原发性心肌病分类与诊断中的几个问题

近年来原发性心肌病发病率在国内外均有显著增加。原发性心肌病的病因、分类、诊断和治疗中仍有很多问题尚未解决,现就其分类与诊断中的几个问题,谈谈个人看法。     

小儿心肌病诊断之我见

小儿心肌病诊断之我见首都儿科研究所（１０００２０）李家宜近年来，随着人们认识的提高和检查方法的改进，小儿心肌病已较过去多见。１９８０年世界卫生组织和国际心脏病学会发表的原发性心肌病的定义和分类国际上已沿用了１０余年。但这种分类法是在原因不明的情况下，...     

小儿扩张型心肌病临床特点,器械检查及治疗

原发性心肌病是病变限于心肌、病因未明的心肌疾病。本病分3型：扩张型心肌病、肥厚型心肌病和限制型心肌病，其中以扩张型心肌病最多见。小儿扩张型心肌病（dilatedcardiomyopathy，DCM）近年来显著增多，这一方面由于对心肌病认识的提高、诊断技术的发展，另一方面由于病毒感     

为提高小儿肝胆外科诊治水平而努力

小儿肝胆外科是小儿腹部外科的重要组成部分。根据我院统计小儿肝胆外科疾病占腹部外科住院总人数的８．７％，其中很多属先天性疾病，如先天性胆道闭锁、先天性胆总管囊肿等。这些疾病东方民族的发病率大大高于西方国家，我国这些病的发病数居世界之首。有些肝胆外科疾病如肝肿瘤、胆石症等病种虽与成人相同，但发生在小儿期其病理类型、临床表现、诊断和治疗等均有其特殊性，故提高小儿肝胆外科疾病的诊治水平至关重要。近年来由于新的诊疗仪器、设备的不断出现，促进了肝胆疾病诊治的发展，如Ｂ超、ＣＴ、－ＲＩ、放射性核素显像技术等已…     

小儿心肌病与病毒性心肌炎的诊断

小儿心肌病与病毒性心肌炎的诊断南京军区福州总医院（３５０００１）陈新民１小儿心肌病的诊断世界卫生组织将原发性心肌病分为三型，其中扩张型约占７０％８０％，肥厚型约占１０％２０％，限制型在１０％以下。现将近年来临床诊断工作中的某些进展作一简述。１１常规...     

儿童高血压的病因和诊断程序

儿童高血压并非少见 ,由于高血压症状差别悬殊 ,除血压升高外 ,其他特异体征少 ,婴幼儿上臂短 ,有时患儿不配合 ,正确测量血压困难 ,因此 ,高血压常不能及时发现。高血压是一种症状 ,引起高血压的病因很多 ,小儿有些病因较难明确 ,因此 ,了解小儿高血压的病因和诊断程序是正确掌握高血压的基础。1　小儿高血压的病因儿童原发性高血压的患病率并不低 ,约为 0 5 %～2 % [1,2 ] ;继发性高血压发病率为 0 1%～ 0 3% ,因起病急、程度重 ,所以就诊者多。对继发性高血压不同病因的发病率报道差异较大 ,2 0 0 0年Goonasekera[1] 报告儿童继发性…     

小儿扩张型心肌病的诊断与治疗

小儿扩张型心肌病的诊断与治疗重庆医科大学儿童医院（６３００１４）钱永如小儿原发性心肌病的病例数近年有上升趋势。据报道，瑞典Ｍａｌｍｏ市年发生率为（５１０）／１０万人口。国内上海和广州５０年代与７０年代比较，心肌病患儿占心脏病住院总数分别由００５％及...     

小儿原发性扩张型心肌病临床探讨(附17例报告)

小儿原发性扩张型心肌病临床探讨（附１７例报告）中国医科大学第二临床学院儿科（１１０００３）富建华，朴英爱，田英中国医科大学第二临床学院心脏外科张贺原发性扩张型心肌病为小儿危重病种之一。大多数病例预后欠佳。本文总结了１９８４年１２月～１９９４年１１月我...     

尿道下裂手术的远期效果

尿道下裂是小儿生殖器官畸形中发病率仅次于隐睾，为第二位的先天畸形，占出生活产男婴的0．8‰～8．2／‰，近年来发病率有上升趋势。由于尿道下裂的发病机制尚未完全明确，且临床表现从轻型到重型有很大不同，故有200余种术式可供选择。随着手术技术的提高及围手术期治疗、护理的进步，短期手术效果已有很大改善。但这些术式很多仅根据短期的排尿功能和早期的手术并     

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应邀专家(以发言先后为序)马沛然教授山东省立医院陈新民教授南京军医福州总医院儿科杜军保教授北京大学第一医院黄国英教授复旦大学附属儿科医院陈树宝教授上海儿童医学中心汪翼教授山东省立医院金梅教授北京安贞医院小儿心脏科钱永如教授重庆医科大学儿童医院田杰教授重庆医科大学儿童医院于宪一教授中国医科大学第二临床学院座谈内容1心肌病命名与分类有哪些新变化?2心肌病的病因及发病机制研究有哪些新进展?3我国小儿心肌病的发病率及临床流行病学调查情况如何?4心肌病的影像学诊断有哪些新进展?如何评价超声心动图对心肌病的诊断价值?5…     

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.