β、白细胞介素-6、白细胞介素-8水平均下降(P<0·05)。治疗组死亡1只(12·5%),对照组死亡6只(75%),两组死亡率差异有显著性(P<0·05)。结论换血治疗能在一定程度上改善MODS新生猪受损器官的功能,改变高炎症反应状态,阻断MODS的序贯发生,提示在治疗新生猪MODS中可能有临床价值。     

新生儿坏死性小肠结肠炎并发肠穿孔20例临床分析

本文报告1985年5月～1991年4月期间收治的20例新生儿坏死性小肠结肠炎(NEC)并发肠穿孔,3例接受外科手术治疗皆死亡,7例自动出院,另10例拒绝外科手术经内科积极保守治疗5例死亡,5例治愈,且无后遗症。作者认为NEC合并肠穿孔的患婴,若家长拒绝手术,经保守治疗,有存活的可能性,本文存活率50％。近年文献报告用内毒素测定法(Limulus Test)阳性者死亡率高,而阴性者预后好,对判断新生儿消化道穿孔的预后有一定价值。     

坏死性小肠结肠炎新生儿血浆D-乳酸水平变化的意义

目的 探讨坏死性小肠结肠炎(NEC)新生儿血浆D-乳酸水平变化的意义.方法 选择诊断为NEC 50例新生儿为NEC组(其中NECⅡ期30例,NECⅢ期20例).选择同期非NEC新生儿50例为对照组.NEC组于NEC确诊24 h内,对照组于相应日龄取外周静脉血2 mL,采用酶联免疫吸附法检测血浆D-乳酸水平,采用受试者工作特性(ROC)曲线确定血浆D-乳酸阳性标准.根据NEC发生时血浆D-乳酸水平分为D-乳酸升高组和D-乳酸正常组,根据NEC患儿病情转归分为病死组和存活组,比较不同组间血浆D-乳酸水平、新生儿危重病例评分(NCIS)分值、并脓毒症的发生率、病死率的差异.结果 NECⅢ期组、NECⅡ期组和对照组血浆D-乳酸水平分别为(36.2±10.3) mg·L-1、(28.6±12.5) mg·L-1和(3.8±2.6)mg·L-1,3组比较差异有统计学意义(F=7.63,P＜0.05).ROC曲线分析显示,血浆D-乳酸阳性标准为≥7 mg·L-1,预测NEC的敏感性为80.0％,特异性为84.6％,假阴性率为15.4％,假阳性率为20.0％.D-乳酸升高组并脓毒症发生率、病死率较高,NCIS评分较低,与D-乳酸正常组比较差异均有统计学意义(Pa＜0.05);病死组血浆D-乳酸水平、并脓毒症发生率较高,NCIS评分较低,与存活组比较差异均有统计学意义(Pa＜0.05).结论 血浆D-乳酸水平能较敏感地反映NEC新生儿的病情,可作为预测NEC程度和预后的指标之一.     

脓毒症患儿中性粒细胞、淋巴细胞CD11b表达的研究

目的探讨脓毒症患儿中性粒细胞、淋巴细胞CD11b的表达意义。方法用流式细胞术检测脓毒症(观察组)患儿27例中性粒细胞、淋巴细胞CD11b表达,并与对照组20例和正常组20例比较。结果在急性期、恢复期,观察组中性粒细胞CD11b[(98·26±1·55)%,(97·74±1·41)%],与对照组[(86·3±6·33)%,(81·48±3·35)%]和正常组[(69·59±9·98)%]比较,P<0·05。急性期,观察组淋巴细胞CD11b[(15·17±10·2)%],低于对照组[(19·2±7·6)%],P<0·05;严重脓毒症淋巴细胞CD11b[(6·54±2·52)%]表达下调,低于脓毒症组[(19·45±8·68)%],P<0·05。结论中性粒细胞、淋巴细胞CD11b表达在脓毒症的演变过程中起到一定的作用,可作为脓毒症的判断依据,预测疾病的发展。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.