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??Inhalation therapy is an effective therapeutic strategy for respiratory diseases. With the continuous improvement of drug preparation and inhalation devices??inhalation therapy in clinical use has been increasingly widespread. With the advantages of the effective drug output and good distribution of drug aerosol in lungs and no specific needs for cooperation during the process of inhalation??nebulization is more suitable for use in clinical pediatric. However??it should be noted that in clinical practice there is still something that is unreasonable??or even not standardized. Combining with some of the phenomena in clinical practice??the standardization points of nebulization therapy in childhren are proposed.     

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??It is well known that a successful eradication of H.pylori dramatically reduces the rate of recurrence of gastritis and peptic ulcers in affected children. The recommended first-line eradication therapy for H.pylori infection has been triple therapy consisting of a proton pump inhibitor and two antibiotics. However??the eradication rate has declined in recent years??due to an increased bacterial resistance to antibiotics or decreased compliance with the patients. The treatment of H.pylori infection in children should be personalized.     

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??Food allergy related digestive disease refers to damage of digestive tract mucosa caused by allergy??and digestive tract symptoms are the main manifestation. Including oral allergy syndrome??anaphylaxis??food protein-induced enteropathy??food protein-induced enterocolitis syndrome??food protein-induced proctocolitis??celiac disease??eosinophilic esophagitis??eosinophilic gastroenteritis. The diagnosis was based on history??manifestation??and food chanllenge. Skin prick test and specific serum IgE test are helpful for screening IgE mediated food allergy. Endoscopy should be based on different disease conditions. The management principles of these diseases include avoiding diet??choosing proper nutrition substitute??following up??carrying out nutrition monitoring and evaluation??and rationally using medicine.     

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??Parenteral nutrition has become a mainstay in the treatment of critically ill children with intestinal or conditions that preclude enteral feeding. Accurate assessment of nutritional status and energy requirements in critically ill children will help to better implement the strategy of parenteral nutrition. The timing of the optimal parenteral nutrition has not been determined??and at present the supplementary parenteral nutrition tends to be started at a later stage. “All in one” is recommended for parenteral nutrition. Pharmacological nutrients have been recommended in critically ill adult patients with different levels??but more extensive research is still needed in critically ill children.     

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??The classification of thalassemia is mainly based on the types of the defective synthesis of the globin chains of adult hemoglobin A and clinical features??the former mainly includes α??β??γ??δ??δβ??etc.??and α or β thalassemia is the most common form of thalassemia??the latter is classified as transfusion-dependent thalassemia and non-transfuion-dependent thalassemia in terms of long-term survival depending on blood transfusion. The diagnostic techniques include screening test and genetic test??the former diagnostic method is mainly based on the morphology of red blood cells and the physical and chemical properities??including routine analysis of blood??red blood cell morphology and hemoglobin electrophoresis??etc.??and the latter is mainly based on PCR technology??including gap-PCR??real-time PCR??gene chip and DNA screening??etc. This paper reviews the classification and laboratory diagnosis techniques in thalassemia??combining comprehesive screening method with technique of genetic diagnosis??which appropriately contributes to the diagnosis of thalassemia.     

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??The standardized resident training is an important step for training high-level professionals in clinical medicine and it is an important part of medical education after graduation. The training of residents’ clinical thinking and clinical judgement should be emphasized in all resident training programs. Some resident programs in China still focus on the teaching of important knowledge that are originated from the medical colleges. Therefore??it is urgent for faculty to adopt new teaching methods aiming at the training of clinical thinking and clinical judgement. The paper introduces a mode for teaching clinical thinking and clinical judgement in the resident training programs. The goal is to improve the level of resident physicians in clinical diagnosis and treatment in China.     

儿童炎症性肌病规范化治疗及预后

儿童炎症性肌病包括感染和免疫相关两类,前者以病毒性肌炎为主,后者以幼年皮肌炎常见。治疗方法根据病因、程度而不同,包括抗感染和免疫治疗,免疫治疗首选激素和免疫抑制剂。是否及时诊治、病损程度、是否合并心肺等器官受累是影响预后的因素。     

“Give me strength” – inflammatory muscle disease in children

Juvenile dermatomyositis is the most common idiopathic inflammatory myopathy of childhood. Management focuses on early aggressive suppression of inflammation to induce sustained remission and prevent complications such as muscle contractures or calcinosis. Advances in diagnostic modalities and treatment have led to improved mortality and morbidity, but long-term risks remain significant. Early disease recognition with appropriate referral and management by a specialist multidisciplinary team is crucial. This review focuses on juvenile dermatomyositis including differential diagnosis from other conditions causing muscle weakness in children.     

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??Idiopathic inflammatory sympathies are a group of rare autoimmune disorders??characterized by inflammation in striated muscle. Dermatomyositis is the most common subtype in children.Though a number of diagnostic criteria have been proposed??the diagnosis of dermatomyositis is based on a combination of clinical??laboratory and electromyography findings.Typical features are subacute-onset?? proximal??symmetric muscle weakness??pathognomonic skin rashes.Muscle biopsy demonstrated inflammatory infiltrates confined to perimysium??often around blood vessels??and perifascicular muscle fiber atrophy. The uses of magnetic resonance imaging of skeletal muscles??detection of myositis-specific autoantibodies and nailfold capillaroscopy have become useful additions to our diagnostic test.     

儿童多发性肌炎和皮肌炎临床分析

目的 探讨儿童多发性肌炎(PM)和皮肌炎(DM)的临床特点与治疗.方法 分析1999年4月-2004年6月诊断为PM和DM 6例患儿的临床特点、实验室检查、肌电图和肌肉活检结果,以及糖皮质激素联合免疫抑制剂治疗疗效和预后.结果 PM和DM患儿均有对称件近端肌无力,皮肤改变为双上眼腧水肿性淡紫红色斑和Gottron征,部分患儿累及关节、心脏、肺脏和消化系统;6例患儿均有肌酶升高,肌电图呈肌源性损害,肌肉活检示肌炎改变;泼尼松联合免疫抑制剂治疗后,肌力恢复至Ⅱ～Ⅲ级约为1～3个月.肌力从Ⅲ～Ⅳ级恢复至正常为7～13个月,四肢肌力早于颈部肌力恢复;血清肌酶1～6个月恢复正常,ESR、CRP、肝功能异常治疗后均恢复正常;6例患儿中5例已停止治疗,最长停药时间为4年,肌力正常,无发热、皮疹等情况,1例治疗中出现对称性腕关节、近端指间关节肿痛,活动受限.结论 PM和DM以肌无力和皮肤损害为突出表现,其他系统亦可受累,各脏器功能评估对诊断和判断疾病严重性非常有益;肌电图和肌肉病理检查是确诊PM和DM及与其他原因引起的肌病鉴别的重要依据;糖皮质激素联合免疫抑制剂治疗PM和DM安全有效,儿童PM和DM预后较好.     

幼年皮肌炎的药物治疗进展

幼年皮肌炎(Juvenile Dermatomyositis,JDM)是一种儿童期可累及全身的自身免疫性炎性肌病,主要累及皮肤和近端肌肉,还可以累及重要器官(如：肺、关节及胃肠道等),常伴有特异性抗体的存在。目前关于JDM的治疗非常困难,一线药物是糖皮质激素和甲氨蝶呤,对于治疗效果不佳的患者,有些二线或三线药物起到一定的支持作用,在难治性患者中,静脉注射免疫球蛋白或环磷酰胺可能是有效的,同时生物制剂和小分子靶向药物也逐渐被应用于临床。而除糖皮质激素外的药物选择往往还需要综合临床症状、血清特异性抗体、病理结果及初步治疗的反应,在治疗过程中不断调整。这篇综述的目的是概述目前用于治疗JDM的药物、治疗效果观察和选择药物的策略,为临床治疗提供思路。     

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??Children inflammatory myopathies are divided into autoimmunemyositises and infective myositises. Autoimmune myositises usually are called idiopathic inflammatory myopathies. Clinical diagnosis of pediatric inflammatory myopathy mainly depends on the clinical manifestation, serum creatine kinase and EMG examination, pathological examination can be further confirmed the diagnosis,the serum antibody examination will be helpful for the treatment and prognosis. The traditional JDM diagnostic criteria is Bohan and Peter standard in 1975.
Keywords?? idiopathic inflammatory myopathies??children??dermatomyositis??diagnosis and differential diagnosis     

幼年类风湿性关节炎96例的预后和转归

目的　探讨幼年类风湿性关节炎 (JRA)的远期预后和转归。方法　对 1988～ 1992年我院诊断为JRA患儿通过门诊、信函进行随访 ,随访 84～ 13 2个月 ,并对随访者病情、治疗经过、目前情况及生活状况进行分析。结果　完成随访 96例 ,1例死于霍奇金病 ;余 95例中全身型 44例 ,2例于发病第 7年死于感染 ,2 3例发展为严重关节炎 ,17例完全缓解 (CR) ,2例部分缓解 (PR) ;少关节炎型 3 8例 ,2 7例在发病 10年后仍有活动性关节炎 ,9例诊断为强直性脊柱炎 ;多关节炎型 13例 ,3例类风湿因子 (RF)阳性均出现关节畸形。结论　JRA预后不良 ,尤其全身型 ,影响预后主要因素有年龄、发热、炎症表现、系统受累及治疗情况。少关节炎Ⅱ型约 3 0 %以上发展为强直性脊柱炎。多关节炎型RF阳性者关节畸形发生率高 ,预后不佳     

经CD34+纯化的自体外周造血干细胞移植治疗小儿皮肌炎1例报告

目的探讨经CD34^＋纯化的自体外周造血干细胞移植治疗小儿皮肌炎的疗效。方法1例3岁皮肌炎患儿接受经CD34^＋纯化的自体外周造血干细胞移植。采用环磷酰胺（CTX）＋粒细胞集落刺激因子（G-CSF）方案动员外周血干细胞后,通过CliniMACS细胞分选仪分选CD34＋细胞,预处理选用CTX、兔抗人T淋巴免疫球蛋白（ATG）和马法兰（MeL）。0天回输CD34^＋细胞数9.45×10^6/kg。观察症状体征变化、造血重建及免疫恢复情况。结果动员获得单核细胞数为1.04×10^7/kg,经纯化获得CD34^＋细胞占94%,CD34^＋细胞回收率达67%,去除3个对数级CD3^＋。＋9d粒系植入,＋14d巨核系植入。＋19d皮损恢复正常,四肢肌力由移植前Ⅱ级或Ⅲ级转为Ⅳ级。＋180d免疫恢复,＋210d肌力恢复Ⅴ级。结论对常规治疗无效的小儿皮肌炎,可选择CD34^＋纯化自体外周造血干细胞移植。