A chemotactic inhibitory factor in the minimal change nephrotic syndrome

This study attempts to investigate the role of neutrophil in the increased incidence of infection in nephrotic syndrome. We investigated the function of neutrophils in the various categories of nephritis and the relationship to the response of steroid therapy in the nephrotic syndrome. We used peripheral blood for the study of chemotaxis in 62 children suffering from nephritis in the acute phase and in remission. These patients included minimal change nephrotic syndrome (9 cases), focal segmental glomerular sclerosis (3 cases), mesangial cell proliferative nephropathy (23 cases), Hepatitis B antigenemia associated membranous glomerulonephropathy (4 cases), poststreptococcal glomerulonephritis (20 cases) and chronic glomerulonephritis (3 cases). The chemotactic index was significantly increased in normal range in the remission stage. The molecular weight of the chemotactic inhibitory factor was estimated to be about 89,000 using Sephacryl S-200 column. All cases of mesangial proliferative nephropathy had nephrotic syndrome with frequent relapses or steroid resistance. These results suggest that the chemotactic index may serve as an important parameter between steroid responsive and non-responsive nephrotic syndrome.     

Failure of clinical and laboratory characteristics to differentiate mesangial proliferative from minimal-change nephrotic syndrome

Twelve clinical and laboratory characteristics of nephrotic syndrome were compared in 24 children with biopsy-proven mesangial proliferative glomerulonephritis (MesPGN) and 17 children with biopsy-proven minimal-change nephropathy (MCNS). The objective of the study was to determine if these characteristics alone, without renal biopsy, could be used to differentiate the two histopathologic entities. Sex, urinary protein level and IgM immunofluorescence were found to be significantly different in the two groups. Discriminant analysis produced two formulae which gave a discriminant rate of 79% for MesPGN and 76% for MCNS. We conclude that the clinical and laboratory characteristics studied could not differentiate MesPGN from MCNS.     

他克莫司在儿童肾脏疾病中的临床应用及作用机制

儿童肾小球疾病的发病机制中免疫因素是主要致病因素之一。他克莫司作为继环孢菌素A之后临床应用的一种强而有效的免疫抑制剂,近年来在儿童肾脏疾病治疗中的地位与作用日渐被重视。他克莫司在针对难治性肾病综合征包括激素依赖型、激素耐药型和频繁复发型等肾小球疾病治疗中取得了良好的效果。该文从作用机制入手,综述他克莫司在肾病综合征、局灶节段性肾小球硬化、系膜增生性肾小球肾炎、膜性肾病、狼疮性肾炎等儿童肾脏疾病中的应用。     

Nephrotic syndrome in mesangial proliferative lupus nephritis

Renal involvement is a major complication of systemic lupus erythematosus (SLE) and occurs in 30-70% of patients with SLE. Lupus nephritis is classified into six classes (I-VI) by the International Society of Nephrology and Renal Pathology Society (ISN/RPS). Although nephrotic syndrome is commonly associated with diffuse (ISN/RPS class IV) or membranous (ISN/RPS class V) lupus nephritis, several reports have described nephrotic syndrome in adult patients with minimal mesangial lupus nephritis (ISN/RPS class I) or mesangial proliferative lupus nephritis (ISN/RPS class II). However, nephrotic syndrome in mesangial proliferative lupus nephritis has rarely been reported in children. Although the pathogenesis of nephrotic syndrome with mesangial lupus nephritis is incompletely understood, three potential mechanisms have been postulated including lupus nephritis itself, non-steroidal anti inflammatory drug (NSAID)-induced minimal change nephrotic syndrome (MCNS) and coincidental occurrence of MCNS. We describe here a child with mesangial proliferative lupus nephritis who developed MCNS.     

Clinicopathological features of childhood nephrotic syndrome in Saudi Arabia.

The clinicopathological features are described in 119 Arab children in Saudi Arabia with the nephrotic syndrome. The clinical and laboratory data are similar to those described in other parts of the world. However, mesangial proliferative glomerulonephritis (MesPGN) was found in 21 of 66 biopsies (31.8%), giving a frequency of 17.6% of all children with the nephrotic syndrome. Minimal-change nephrotic syndrome (MCNS) was diagnosed in 17 biopsies (25.8%) and in 58 patients (48.7%). Onset of the nephrotic syndrome was at less than 1 year of age in 17 patients (14.3%). Seven children had 11 episodes of peritonitis. Seven children had positive hepatitis B surface antigen (HBsAg) in their serum: renal biopsy carried out on four of them showed membranous glomerulonephritis (MGN) in three, and four of the seven patients developed end-stage renal disease (ESRD). There were nine deaths, all in patients with end-stage renal disease: six of the deaths occurred in infants. The pattern of childhood nephrotic syndrome in Saudi Arabia is different from the pattern in tropical countries.     

Hepatitis B-associated nephrotic syndrome in Jamaican children

Between December 1984 and November 1996, 171 children under 12 years old presented to the University Hospital of the West Indies with nephrotic syndrome. Hepatitis B surface antigen (HBsAg) was found in ten (6%) of these children, eight of whom had membranous nephropathy (MN), and one each had mesangial proliferative glomerulonephritis (MesN) and minimal change nephrotic syndrome (MCNS). Only those children with MesN and MCNS were steroid-sensitive. The HBsAg-positive status was identified incidentally on screening. At a mean follow-up of 34 months, seven of ten children had experienced complete or partial remission and three had persistent nephrotic syndrome, although none was in renal failure. Six of the ten had biochemical hepatitis. All the children were still HBsAg-positive. Hepatitis B virus (HBV) is a factor contributory to nephrotic syndrome in Jamaican children. As diagnostic clinical markers for HBV-associated nephropathy are usually absent, all children presenting with nephrotic syndrome should be screened for HBsAg. A policy should be implemented in Jamaica for screening pregnant women and at-risk groups for HBsAg, as well as for immunising susceptible neonates, in order to reduce the incidence of HBV-associated pathology.     

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??Objective??To explore and conclude the pathological categories and clinical data of childhood renal diseases and understand the importance of renal biopsy in childhood renal diseases. Methods??Totally 753 patients who underwent percutaneous renal biopsy from 1995 to 2015 were selected as study subjects??and their clinical and pathological information was analyzed retrospectively. Results??Among 753 patients who underwent percutaneous renal biopsy??428 cases??56.84%?? had primary glomerular disease??306 cases??40.64%?? had secondary glomerular disease??17 cases??2.26%?? had heritage glomerular disease??and 2 cases??0.27%?? had renal tubular interstitial disease. The most common clinical diagnosis were primary nephritic syndrome. The most common clinical diagnosis and pathological category in primary glomerulary disease were primary nephritic syndrome and IgA nephropathy respectively?? and in secondary glomerulary disease they were purpura nephritis and mesangial proliferative glomerulonephritis respectively. Thin basement membrane disease and Alport’s syndrome are the most common pathological category of the heritage glomerular disease. The 8 repeated renal biopsies showed pathological and clinical progression. Conclusion??The primary glomerulary disease is the main type of childhood glomerulary diseases??The most common clinical diagnosis and pathological category are primary nephrotic syndrome and mesangial proliferative glomerulonephritis respectively. The repeated renal biopsy is beneficial to control the transformation of pathological types and adjust new treatments timely.     

环孢素A治疗儿童不同病理类型肾病综合征83例的疗效观察

目的　研究环孢素A(CyA)治疗儿童不同病理类型肾病综合征的临床疗效及意义。方法　 83例肾病综合征患儿入院后逐渐减用激素 ,给予口服CyA ,剂量 5mg/ (kg·d) ,疗程 3～ 6个月 ,并监测血浓度调整CyA的剂量。结果　 83例患儿经治疗后 ,尿蛋白转阴者 4 5例 (完全缓解率 5 4 % ) ,尿蛋白减少者 2 3例 (部分缓解率 2 8% ) ,未缓解 15例 (18% ) ;总有效率达 82 %。不同病理类型治疗反应 :微小病变型肾病有效率为 86 % ,系膜增殖性肾小球肾炎为 84 % ,膜增殖性肾小球肾炎为 3/ 5 ,局灶节段性肾小球硬化为 2 / 4。显效时间为 7～ 4 5d ,其效应多出现于用药 1个月内。服药后分别于 1周和 2周末 ,测定CyA的血药浓度 ,有效血浓度维持在 10 0～ 2 0 0 μg/L ,可使大部分患儿病情顺利缓解 ,疗程一般在 3～ 6个月。 83例患儿都进行了随访 ,其中 6 8例经CyA治疗缓解后的 17例在减量或停药后出现复发 ,复发率为 2 5 % ,复发的患儿重新服用CyA仍然有效。治疗过程中 5例患儿出现一过性尿肌酐的增加 ,8例尿N 乙酰 β D 氨基葡萄糖苷酶轻微增加 ,一般减量或停药后可逆转。 结论　CyA是替代皮质激素治疗难治性肾病的较好方法之一 ,能有效而快速达到治疗难治性肾病的目的 ,其治疗效果与有效的血药浓度和病理类型有关     

江西地区肾脏病儿童757例肾活检的病理与临床资料分析

目的 探讨儿童肾脏疾病的病理特点及其与临床表现的关系.方法 回顾性分析2002年2月-2010年6月在江西省儿童医院行肾活检的757例肾病患儿的病理及临床资料.将肾活检组织分别行光镜、免疫荧光、免疫组织化学及电镜检查.肾活检组织均作苏木精-伊红(HE)、过碘酸雪夫反应(PAS)、六胺银(PASM)及Masson染色;免疫荧光检测IgG、IgA、IgM、C3、C4、C1q.有乙型肝炎病毒感染证据者肾组织同时行乙型肝炎表面抗原(HBsAg)、乙型肝炎e抗原(HBeAg)、乙型肝炎核心抗原(HBcAg)免疫组织化学.参照中华医学会肾脏病分会2000年制定的标准进行病理分型,结合临床和病理资料进行统计分析.结果 1.肾活检病例757例中原发性肾小球疾病537例(70.97%),其中肾病综合征265例(49.35%),孤立性血尿99例(18.44%);继发性肾小球疾病211例(27.84%),其中紫癜性肾炎144例(68.25%),乙肝相关性肾炎47例(22.27%);遗传性肾小球疾病9例(1.19%).2.原发性肾小球疾病病理类型最多的是系膜增生性肾小球肾炎277例(51.58%);继发性肾小球疾病中紫癜性肾炎最多,为144例(68.25%),其病理分级以Ⅱb～Ⅲb为主,占79.17%;遗传性肾小球疾病中Alport综合征8例;薄基底膜肾病1例.结论 江西地区儿童肾脏疾病以原发性肾小球疾病为主,病理改变以系膜增生性肾小球肾炎占绝大多数;继发性肾小球疾病中除以紫癜性肾炎为主外,乙肝相关性肾炎并不少见.     

儿童C1q肾病10例临床与病理分析

目的探讨C1q肾病的临床与病理改变的关系。方法对10例经肾活检确诊为C1q肾病患儿临床表现、肾小球、肾小管及免疫病理特征进行分析比较,6例肾病综合征中环磷酰胺冲击治疗3例,环胞素、霉酚酸酯和甲泼尼龙冲击治疗各1例。结果临床表现为单纯性血尿2例,肾炎综合征、急性肾炎各1例,肾病综合征6例;病理类型为轻微病变、系膜增生性肾小球肾炎各2例,局灶节段性肾小球硬化5例,新月体肾炎1例;肾小管间质1例无改变,Ⅰ级和Ⅱ级各3例,Ⅲ级2例,Ⅳ级1例;免疫荧光:系膜区均有娃著的以C1q为主的沉积。10例患儿平均随访25.7个月;6例肾病综合征均对激素抵抗,加用免疫抑制剂治疗,5例缓解,1例无效,肾功能渐减退。结论C1q肾病临床病理改变多样化,临床以肾病综合征为主,病理以局灶节段性肾小球硬化为主,对激素多不敏感,预后与间质损害程度相关,与C1q沉积无相关性。     

Glomerulopathy with mesangial IgM deposits: Long-term follow up of 64 children

BACKGROUND: The aim of the present study was to investigate to what extent IgM nephropathy in children with minimal change nephrotic syndrome (MCNS) and diffuse mesangial hypercellularity (DMH) evolves to focal segmental glomerulosclerosis (FSGS). METHODS: Tissues from renal biopsies were examined by light microscopy (LM), immunofluorescence (IF) and, in four cases, by electron microscopy (EM). From a total of 352 nephrotic children, 121 had renal biopsy results as steroid dependent or resistant. A diagnostic renal biopsy was also performed in 331 children with non-nephrotic proteinuria and/or hematuria. A second renal biopsy was performed in 16 children whose renal function was impaired during the follow up. The clinical course of IgM-positive children was compared with that of IgM-negative children. RESULTS: Of the 121 nephrotic children with renal biopsy, 85 were MCNS. Twenty were IF positive mainly for IgM, six of whom (30%) presented evolution to FSGS, while of the remaining 65 IF-negative children, only three (4.6%) presented evolution to FSGS. Of the total 331 children with non-nephrotic proteinuria and/or hematuria, 139 were diagnosed as IgA--IgG nephropathy, 44 had positive IF for IgM and 148 were IF negative. Of the 44 children IF positive for IgM, seven (15.9%) presented evolution to FSGS, while none of the 148 IF-negative children presented evolution to FSGS. The follow-up time for all children ranged from 1 to 14 years. CONCLUSIONS: Of IgM nephropathy patients with MCNS and DMH, a significant percentage develop impaired renal function, due to the evolution of FSGS, as revealed by repeat biopsy during long-term follow up.     

Late resistance to corticosteroids in nephrotic syndrome

Corticosteroid resistance appeared late in the course of relapsing nephrotic syndrome in 12 patients who previously had steroid-sensitive relapses for 0.8 to 13 years. In 11 patients, renal histology performed earlier in the course of the disease showed minimal change in eight, mesangial proliferative glomerulonephritis (MesPGN) in two, and focal segmental glomerulosclerosis (FSGS) in one. Renal biopsy in another patient and a repeat procedure in four of eight patients who initially showed minimal change was done after they had developed steroid resistance, and showed FSGS. Cyclophosphamide was given to 11 patients after they became steroid resistant, and induced remission in eight that continued for 1 to 2 years in two patients. The other six had relapses that were steroid sensitive, but three of them (two with FSGS and one with MesPGN) later became resistant to steroids as well as to cyclophosphamide. Of six patients with FSGS, four with initial or subsequent resistance to cyclophosphamide eventually developed renal insufficiency. The other two have remained in remission for 12 to 16 years; one of these did not receive cyclophosphamide. Our observations suggest that patients with late steroid resistance comprise a heterogeneous group; those with FSGS and resistance to cyclophosphamide therapy may have a poor outcome.     

Forms of nephrotic syndrome more likely to progress to renal impairment

In this article, emphasis is placed on those conditions that appear as the idiopathic nephrotic syndrome but that are more likely to result in progressive renal failure. Four conditions, membranoproliferative glomerulonephritis (mesangiocapillary glomerulonephritis), mesangial proliferative glomerulonephritis, membranous nephropathy, and focal segmental glomerulocosclerosis, account for 12 to 15 per cent of cases of idiopathic nephrotic syndrome during childhood. The further aim of this article is to focus on recent progress in our understanding of these conditions and to remind readers that little is known regarding the etiology and pathogenesis of each, and that effective, clearly proven forms of treatment for each are not apparent currently.     

Nephrotic syndrome associated with varicella infection

A 4-year-old boy developed nephrotic syndrome following varicella infection. Serologic studies during the early phase of the disease demonstrated a decrease in serum C3, C4, and properdin factor B. Renal biopsy revealed an acute proliferative glomerulonephritis with deposition of immunoglobulins A (IgA) and M, C3, C1q, and varicella virus antigen in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, this suggested a postinfectious immune complex glomerulonephritis. These phenomena suggested that varicella virus antigen antibody complexes were deposited in the glomerulus and activated the classic and alternative pathway of complements, leading to an immune complex glomerulonephritis. During the nephrotic phase, an increase in OKT8 cells and decrease of the OKT4 cells were demonstrated. Two months later, this alteration returned to normal as the renal disease was in remission. This change of lymphocyte subsets during varicella infection may play a role in the pathogenesis of nephrotic syndrome.     

婴幼儿227例肾穿刺及临床病理学特点

目的 分析婴幼儿肾脏疾病临床病理的特点及婴幼儿肾穿刺安全进行的方法和意义.方法 对临床诊断为肾脏疾病的227例婴幼儿进行经皮肾穿刺活检,肾脏病理组织分别进行光镜、免疫荧光和电镜观察.光镜标本做苏木精-伊红(HE)、过碘酸雪夫反应(PAS)、六胺银(PASM)和Masson染色,免疫荧光检测其肾组织中的IgG、IgM、IgA、C3、C4、C1q、Fibrin,204例标本同时行电镜检查.结果 所有患儿肾穿刺均获成功,术后无明显并发症.有肾穿刺适应证的227例婴幼儿肾脏疾病中最常见的是肾病综合征(38.3%)、孤立性血尿(37.0%)和急性肾炎综合征(9.3%),继发性肾脏疾病相对较少(5.3%).肾脏病理类型中最常见的是系膜增生性肾小球肾炎(62.6%)、IgA肾病(8.4%)和局灶节段性肾小球硬化(5.7%).87例肾病综合征病理类型最常见的是系膜增生性肾小球肾炎(50例)、微小病变(11例)和局灶节段性肾小球硬化(9例);84例孤立性血尿病理类型最常见的是系膜增生性肾小球肾炎(68例)和IgA肾病(9例).结论 在不盲目扩大适应证的基础上,安全有效地进行婴幼儿肾穿刺,可以提高婴幼儿肾脏疾病的诊治水平.     

Lupus nephritis. Collaborative study by the French Society of Paediatric Nephrology.

Sixty two children were included in a collaborative study to determine the prognosis for lupus nephritis. Renal involvement was confirmed by histologic study of renal biopsy specimens which were classified into five categories: minimal lesions (11 cases, 18%); focal segmental glomerulonephritis (15, 24%); diffuse proliferative glomerulonephritis (30, 48%); membranous nephropathy (5, 8%); and glomerular sclerosis (1,2%). That the predictive value of the early biopsy is limited was indicated by the most recent status of 37 patients five years after onset--total remission (13, 35%); urinary abnormalities or nephrotic syndrome (7, 19%); moderate renal failure (4, 11%); chronic renal failure (7, 19%); and hypertension (6, 16%). Treatment did not always prevent the development of severe renal failure; in particular, plasmapheresis failed to avert the death of one patient and the development of chronic renal failure in two others.     

Cyclosporine in steroid dependent and resistant childhood nephrotic syndrome

OBJECTIVE: To evaluate the efficacy of cyclosporine (CyA) monotherapy in steroid resistant (SRNS) and steroid dependent (SDNS) nephrotic syndrome in children. DESIGN: A retrospective study. SETTING: Tertiary kidney care center for children at Bangalore. METHODS: Forty-one children with SDNS and SRNS with normal renal functions were treated with CyA at a dose of 6 mg/kg/day initially and maintained at 3 to 4 mg/kg/day if remission was sustained. The dosage was adjusted according to the CyA blood levels in non-responders. RESULTS: The median age of patients was 93 months (range 48-936) months. Thirteen children had minimal change disease (MCNS), 10 had mesangial proliferative glomerulonephritis (GN). Ten had membrano-proliferative (GN) (MPGN) and 8 had focal segmental glomerulosclerosis (FSGS). Median age at onset of disease and median time for CyA usage from disease onset was 22 months and 16 months respectively. Median duration of CyA therapy was 24 months (range 6-72) months. The data was analyzed to determine significance of variables on the outcome. Median follow up was 71 months (range 20-205) months. Eleven children were CyA resistant. Of the remaining 30 who were CyA responders, 22 (73.33%) were CyA dependent. Seven children developed chronic renal failure (CRF). CONCLUSIONS: The predictors for CyA non-responsiveness were steroid resistance, non MCNS on biopsy and longer duration between onset of nephrotic syndrome and CyA usage, irrespective of the age of onset of the disease. There was a higher incidence of CyA dependence among young responders. Patients with CyA resistance are at high risk for significant infections and CRF.     

单中心儿童肾活检1 579例临床和病理类型分析

目的 了解肾穿刺患儿病理特点及其与临床表现的关系,以及疾病谱变迁。方法 调取华中科技大学同济医学院附属同济医院儿科1989至2012年行肾活检病例的临床分类和病理学分型资料,依据年龄（<1、～3、～6、～12和～18岁）和性别分组进行构成比的比较和分析,以2001年为时间节点分为2个阶段分析疾病谱的变化趋势。结果 1 579例肾活检患儿进入分析,平均肾穿刺年龄（9.3±3.2）岁,男女比例1.92∶1。①肾活检患儿中原发性肾小球疾病949例（601%）,继发性肾小球疾病493例（31.2%）,遗传性肾脏疾病130例（8.2%）;原发性肾小球疾病中肾病综合征、单纯血尿、急性肾炎分别占44.8%、26.2%和15.3%;继发性肾小球肾炎中紫癜性肾小球肾炎、HBV相关性肾炎（HBVGN）和狼疮性肾炎（LN）分别占55.4%、22.9%和18.4%;遗传性肾脏疾病中薄基底膜病和Alport综合征分别占50.0%和462%。②949例原发性肾小球疾病的病理类型以轻微病变/微小病变(24.8%)、IgA肾病(21.0%)和系膜增生性肾小球肾炎(191%)为主;女性新月体性肾小球肾炎构成比显著高于男性。③肾病综合征构成比随年龄增长呈逐渐减少趋势,单纯血尿构成比在～12岁组最高（31.0%）,急性肾炎和慢性肾炎构成比随年龄增长呈逐渐增加趋势;HBVGN主要分布于～3岁组(71.4%),构成比随年龄增长而呈下降趋势。LN主要见于～18岁组,溶血尿毒综合征主要分布于～3岁和～6岁组。④原发性肾小球疾病主要病理类型：肾病综合征为微小病变/轻微病变(31.1%),急性肾炎为毛细血管内增生性肾小球肾炎(28.3%),慢性肾炎为硬化性肾炎(59.4%)。⑤2002至2012年肾病综合征、紫癜性肾炎、IgA肾病构成比较1989至2001年显著增高,急性肾炎、 HBVGN构成比显著下降。结论 肾活检患儿肾小球疾病临床和病理类型与年龄、性别有一定相关性,23年间某些肾小球疾病的构成比发生变化。     

儿童IgA肾病72例临床与病理分析

目的探讨儿童IgA肾病(IgAN)的临床、病理特点及其相关关系。方法对本院2005年5月-2011年8月经肾穿刺活检确诊为IgAN的72例患儿的临床表现、临床分型、病理特点及免疫分型进行回顾性总结,并分析它们之间的相关关系。结果本组72例。男48例,女24例;年龄1岁5个月～17岁[(8.99±2.94)岁];入院时病程2 d～9 a(平均12.86个月)。临床以血尿起病者58例(包括38例肉眼血尿及5例伴水肿者),以单纯水肿起病者12例,以蛋白尿起病者2例。临床分型为肾病综合征型28例(38.89%)、孤立性血尿型19例(26.39%)、血尿和蛋白尿型13例(18.05%)、急性肾炎型10例(13.89%)、孤立性蛋白尿型2例(2.78%)。病理改变:系膜增生型肾小球肾炎40例,局灶增生型肾炎25例、毛细血管内增生型肾炎6例、新月体型肾炎1例。其中伴新月体形成者17例(占23.61%)。免疫组织化学可见多种免疫球蛋白沉积。沉积类型为满堂亮型1例、IgA+IgG+C32例、IgA型8例、IgA+IgM+IgG+C3型17例、IgA+IgM+C3型44例。结论 IgAN的临床表现形式多样,其病情轻重与起病形式无关。病理表现以系膜增生型肾小球肾炎为主,免疫球蛋白沉积以复合型为主。临床表现为肾病综合征型及血尿和蛋白尿型者病理较重,应尽早行肾穿,及时治疗。     

他克莫司联合小剂量激素治疗儿童激素抵抗型肾病综合征21例临床分析

目的 分析评估他克莫司对治疗儿童激素抵抗型肾病综合征的疗效及其安全性.方法 采用回顾性纵向研究分析21例激素抵抗型肾病综合征患儿,他克莫司初始剂量0.10 ～0.15 mg/(kg·d),每12小时1次,定期监测血药浓度、尿常规、血常规及肝肾功能等指标.同时口服小剂量泼尼松0.20 ～0.75 rmg/( kg·d).结果 1～3个月后观察近期疗效,完全缓解者14例,部分缓解者7例,完全缓解率66.7％.16例患儿接受了肾活检,其中6例微小病变型肾病患儿中3例完全缓解,3例部分缓解;4例局灶节段性肾小球硬化患儿中2例完全缓解,2例部分缓解;5例lgM肾病及1例系膜增生性肾小球肾炎患儿均完全缓解.服药期间6例患儿出现一过性不良反应,经对症处理后均缓解.20例患儿获随访,1年内共4例复发,第2年共4例6次出现复发.结论 他克莫司对儿童激素抵抗型肾病综合征有较好的疗效,不良反应较少,大多可耐受,但服药1～2年内复发率较高,因此其长期疗效仍有待于进一步随访观察.