自身免疫性甲状腺疾病患者血清TPO-Ab测定的临床应用

自身免疫性甲状腺疾病（autoimmune thyroid disease,AI-TD）主要包括Graves＇病（GD）和桥本甲状炎（HT）,有关AI-TD的发病机制迄今尚未阐明。甲状腺过氧化物酶抗体（TPO-Ab）是主要的甲状腺组织自身抗体,与甲状腺组织免疫性损伤密切相关。本文对177例AITD患者和64例非AI-TD患者进行了血清TPO-Ab的测定,现报道如下。     

成人IgA肾病的临床与病理探讨

目的 回顾分析73例IgA肾病（IgAN）的临床表现与病理改变及其内在联系。方法 经常规肾穿刺活检获取年龄大于18岁的IgAN忠者的病理结果，并将临床表现及病理结果进行分类分型，同时获取IgAN的病理资料。结果 成人IgAN的临床表现以慢性肾炎为常见。占30例（41．1％）．其次是无症状血尿／尿检异常及肾病综合症。病理类型以系膜增生型肾小球肾炎常见，占39例（53．4%）。其次为局灶节段硬化性肾小球肾炎。慢性肾炎常见的病理类型为系膜增生性肾小球肾炎，其次为局灶节段硬化性肾小球肾炎和膜增殖性肾小球肾炎；无症状血尿／尿检异常及肾病综合症常见症理类为系膜增生型肾小球肾炎，其次为局灶节段硬化型肾小球肾炎；急性肾炎综合症以局灶节段增生性肾小球肾炎和系膜增生性肾小球肾炎为主，急进性肾炎主要表现为新月体肾炎．肾病综合征表现为系膜增生性肾小球肾炎和局灶节段硬化性肾小球肾炎。IgAN的病理特点以轻系膜增生为主，伴有部分及全球的硬化及间质的慢性化表现。结论 成人IgAN的临床表现以慢性肾炎最常见，病理类型以系膜增生性肾小球肾炎常见．慢性肾炎除局灶节段增生性肾小球肾炎外，其余各种病理类型均可以见到。急进性肾炎仅见于无症状性血尿／尿检异常，除新月体肾炎外。各种病理类型均可见到。IgAN的病理特点以轻系膜增生为主，同时中度及重度系膜增生占一定比例，并伴有肾小球硬化及间质纤维化，提示预后差，建议早期肾活检。积极干预治疗。     

291例自身免疫性甲状腺疾病患者血清TPO-Ab等检测分析

自身免疫性甲状腺病（AITD）主要包括Graves’（GD）、桥本氏甲状腺炎（HT）和特发性黏液性水肿（PM）。抗甲状腺过氧化物酶抗体（TPO—Ab）与AITD的发生、发展密切相关。笔者通过对2007年7月～2010年10月我院诊断为GD、HT、PM291例患者进行回顾性分析，即重点分析血清TPO-Ab的浓度，结果发现，AITD的患者血清TPO-Ab浓度明显增高，并进一步分析和探讨其对上述疾病的诊治临床意义。现将结果报道如下。     

膜性肾病合并IgA肾病的临床病理特点

目的探讨膜性肾病合并IgA肾病的临床病理特点。方法回顾性研究北京大学第一医院肾内科和北京大学肾脏病研究所1998年1月—2006年4月问的肾活检病例9572例,对11例膜性肾病合并IgA肾病的临床病理特点进行分析,结合免疫电镜标记方法,对其病理诊断及发病机制进行探讨。结果11例患者以中年为发病高峰,平均年龄39．9岁,女性多于男性（男：女为1：2．9）,临床表现为蛋白尿,其中7例（63．6％）出现肾病综合征水平的蛋白尿,7例（63．6％）合并镜下血尿,肾功能均正常,除外了肝炎病毒感染、系统性红斑狼疮等继发性疾病。光镜下可见肾小球基底膜空泡变性和增厚,系膜细胞和基质轻度增生,2例可见少数肾小球伴有新月体形成。免疫荧光检查见IgG和c3颗粒样沿肾小球毛细血管壁沉积;IgA团块状在肾小球系膜区沉积。电镜检查可见肾小球上皮细胞下多数块状电子致密物沉积,系膜区可见团块状电子致密物沉积。免疫电镜标记结果显示,IgG定位于肾小球上皮细胞下的电子致密物,IgA定位于肾小球系膜区的电子致密物。结论膜性肾病合并IgA肾病兼具有膜性肾病和IgA肾病的临床病理特点,其发生过程可能为各自独立发生的两种疾病的叠加所致。     

TPO-Ab及TGA与淋巴细胞亚群联检在AITD中的临床价值

目的:探讨自身免疫性甲状腺疾病(AITD)发病与甲状腺自身抗体之间存在的作用关系.方法:分别采用电化学发光法(ECL)检测了甲状腺过氧化物酶抗体(TPO-Ab)水平,采用放射免疫分析检测了89例男性及81例女性AITD患者和相应正常对照组的甲状腺球蛋白抗体(TGA),并采用免疫荧光标记单克隆抗体和流式细胞仪联检T淋巴细胞亚群(CD4 /CD8 )的活性.结果:AITD患者TPO-Ab以及TGA的水平均显著高于对照组(P＜0.01),相关分析显示,抗体水平与淋巴细胞T细胞亚群(CD4 /CD8 )活性变化没有明显相关(P＞0.05).结论:甲状腺自身抗体的水平变化可作为AITD部分病因的推测和提示.     

肾炎患者尿液单个核细胞尿激酶受体的表达及其意义

尿液中的免疫活性细胞与肾小球肾炎的发生、发展具有直接关系,而尿激酶受体（urokinasereceptor,u－PAR）作为淋巴细胞活化抗原,在肾小球肾炎尿液中单个核细胞（UMC）的表达情况及其意义尚不清楚。我们采用流式细胞仪,对１０７例肾炎患者的尿液进行了分析并探讨了其意义。１材料和方法１．１材料表现有大量蛋白尿、低蛋白血症和高胆固醇血症等肾病综合征特征的肾小球肾炎住院患者１０７例,男６３例,女４４例,年龄８～５４岁。按其病理类型分为两组：增生性肾小球肾炎组（以下简称增生组）４０例,包括：系膜增生性肾炎１６例、膜…     

原发性肾病综合征患儿539例病理类型及随访

目的分析原发性肾病综合征（PNS）患儿的病理类型、治疗及随访情况。方法纳入2005年1月至2009年12月在湖南省儿童医院肾内科住院的诊断为PNS,进行肾脏病理学检查且随访时间≥6个月的患儿,根据年龄分为〈2岁组、～5岁组、～10岁组和〉10岁组,对不同年龄组肾脏病理类型、对糖皮质激素的反应、转归及随访资料进行分析。结果共纳入539例PNS患儿,男性402例,女性137例,男女比例为2.9∶1;〈2岁组159例（29.5%）,～5岁组269例（49.9%）,～10岁组84例（15.6%）,〉10岁组27例（5.0%）（P〈0.01）。①微小病变274例（50.8%）,局灶节段性肾小球硬化79例（14.6%）,系膜增生性肾小球肾炎173例（32.1%）,膜增生性肾小球肾炎6例（1.1%）,膜性肾病4例（0.74%）,增生硬化性肾小球肾炎2例（0.37%）,脂蛋白肾病1例（0.18%）。不同年龄组间肾脏病理类型的分布差异有统计学意义（P〈0.05）。②对糖皮质激素敏感和依赖的PNS患儿以微小病变为主（分别为62.4%和69.6%）,对糖皮质激素耐药的PNS患儿以系膜增生性肾小球肾炎为主（48.4%）。③539例中239例完全缓解,75例部分缓解,61例无效,158例症状控制,尿蛋白（-）,但仍在服用泼尼松。6例患儿分别因对糖皮质激素耐药,病情不能缓解,合并严重感染或进展至终末期肾病放弃治疗而死亡。结论儿童PNS的发病高峰年龄、肾脏病理类型以及对糖皮质激素耐药的发生率可能已发生改变,并且在不同年龄患儿间存在差异。     

慢性肾小球肾炎临床和病理分型与肾功能衰竭

观察慢性肾小球肾炎（ＣＧＮ）１５０例,男７３例,女７７例,年龄１７～５９岁。临床分型：活动型ＣＧＮ２７例,非活动型ＣＧＮ７５例,肾病型ＣＧＮ３２例,肾病高血压型ＣＧＮ１６例。病理形态学分型：系膜增殖性肾小球肾炎８０例,膜性肾小球肾炎１１例,系膜毛细血管性肾小球肾炎３６例,局灶节段性肾小球透明变性或硬化１３例,类脂性肾病１０例。随诊７年以上,有３２例发展为慢性肾功能衰竭（ＣＲＦ）。①把活动型ＣＧＮ２７例和肾病高血压型ＣＧＮ１６例列为经过不顺利的临床型共４３例,其中有３０例（６９７％）发展为ＣＲＦ…     

与抗中性粒细胞浆抗体相关的肾小球疾病6例报告

与抗中性粒细胞浆抗体相关的肾小球疾病６例报告本文首次报告上海儿科所见６例与抗中性粒细胞胞浆抗体（ＡＮＣＡ）相关的肾小球疾病。其中原发性肾小球疾病为急进性肾炎、肾炎综合征、急性肾小球肾炎和肾病综合征各１例。继发性为狼疮性肾炎和紫癜性肾炎各１例。血浆ＡＮ...     

TPO-Ab在甲状腺自身免疫性疾病中的应用

目的：探讨甲状腺过氧化物酶抗体（TPO-Ab）在甲状腺功能亢进（甲亢）、甲状腺功能减退（甲减）、单纯性甲状腺肿大患者的临床价值。方法：采用放射免疫分析检测甲状腺疾病患者血清TGA、TMA、TPO-Ab浓度及血清FL、FT4、TSH水平,分析TPO-Ab在69例甲亢、53例甲减及45例单纯性甲状腺肿的阳性率。结果：TPO-Ab的阳性率（82％-92．5％）明显高于同组的TGA（44．2％）、TMA（69．8％）,甲减组TPO-Ab的阳性值（平均57．4％）明显高于甲亢组（31．2％）和单纯甲状腺肿组（17．6％）,差异有显著意义（P〈0．01）。结诊!TPO-Ab对自身角癌件甲状腺疾病的诊断、治疗和预后评估具右一定的临床价值．     

Acquired glomerular lesions in patients with Down syndrome

The long-term survival of persons with Down syndrome has dramatically increased over the past 50 years. There are no studies addressing the spectrum of glomerular lesions in these patients. We reviewed the clinical-pathologic characteristics of 17 patients with Down syndrome who underwent renal biopsy. The cohort consisted of 12 whites and 5 African Americans with mean age of 29 years (range, 6-45 years). History of hypothyroidism was present in 8 patients. Renal presentations included renal insufficiency (15 patients, mean serum creatinine 3.4 mg/dL), proteinuria (all patients, including 3 with nephrotic syndrome, mean 24-hour urine protein 4.2 g), and hematuria (14 patients, including 4 with gross hematuria). The glomerular diseases found on biopsy were IgA nephropathy (n = 5 patients), focal segmental glomerulosclerosis (n = 4), membranoproliferative glomerulonephritis (n = 2), acute postinfectious glomerulonephritis (n = 2), pauci-immune crescentic glomerulonephritis (n = 2), membranous glomerulonephritis (n = 1), and lupus nephritis (n = 1). Follow-up (mean, 47 months; range, 2-141 months) was available on 16 patients (94%). Two patients (1 with membranous glomerulonephritis and 1 with acute postinfectious glomerulonephritis) had complete remission; 8 patients (4 with IgA nephropathy, 2 with focal segmental glomerulosclerosis, 1 with lupus nephritis, and 1 with acute postinfectious glomerulonephritis) had chronic kidney disease; and 6 patients (2 with pauci-immune crescentic glomerulonephritis, 2 with membranoproliferative glomerulonephritis, 1 with IgA nephropathy, and 1 with focal segmental glomerulosclerosis) progressed to end-stage renal disease, 4 of whom died. In summary, a wide spectrum of glomerular diseases can be seen in patients with Down syndrome, with IgA nephropathy and focal segmental glomerulosclerosis being the most common. Renal biopsy is necessary to determine the type of glomerular lesion and appropriate treatment.     

2013~2017年我院402例肾活检临床病理分析

目的 分析北部湾沿海钦州地区肾脏疾病临床及病理特点。方法 对广西钦州市第一人民医院2013年~2017年共402例肾活检患者的临床和病理资料进行回顾性分析。结果 402例肾活检患者,原发性肾小球疾病(PGN)297例,占73.88%;继发性肾小球疾病(SGN)88例,占21.89%。PGN病理分型中最常见为膜性肾病,其次为微小病变型肾病及IgA肾病,SGN中狼疮性肾炎居于首位,其次为乙肝相关性肾炎。结论 本地区肾活检患者临床表现以肾病综合征为主,PGN最常见的病理类型是膜性肾病,非肾病综合征最常见的病理类型是IgA肾病,本地区无症状尿检异常有肾活检指征患者检出率低。     

Pattern of glomerulonephritis in Hong Kong

A retrospective analysis of all renal biopsies (961) performed in two regional hospitals in Hong Kong during 1977-1985 revealed that IgA nephropathy was the most frequently encountered glomerulopathy. Lipoid nephrosis (minimal change nephrotic syndrome) remained the commonest cause of nephrotic syndrome in children. The frequencies of mesangiocapillary glomerulonephritis, focal glomerulosclerosis, and idiopathic membranous nephropathy were lower than in other populations. Membranous nephropathy was frequently associated with hepatitis B virus antigenemia, especially in children. Other chronic infections did not have a significant pathogenetic role in glomerular diseases. Lupus nephritis was the commonest secondary glomerular disease in our study, and over seventy percent of the renal biopsies showed advanced pathologies with either diffuse proliferative glomerulonephritis or membranous nephropathy.     

Crescentic glomerulonephritis related to hepatitis B virus.

Crescentic glomerulonephritis was diagnosed in two chronic hepatitis B surface antigen carriers. In all three biopsies performed, hepatitis B e antigen was found in glomerular capillary tufts. The first patient presented with nephrotic syndrome and acute renal failure. The glomerular pathology revealed crescentic glomerulonephritis with endocapillary proliferation, and she recovered spontaneously with normal renal function over the following 6 mo despite a persistent hepatitis B surface antigenemia. The other patient presented with nephrotic syndrome and normal renal function. Hepatitis B virus-related membranous nephropathy was diagnosed on the first biopsy, and he did well on symptomatic treatment for 20 mo. The nephrotic syndrome recurred and subsequently he developed acute renal failure a month following interferon treatment. The repeated biopsy revealed a crescentic glomerulonephritis that was associated with mixed membranous and membranoproliferative glomerulopathies. This patient responded to immunosuppression and plasma exchange with residual impairment of renal function. He has been stable after 18 mo follow-up.     

Immunohistochemical study of the membrane attack complex of complement and S-protein in idiopathic and secondary membranous nephropathy.

Twenty-eight renal biopsies from 12 patients with idiopathic membranous nephropathy (MN), eight patients with lupus MN, and eight patients with hepatitis B virus-(HBV) related MN were investigated by immunofluorescence for the presence of C5b-C9 neoantigens of the terminal sequence of complement and for S-protein, which is a regulatory component of the membrane attack complex (MAC). Glomerular MAC was detected in 50% of patients with idiopathic MN, in 75% of patients with lupus MN, and in only 12.5% of the HBsAg carrier with MN. Glomerular adhesions to Bowman's capsule were associated with a high incidence of glomerular MAC deposition only in patients with idiopathic MN. Lupus patients had a high incidence of MAC deposition and patients with HBV-related MN had a low incidence of MAC deposition, in both cases regardless of the presence of glomerular capsular adhesions. It is unlikely that deposition of S-protein could inhibit the glomerular damage in idiopathic or lupus MN because significant glomerular capsular adhesions and MAC deposition were observed despite the concomitant glomerular deposition of S-protein. It was concluded that activation of terminal components of complement may play a role in glomerular injuries in idiopathic and lupus MN. The rare occurrence of glomerular MAC deposition in HBV-related MN could be related to its distinct immunopathogenetic mechanism and its indolent clinical course.     

Systemic lupus erythematosus and thyroid disease – Experience in a single medical center in Taiwan

BackgroundTo investigate the association of systemic lupus erythematosus (SLE) with thyroid diseases in a medical center in central Taiwan.MethodsThis is a retrospective cohort of 2796 SLE patients in a tertiary referral medical center from 2000 to 2013. We screened SLE by catastrophic illness registration from national insurance bureau; and thyroid diseases by ICD 9 codes, then confirmed by thyroid function test, auto-antibody, medical and/or surgical intervention. We compared the rate of hyperthyroidism, hypothyroidism and autoimmune thyroid disease (AITD) in SLE patients and the 11,184 match controls. We calculated the rate of these thyroid diseases and positive antibodies to thyroglobulin (ATGAb), thyroid peroxidase (TPOAb) in SLE patients grouped by the presence of overlap syndrome and anti-dsDNA antibody. We also compared the association of thyroid diseases to severe SLE conditions, including renal, central nervous system (CNS) involvement, and thrombocytopenia.ResultsCompared to the matched controls, the cumulative incidence of thyroid disease, including hyperthyroidism, hypothyroidism and AITD, were all higher in SLE patients (p < 0.0001). The average age of SLE patients with thyroid diseases patients were older than those without thyroid diseases (p = 0.002). Those had euthyroid AITD were younger than other patients with thyroid diseases (p = 0.02). Up to 30.3% SLE patients had overlap syndrome and had higher relative risk of thyroid diseases than those without overlap syndrome, in terms of hypothyroidism and AITD, but not hyperthyroidism. SLE patients with thyroid diseases also carry higher risk for severe complications such as renal involvement (p = 0.024) central nervous system involvement (p < 0.0001).ConclusionSLE patients had significantly higher rate of hyperthyroidism, hypothyroidism, and AITD than the matched control. Among lupus patients, the risks of thyroid diseases are even higher in the presence of overlap syndrome. SLE patients with thyroid diseases had higher risk of renal and CNS involvement.     

Visfatin与自身免疫性甲状腺疾病关系的研究

探讨内脂素（visfatin）与自身免疫性甲状腺疾病的关系,进而为自身免疫性甲状腺疾病的诊断、治疗与预后提供新的思路。采用ELISA法测定不同组别自身免疫性甲状腺疾病患者和健康人群血浆visfatin水平,并分析visfatin与FT3、FT4、TSH、TPO-Ab、TG-Ab、体重指数（BMI）和腰臀比的关系。结果显示,自身免疫性甲状腺疾病患者血浆visfatin水平明显高于对照组（P〈0.05）;桥本甲状腺炎甲减组血浆visfatin水平高于桥本甲状腺炎甲亢组和Graves病组（P〈0.05）;桥本甲状腺炎甲亢组血浆visfatin水平高于Graves病组（P〈0.05）;相关分析显示,Visfatin与FT3、FT4、TSH、TPO-Ab、TG-Ab、BMI和腰臀比不相关（P〉0.05）。结论：Visfatin可能参与了自身免疫性甲状腺疾病的发生发展;Visfatin可能做为诊断自身免疫性甲状腺疾病的参考指标。     

Thin basement membrane nephropathy as a cause of recurrent haematuria in childhood

A survey of 69 children presenting with recurrent or persistent haematuria and submitted to percutaneous renal biopsy at this hospital over a 17-year period, was performed to establish the incidence of thin basement membrane nephropathy (TBMN). A diagnosis of primary glomerular disease was established in 44 (IgA nephropathy in 16, Alport's syndrome in 13 and other varieties of glomerulonephritis in 15). Of the remaining 25 patients in whom light microscopical and immunochemical examination revealed no abnormalities, material for electron microscopy was available in 11. In eight of these (five of whom had a family history), TBMN was diagnosed on the basis of ultrastructural morphometric evaluation of glomerular basement membrane thickness. Assuming a similar proportion of the remaining 14 patients with renal biopsy specimens normal by light microscopy had TBMN, the probable frequency of this abnormality in the whole series would be 26%, very similar to that of IgA nephropathy. In the eight TBMN patients the mean glomerular basement membrane thickness ranged between 181 and 236 nm, whilst in 'control' biopsies from children with 'minimal change' nephrotic syndrome or IgA nephropathy, the mean thickness ranged between 242 and 333 nm.     

从复旦大学附属儿科医院31年肾脏病理及临床资料反思肾活检指征

目的了解复旦大学附属儿科医院肾脏风湿科肾脏疾病的病理学类型及临床特点,并对肾活检指征进行反思。方法回顾性分析1979至2009年肾活检病理学分型和临床资料,并以10年为一个间期分3个阶段进行分析比较。结果 31年中肾活检1 633例,其中1 419例满足入选条件进入分析。①原发性、继发性和遗传性肾脏疾病分别占63.9%（907例）、23.2%（329例）和12.1%（172例）。②原发性肾脏疾病中,IgA肾病（26.6%,241/907例）、微小病变病（23.0%,209/907例）和轻微病变（18.1%,164/907例）所占比例较高,局灶节段肾小球硬化仅占3.0%（27/907例）;继发性和遗传性肾脏疾病分别以紫癜性肾炎（47.1%,155/329例）和薄基膜肾病（80.8%,139/172例）所占比例最高。③31年间肾活检病理学类型构成比的变化趋势为IgA肾病、轻微病变和紫癜性肾炎在各阶段所占比例逐渐增加,系膜增生性肾小球肾炎及HBV相关性肾炎所占比例逐渐减少。④肾活检临床表现以血尿（38.8%,551/1 419例）和原发性肾病综合征（30.9%,439/1 419例）多见。血尿中单纯性镜下血尿占14.5%（206/1 419例）,其病理类型主要为薄基膜肾病（52.9%,109/206例）和轻微病变（23.3%,48/206例）;原发性肾病综合征初发为单纯性肾病的患儿中激素依赖及频复发占11.1%（157例）,其病理学类型主要为微小病变（61.8%,97例）和轻微病变（17.2%,27例）。结论肾活检病理学的构成比中仍以原发性肾脏疾病多见,主要为IgA肾病和微小病变,临床表现以血尿和原发性肾病综合征为主。肾活检对于单纯性镜下血尿患儿意义相对有限,临床上可密切随访尿蛋白和尿微量蛋白,如有异常再考虑行肾活检。而对于原发性肾病综合征激素依赖及频复发,特别是初发表现为单纯性肾病的患儿,临床上应关注糖皮质激素的不良反应,如出现严重不良反应,需加用免疫抑制剂特别是环孢素A和他克莫司前应考虑行肾活检。