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Dermatitis herpetiformis. 疱疹样皮炎。
Pub Date : 2020-03-25 DOI: 10.1201/9780429165122-2
L. Fry
Dermatitis herpetiformis (DH) is a relatively rare skin disorder with an estimated incidence of 1:10,000 in the UK. It is characterized by urticarial plaques and blisters on the elbows, buttocks, and knees, although other sites may also be involved. The eruption tends to be persistent: only 10-15% of patients have spontaneous remission over a 25-year study period. The disease is characterized by the presence of IgA deposits in the upper dermis of uninvolved skin and the diagnosis should not be made in the absence of these deposits. Two-thirds of patients have a small intestinal enteropathy with villous atrophy as seen in coeliac disease (CD). However, the remaining third also show evidence of a gluten sensitivity in the intestine, as judged by increased lymphocytic infiltration of the epithelium. Villous atrophy also ensues after gluten challenge in those patients with previous normal villous architecture. The initial treatment of the rash is with one of the following three drugs, dapsone, sulphapyridine or sulphamethoxypyridazine. However, the rash also clears with gluten withdrawal. It must be stressed, however, that the average time to achieve significant reduction in drug requirements is 6 months and it can be over 2 years before drugs are no longer required. On re-introduction of gluten the eruption recurs. Patients with DH have a high incidence of auto-immune disorders, thyroid disease, pernicious anaemia, and insulin-dependent diabetes, and should be screened for those diseases on a yearly basis. As with coeliac disease there is also an increased incidence of lymphoma and a gluten-free diet appears to protect patients from this complication. The mechanism by which gluten causes the skin lesions has still to be elucidated, but current investigations implicate lymphocytes and cytokines in the pathogenesis. The original hypothesis of an antigen-antibody reaction in the skin with complement activation causing the skin lesions, may not be correct.
疱疹样皮炎(DH)是一种相对罕见的皮肤病,在英国的发病率估计为1:10 000。它的特征是在肘部、臀部和膝盖上出现荨麻疹斑块和水泡,尽管其他部位也可能受累。皮疹往往是持续性的:在25年的研究期间,只有10-15%的患者自发缓解。该疾病的特征是在未受累皮肤的上真皮层存在IgA沉积,在没有这些沉积的情况下不应作出诊断。三分之二的患者有小肠病伴绒毛萎缩,如乳糜泻(CD)所见。然而,其余三分之一的人也表现出肠道麸质敏感的证据,这可以通过上皮淋巴细胞浸润增加来判断。在那些以前绒毛结构正常的患者中,麸质挑战后绒毛萎缩也随之发生。皮疹的最初治疗是使用以下三种药物之一,氨苯砜,磺胺吡啶或磺胺甲氧基吡啶。然而,皮疹也会随着麸质的消失而消失。然而,必须强调的是,实现药物需求量显著减少的平均时间为6个月,而不再需要药物的时间可能超过2年。一旦再次引入谷蛋白,就会复发。DH患者自身免疫性疾病、甲状腺疾病、恶性贫血和胰岛素依赖型糖尿病的发病率很高,应每年筛查这些疾病。与乳糜泻一样,淋巴瘤的发病率也会增加无麸质饮食似乎可以保护患者免受这种并发症的影响。谷蛋白引起皮肤病变的机制仍有待阐明,但目前的研究表明淋巴细胞和细胞因子在发病机制中起作用。最初的假设是皮肤中的抗原抗体反应与补体激活导致皮肤病变,这可能是不正确的。
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引用次数: 0
Chapter 40 – Wilson Disease 第四十章威尔逊病
Pub Date : 2015-01-01 DOI: 10.1016/B978-0-12-410529-4.00040-1
Golder N Wilson
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引用次数: 5
Hepatitis E. 肝炎E。
Pub Date : 2014-09-01 DOI: 10.1055/s-0034-1377579
E. Mast, M. Purdy, K. Krawczyński
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引用次数: 1
5 Nutrition in inflammatory bowel disease 5炎症性肠病的营养
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90005-7
Simon H. Murch PhD, FRCP, FRCPCH (Senior Lecturer Honorary Consultant in Paediatric Gastroenterology), John A. Walker-Smith MD, FRCP, FRACP, FRCPCH (Professor of Paediatric Gastroenterology)

Nutrition is clearly disturbed by active intestinal inflammation. Appetite is reduced, yet energy substrates are diverted into the inflammatory process, and thus weight loss is characteristic. The nutritional disturbance represents part of a profound defect of somatic function. Linear growth and pubertal development in children are notably retarded, body composition is altered, and there may be significant psychosocial disturbance. Macrophage products such as tumour necrosis factor-α and interleukins-1 and 6 may be the central molecules that link the inflammatory process to this derangement of homeostasis. Intriguingly, there is also increasing evidence that an aggressive nutritional programme may in itself be sufficient to reduce the mucosal inflammatory response. Recent evidence suggests that enteral nutrition alone may reduce many pro-inflammatory cytokines to normal and allow mucosal healing. In addition, specific nutritional components, such as n-3 polyunsaturated fatty acids, may have an anti-inflammatory effect as they may alter the pattern of leukotrienes generated during the immune response. The recent discovery of the specific molecular mediators of appetite and body composition, such as leptin and myostatin, may allow increased therapeutic specificity and further improvement in the nutritional treatment of the inflammatory bowel diseases.

营养显然受到活跃的肠道炎症的干扰。食欲减少,但能量底物被转移到炎症过程中,因此体重减轻是典型的。营养失调是躯体功能严重缺陷的一部分。儿童的线性生长和青春期发育明显迟缓,身体成分发生改变,并可能存在明显的社会心理障碍。巨噬细胞产物如肿瘤坏死因子-α和白细胞介素-1和6可能是将炎症过程与这种体内平衡紊乱联系起来的中心分子。有趣的是,也有越来越多的证据表明,积极的营养计划本身可能足以减少粘膜炎症反应。最近的证据表明,单独肠内营养可以使许多促炎细胞因子恢复正常,并使粘膜愈合。此外,特定的营养成分,如n-3多不饱和脂肪酸,可能具有抗炎作用,因为它们可能改变免疫反应过程中产生的白三烯的模式。最近发现的食欲和身体组成的特异性分子介质,如瘦素和肌肉生长抑制素,可能会增加治疗特异性,并进一步改善炎症性肠病的营养治疗。
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引用次数: 14
2 Assessment of nutritional status in clinical practice 2临床营养状况评估
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90002-1
Olivier Goulet MD, PhD (Professor of Pediatrics)

As most diseases can have nutritional consequences, the assessment of nutritional status may help to detect an underlying disease, to identify nutritional disorders related to a given disease, and to quantify the impact of nutritional therapy. The aims and methods used for nutritional assessment depend on the circumstances in which nutritional assessment is performed.

Whatever the context or aim, nutritional status is assessed through a simple, mainly clinical approach, based on the past history, dietary intake, auxological analysis, anthropometric measurements, body compartment and biological parameters. Accurate techniques for measuring body compartments are available in children such as dual-energy X-ray absorptiometry to assess fat body mass or bioelectrical impedance analysis for body water and lean body mass. Measuring energy expenditure allows for a more accurate monitoring of the patient's energy needs and decreases the risks associated with underfeeding or overfeeding.

In clinical practice, the analysis should be longitudinal and take into account situations carrying a risk of malnutrition. Preventive use of nutritional assessment allows nutritional support to be introduced in a timely fashion, thereby avoiding morbidity/mortality and limiting the long-term impact of malnutrition on growth and development.

由于大多数疾病都可能对营养产生影响,对营养状况的评估可能有助于发现潜在疾病,确定与特定疾病相关的营养失调,并量化营养治疗的影响。营养评估的目的和方法取决于进行营养评估的环境。无论背景或目的如何,营养状况都是通过一种简单的、主要是临床的方法来评估的,该方法基于过去的历史、饮食摄入、生理分析、人体测量、身体间隔和生物学参数。测量儿童身体隔室的精确技术是可用的,例如用于评估脂肪体重的双能x射线吸收仪或用于评估身体水分和瘦体重的生物电阻抗分析。测量能量消耗可以更准确地监测患者的能量需求,并降低与喂养不足或过度喂养相关的风险。在临床实践中,分析应该是纵向的,并考虑到有营养不良风险的情况。预防性使用营养评估可以及时提供营养支持,从而避免发病率/死亡率,限制营养不良对生长发育的长期影响。
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引用次数: 25
3 Nutritional support of infants and children: Supply and metabolism of lipids 3婴幼儿营养支持:脂质供给与代谢
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90003-3
Berthold Koletzko MD (Professor of Paediatrics), Hans Demmelmair PhD, Piotr Socha PhD

The quantity and quality of dietary lipids and their metabolism are of major importance for the growth, body composition, development and long-term health of children, both in health and disease. Lipids are the major source of energy in early childhood and supply essential lipid-soluble vitamins and polyunsaturated fatty acids that are required in relatively high amounts during early growth. Lipids affect the composition of membrane structures, and modulate membrane functions as well as the functional development of the central nervous system. Some long-chain polyunsaturated fatty acids serve as precursors for bioactive lipid mediators, including prostaglandins, thromboxanes and leukotrienes, which are powerful regulators of numerous cell functions such as thrombocyte aggregation, inflammatory reactions and immune functions. Here we review some aspects of the biochemistry and physiology of lipids and their implications for lipoprotein metabolism, energy balance and the lipid supply during early childhood through the placenta, human milk, enteral diets and parenteral lipid emulsions.

膳食脂质的数量和质量及其代谢对儿童的生长、身体组成、发育和长期健康具有重要意义,无论是在健康方面还是在疾病方面。脂质是儿童早期能量的主要来源,并提供早期生长所需的相对大量的必需脂溶性维生素和多不饱和脂肪酸。脂质影响膜结构的组成,调节膜的功能以及中枢神经系统的功能发育。一些长链多不饱和脂肪酸作为生物活性脂质介质的前体,包括前列腺素、血栓烷和白三烯,它们是许多细胞功能的强大调节剂,如血栓细胞聚集、炎症反应和免疫功能。本文综述了脂质生物化学和生理学的一些方面,以及它们对早期儿童通过胎盘、人乳、肠内饮食和肠外脂质乳的脂蛋白代谢、能量平衡和脂质供应的影响。
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引用次数: 35
1 Protein and energy requirements in healthy and ill paediatric patients 1健康和患病儿童的蛋白质和能量需求
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90001-X
Jean-Louis Bresson MD (Professor of Nutrition)

A reappraisal of available data, together with new studies, suggests that normal infants' energy and protein requirements might be substantially lower than previously estimated. For example, the safe level of protein intake would amount to only 10 g per day during the first 2 years of life and to about 12 g per day during the third. This has direct consequences for the management of malnourished children, particularly for defining an optimal protein:energy ratio. A reduced food intake has long been accepted as the main cause of malnutrition. However, evidence has accumulated suggesting that metabolic dysregulation may also play a part. This is particularly true for proteins. Net protein deposition in the growing child results from protein synthesis rates being higher than protein breakdown. However, this setting can be disrupted by a significant increase in protein breakdown in response to cytokines. This mechanism, which is found in acute as well as in chronic inflammatory processes, may lead to severe protein malnutrition and is not always amenable to nutritional support.

对现有数据的重新评估以及新的研究表明,正常婴儿的能量和蛋白质需求可能大大低于先前的估计。例如,在生命的头两年里,蛋白质摄入量的安全水平是每天10克,在第三年里,每天12克。这对营养不良儿童的管理有直接影响,特别是对确定最佳蛋白质:能量比例有直接影响。长期以来,人们一直认为食物摄入量减少是营养不良的主要原因。然而,越来越多的证据表明,代谢失调也可能起到一定作用。对于蛋白质来说尤其如此。生长中的儿童的净蛋白质沉积是由于蛋白质合成速率高于蛋白质分解速率。然而,这种设置可能会被响应细胞因子的蛋白质分解显著增加所破坏。这种机制在急性和慢性炎症过程中都有发现,可能导致严重的蛋白质营养不良,并且并不总是适合营养支持。
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引用次数: 8
4 Nutritional management in diarrhoeal disease 4腹泻病的营养管理
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90004-5
Stefano Guandalini MD (Professor of Pediatrics), Ayse Pinar Dincer MD

Adequate nutritional intervention in diarrhoeal disease in children is crucial in obtaining optimal control of a disorder that may become life-threatening. During recent years, important advances have been made in our understanding of the pathophysiology of diarrhoeal states, in the formulation of oral rehydration solutions and in the role of micro- and macronutrients in diarrhoeal disorders. This chapter outlines some of the relevant concepts in the pathophysiology of diarrhoeal disease and provides a rationale for nutritional intervention. Guidelines for nutritional management in the settings of acute-onset diarrhoea, post-enteritis protracted diarrhoea and chronic non-specific diarrhoea are provided, mostly based on controlled clinical trials and meta-analyses of evidence-based medicine.

对儿童腹泻病进行适当的营养干预对于获得对可能危及生命的疾病的最佳控制至关重要。近年来,我们对腹泻状态的病理生理学、口服补液溶液的配方以及微量和大量营养素在腹泻疾病中的作用的理解取得了重要进展。本章概述了腹泻病病理生理学中的一些相关概念,并提供了营养干预的基本原理。提供了急性腹泻、肠炎后持续性腹泻和慢性非特异性腹泻的营养管理指南,主要基于对照临床试验和循证医学的荟萃分析。
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引用次数: 10
12 Home enteral and parenteral nutrition in children 儿童的家庭肠内和肠外营养
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90012-4
Virginie Colomb MD, PhD (Consultant in Paediatric Gastroenterology and Nutrition), Olivier Goulet MD, PhD (Professor of Paediatrics), Claude Ricour MD, PhD (Professor of Nutrition)

The prevalence of home enteral and parenteral nutrition programmes is rising rapidly all over the world, in children as in adults. Home artificial nutrition, especially parenteral nutrition, is an expensive technology but is life-saving for many patients. The only possible alternative to home treatment is keeping patients in hospital, and cost-benefit studies have demonstrated that home nutrition is about 70% more cost-effective than hospital-based therapy. Although home nutrition is usually considered by children and families to lead to an improvement in their quality of life, the complications of these techniques, including psychological consequences, have to be carefully assessed and prevented.

在世界各地,无论是儿童还是成人,家庭肠内和肠外营养规划的普及程度正在迅速上升。家庭人工营养,特别是肠外营养,是一项昂贵的技术,但对许多患者来说是挽救生命的。家庭治疗的唯一可能替代方案是让患者住院,成本效益研究表明,家庭营养比医院治疗的成本效益高70%左右。虽然儿童和家庭通常认为家庭营养可以改善他们的生活质量,但必须仔细评估和预防这些技术的并发症,包括心理后果。
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引用次数: 39
9 Cystic fibrosis: Nutritional consequences and management 囊性纤维化:营养后果和管理
Pub Date : 1998-12-01 DOI: 10.1016/S0950-3528(98)90009-4
Dominique Turck MD (Professor of Paediatrics Director), Laurent Michaud MD (Staff Member)

Malnutrition is an adverse prognostic factor in cystic fibrosis, influencing the course of pulmonary disease and correlating inversely with survival. A positive energy balance between energy intake and the combination of total energy expenditure, energy losses and growth-related energy cost is essential to maintain normal nutritional status. Before starting nutritional supplementation, it is important to rule out pathological conditions that may have a deleterious effect on nutritional status: persistent exocrine pancreatic insufficiency, chronic bacterial pulmonary colonization, impaired glucose tolerance, specific nutritional deficits and associated disorders leading to a decrease of energy intake. Several methods are available, ranging from boosted oral nutrition to behavioural intervention, oral supplementation, enteral nutrition and, rarely, parenteral nutrition. The use of elemental nutrients for either oral supplementation or enteral nutrition seems of no nutritional benefit and is more expensive than conventional polymeric nutrients. Provided that the goals of the nutritional supplementation are fulfilled, simpler is often better.

营养不良是囊性纤维化的不良预后因素,影响肺部疾病的病程,并与生存率呈负相关。能量摄入与总能量消耗、能量损失和生长相关能量消耗之间的正能量平衡对于维持正常的营养状况至关重要。在开始营养补充之前,重要的是要排除可能对营养状况产生有害影响的病理状况:持续性外分泌胰腺功能不全,慢性细菌肺定植,糖耐量受损,特异性营养缺乏和导致能量摄入减少的相关疾病。有几种可用的方法,从增强口服营养到行为干预、口服补充、肠内营养以及很少使用的肠外营养。使用元素营养素进行口服补充或肠内营养似乎没有营养效益,而且比传统的聚合营养素更昂贵。如果营养补充品的目标得以实现,那么通常越简单越好。
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引用次数: 0
期刊
Bailliere's clinical gastroenterology
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