幼年皮肌炎诊治进展

幼年皮肌炎是一种以皮肤与肌肉组织病变为主的系统性自身免疫性疾病.该文总结了幼年皮肌炎的特异性临床表现、实验室诊断方法如MRI的应用价值,并对皮肌炎诊断标准提出了建议,介绍了近期应用于该病的免疫抑制剂与生物制剂的治疗进展.     

幼年皮肌炎诊治进展

幼年皮肌炎是一种以皮肤与肌肉组织病变为主的系统性自身免疫性疾病.该文总结了幼年皮肌炎的特异性临床表现、实验室诊断方法如MRI的应用价值,并对皮肌炎诊断标准提出了建议,介绍了近期应用于该病的免疫抑制剂与生物制剂的治疗进展.     

幼年皮肌炎慢作用药物治疗进展

幼年皮肌炎是儿童少见的风湿性疾病之一，常用的治疗方案是激素联合慢作用药物。由于发病率较低，缺少大样本随机对照试验研究，选择哪种慢作用药物、了解药物之间的转换以及确定疗程是比较困难的。该文对治疗幼年皮肌炎的常用慢作用药物及其作用机制、剂量、不良反应和适应证进行介绍，有助于临床医师对药物选择进行权衡。     

风湿病及结缔组织病

073867儿童不典型系统性红斑狼疮的诊断与治疗/曹兰芳∥实用儿科临床杂志.-2007,22(9).-641~643073868儿童系统性红斑狼疮国内外诊断标准比较/陈瑜…∥实用儿科临床杂志.-2007,22(9).-715~717073869多发性肌炎/皮肌炎研究进展/宋晓颖…∥实用儿科临床杂志.-2007,22(9).-704~706073870小儿皮肌炎的临床特征/楚海峰…∥实用儿科临床杂志.-2007,22(9).-689~690回顾性分析15例小儿皮肌炎(JDM)的临床特点,对其血清肌酶、肌肉活检的病理改变及患儿对糖皮质激素的疗效和预后资料进行分析。结果:JDM患儿均有对称性近端肌无力;最常见皮肤改变是眼…     

儿童皮肌炎的诊断与治疗

皮肌炎是横纹肌非化脓性炎性肌病,临床表现多样,主要累及皮肤、肌肉、胃肠,多伴有特征性的皮肤损害。儿童皮肌炎有其自身特点,较成人型更易引起软组织钙化,雷诺现象及并恶性肿瘤者少见。目前药物治疗仍以糖皮质激素为首选,合并免疫抑制剂治疗可缩短激素使用时间,减少激素用量。难治型、反复型皮肌炎可选用IVIG、血浆置换等治疗。     

血液灌流在儿童重型皮肌炎治疗中的应用

目的观察血液灌流治疗儿童重型皮肌炎的效果。方法回顾性分析2例重型皮肌炎接受血液灌流治疗患儿的临床资料。结果 2例患儿血液灌流过程顺利,灌流后肌痛症状明显好转,肌酶明显下降;其中1例患儿在血液灌流后拒绝接受治疗,病情反复;另1例患儿灌流后继续给予免疫抑制剂等积极治疗,最终好转出院。结论血液灌流可以作为重型皮肌炎的辅助治疗方法,可用于一般治疗无效者。     

儿童寡肌病性皮肌炎合并肺间质病变2 例报告

目的探讨托珠单抗对儿童寡肌病性皮肌炎合并肺间质病变的疗效。方法回顾分析2例寡肌病性皮肌炎合并肺间质病变患儿的临床特点及治疗与预后,并复习相关文献。结果男女各1例,女性患儿10岁11个月、男性患儿8岁5个月,起病时均有气促,但无肌肉损害的临床表现;均有典型皮疹,但肌力及肌张力正常。实验室检查以血清铁蛋白、乳酸脱氢酶、谷氨酸氨基转移酶及天冬氨酸氨基转移酶升高为主,肌酸肌酶除首次入院时稍高,复查始终在正常范围。高分辨CT示肺间质病变。临床诊断为寡肌病性皮肌炎合并肺间质病变。女性患儿经大剂量激素、环磷酰胺、环孢素、吡菲尼酮及丙种球蛋白等治疗无效死亡。男性患儿在常规激素治疗的基础上,加用托珠单抗(240 mg/次,2次),病情稳定,随访复查各指标均在正常范围内。结论儿童寡肌病性皮肌炎临床表现以及实验室检查结果不典型,死亡发生率高。联合托珠单抗治疗有效。     

小儿皮肌炎16例临床分析

本文报道１６例小儿皮肌炎的临床表明、实验室检查、治疗和疗效观察。１６例中特征性皮肤损害和肌无力在１－２月内相继出现者有１５例，仅１例在肌无力发生１年后才出现皮损。所有病例追踪随访２－１９年。最后对小儿皮肌炎的病因，临床、诊断和预后结合文献予以简要复习和讨论。     

血浆置换术治疗幼儿皮肌炎二例

幼儿皮肌炎是一种严重而比较少见的结缔组织病。以皮肤损害及横纹肌非化脓性炎症为特征。临床表现为严重皮肤溃疡、进行性对称性肌无力、肌痛；随着肌无力的进展可产生吞咽困难、呼吸困难而危及生命。自从采用血浆置换术以来，严重幼儿皮肌炎取得满意疗效。现将我们治疗的...     

小儿皮肌炎的诊治进展

小儿皮肌炎属风湿性疾病、结缔组织病、自身免疫性疾病,确切的病因尚不完全清楚。其发病率较成人为低,约１０％～２０％。在小儿结缔组织病中,据北京儿童医院住院病例数的统计,小儿皮肌炎与全身性红斑狼疮的病例数相近,所以,小儿皮肌炎并非罕见。对于皮肌炎与多发性...     

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     

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.     

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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.     

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.     

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.