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??Abstract?? Objective To investigate the children with idiopathic membranous nephropathy??IMN?? about the clinical and pathological characteristics?? efficacy and prognosis?? providing the reference for the clinical diagnosis and treatment.Methods Summarize and analyze the clinical manifestation?? pathological features?? treatment and prognosis of 20 cases of IMN patients in Paediatric Nephrology of Shengjing Hospital of China Medical University between 2006.2 and 2014.2. Results ??1??In 20 cases of children with IMN??there were 13 male cases and 7 female cases, male to female ratio 1.86??1. The age ranged from 4 to 14 years?? the mean age being 10.65 ± 3.18 years??from onset to renal biopsy the was 7 to 190 days??with an average of 59.1 ± 55.9 days.There were 5 cases of below nephrotic proteinuria in clinical manifestations?? ??including simple proteinuria in 1 case?? hematuria and proteinuria in 4 cases????and 15 cases of nephrotic proteinuria?? of which 1 case with acute renal insufficiency.??2??Renal pathology:light microscopy showed that there were 2 cases of stage I membranous nephropathy??17 cases of stage II membranous nephropathy and 1 cases of stage III membranous nephropathy??among which 16 cases were with mild mesangial cells proliferation and mesangial matrix increase??4 cases with partial glomerular sclerosis??percentage of glomerular sclerosis was 2.4%??3.4%??3.7% and 5.5%??respectively????and 7 cases with focal tubular atrophy and interstitial fibrosis.Immunofluorescence showed there was IgG and C3 deposition?? partly with IgM??Fg??C1q??and IgA deposition.??3??According to the level of proteinuria the patients were treated with glucocorticoid and renin angiotensin converting enzyme inhibitor??ACEI?? or combined with immunosuppressive therapy with a result of 12 cases completely relieved and 8 parthy relieved.??4??In the 19 cases followed up for 2-7 years??12 cases obtained complete remission?? of which 2 cases replased in 1.5 year and 2 years after drug withdrawal. Eight cases had partial remission??of which 7 cases were still receiving sequential treatment??while 1 case lost follow-up because of refusing the use of immunosuppressant. Conclusion The onset of IMN is mainly in elder children and male is more than female.Nephrotic syndrome is the major clinical manifestations of IMN. Glucocorticoids combined withimmunosuppressive drugs can achieve satisfactory therapeutic effect.     

Treatment of recurrent papillary thyroid carcinoma in children and adolescents

Mortality for children with papillary thyroid carcinoma (PTC) is low (< or = 10%), but recurrence is frequent (20%). In adults, recurrent PTC has a poor prognosis (50% remission) and a high mortality (16-63%). We hypothesized that treatment of recurrent PTC would be more effective in children than in adults. We reviewed the clinical course of 42 children with PTC in remission. Seven (7/42, 17%) recurred and records were available for six. All six received RAI (median = 130 mCi), and one had a cervical node removed. Five of the six (83%) achieved remission (median duration 67 mo, range 10-99 mo), one had a second recurrence and a third remission, and one patient (17%) had persistent disease. There were no deaths. In conclusion, although we had only one child less than 10 years of age in our study, these and previous data suggest that treatment of recurrent PTC in children is generally effective.     

儿童特发性膜性肾病临床病理特点及治疗探讨

目的 了解儿童特发性膜性肾病(IMN)的临床病理特点,探讨其治疗方案.方法 回顾性分析25例病理确诊的IMN患儿的临床病理特点,总结其不同治疗方法 的疗效.结果 儿童IMN占同期所有肾穿刺活检(简称肾穿)患儿的3.81%.25例IMN中男9例,女16例;起病年龄2～14岁,平均(9.4±3.4)岁;肾穿时病程0.4～11.0个月,中位数2.5个月.临床表现为肾病综合征肾炎型21例(84%),肾小球肾炎4例(16%).全部患儿均伴血尿,其中肉眼血尿7例,高血压4例,并发血栓2例,肾功能不全1例.病理分期IMNⅡ期21例(84%).伴中重度小管间质损害者6例,伴局灶节段硬化2例.22例肾病综合征及肾病水平蛋白尿患儿中,21例首选糖皮质激素治疗,其中20例符合评价激素疗效标准:激素敏感1例(复发后转为激素耐药),19例为激素耐药(95%).后续治疗包括继续单纯激素减量隔日治疗8例,其中完全缓解5例,部分缓解3例;激素联合免疫抑制剂治疗12例,该12例连同首选联合免疫抑制剂治疗1例、激素治疗5周联合免疫抑制剂治疗1例,共14例.结论 本组患儿IMN临床表现以肾病综合征为主,均伴有不同程度血尿.绝大多数初治激素耐药,但部分病例减量隔日治疗过程中获缓解,联合免疫抑制剂治疗及疗效尚需进一步临床验证.

Abstract：

Objective To investigate the clinicopathological feature and treatment of idiopathic membranous nephropathy(IMN)in children.Method A retrospective analysis of 25 cases of biopsyproven IMN seen between January 2004 and December 2009.Result The incidence of IMN was 3.81% in all the children patients who underwent renal biopsy.Of 25 patients with IMN,nine were boys and sixteen were girls.The mean age at onset was(9.4±3.4)years with a range of 2-14 years.Renal biopsies were performed at a median 2.5 months(range 0.4-11 months)after onset.The clinical manifestations included nephrotic syndrome(NS)nephritic type in 21 cases(84%)and glomerulonephritis in 4 cases.All patients presented with hematuria,and 7 had macroscopic hematuria.Hypertension was noted in 4 patients.Two patients were complicated with thrombosis.One patient was in a chronic renal insufficiency(CRI)state.According to the MN staging criteria,21 cases were in stage Ⅱ IMN(84%).Six patients showed moderate or severe tubulointerstitial lesion.Focal segmental glomerulosclerosis(FSGS)was found in two patients.Of the 22 patients with NS and nephrotic proteinuria,21 cases were treated with prednisone initially and in 20 of them the efficacy of corticosteroid therapy was evaluated:one of them was steroid sensitive(became steroidresistant after relapse)and all the others were steroid-resistant(95%).The subsequent treatment:eight of them were treated with prednisone followed by a taper to alternate-day therapy.Five of them had complete remission and three partial remission.Twelve cases were treated with combined therapy of prednisone and immunosuppressive agents. Of these 12 cases together with one case who received initially combined treatment with prednisone and immunosuppressive agent and one case treated with prednisone initially for five weeks then with combined therapy contained another immunosuppressive agent,totally 14 cases,5 had complete remission,2 partial remission,3 did not achieve remission,and 3 had unknown response.Conclusion Of the patient cohort,the predominant presenting feature was nephrotic syndrome,and with different degree hematuria.Almost all of them were steroid resistant,but followed by a taper to alternate-day therapy,some could achieve remission.The effect of a combination of prednisone and immunosuppressive agent is needed to be further proven in children.     

激素联合免疫抑制剂治疗儿童原发性IgA肾病有效性和安全性的Meta分析

目的 系统评价激素联合免疫抑制剂治疗儿童原发性IgA肾病的有效性和安全性。方法 检索中英文电子数据库,纳入对比激素联合免疫抑制剂和单用激素治疗儿童原发性IgA肾病的有效性、安全性的研究。结局指标为尿蛋白缓解率、尿蛋白定量、不良反应发生率、估算肾小球滤过率和肾功能损害发生率。使用Review Manager 5.3软件进行数据分析。结果 最终纳入7项研究,包括381例患儿（基线平均尿蛋白定量为中到大量水平）。Meta分析结果显示,激素联合免疫抑制剂组比单用激素治疗组达到更高的尿蛋白缓解率（RR=1.36,95% CI：1.19~1.55,P < 0.001）,更低的尿蛋白定量（SMD=-0.82,95% CI：-1.23~-0.41,P < 0.001）;两组不良反应发生率差异无统计学意义（RR=1.28,95% CI：0.92~1.77,P=0.14）。结论 现有证据显示,对于伴有中到大量蛋白尿的原发性IgA肾病儿童,与单用激素治疗相比,激素联合免疫抑制剂治疗的疗效更佳,且不增加不良反应的发生。     

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.     

小儿肾病综合征的中长期随访研究

目的探讨小儿肾病综合征（ＮＳ）的预后及有关因素。方法对７２例小儿ＮＳ患儿作中、长期随访,其中１６例随访１０年,４９例随访５年,７２例随访３年。结果３组的完全缓解率分别为７５％、８４％及７９％。基本痊愈率在５年组为４９％,１０年组为６９％。死亡３例。结论肾小球的组织学改变与预后有关。小儿ＮＳ总的预后较好;微小病变、膜性肾病的预后较局灶硬化、弥漫性硬化及膜增殖型要好;轻、中度系膜增殖型预后好,严重增殖者预后较差。延长激素疗程,缓慢停药可增加缓解率,减少反复     

Posttransplant de novo membranous nephropathy in childhood

Posttransplant proteinuria is a recognized, but relatively uncommon, presentation of renal transplant dysfunction. Significant proteinuria occurs in around 10-15% of renal transplant recipients. We present a case of de novo posttransplant membranous nephropathy in childhood complicating renal transplantation for severe congenital obstructive uropathy and review the pathology, pathogenesis, and clinical implications of this condition. In the majority of cases, the cause of posttransplant proteinuria is either related to chronic allograft nephropathy or recurrence of the glomerulonephritis for which transplantation was indicated. In a minority, however, de novo posttransplant membranous nephropathy (DNPMN) is identified on biopsy. The histopathological findings in some cases may either be similar to those of classical membranous nephropathy, or may be more subtle, showing focal segmental variation in severity, often in conjuction with the features of chronic allograft nephropathy. The use of ancillary techniques including immunohistochemistry and electron microscopy may be required to confirm the diagnosis. The presence of posttransplant de novo membranous nephropathy may be associated with an increased risk of graft loss.     

儿童特发性膜性肾病的临床病理特点及治疗转归（附22例报告）

目的　总结儿童特发性膜性肾病（IMN）的临床病理特点、治疗及预后转归。方法　回顾性分析2005年1月至2017年2月山东大学附属省立医院小儿肾脏风湿免疫科经病理证实的22例IMN患儿的临床表现、病理特征、治疗及预后等资料。结果　22例患儿中男12例,女10例,发病年龄3～15岁。临床表现：血尿蛋白尿4例（18.18%）;原发性肾病综合征18例（81.82%）。初次肾活检结果：膜性肾病Ⅰ期8例（36.36%）、Ⅰ～Ⅱ期5例（22.73%）,Ⅱ期5例（22.73%）、Ⅱ～Ⅲ期1例（4.54%）、不典型膜性肾病3例（13.64%）。22例患儿肾组织M型磷脂酶A2受体（PLA2R）阳性者12例（54.54%）,83.33%（10/12）PLA2R阳性的患儿起病年龄≥10岁。治疗方案：4例血尿蛋白尿患儿中2例给予激素及血管紧张素转化酶抑制剂（ACEI）治疗,2例仅给予ACEI治疗,均获得完全缓解。18例肾病综合征患儿全部予以激素、ACEI治疗,激素耐药者加用免疫抑制剂治疗。随访发现22例患儿中18例（81.82%）获完全缓解,3例（13.64%）获部分缓解,1例（4.54%）失访。结论　儿童IMN临床多表现为肾炎型肾病综合征,以激素耐药型为主。病理表现以Ⅰ～Ⅱ期多见。儿童IMN的PLA2R阳性以青少年为主,小年龄儿童阳性率较低。激素联合ACEI、免疫抑制剂治疗缓解率较高。     

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??Objective??To summarize the clinicopathological features??treatment and prognosis of childhood idiopathic membranous nephropathy??IMN??. Methods??The clinical manifestations??pathologic features??treatment and prognosis of 22 IMN children who were diagnosed by pathology from January 2005 to February 2017 were retrospectively analyzed. Results??Twenty-two children??12 males and 10 females?? had an onset age range of 3 to 15 years. Clinical manifestations??hematuria and proteinuria in 4 cases??18.18%????nephrotic syndrome??NS?? in 18 cases??81.82%??. Renal biopsy results??8 cases??36.36%?? in stage??5 cases??22.73%?? in stage ??-??5 cases??22.73%?? in stage ??1 case??4.54%?? in stage ??-??atypical membranous nephropathy in 3 cases??13.64%??. Among them??12 cases??54.54%?? of M-type phospholipase A2 receptor??PLA2R?? positive were detected and 83.33%??10/12?? PLA2R positive children with an onset age older than 10 years old. Immunofluorescence showed predominantly IgG and C3 deposition. Treatment programs??in the 4 cases whose clinical manifestations were hematuria and proteinuria??2 cases were given glucocorticoid and ACEI drug treatment??the other 2 cases were given ACEI drugs alone??and they had complete remission. The 18 patients with NS all were treated with glucocorticoid and ACEI??and immunosuppressive agents were given to steroid-resistant patients. During the follow-up??18 cases??81.82%?? were completely relieved??3 cases??13.64%?? were partially relieved??and 1 patient??4.54%?? was lost of follow-up. Conclusion??Clinical manifestations of IMN are mostly nephritis nephrotic syndrome??mainly steroid-resistant nephrotic syndrome. Pathological performance mainly is??-??stage. The positive rate of PLA2R in junior age was lower than adolescence. Glucocorticoid combined with immunosuppressive agents has a higher response rate.     

儿童血清学阴性乙型肝炎病毒相关性肾炎13例临床与病理分析

目的 回顾性分析儿童血清学阴性乙型肝炎病毒相关性肾炎（sn HBV-GN）的临床及病理学特征,并初步探讨免疫抑制剂治疗的安全性和有效性。方法 收集 2006 年 1 月至 2011年12月经首都医科大学附属北京儿童医院肾活检诊断为HBV-GN病例,根据血清学结果筛选出HBsAg和HBV-DNA阴性患儿,分析其临床、病理资料和治疗情况。结果 13例sn HBV-GN患儿进入分析,男8例,女5例,平均年龄11.8岁。临床表现以肾病综合征为主（11例）,9例伴有血尿;2例表现为肾炎综合征。①11例以不典型膜性肾病为主要病理表现,免疫荧光以IgG、C3沉积为主, 亦可见IgA、IgM、C1q沉积。②肾组织HBsAg与HBcAg阳性率分别为100%（13/13）和76.9%（10/13）,强阳性率分别为23.1%（3/13）和15.4%（2/13）。③2例肾炎综合征患儿经抗凝和卡托普利治疗完全缓解;11例肾病综合征患儿,单纯抗病毒治疗2例均未缓解;单纯糖皮质激素治疗2/3例部分缓解,糖皮质激素加环孢素治疗2/3例部分缓解,糖皮质激素加用麦考酚酸酯治疗3例均完全缓解。加用免疫抑制剂患儿均未发现肝功能异常及HBV-DNA活动表现。结论儿童sn HBV-GN的病理以不典型膜性肾病为主要表现;免疫荧光显示免疫复合物的种类少,沉积强度低;麦考酚酸酯联合激素治疗较单用激素或激素联合环孢素治疗可能有效,单纯抗病毒治疗可能无效。     

儿童原发性膜性肾病16例病理特点与远期预后

目的 了解儿童原发性膜性肾病(IMN)病理特点及其远期预后。方法 回顾性收集1979至2010年6月复旦大学附属儿科医院肾脏风湿科经病理诊断为IMN的连续病例为研究对象,分析一般情况、临床表现、病理特点、治疗和随访情况,探讨其远期预后。结果 16例IMN患儿进行分析,占同期肾穿刺活检病例（1 710例）的0.94%。男10例,女6例,年龄2～12岁,平均（5.2±2.6）岁。①临床表现以肾病综合征为主（11例,68.8%）,无症状性蛋白尿5例（31.2%）,伴有高血压2例(12.5%）,起病时伴有肾功能不全2例（12.5%）。②14/16例行肾组织电镜检查,其中Ⅰ期6/14例（42.9%）,Ⅰ~Ⅱ期6/14例（42.9%）,Ⅱ期1/14例（7.1%）,Ⅱ~Ⅲ期1/14例（7.1%）。病理学检查均未见肾小管萎缩、间质纤维化等肾小管间质损伤。③未达到大量蛋白尿标准的7例患儿予随访观察,其中1例病情进展予激素和免疫抑制剂治疗;达到大量蛋白尿标准的9例患儿均予足量激素(2 mg·kg-1·d-1)治疗,其中5例激素依赖或耐药加用免疫抑制剂治疗。至2010年6月,2例失访,14例IMN患儿随访12~91个月,平均（34.0±18.7）个月,在起病后3~16个月均达完全缓解,无一例进展至慢性肾脏疾病;2例起病时伴肾功能不全的患儿肾功能均恢复。结论 儿童IMN临床表现以肾病综合征为主,小年龄、病理分期较轻且不伴肾小管间质损伤患儿的预后相对较好。     

Persistent asymptomatic isolated hematuria in children: clinical and histopathological features and prognosis

Background

This study involving 351 children who had undergone kidney biopsy secondary to persistent asymptomatic isolated hematuria was undertaken to assess histological diagnosis of the disease and its natural history and prognosis.

Methods

The patients were divided into two groups: 215 patients with asymptomatic isolated microhematuria (AIMH; proteinuria <0.1 g/day) and 136 patients with persistent asymptomatic microhematuria, recurrent macrohematuria and/or proteinuria (AMHP; proteinuria 0.1–0.25 g/day). After kidney biopsy, the patients were monitored for 2–10 years.

Results

Normal biopsies or minor abnormalities were more frequent in AIMH patients than those in AMHP patients, who exhibited IgA nephropathy more frequently. During the 2- to 10-year follow-up period, adverse renal events (i.e., development of proteinuria, hypertension, or impaired renal function) were observed in 13/215 (6.0%) patients with AIMH and 31/136 (22.8%) patients with AMHP (χ²=15.521, P<0.001).

Conclusions

Normal biopsies or minor abnormalities were more frequently observed in AIMH patients, whereas IgA nephropathy and adverse renal events were more frequent in AMHP. Microscopic hematuria, especially when accompanied by macroscopic hematuria and proteinuria, may represent an important risk factor for the development of chronic kidney disease.     

Treatment of hepatitis B virus-associated membranous glomerulonephritis with interferon alfa in a 7 year old boy

BACKGROUND: The association between Hepatitis B virus infection and membranous nephropathy has been confirmed by sources in several countries. Most commonly, the illness is seen as a nephrotic syndrome. Optimal treatment remains undefined. Antiviral therapies observed with recombinant human interferon alpha may be the best treatment option. CASE REPORT: We present a 7-year old boy with membranous glomerulonephritis and nephrotic syndrome. Twelve months after the initial hospitalization therapy was started with recombinant alpha-interferon s.c. three times weekly for six months. After the therapy the patient is stable, without proteinuria, edema or renal failure. He was seronegative for HBsAg, HBV-DNA and antibody to HBeAg. CONCLUSIONS: This case report suggests that alpha interferon is effective in the complete resolution of proteinuria in HBV membranous nephropathy.     

Prevalence and clinical correlates of glomerulopathy in children with sickle cell disease

OBJECTIVES: Glomerular disease and renal failure cause substantial morbidity for patients with sickle cell disease (SCD). Proteinuria is an early manifestation of sickle nephropathy, but the prevalence of proteinuria and its clinical correlations in children with SCD are unknown. STUDY DESIGN: Data were collected prospectively on children with SCD for 10 years including physical measurements, laboratory test results, and clinical complications. Persistent proteinuria was defined as > or =1+ protein on urinalysis for at least 6 months. The glomerular filtration rate was estimated with serum creatinine concentration and height. Proteinuria was correlated with other variables by chi(2) analysis. RESULTS: Proteinuria occurred in 20 of 442 pediatric patients including 15 (6.2%) with sickle cell anemia. Proteinuria increased with age, affecting 12% of older teenagers with sickle cell anemia. Proteinuria was significantly associated with lower hemoglobin concentration, higher mean corpuscular volume, and higher leukocyte count. For children of some ages, proteinuria was associated with complications including stroke, acute chest syndrome, cholelithiasis, and hospitalizations. Glomerular filtration rate hyperfiltration occurred early in life, followed by normalization. CONCLUSIONS: Sickle nephropathy, manifested as persistent proteinuria, begins early in life, occurs in all forms of SCD, and is associated with severity of disease. Early detection of proteinuria may allow therapy to prevent progressive renal insufficiency.     

Paired Trial of Cyclophosphamide and Prednisone in Children with Nephrosis

Cyclophosphamide and prednisone were compared as the primary treatment for the nephrotic syndrome in 27 boys and 4 girls. The patients, aged between 2½ and 11, were paired according to their renal histology; 19 belonged to the minimal change type, 8 to the mild proliferative type, and 4 had diffuse membranous glomerulonephritis but with selective type of proteinuria. The two groups of patients were comparable as regards period of observation before treatment, and duration of follow-up. About half the patients of each group went into spontaneous diuresis, or had no fluid retention, before treatment was started.Cyclophosphamide induced a remission more slowly than prednisone, but remission was less likely to be followed by relapse. Two patients treated with prednisone and one treated with cyclophosphamide still had persistent proteinuria at the time of review.The incidence of cyclophosphamide resistance in nephrosis remains unknown, but is probably not high. Among patients who responded to treatment, duration of persisting proteinuria varied widely, and therefore slow response must be distinguished from true resistance to treatment.Cyclophosphamide should be useful in all patients who relapse after a steroid-induced remission, but its use must be balanced against potential long-term hazards.     

A controlled trial of cyclophosphamide and azathioprine in Nigerian children with the nephrotic syndrome and poorly selective proteinuria.

In a controlled trial, symptomatic treatment alone, or 12 weeks of cyclophosphamide or azathioprine were compared in Nigerian children with nephrotic syndrome (mainly quartan malarial nephropathy) and poorly selective proteinuria. Full remission in 2 patients in each of the two groups treated with drugs, and diminution of proteinuria in most patients in the cyclophosphamide group showed possible evidence of benefit. Infections during treatment were significantly more common in the drug-treated groups but were controllable. Mortality from renal failure in the 2nd year after treatment was significantly greater in the azathioprine-treated group than in the other two, suggesting that the drug may have exacerbated the nephritis. The 5-year survival rate was similar in the cyclophosphamide and the control group.     

Long-term follow-up in children with steroid-resistant nephrotic syndrome.

A prospective study was performed in order to evaluate the efficacy of oral cyclophosphamide and chlorambucil in inducing a remission in children with steroid-resistant primary nephrotic syndrome (NS). Out of 215 children with steroid-resistant primary NS, 164 had been followed from one to 10 years. The children had a mean age of 8.2 years, with a range from one to 16 years. Steroid resistance was more common in children over six years of age compared with the other age groups. Hematuria was seen in 68 of the 164 children (41%); hypertension in 41 (25%); and hyperlipidemia in 112 (68%). Hypocomplementemia was noted in 24 of the 65 (37%) children in whom complement concentrations were determined. Renal biopsy was performed in 117 of the children. Pathologic changes consisted of minimal change nephrotic syndrome (MCNS) in 14 children (12%), membranoproliferative glomerulonephritis (MPGN) in 45 (38%), focal segmental glomerulosclerosis (FSGS) in 20 (25%), mesangial proliferation (MP) in 23 (20%), and membranous glomerulonephritis in six children (5%). Cyclophosphamide (2 mg/kg/day) was given to 164 patients, with complete remission and partial remission rates of 20.7% (34 of 164 children) and 24.4% (40 of 164 children), respectively. In this group, sustained remission and sustained partial remission rates were found in 20% (32 children) and 13% (21 children), respectively. Chlorambucil was given to 40 children with steroid- and cyclophosphamide-resistant nephrotic syndrome, with total remission and partial remission rates of 20% (eight children), and 12.5% (five children), respectively. These rates did not change during the follow-up. Thus, cyclophosphamide is valuable in the treatment of children with steroid-resistant NS with a variety of histologic changes.     

Membranous nephropathy associated with ovarian tumour in a young girl: recovery after removal

We report a 7-year-old girl who presented with membranous glomerulonephritis and steroid-resistant nephrotic syndrome in association with a benign ovarian tumour. Surgical excision of the tumor led to complete disappearance of the proteinuria within 2 weeks. Tumour-associated membranous nephropathy in children is rare, as a review of the literature shows.Abbreviations CEA carcino embryonic antigen - NCA non-specific cross-reacting antigen     

Haematuria in children. I. Differential diagnosis of haematuria in childhood (author's transl)

Haematuria in children is either of glomerular or nonglomerular origin. In the latter case intravenous urography should always be performed. A renal biopsy is indicated if unexplained haematuria persists for at least one year or if an unfavorable prognosis is indicated by the appearance of hypertension, significant proteinuria or persistently low levels of serum complement (C3). The importance of screening the families of haematuric patients is emphasized. More than half of our cases with persistent or intermittent haematuria undergoing renal biopsy showed no or only minimal glomerular changes. In other children with a similar clinical picture more severe histological lesions were detected. In any case the kidney tissue obtained by biopsy should be examined by immunofluorescence and by electron microscopy. One of the most frequent causes for persistent or intermittent haematuria during childhood is Berger's disease (IgA/IgG nephropathy).     

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.