23例格林-巴利综合征临床分析

格林 巴利综合征 (GBS)又称急性炎症性脱髓鞘性多发性神经病 ,其病理改变为周围神经和神经根的脱髓鞘及小血管周围淋巴细胞和巨噬细胞的炎性反应。现对 2 3例GBS的临床 ,肌电图及病理改变进行分析 ,报告如下。资料与方法一、临床资料　我院 1990年 6月～ 2 0 0 2年 6月诊断GBS患儿 2 3例 ,男 15例 ,女 8例 ;11个月 1例 ,1～ 3岁 7例 ,～ 6岁 9例 ,～ 14岁 6例。急性起病者 18例 ,亚急性起病 5例。发病前 1个月内有感染史 8例 ,其中 6例有肠道感染史 ,2例有上呼吸道感染史。首发症状为四肢麻木、疼痛等感觉障碍 13例 ,双下肢无力 7例 ,四…     

小儿急性偏瘫41例报告

我院1981～1991年收治小儿急性偏瘫41例,现经随访,结合原始资料,总结如下。临床资料一、一般资料本组患儿41例,男25例,女16例。年龄9个月～14岁,平均6岁3个月,其中≤1岁者3例,～3岁6例,～6岁12例,～9岁9例,～14岁11例。发病在3～5月份者4例,～8月份11     

急性脑梗塞患儿的磁共振改变

近年来,急性脑梗塞患儿有逐渐增加趋势,且临床表现多样,病因与成人有很大差异,有一定致残率。因此,早期诊断、及时治疗十分重要。本文对于1995年9月—2 0 0 3年8月间在我院儿科住院的5 8例急性脑梗塞患儿进行了头部MRI检查,现将其结果与临床表现进行对比分析,报告如下。1　材料与方法1 1　一般资料　在5 8例中,男34例,女2 4例,男女之比约1 5∶1。年龄4 5月～14岁之间,平均年龄4 5岁。病程0 5个月～2 5个月,平均1 5个月。起病形式:均为急性起病、进展较快。在5 8例患儿中,36例发病前1～3周内有急性上呼吸道、消化道、泌尿道感染史。12…     

小儿脱髓鞘疾病糖皮质激素治疗效果初步探讨

目的探讨小儿脱髓鞘疾病的临床特征和糖皮质激素的治疗效果.方法总结11例脱髓鞘患儿的临床资料和磁共振(MRI)特点,观察其临床转归和糖皮质激素的治疗效应.结果11例患儿,以急性或亚急性起病,7例起病前1～4周有发热、呼吸道或消化道感染症状,出现神经系统异常时感染征象已消失.主要表现有乏力、嗜睡、头痛、颅神经异常和肢体瘫痪.头部MRI检查全部异常,表现为白质、基底节、脑干和小脑T1WI等信号、低信号病灶和T2WI高信号病灶.甲基泼尼松龙或泼尼松治疗4～7天症状好转,4～12周后治愈6例,好转5例.其中3例有复发.结论儿童急性脱髓鞘疾病①多数与感染有关;②临床多样化,颅神经受累和肢体瘫痪是主要神经系统表现;③MRI为诊断本病的主要辅助手段之一;④糖皮质激素治疗有效,但部分患儿有复发     

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本院自2002-11—2004-11收治11例病毒性脑干脑炎患儿,现将临床资料分析如下。临床资料:11例患儿中,男性8例,女性3例,年龄为11个月至14岁。其中11个月至3岁3例,~9岁6例,~14岁2例。多数呈急性起病且进展迅速,病前有呼吸道感染7例,有消化道感染4例,发病时间多见于11月至来年2月。临床表现:因病毒性脑干脑炎波及部位及程度各异,临床表现呈多样化,但所有患儿发病前均有恶心呕吐,多数为非喷射状呕吐。其中头痛9例,视物不清2例,头晕3例,呛咳5例,精神烦躁异常者5例,肢体运动障碍7例,心慌胸闷1例,失语2例,尿便失禁1例。所有病例发病后均有不同程度的…     

小儿脑性瘫痪26例临床分析

我们自1981年至1992年诊治小儿脑性瘫痪(脑瘫)26例,现分析如下。 临床资料 男14例,女12例;5月～1岁9例,～3岁11例,～6岁5例,～12岁1例。新生儿缺氧缺血性脑病11例,产伤7例,新生儿期中枢神经系统感染5例,早产儿3例,核黄疸2例,先天畸形1例,原因不明5例。痉挛型18例,肌张力低     

大剂量丙种球蛋白治疗原发性血小板减少性紫癜26例临床分析

原发性血小板减少性紫癜 (简称 ITP)是小儿最常见的出血性疾病 ,其特点是出血呈紫癜性质、末梢血中血小板减少 ( <1 0 0×1 0 9/L )。根据病程分为急性型 (病程小于 6个月 )与慢性型两种。我们用大剂量丙种球蛋白治疗 ITP,效果显著 ,现分析报告如下 :一、临床资料1 .一般资料 :2 6例中男 1 6例 ,女 1 0例。<1岁 4例占 1 5 .4% ,～ 3岁 6例占 2 3.1 % ,～ 7岁 1 2例占 46 .1 % ,～ 1 2岁 4例占1 5 .4%。急性型 2 1例 ,慢性型 5例 ,慢性反复发作 3例。2 .发病季节及诱因 :一年四季均可发病 ,以冬春季节发病多见占 1 6例 ,秋季发病最少占 3…     

以肾病综合征起病的毛细血管内增生性肾炎6例报告

毛细血管内增生性肾小球肾炎(ECPGN)临床主要表现为急性肾炎综合征,以肾病综合征起病者较少见。现将该类病例临床与病理资料报告如下。 临床资料6例均为男性;年龄6～12岁;起病距肾活检时间15天～6周。5例分别在发病前1～2周有明显的前驱病感染史,全部病例均以浮肿、排泡沫状尿就诊,4例见肉眼血尿,2例为镜下血尿,高血压3例,急性肾功能不全1例。尿     

小儿病毒性脑干脑炎九例临床分析

病毒性脑干脑炎是指各种病毒感染所引起的脑实质及其脑干的炎症。小儿时期病毒性脑干脑炎并不多见,由于其可引起脑干重要生命中枢功能障碍,预后大多凶险。为提高对病毒性脑干脑炎的认识,现将我院收治的9例病毒性脑干脑炎患儿的临床资料作一回顾性分析。1临床资料1.1一般资料9例患儿中男5例,女4例,年龄2～14岁,平均(4.6±3.8)岁。急性起病者8例,亚急性起病1例。临床症状1周内达高峰者6例,1～2周内达高峰者3例。四季均有发病,7例发病前有上呼吸道感染史,2例发病前有腹泻病史。1.2临床表现头痛、头晕6例,复视2例,眼球运动障碍3例,口角歪斜5例,…     

自身免疫性溶血性贫血12例治疗体会

溶血是小儿贫血的原因之一。 1994年 1月～ 1998年 12月 ,我们收治了 12例自身免疫性溶血性贫血 (ＡＩＨＡ)患儿 ,其中Ｅｖａｎｓ综合征 3例。为了探讨ＡＩＨＡ的激素用量及疗程长短 ,对我们收治及复诊的 12例患儿进行回顾性临床分析 ,报告如下。一、临床资料1 对象　男 8例 ,女 4例。年龄 :2个月1例 ,6个月 1例 ,～ 1岁 4例 ,～ 3岁 6例。2 发病前感染因素　上呼吸道感染 5例 ,支气管肺炎 4例 ,腹泻 2例 ,水痘 1例。3 症状及体征　急性起病 10例 ,缓慢起病 2例 ;发热 10例 ;烦躁、寒战、呼吸困难 3例 ;黄疸 10例 ;皮肤、粘膜出血点 3例。…     

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.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.