不同性别系统性红斑狼疮患儿的临床特点

目的 探讨不同性别SLE患儿临床特点,为临床医师正确认识和诊断SLE提供参考.方法 回顾性分析1993年12月-2007年9月本院收治的88例SLE患儿临床和实验室检查资料,88例SLE患儿男女比例为1.0：4.9,高峰发病为青春发育期(10～15岁)的女童,占全部患儿的60.2%.男女平均起病年龄无差异.采用美国SLE疾病活动积分(SLEDAI)评分法判断病情,并依据不同性别分别进行对比分析,临床表现和实验室检查采用Spearman相关分析.结果 男童与女童的首发症状构成比及临床症状构成比具有统计学差异,男童以皮肤损害起病多见,而女童以发热起病多见.男女各自系统累及率比较无统计学意义,但女童贫血和肝功能异常的发生率高于男童,脾大、脑电图异常、蝶形红斑在女性患儿中有多发趋势,胃肠道症状、浆膜炎、眼损害在男性患儿中有多发趋势.2组肾脏受累类型均以肾病综合征型为主(男40.0%,女45.7%).未发现常见的实验室指标在男女童之间存在分布差异.相关分析显示,女童患儿中C3下降与多器官损害相关,双链脱氧核苷酸(dsDNA)阳性变化与肾脏损害显著相关.结论 SLE男童和女童以不同的临床表现起病,各个系统累及率可能与性别无关,常见实验室指标检出率无性别差异.C3可作为判断女童SLE病情严重程度的指标之一.     

儿童期起病的男性系统性红斑狼疮患儿30例临床特征分析

目的　探讨儿童期起病男性系统性红斑狼疮（SLE）患儿临床特点及预后。方法　回顾性分析2011年1月至2016年10月于重庆医科大学附属儿童医院住院初诊为SLE的男性患儿临床资料及预后,与同期初诊女性SLE患儿进行比较。结果　SLE患儿男30例,女129例,中位随访时间45个月。男性患儿平均年龄11.57±2.78）岁,肝脾大为最常见首发表现（70.0%）,其次为皮肤黏膜表现、发热、淋巴结肿大、血液系统受累、肾脏受累,发生率均高于50%。其中肝脾大、浆膜炎及口腔溃疡者发生率均高于女性（P＜0.05）。13例男性SLE患儿完成肾活检,Ⅳ型狼疮性肾炎发生率为76.9%,与女性患儿差异无统计学意义（P＞0.05）。男性患儿出现慢性肾脏病Ⅲ期及以上者高于女性患儿（P＜0.05）,2例死亡病例死因为肾功能衰竭。不同性别患儿存活率及并发症发生率差异无统计学意义（P＞0.05）。结论　男性SLE患儿首发表现肝脾大、浆膜炎及口腔溃疡较女性患儿多见。男性SLE患儿远期更易发生严重的肾脏损害,且为男性SLE患儿死亡的主要原因。     

小儿系统性红斑狼疮113例临床分析

我们收集了近15年在我院及外院住院治疗的系统性红斑狼疮(SLE)病例113例,从发病、临床特点、实验室检查及治疗转归几方面对小儿SLE做一全面的回顾性分析。 1.临床资料 本组患儿女性多见,男:女=1∶4.13。年龄5～14岁,以10岁以上多见(占81％)。病程最短半个月,最长者12年。患儿起病急,首发症状各异,可以表现为发热(42.5％)、皮疹(13.3％)、淋巴结肿大(6.2％)、关节痛(15.9％)等全身症状,也可在病初即出现器官受累的症状.如头痛、抽搐(3.5％),尿少、浮肿(11.5％),腹痛、腹泻(4.4％)及出血点,瘀斑(2.7％)。临床     

儿童与成人系统性红斑狼疮临床特点比较

目的探讨儿童系统性红斑狼疮(SLE)的临床特点。方法对127例儿童SLE和120例成人SLE首次住院病史进行回顾性分析。从性别、临床表现、实验室检查及肾脏病理等方面,比较儿童与成人SLE特点。结果儿童SLE男女比例1:6.1,成人SLE男女比例1∶5,两组性别构成比差异无统计学意义;儿童SLE患者发病初的平均疾病活动度指数(SLEDAI)明显高于成人,差异有统计学意义(Z=4.15,P<0.000);儿童SLE患者平均累及器官数多于成人,差异有统计学意义(Z=6.30,P<0.000)。儿童SLE肾脏病理类型依次为Ⅳ型57.1%、Ⅲ型26.3%、Ⅴ型18.4%、Ⅲ型+Ⅴ型10.5%、Ⅳ型+Ⅴ型2.6%,成人SLE依次为Ⅳ型45.5%、Ⅴ型22.7%、Ⅲ型+Ⅴ型13.6%、Ⅱ型和Ⅲ型均为9.1%。结论儿童SLE与成人SLE相似,女性多见;儿童SLE较成人SLE疾病活动性强,累及器官多,肾脏病理改变同成人以重型多见,而无症状的肺脏病变较成人多见,应引起重视。     

幼年系统性红斑狼疮患儿首发临床症状分析

目的 回顾性分析幼年系统性红斑狼疮患儿的首发临床症状.方法 采用流行病学调查的方法,抽取我院2000年至2010年135例幼年SLE患儿的病历,了解其自发病以来的临床表现及发病后的就诊情况,分析其首发症状.结果 首发症状以面部红斑最多,占60.0％(81例),其次为关节痛(炎)40.7％(55例)、发热38.5％(52例)、肾损害症状23.0％(31例).从出现症状到患儿住院的时间:发热19.9个月、肾损害症状21.2个月、关节痛(炎)35.7个月、面部红斑43.9个月.男女发病比例为1:12.5,男性以面部红斑最多见,其次为发热,均高于女性,关节痛、肾损害发生率,男性略低于女性.结论 面部红斑、关节痛(炎)、发热是幼年系统性红斑狼疮最常见的首发症状,是患儿住院的主因.女性发病率明显高于男性,且以关节痛(炎)、肾损害症状就诊多见,而男性患儿皮疹、发热发生率高.此外,患儿出现发热、浮肿等症状时就诊时间短,而以关节痛(炎)、皮疹为首发症状的患者就诊时间长.     

系统性红斑狼疮患儿T辅助细胞亚群的检测及分析

近年来 ,T辅助细胞 (TH 细胞 )亚群及其分泌的细胞因子在系统性红斑狼疮 (SLE)发病中的作用越来越得到重视。本文利用酶联免疫斑点法(ELISPOT) ,检测SLE患儿细胞因子的表达 ,以观察SLE患儿TH 亚群平衡的改变以及与受累器官之间的关系。一般资料 :4 0例SLE患儿来自2 0 0 0年 1～ 12月本院风湿病门诊和病房 ,符合 1982年ARA的SLE诊断标准 ,均除外合并感染。年龄 5～ 16岁 ,男女之比为 6∶34,其中活动期 17例 ,非活动期 2 3例。肾脏受累者 2 2例(5 5 % ) ;合并血液系统损害者 15例(37 5 % ) ,合并中枢神经系统损害者 7例 (17 5 % )…     

儿童系统性红斑狼疮100例

目的探讨儿童SLE的临床和实验室指标特点及狼疮性肾炎(LN)的肾脏病理类型。方法选取2001年1月-2011年1月本院住院确诊的SLE患儿100例,其中LN 82例,分析其临床表现、实验室和病理检查资料,应用SAS 9.1软件对SLE及各病理类型LN按临床及实验室检查特点行χ2检验。结果 100例SLE患儿,男19例,女81例;发病年龄(10.28±2.98)岁。发病高峰为9～14岁的女性患儿。新生儿1例。临床表现以发热(54%)、皮肤损害(54%)最常见,其次为水肿(34%)、关节炎(22%)、肝脾大(18%)、淋巴结大(15%)、口腔溃疡(4%)。SLE患儿的肾脏累及率为82%。其中肾炎性肾病占33%;血液系统累及率较高,其中贫血59%、低清蛋白血症53%、血小板降低20%;呼吸系统感染40%,肝损害26%,中枢神经受累16%,浆膜炎13%。免疫学检查血清ANA阳性(82%)、补体C4降低(77%)、补体C3降低(70%)、ESR加快(59%)、dsDNA阳性(52%)。48例肾活检病例均有病理异常,病理类型以Ⅳ型最多,占27%;肾脏累及和无肾脏累及组患儿在补体C3降低(P=0.040 9)、抗dsDNA阳性(P=0.023 0)、水肿(P=0.023 6)方面比较差异均有统计学意义。将48例肾活检病例分为增殖性和非增殖性肾炎2组,对Ⅲ、Ⅳ、Ⅴ型+Ⅲ型和Ⅴ型+Ⅳ型(增殖性病变)和Ⅰ、Ⅱ和Ⅴ型(非增殖性病变)进行统计学分析,发现补体C3降低(P=0.042)、抗ANA阳性(P=0.000 6)、ESR加快(P=0.021 9)、贫血(P=0.003 8)差异有统计学意义。结论儿童SLE肾脏、血液系统及呼吸系统累及率最高。补体C3降低、dsDNA阳性和首发表现为水肿的SLE患儿可能更易累及肾脏。肾脏病理表现为增殖性病变的患儿更易发生贫血,补体C3降低、抗ANA阳性、ESR加快提示增殖性病变处于活动期。     

儿童狼疮性肺损伤8 例临床分析

目的分析儿童狼疮性肺损伤的临床特点。方法回顾分析2011年1月至2015年12月收治的8例以呼吸系统受累为首发表现的系统性红斑狼疮(SLE)患儿的临床资料。结果 8例患儿的年龄6~15岁,男女各4例,均有咳嗽表现,发热7例,气促5例,咳痰5例,咯血3例,胸痛3例,呼吸困难3例,紫绀3例。狼疮性肺损伤类型分别为胸腔积液5例(62.5%)、急性狼疮性肺炎4例(50.0%)、慢性间质性肺炎2例(25.0%)、肺出血2例(25.0%);其中7例患儿胸部高分辨CT检查主要有磨玻璃样影5例、胸腔积液5例、纵膈或淋巴结肿大5例、节段性肺实变4例。7例患儿经治疗原发病后呼吸系统症状及肺部影像改变迅速好转,1例合并肺出血者死亡。结论以呼吸系统受累为首发症状的SLE患儿临床表现缺乏特异性,易与呼吸系统感染混淆,但多合并其他脏器受累;胸部影像学以磨玻璃样影、节段性肺实变、胸腔积液多见。     

85例儿童过敏性紫癜临床分析

目的分析小儿过敏性紫癜(HSP)的临床特点,提高临床诊治水平。方法对2003年1月至2005年1月在我科治疗的85例过敏性紫癜患儿的发病特点、临床表现、相关实验室检查等方面进行回顾性分析。结果(1)HSP主要发生于学龄前儿童和学龄儿童共84例,占98.82%。(2)诱因:感染62例,占72.94%,进食特殊食品8例,占9.41%。(3)出现肾脏损害者30例,发生率35.29%。(4)在64例做心电图检查者中出现心电图异常者23例,发生35.94%。(5)在35例心肌酶谱检查者中9例患儿一项或多项异常,发生率25.71%。结论(1)HSP发病诱因仍以感染为第一位。(2)多部位受累者发生肾脏受累和心电图改变。(3)单一器官的严重损害比多个器官的较轻损害对患儿预后影响更大。     

贝利尤单抗治疗以呕吐尿频为首发症状的儿童系统性红斑狼疮一例

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种以多系统受累为特征的自身免疫性疾病。多数患者以不明原因发热、皮疹或关节肿痛就诊,此外,血液系统、肾脏、神经系统也常受累,但消化道和膀胱的症状通常较轻。故以消化道症状为首发症状,伴有明显的尿频,而其他器官受累不明显的病例,容易被延误诊断及治疗。SLE标准治疗包括诱导期积极应用免疫抑制剂,即大剂量的糖皮质激素与环磷酰胺或霉酚酸酯伍用,但耐药患者的疗效差。贝利尤(belimumab)单抗是目前唯一获批用于治疗SLE生物制剂,国外已有报道该药应用于治疗常规治疗无效的狼疮性肾炎(LN)、皮肤狼疮等案例,但迄今尚无用于治疗以平滑肌受累为主要表现的SLE的报道。由于该药在国内刚刚上市,临床经验甚少。现报道1例以呕吐、尿频等平滑肌受累为主要表现的SLE患儿,其常规治疗效果不佳,使用贝利尤单抗治疗后症状明显改善,疾病活动度显著降低。     

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.     

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   

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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.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

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.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.