幼年特发性关节炎37例

目的探讨幼年特发性关节炎(JIA)的临床特点及治疗方法。方法按照国际风湿病学联盟(ILAR)新的分类标准对JIA患儿进行分型,总结37例JIA患儿的临床表现、实验室和影像学检查结果及药物治疗及其转归。结果全身型22例(59.46%),少关节型7例(18.92%),多关节型5例(13.51%),其他关节炎型3例(8.10%)。全身型以非类固醇性抗炎药(NSAIDs) 缓解病情抗风湿药物(DMARDs) 激素治疗为主,其中8例加用细胞毒药物(CTX);少关节型、多关节型及其他关节炎型以NSAIDs DMARDs 小剂量激素治疗,基本能够控制病情,无1例发生关节功能障碍。结论JIA以全身型最多见,其次为少关节型;JIA治疗应提倡早期联合治疗,以尽快控制炎症,改善病情,防止残疾发生。     

沙利度胺治疗幼年特发性关节炎的效果分析

目的：评价沙利度胺治疗幼年特发性关节炎（juvenile idiopathic arthritis,JIA）的临床疗效和安全性。方法：12例传统治疗方案治疗病情无缓解的JIA患儿,应用沙利度胺治疗（每日2 mg/kg）,记录治疗前后患儿的症状、体征、实验室指标及沙利度胺治疗的不良反应。结果：沙利度胺治疗6个月后,患儿泼尼松用量由每日1.92±0.16 mg/kg 降至0.49±0.42 mg/kg (P<0.01),并有4例能停用泼尼松治疗。12例患儿外周血白细胞计数、ESR、CRP、血清铁蛋白（SF）较治疗前均明显下降（P<0.01）; 8例患儿血红蛋白明显升高(P<0.01)。多关节型JIA患儿平均受累关节数由5个以上降至0～2个（P<0.01）,附着点炎症型患儿双“4”字征转阴,Schober′s征转阴。治疗过程中未出现严重不良反应。结论：沙利度胺对JIA有显著的临床疗效,可以作为传统治疗方案无效后的治疗措施。     

鹿瓜多肽注射液辅助治疗幼年特发性关节炎近期疗效观察

目的 观察鹿瓜多肽注射液治疗幼年特发性关节炎的疗效和安全性.方法 将住院的104例幼年特发性关节炎患儿随机分为治疗组和对照组各52例.两组基础治疗相同,治疗组加用鹿瓜多肽注射液0.2 mg/(kg·d)静脉滴注,每日1次,疗程10 d.观察两组治疗10 d后活动性指标变化,并判定疗效.结果 两组治疗10 d后总有效率分别为96.15%、78.85%(P<0.01).两组总有效率、控制疼痛关节数、肿胀关节数、ESR、CRP、CD71/CD4+比较,差异均有统计学意义(P均<0.01).该制剂未见明显的不良反应.结论 鹿瓜多肽注射液治疗幼年特发性关节炎疗效显著,安全性好.     

幼年特发性关节炎229例的诊断和分型

目的了解我国北方地区幼年特发性关节炎（JIA）各型发病比率,对JIA的诊断和分类标准使用提供参考意见。方法对2000年1月-2001年12月本院住院的慢性关节炎患儿,用2004年修订的JIA分类标准进行回顾性分析。结果229例中以JIA分类标准分析诊断成立者196例。JIA 196例中全身型55例（28%）;少关节型21例（10.7%）;多关节型11例（5.6%）,其中类风湿因子（RF）阳性3例,RF阴性8例;与附着点炎性反应相关关节炎（ERA）106例（54%）;未分类3例（1.5%）;未见到银屑病性关节炎,无少关节扩展型。结论我国北方地区JIA全身型、ERA发病比率明显高于国外,而少关节型比率较国外明显降低,多关节型略少于国外。JIA诊断分类标准优于幼年类风湿关节炎。     

不同剂量丙种球蛋白联合糖皮质激素治疗小儿中重度急性吉兰-巴雷综合征的疗效比较

目的 观察并比较静脉注射小剂量丙种球蛋白与大剂量丙种球蛋白联合糖皮质激素冲击治疗小儿中重度急性吉兰-巴雷综合征（GBS）的临床疗效与安全性。方法 将100例中重度急性GBS患儿随机分为小剂量组（48例）和大剂量组（52例）。小剂量组和大剂量组分别采用0.2 g/（kg·d）和0.4 g/（kg·d）丙种球蛋白联合甲基强的松龙治疗。比较治疗后两组症状改善时间、外周血炎性因子水平的变化、有创通气比例、治疗有效性及不良反应。结果 治疗5 d后,小剂量组和大剂量组血清TNF-α、IL-6、CRP水平均较治疗前明显下降,两组TNF-α、IL-6、CRP下降幅度差异均无统计学意义。两组患儿呼吸机麻痹恢复时间、肌力提升1级时间、感觉障碍恢复时间及住院时间比较差异均无统计学意义。小剂量组和大剂量组总体有效率（90% vs 92%）比较差异无统计学意义。两组患儿发热、头痛、恶心、心慌发生率比较差异均无统计学意义。结论 小剂量与大剂量丙种球蛋白联合甲基强的松龙冲击治疗小儿中重度急性GBS临床疗效与安全性相当。     

幼年特发性关节炎患儿生长发育状况及生长迟缓影响因素的病例对照研究

目的:了解幼年特发性关节炎（JIA）患儿生长发育状况和生长迟缓的影响因素。方法:病例对照研究,以JIA治疗随访中身高Z值(HAZ)＜-2 SD为生长迟缓组,HAZ≥-2 SD为生长正常组,通过问卷调查考察JIA患儿治疗中生长发育的影响因素,通过自制调查问卷采集基本情况、疾病史、饮食情况、生活习惯和家庭情况5个方面21个问题,风湿科医生在门诊对JIA患儿行诊断和治疗随访的同时,儿童保健科医生随诊中以面对面询问方式行问卷调查,并行体格测量。对调查问卷的问题行起病年龄、症状体征、缓解病情药物、确诊间隔、病程、父母亲文化程度和父母亲工作的整合,通过单因素和多因素Logistic回归分析JIA患儿生长迟缓的危险因素。结果:2018年9月至2019年4月符合纳入排除标准的221例JIA患儿进入本文分析,其中男103例（46.6%）,平均年龄（7.8±3.7）岁,全身型58例、RF（-）多关节型16例、RF（+）多关节型36例、少关节型108例、点附着型2例、银屑病型1例。糖皮质激素（GCs）治疗88例,生物制剂治疗72例,其他治疗61例。33例（14.9%）合并生长迟缓,少关节型、全身型、多关节型的HAZ分别为-0.34 SD、-1.53 SD和-0.80 SD。单因素分析结果显示,影响JIA患儿线性生长的因素包括疾病亚型、病程、疾病活动度、GCs治疗、挑食和户外活动时间少（P＜0.05）;多因素Logistic回归分析结果显示,GCs治疗（OR=7.227,95%CI：1.877~27.817）、病程≥3年（OR=4.278,95%CI：1.322~13.843）和户外活动＜1 h（OR=4.078,95%CI：1.252~13.288）是JIA患儿生长迟缓的独立影响因素（P均＜0.05）。结论:JIA并发生长迟缓的发生率高,病程长、GCs治疗和户外活动时间少是影响患儿线性生长的危险因素。     

重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白治疗幼年特发性关节炎的随机对照研究

目的 运用随机对照试验研究重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白( rhTNFR:Fc)治疗幼年特发性关节炎( JIA)患儿的疗效.方法 运用随机对照原则,将124例JIA患儿分为对照组(62例)及治疗组(62例),两组基础治疗为应用一种以上抗风湿慢作用药物,或非甾体类药物,或肾上腺皮质激素;两组临床分型[其中少关节型17例(27.42％),多关节型15例(24.19％),全身型30例(48.38％)]及基础治疗差异无统计学意义(P＞0.05).治疗组JIA患儿皮下注射rhTNFR:Fc每周0.8 mg/kg,疗程6个月.两组于用药后2周、1个月、3个月、6个月记录评价指标:ACR Pedi 30、50、70和药物不良反应.结果 治疗组不同分型的JIA患儿,用药后2周、1个月达ACR Pedi 30,3个月、6个月后达ACR Pedi 50,6月后达ACR Pedi 70的缓解率不同(P＜0.05);全身型患儿的临床缓解率较少关节型和多关节型的低(P＜0.05),用药后3个月44％ ACR Pedi 50缓解,用药后6个月41.7％ ACR Pedi 50缓解,29.2％达ACR Pedi70缓解;少关节型和多关节型患儿在不同时间点的ACR Pedi 30、50、70的缓解率差别不大,80％以上患儿在用药后6个月达ACR Pedi 50缓解,50％以上患儿达ACR Pedi 70缓解,与对照组比较差异有统计学意义(P＜0.05).治疗组全身型患儿中,3例合并巨噬细胞活化综合征常规治疗无效的患儿使用rhTNFR:Fc后效果明显,2例患儿治疗1周后出现全身酸痛等不适,3例患儿在治疗期间多次出现上呼吸道感染、腹泻等感染症状,其中1例感染水痘,停药和对症治疗后好转.治疗组全身型患儿不良反应发生率为16.7％,少关节型和多关节型患儿未见不良反应发生.结论 rhTNFR:Fc对少关节型和多关节型JIA患儿具有良好的治疗效果,中短期(6个月)内的不良反应少见;对全身型JIA有一定的临床缓解治疗作用,应用时须严加防范感染等不良反应的发生.对常规治疗无效的全身型JIA患儿及合并巨噬细胞活化综合征的全身型JIA患儿可以考虑使用rhTNFR:Fc.     

来氟米特联合柳氮磺吡啶治疗幼年特发性关节炎的疗效

目的探讨来氟米特与柳氮磺吡啶(SASP)联合用药治疗幼年特发性关节炎(JIA)的疗效。方法JIA 15例,至少3个月内使用过糖皮质激素和(或)SASP,仍反复发作,关节畸形加重。予来氟米特10~20 mg,1次/d;SASP 1.0 g,1次/d,连服8个月。随访8~12个月,观察远期疗效。结果15例JIA联合用药2、4、8、12周显效率分别为69.1%、78.3%、83.6%、92.8%。联合用药6个月后疗效为:显效86.6%,有效13.4%。不良反应:胃肠道反应2例次,皮疹1例次,转氨酶升高3例次,白细胞减少2例次。结论来氟米特 SASP联合用药对JIA安全有效,能迅速改善临床表现,改善关节功能。     

幼年特发性关节炎50例临床分析及远期随访

目的探讨幼年特发性关节炎(JIA)临床特点、治疗及预后。方法总结50例JIA患儿临床表现、实验室和影像学检查结果,并随访16例患儿治疗结果,以国际风湿病学联盟(ILAR)儿科专家组的分类标准讨论稿进行分析。结果全身型8例中1例对NSAID有效;6例两药联用有效;1例加MTX,复发。JIA多关节型6例,2例对NSAID有效;3例加MTX有效;1例加CTX,复发。少关节型1例对NSAID有效。结论JIA中以全身型多见,其次为多关节型,该病临床表现多样,需根据不同临床分型予不同治疗方案,联合用药并个体化。糖皮质激素不良反应相对大,患儿耐受较差。MTX对各型均有较好疗效,主要用于多关节型以及全身型。JIA预后较好,好转率>75%。     

不同亚型幼年特发性关节炎患儿血脂水平变化研究

目的分析不同亚型幼年特发性关节炎（JIA）患儿活动期及缓解期血脂水平的变化，初步探讨JIA患儿远期发生动脉粥样硬化的风险。方法将128例初诊为JIA活动期患儿根据亚型分为少关节型48例，多关节型38例，全身型22例，附着点型20例，其中38例多关节型JIA患儿又根据类风湿因子（RF）是否阳性分为RF阳性多关节型15例，RF阴性多关节型23例；同期另随机选取45例行健康体检儿童作为健康对照组。检测各组血清总胆固醇（TC）、甘油三酯（TG）、低密度脂蛋白胆固醇（LDL-C）、高密度脂蛋白胆固醇（HDL-C）等水平进行比较分析。对87例治疗后处于缓解期的患儿进行血脂指标的复查，并与活动期各血脂指标水平进行比较分析。结果在JIA活动期，全身型、RF阳性多关节型组与健康对照组比较，HDL-C水平明显下降，TG水平明显升高（P < 0.05），而TC、LDL-C水平差异无统计学意义（P > 0.05）；其他亚型各血脂指标水平与健康对照组比较差异均无统计学意义（P > 0.05）。RF阳性多关节型JIA患儿缓解期与活动期比较，血浆HDL-C明显升高（P < 0.05）；其他亚型JIA患儿缓解期与活动期比较，血浆各血脂指标水平差异均无统计学意义（P > 0.05）。结论全身型、RF阳性多关节型JIA活动期存在血脂代谢紊乱，RF阳性多关节型JIA缓解期血脂紊乱有所改善，其远期发生动脉粥样硬化的风险是否增大尚需进一步观察。     

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     

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.     

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.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

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.     

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.