小儿全肠外营养108例分析

目的探索更合理的小儿全肠外营养（ｔｏｔａｌｐａｒｅｎｔｅｒａｌｎｕｔｒｉｔｉｏｎ，ＴＰＮ）支持的方案。方法收集１９８７年１月至１９９６年１２月中住院患儿应用ＴＰＮ≥５天的临床资料共１０８例；年龄３个月～１２岁，平均ＴＰＮ持续１７天（５～１３２天）。经周围静脉ＴＰＮ１０３例，中心静脉ＴＰＮ５例。结果１０８例中出院１０２例，死亡６例。出院患儿中体重增加４８例，不变３３例，下降２１例。发生ＴＰＮ有关的并发症共１２例（占１１．８％），其中肝损害及胆汁郁积５例，高血糖２例，高脂血症２例，低磷、低钙血症２例，导管感染１例。结论（１）一般热卡在１６７～３３５ｋＪ·ｋｇ－１·ｄ－１，可获得正氮平衡及体重增加。推荐氨基酸剂量婴幼儿为２．０～２．５ｇ·ｋｇ－１·ｄ－１，儿童为１．５～２．０ｇ·ｋｇ－１·ｄ－１；脂肪乳剂婴幼儿推荐量为１～３ｇ·ｋｇ－１·ｄ－１，儿童为１～２ｇ·ｋｇ－１·ｄ－１。（２）婴幼儿应选择小儿氨基酸；肝功能不全或肿瘤化疗患儿应选含中链甘油三脂的脂肪乳剂。（３）脂肪乳剂输注应＞１６小时；葡萄糖８～１４ｍｇ·ｋｇ－１·ｍｉｎ－１，提倡“全合一”输注。（４）应常规补充钙磷。     

译文摘要

译文摘要儿童结肠血管发育异常作者报道１９７０～１９９３年９例结肠血管发育异常的患儿，发病年龄为１～１ｌ岁（平均２．３岁），其中０例症状出现于１岁前。诊断年龄为１岁５个月～１３岁（平均５．３岁），平均延误诊断２．９年。９例患儿都有便血，其中３例黑便，血...     

全肺静脉异位回流的外科治疗：附21例报告

报告手术治疗２１例小儿全肺静脉异位回流，年龄６个月－１４岁（平均６．７岁）；体４．９－３０ｋｇ（平均１５．８ｋｇ）。３周岁１０ｋｇ以下的婴幼儿５例。心上型１０例，心内型８例，混合型３例。手术死亡３例，手术死亡率为１４．３％。本文介绍了根据不同病变类型的各种手术方法，并就吻合口大小、术前肺动脉高压、术后心律失常、术后吻合口狭窄等问题进行了讨论。     

221例新生儿休克的临床分析

２２１例新生儿休克的临床分析福建省泉州市儿童医院（３６２０００）高璇璇柯丹红１临床资料１．１发病和死亡情况我院１９９０年４月～１９９５年１０月共收治新生儿３０９５例，其中新生儿休克２２１例（占７．１％）。男１８４例，女３７例；日龄≤３天１０１例（占４...     

住院学龄前儿童236例死亡分析

本文对我院近５ ａ年来２３６例住院学龄前儿童死亡病因进行统计分析,现总结如下。 资料与方法 资料来源于我院医务处统一管理的住院病人死亡统计工作报表。参考 ＩＣＤ－９分类方法,根据死亡病因第一诊断确定进行统计分析。 结果 学龄前儿童住院人数与死亡人数见表１。死亡病例的年龄分段新生儿期 １２８例（５４．２％）,婴儿期 ５１例（２１．６％）,幼儿期 ３６例（１５．３ ％）,学龄前期死亡 ２１例（８．９ ％）。死因顺位统计早产儿低出生体重８３例（３５．２ ％）,新生儿窒息３８例（１６．１％）,先天异常疾病２６例（１…     

92例学龄前儿童心脏瓣膜成形术

目的 探讨学龄前儿童心脏瓣膜成形术的手术方法、效果。方法 回顾92例在中度低温体外循环下行心脏瓣膜成形术的各种心脏瓣膜病患儿的瓣膜病理改变、外科瓣膜处理方法。结果 术后早期死亡2例(占2．2％)。远期随访89例(占98．9％)，残留中度以上瓣膜返流6例(占6．5％)，心胸比、左房径均明显下降，无远期死亡。结论 学龄前儿童心脏瓣膜病可以采取瓣膜成形术，并能取得良好的术后效果。根据不同瓣膜病变，采用不同的手术方法，是学龄前儿童心脏瓣膜成形术取得满意效果的关键。     

我国儿童钙营养状况

从我国１９９２年全国第３次营养抽样调查结果来看，我国居民平均每日钙摄入量为４０５－４ｍｇ，城市居民为４５７－９ｍｇ，农村居民为３７８－２ｍｇ，分别占我国营养学会推荐的每日膳食中钙供给量（ＲＤＡ）的４９－２％，５３－３％及４７－１％［１］。这是包括儿童在内的我国整体人群钙营养状况，这种低钙营养状况在儿童期尤为明显，我国成人钙摄入量达到推荐ＲＤＡ者约占１０％，儿童仅占３％～４％，不足成人的一半。我国居民每日摄入多少钙才能达到ＲＤＡ呢？初生至６个月４００ｍｇ，约５０～８０ｍｇ／ｋｇ；７个月至２岁６００…     

5kg以下婴儿室间隔缺损的手术治疗

１９９４年３月～１９９８年６月，笔者对２３例５ｋｇ以下婴儿室间隔缺损（ＶＳＤ）伴肺动脉高压者施行手术治疗，占同期６２例１周岁以下婴儿ＶＳＤ修补术的３７．１％。临床资料本组２３例，男１３例，女１０例。年龄为３～１１个月（平均为６．７８±０．５３个月），体重为３．２～５．０ｋｇ（平均为４．３８±０．２１ｋｇ）。术前反复呼吸道感染２３例，反复心衰１７例。１１例因难治性肺炎或心衰，经内科控制下急诊手术。全组均经Ｘ线胸片、心电图、超声心动图、心导管和心血管造影检查确诊。全组均伴有中度以上肺动脉高压，肺动脉…     

小儿瓣膜置换术

小儿瓣膜置换术肖诗亮胡志伟蔡俊坚叶世铎张凯伦杜心灵报告１９８１年１１月～１９９６年１１月我院１１例儿童瓣膜置换及随访结果。资料与方法：男５例，女６例，年龄４～１４岁（平均１１．３±４．３岁）。风湿性二尖瓣狭窄并关闭不全５例，先天性二尖瓣关闭不全３例，...     

婴幼儿肺炎病毒病原与流行病学观察：附52例报告

本文应用ＥＬＩＳＡ法对５２例肺炎患儿进行ＲＳＶ，ＰＦＶ（１，２，３型）和ＡＤＶ（３，７型）特异型抗体检测，阳性２４例（阳性率４６．２％），以ＰＦＶ居首（占２７％），其中ＰＦＶ３和ＰＦＶ１分别占１３．５％和９．６％；ＲＳＶ次之占（占１１．５％）。三种病毒感染主要发生在３岁以内小儿，以１岁以下婴儿发病率最高（占６６．７％）。１２月－１月为流行高峰（占７０．８％）。     

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.     

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.     

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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.     

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.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.