原发性免疫球蛋白A肾病55例临床病理分析

目的探讨儿童原发性免疫球蛋白A肾病(IgAN)的临床、病理特征及预后。方法对1996~2005年经肾活检确诊为原发性IgAN的患儿55例进行详尽的临床病理分析。本组男35例,女20例,发病年龄2~16岁,平均9岁,占同期肾活检的10.5%。结果临床表现为肾病综合征占30.9%、孤立性血尿占25.5%、血尿蛋白尿占23.6%、急性肾炎综合征占18.2%、慢性肾炎综合征占1.8%;病理分级以Ⅲ级多见(61.8%),其次为Ⅳ级(21.8%)和Ⅱ级(12.7%),Ⅰ级仅占3.6%;免疫病理分型IgA IgM IgG( C3)型占45.5%,IgA IgM( C3)型30.9%,IgA单独沉积21.8%,满堂亮者1.8%。双向有序χ2检验表明临床表现的严重程度与病理分级间存在线性关联,伴蛋白尿者病理改变较重;且临床表现与免疫病理分型间也具有一定相关性,孤立性肉眼血尿患儿中,以IgA型较多见,而表现为肾病综合征患儿中,IgA IgM IgG( C3)型最多见。对其中24例平均随访39个月,除1例孤立性血尿尿检无改变,1例血尿蛋白尿蛋白尿好转血尿无改善外,其他患儿均明显好转,仅有轻微血尿或微量蛋白尿。结论儿童原发性IgAN的临床表现与病理特征存在一定程度关联。临床表现为肾病综合征及肾炎综合征者病理改变较重,以Ⅲ、Ⅳ级为主,而孤立性血尿者病变较前者轻。     

33所医院儿童原发性IgA肾病临床和病理表现调查分析

目的调查分析我国儿童原发性IgA肾病（IgAN）临床与病理特征,了解IgAN患儿临床、病理特征,以及治疗和转归情况。方法由中华医学会儿科学分会肾脏病学组统一制订调查表格,对经肾穿刺活检确诊的原发性IgAN患儿进行回顾性登记,由各地初步归纳分析,然后汇集并进行统计分析。结果1995年1月至2004年12月全国33所医院≤14岁住院IgAN患儿共1349例,占同期住院泌尿系疾病患儿的1．37％,肾穿刺活检患儿的11．18％。1203例完整资料分析显示,男：女为2．07：1,发病年龄和确诊年龄中位数均为9．0岁,确诊前病程中位数为4个月。55．94％患儿起病有诱因。临床表现以复发性肉眼血尿型最常见（41．15％）,其次为肾病综合征型（23．77％）和血尿蛋白尿型（20．78％）。病理分级以Ⅲ级（41．40％）和Ⅱ级（28．51％）最多见。免疫荧光检查：单独IgA沉积占34．50％,IgA沉积强度以血尿蛋白尿型和急进性肾炎型最强（＋＋＋）。治疗与随访：无统一治疗方案,部分患儿采用皮质激素和免疫抑制剂治疗。69．24％患儿临床好转,10．39％无变化,2例恶化。仅23．35％进行随访（平均24．4个月）。结论我国儿童原发性IgAN发病年龄在6岁以上。临床表现以血尿和肾病综合征型为多见,病理表现以Ⅱ-Ⅲ级为主。目前国内尚元统一治疗方案,随访率低,提示治疗亟待规范化,应加强随访。     

279例儿童紫癜性肾炎临床与病理分析

目的了解儿童紫癜性肾炎的临床和病理特点。方法回顾性分析279例儿童紫癜性肾炎患儿的临床和病理资料。结果279例儿童紫癜性肾炎的临床分型以血尿和蛋白尿型最多（107例,38．4％）,其次是肾病综合征型（69例,24．7％）,孤立性蛋白尿型（40例,14．3％）,孤立性血尿型（29例,10．4％）,急性肾炎型（21例,9．3％）,急进性肾炎型（8例,2．9％）,慢性肾炎型（5例,1．8％）。根据国际儿童肾脏病研究中心标准,279例儿童紫癜性肾炎的病理分级以Ⅱ级和Ⅲ级最多,分别为133例（47．7％）和109例（39．1％）,血尿和蛋白尿型的病理分级以Ⅱ级（61例,57．0％）和Ⅲ级（35例,32．7％）多见,肾病综合征型的病理分级以Ⅲ级多见（41例,59．4％）。免疫病理分型以IgA＋IgM沉积型多见（108例,38．7％）,IgA＋IgM＋IgG沉积型次之（86例,30．8％）。肾病综合征型的病理改变相对较重（X^2=35．989,P〈0．05）,免疫病理分型与病理分级无相关性（P〉0．05）。结论儿童紫癜性肾炎临床以血尿和蛋白尿型及肾病综合征型为主,病理分级以Ⅱ级和Ⅲ级常见,但临床症状与病理损伤的程度不完全一致,肾病综合征型的病理改变相对较重。应根据临床类型和病理分级制定治疗计划,改善预后。     

儿童IgA肾病病理特点与临床关系探讨:附32例分析

为探讨儿童IgA肾病病理特点与临床关系，对肾活检确诊为IgA肾病的32例病例进行临床分型、病理分级及免疫分型。结果发现临床表现为单纯性肉眼血尿15例（46.9%)，肾病综合征10例（31．3％），肾炎综合征5例（15．6％），单纯性蛋白尿2例（6．2％）。病理损害按Meadow分级共V级，以第Ⅲ级为主（50．0％），其次为第Ⅱ及第Ⅳ级，各占18．8％和15．6％；单纯性肉眼血尿以第Ⅲ级为主（53．3％）；肾病综合征以Ⅲ及Ⅳ级为主，且肾炎性肾病的病理分级相对比单纯性肾病重。提示蛋白尿的程度与肾脏组织学改变密切相关；以肾病综合征为表现的IgA肾病病理改变均较重。     

儿童IgA肾病临床与病理的关系

目的探讨儿童IgA肾病(IgAN)的临床特点及其与病理的关系。方法对肾活检确诊为IgAN 21例进行临床分型、病理分级及免疫分型,并分析之间的相互关系。结果本组IgAN发病率男童多于女童(2.5∶1.0),临床表现为单纯性肉眼血尿14例(66.7%),血尿伴蛋白尿4例(19.1%),肾病综合征1例(4.7%),肾炎综合征2例(9.5%),病理改变以Ⅲ级为主,免疫球蛋白沉积以复合型为主。结论随着对无症状血尿、蛋白尿者肾活检的增多,小儿IgAN的诊断有逐年增加趋势。IgAN临床表现多样,几乎包括肾小球疾病的所有类型,且临床与病理有一定关系。单纯血尿者病理改变相对较轻,预后较好;蛋白尿者病理改变较重,应早期诊断,早期治疗。     

儿童IgA肾病临床表现与病理分析

目的探讨IgA肾病临床表现与病理的关系。 方法对1996—2005上海交通大学附属儿童医院住院的77例患儿,进行肾组织活检确诊为原发性IgA肾病。参照Lee修改的Meadow病理分级标准将IgA肾病分级。 结果血尿蛋白尿33例,孤立性血尿22例,肾病综合征12例,急性肾炎9例,孤立性蛋白尿1例。病理分级：Ⅰ级11例,Ⅱ级14例,Ⅲ级47例,Ⅳ级5例。 结论呈孤立性血尿的IgA肾病病理改变相对较轻,随着蛋白尿的增多,肾损害逐渐加重。     

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目的探讨IgA肾病临床表现与病理的关系。方法对1996—2005上海交通大学附属儿童医院住院的77例患儿,进行肾组织活检确诊为原发性IgA肾病。参照Lee修改的Meadow病理分级标准将IgA肾病分级。结果血尿蛋白尿33例,孤立性血尿22例,肾病综合征12例,急性肾炎9例,孤立性蛋白尿1例。病理分级:Ⅰ级11例,Ⅱ级14例,Ⅲ级47例,Ⅳ级5例。结论呈孤立性血尿的IgA肾病病理改变相对较轻,随着蛋白尿的增多,肾损害逐渐加重。     

IgA肾病97例的临床和病理分析

目的探讨儿童IgA肾病的临床与病理特点的关系。方法对2005年1月-2010年6月经本院肾脏病理室确诊为IgA肾病的97例患儿的临床表现,病理特点及相关实验室检查进行回顾性分析。结果 97例患儿中男女比例为2.61,发病年龄(10.6±2.9)岁。临床表现以肾病综合征最多见(40.2%),其次为孤立性血尿(30.9%),病理类型以轻度系膜增生性IgA肾病最多见(29.9%),组织分级以Ⅲ级改变为主(52.6%),免疫荧光分型以IgA+IgM型多见(45.4%)。将临床表现由轻到重分为孤立性血尿、血尿蛋白尿、肾病及肾炎综合征3组,病理改变分为Ⅰ～Ⅱ级、Ⅲ级、Ⅳ～Ⅴ级3个级别,行双向有序χ2检验,差异无统计学意义(χ2=4.081,P=0.395);将免疫病理分为单纯IgA沉积型与复合沉积型2组,其临床表现不同,差异有统计学意义(χ2=8.421,P=0.015)。结论儿童IgA肾病以学龄期多见,男性多于女性。临床表现与免疫病理分型相关,单纯IgA沉积者临床表现较轻。临床分型与组织分级间未发现显著联系。     

单中心原发性儿童IgA肾病临床及病理特征分析（附303例报告）

目的探讨儿童原发性IgA肾病的临床表现、病理特点及其相关关系。方法回顾性分析浙江大学医学院附属儿童医院肾内科2014年1月至2019年12月经肾穿刺活检确诊的303例原发性IgA肾病患儿的临床表现、临床分型、病理特点及免疫分型。结果共纳入303例患儿,男196例(60.7%),女107例(35.3%),平均(10.2±2.4)岁,55.8%的患儿病前有诱因。临床分型中以孤立性血尿型占首位(199例),其次分别为血尿和蛋白尿型(31例)。临床分型中,病理改变总体以Ⅱ级为主;除慢性肾炎型的免疫复合物沉积以IgA+IgM+IgG为主和急进性肾炎型以IgA+IgG、IgA+IgM+IgG为主外,其余各型的免疫复合物沉积均以IgA+IgG为主。病理分级中,Ⅰ级以IgA型最多,Ⅱ、Ⅲ级以IgA+IgG型最多,而Ⅳ、Ⅴ级以IgA+IgM+IgG型最多。牛津分类病理类型以M0E0S0T0C0最多见,1/3的患儿出现两种及以上病变。孤立性血尿型中M0E0S0T0C0为最常见的病理类型,血尿蛋白尿型、急性肾炎型、孤立蛋白尿型和慢性肾炎型中,非M0E0S0T0C0占大多数,肾病综合征型和急进性肾炎型中,所有病例均为非M0E0S0T0C0。Lee氏病理分级中Ⅰ级和Ⅱ级的病理改变均以M0E0S0T0C0为主,随着级别的增高,非M0E0S0T0C0所占的比例上升,Ⅳ级和Ⅴ级的所有患儿的病理改变均为非M0E0S0T0C0。结论儿童原发性IgA肾病的临床表现形式多样,以孤立性血尿型者居多,以病理类型Ⅱ级为主,免疫复合物沉积与病理类型相关,级别高者免疫复合物沉积以IgA+IgM+IgG型多见,Lee氏病理分级与牛津分型之间有很好的相关性和一致性。IgA肾病临床表现的多样性及病理改变对指导治疗与控制其进展具有重要的意义。     

儿童紫癜性肾炎临床与病理相关性分析

目的：通过对95例紫癜性肾炎（HSPN）患儿临床表现及肾脏病理分析,阐明其临床及病理之间的联系。方法：对HSPN患儿进行临床分型及病理分级,对其进行综合分析。结果：①临床分型以肾病综合征型(27.4%)、蛋白尿＋血尿型(24.2%)多见,病理分级以Ⅲb(42.1%)最多见;②尿检正常者可见肾脏病理改变。尿检正常型、孤立性血尿或蛋白尿型以及血尿和蛋白尿型病理改变差异无显著性(P>0.05);③孤立性血尿或蛋白尿型以及血尿和蛋白尿型病例,病程越长病理分级也越重(P＜0.05);④免疫复合物沉积以IgA+IgG+IgM（58%)同时存在比例最高;病理分级越重,病程越短,IgA+IgG+IgM比例越高。结论：HSPN患儿临床表现为肾病综合征和肾炎型者病理改变相对较重,临床症状与病理不一定平行,尿检正常者病理改变也很明显,病程越长,病理改变呈加重趋势。免疫复合物沉积为IgA+IgG+IgM的病理改变相对较重。［中国当代儿科杂志,2007,9（2）：129-132］     

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.