Acquired renal cystic disease in children and young adults on maintenance dialysis

Acquired renal cystic disease (ARCD) is a well-known complication of end-stage renal disease (ESRD). We studied 24 patients, aged 8 – 27 years (mean 19.8±5.3 years), on chronic maintenance dialysis in our service. The duration of dialysis ranged between 13 and 192 months (mean 77.8±44.3 months). High-resolution ultrasonography revealed ARCD in 11 (45.8%) patients. No cysts were seen in 7 (29.1%) patients and solitary cysts in one or both kidneys were seen in 6 (25%) patients. Renal malignancy was diagnosed in 2 patients. One, 15 years old, had renal cell carcinoma after being on dialysis for 6 years. She did well after bilateral nephrectomy, left salpingo-oophorectomy, and regional lymphadenectomy. The second patient, 23 years old, had been on dialysis for 16 years when she developed renal oncocytoma. She died of congestive cardiomyopathy 6 months later. We conclude that ARCD is common in children and young adults with ESRD. Neoplastic transformation, although rare, is a potential complication. Annual follow-up with ultrasonography with selective use of computed tomography or magnetic resonance imaging is advised. Received July 29, 1996; received in revised form and accepted November 15, 1996     

Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis.

Acquired renal cystic disease (ARCD) is defined as the development of multiple cysts in the renal cortex and medulla in patients with chronic renal failure who are free from congenital polycystic kidney disease. ARCD develops generally in contracted kidneys. We report a case of grotesque enlargement of a single kidney in a patient who had been receiving hemodialysis for 18 years. Although the exact causes of ARCD are not known, 3 factors may contribute to the development of nephromegaly; the sex, the duration of hemodialysis and previous unilateral nephrectomy. As in polycystic disease, when the involved kidney reaches considerable size, ARCD may have a favorable effect on anemia caused by chronic renal failure.     

Prevalence of renal cell carcinoma in patients with ESRD pre-transplantation: a pathologic analysis

BACKGROUND: Acquired renal cystic disease (ARCD), renal adenoma (AD), and renal cell carcinoma (RCC) are more common in patients with end-stage renal disease (ESRD). However, the prevalence of these conditions in patients undergoing transplantation, and the clinical characteristics associated with their occurrence are unclear. METHODS: At our institution, the majority of patients undergo an ipsilateral native nephrectomy at the time of transplantation, providing a unique opportunity to study the prevalence and pathology of ARCD, AD and RCC in ESRD. We retrospectively reviewed all consecutive nephrectomy pathology reports over a six year period. Demographic and clinical characteristics associated with these lesions were identified. RESULTS: Two hundred and sixty nephrectomy reports were reviewed: ARCD, AD, RCC and oncocytoma were found in 33%, 14%, 4.2% and 0.6% of cases, respectively. On multivariable analysis, ARCD was positively associated with male sex and longer dialysis duration and negatively associated with peritoneal dialysis. Similarly, AD was positively associated with male sex, longer dialysis duration and greater age. There was a trend for RCC cases to share similar associations although the small total number of cases precluded findings of statistical significance. CONCLUSION: By pathologic analysis, renal tumors are more common in the pre-transplant ESRD population than previously reported (using radiologic methods). Our study also identifies risk factors for their occurrence. This may prove useful in designing screening studies for renal tumors in this patient population.     

Acquired renal cystic disease in HD: a study of 82 nephrectomies in young patients.

In order to study the development of acquired renal cystic disease (ARCD) and its potential complications, we studied, macro- and microscopically, 82 surgical specimens of nephrectomy carried out on young patients with chronic renal failure previous to renal transplantation. Statistical correlation of pathological findings with age, sex and time on hemodialysis (HD) have been done. There were 72 cases of ARCD (87.8%). It was statistically correlated with male sex (p less than 0.02) and prolonged time on HD (p less than 0.001) as has been previously reported. Hyperplasia of the cystic epithelium was found in 42 cases (52%), with 18 (22%) showing marked papillary proliferation. Also, there were 22 cases (27%) with renal adenomas. This incidence of hyperplastic and neoplastic proliferations, more than would be common in such a young population (males: 33.5 +/- 9.3 years; females: 35.4 +/- 11.7 years), suggests the potential of patients affected by ARCD to develop neoplasms. Thus, we consider that these patients must be checked periodically to detect possible malignant neoplasms.     

Acquired renal cystic disease: two cases of associated adenocarcinoma and a renal ultrasound survey of a peritoneal dialysis population

Long-term dialysis patients frequently develop acquired renal cystic disease (ARCD). The discovery of ARCD and renal cell carcinoma in one of our hemodialysis patients led us to review the literature. ARCD has been described mainly in the maintenance hemodialysis (MH) population. Therefore, we investigated 20 peritoneal dialysis (PD) patients for ARCD using ultrasonography. Seven patients (35%) had detectable cysts and two patients (10%) had multiple bilateral cysts. One patient had a large asymptomatic complex cyst that proved to be an adenocarcinoma. Our study suggests that ARCD is relatively common in the PD population, and we speculate that it may be related more to length of time in end-stage renal disease (ESRD) than to the mode of dialysis. The potential for malignant change appears to justify a routine screening examination with ultrasonography and/or computerized tomography (CT) to detect this recently described and probably underrecognized entity.     

The use of cadaver kidneys for transplantation in young children

The role of cadaver kidney transplantation in the management of end-stage renal disease in young children is controversial. To assess the current risk-benefit ratio of cadaver first and second kidney transplants in recipients under 6 years of age, we compared the outcome of 19 transplants performed between 1984 and 1989 using a quadruple-drug regimen (Minnesota antilymphocyte globulin, azathioprine, prednisone, cyclosporine) with the outcome of 25 transplants performed prior to 1984 without the use of cyclosporine at a single institution. Twenty-five transplants were in children under the age of 3 years. In the last decade patient survival has significantly improved. One-year patient survival improved from 53% before 1979 to 90% since 1979 (P less than 0.05). The use of the quadruple-drug regimen since 1984 was associated with a significant improvement in one-year cadaver graft function from 40% before 1979 to 78% in recipients under 6 years of age, and from 22% to 82% in recipients under 3 years of age (P less than 0.05). With the quadruple-drug regimen, one-year and four-year graft function rates for children under 6 years of age were 83% for first cadaver transplants and 72% for second cadaver transplants, which were essentially the same results as in older children and adults. Children who received kidneys from donors over 4 years of age achieved the best result, with 87% one-year graft function compared with 50% for kidneys from donors under 4 years old. In 15 children with successful transplants, 8 (53%) showed accelerated growth, 5 (33%) had normal-velocity growth, and only 2 children (14%) with suboptimal renal function had poor growth following transplantation. Therefore, we believe that with a quadruple-drug immunosuppressive protocol, cadaver renal transplantation using kidneys from adults or pediatric donors over 4 years old is an acceptable form of treatment in young children with end-stage renal disease for whom there are no suitable living-related donors.     

细胞外基质成分异常与获得性肾囊肿大鼠肾小管间质损害的关系

目的探讨细胞外基质(ECM)成分异常与获得性肾囊肿大鼠肾小管间质损害的关系。方法建立化学诱导的获得性肾囊肿疾病(ARCD)大鼠模型。采用免疫组织化学检测不同损害级别的小管间质ECM及α-平滑肌肌动蛋白(SMA)的表达。应用RT-PCR方法检测其mRNA的表达，并分析两者之间的相关性。结果ARCD组不同损害级别的小管间质肾脏组织中，胶原(Col)Ⅰ、Ⅲ、纤连蛋白(FN)及α-SMA表达均显著增加，与小管间质的损害程度呈正相关；层粘连蛋白(LN)的表达组间比较无显著差异，与小管间质的损害程度亦无显著相关性。结论ECM成分异常导致其结构重塑，可能参与了ARCD的形成过程。     

Tacrolimus rescue therapy for children with acute renal transplant rejection

Seventeen children with renal transplants (11 living-related, age 2–18 years) were converted from cyclosporine to tacrolimus because of acute rejection that failed to respond to high-dose corticosteroids. Resistance to corticosteroids was confirmed by renal biopsy in 14 patients, and assumed in 3 patients because of failure of serum creatinine to improve to baseline values. Four patients were also treated with OKT3, and 15 children had been receiving mycophenolate maintenance therapy prior to conversion to tacrolimus. Rejection occurred at 2–174 weeks post transplant (mean 52 weeks). Actuarial 1- and 2-year graft survival was 87% and 78%. Three children progressed to end-stage renal disease after 4, 12, and 13 months of tacrolimus. The remaining 14 children have functioning allografts after 20–168 weeks of treatment (mean 80 weeks). All 14 children exhibit stable or improved renal function: serum creatinine 1.1±0.7 mg/dl versus 2.0±0.9 mg/dl prior to tacrolimus. In conclusion, tacrolimus was effective therapy for both early and late acute rejection in children who failed to respond to high-dose corticosteroids. No significant short-term adverse effects were encountered. Received: 29 August 2000 / Revised: 31 July 2001 / Accepted: 31 July 2001     

Ten year survival after paediatric heart transplantation: a single centre experience.

OBJECTIVE: To report 10 years survival in children under the age of 16 years undergoing heart transplantation in a single institution. METHODS: One hundred and thirty nine/one hundred and ninety three patients (73%) survived more than 1 year after transplant. Seventy four (53%) of these survived more than 10 (10.0-20.1) years. Age at operation was 10 days-15.5 (mean 8.1) years. Patients were maintained on ciclosporin and azathiaprine alone. Routine steroids only given to 4 patients for either persistent rejection or deteriorating renal function. Rejection diagnosed on clinical or echocardiographic grounds. No routine biopsies were performed. Bi-annual coronary angiography was used to diagnose graft coronary disease. RESULTS: Graft coronary disease was found in 8 patients (11%), 2 were re-transplanted and have survived 4.3-7.2 years since. Two patients are alive without intervention 2.0-13.0 years from initial diagnosis. Two patients have undergone interventional procedures 11 and 16 years after transplantation and are alive 3 and 4 years, respectively, later. Seven patients have had post transplant lymphoproliferative disease (PTLD) and 6 have had no recurrence for 3-13 years after treatment. Impaired renal function with abnormal serum creatinine levels is increasingly common-11 patients have developed end stage renal failure, 7 requiring renal transplantation, hypertension occurred in only 3 patients other than those in renal failure. Late rejection episodes associated with probable non-adherence occurred in 7 patients. There were 10 late deaths; 2 from graft coronary disease; 1 from PTLD; 3 from renal failure; 3 from acute rejection and 1 from infection. Conditional actuarial survival from 1 year post transplant was 76 and 67% at 10 and 15 years, respectively. CONCLUSIONS: Survival for more than 10 years is increasingly realistic. In this age group adherence and deteriorating renal function are major challenges.     

End-stage renal disease of the Tunisian child: epidemiology,etiologies, and outcome

From December 1989 to December 1993, 90 children under 15 years were admitted to our department for end-stage renal disease; 9 children were less than 5 years and 28 were aged between 5 and 10 years. The sex ratio (M/F) of the children was 2.1. The estimated incidence of pediatric end-stage renal disease in Tunisia is 7 new cases per year and per million child population under 15 years. The chief etiologies of end-stage renal disease are glomerulonephritis (19%), hereditary nephropathies (29%), and malformative uropathies (13%); 26% of the end-stage renal diseases are of unknown etiologies. Our findings, compared with the European data, show a particularly high frequency of primary hyperoxaluria (13.5%) and an unusual proportion of male subjects. Received April 13, 1995; received in revised form and accepted January 4, 1996     

Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries

Crescentic glomerulonephritis (CsGN) is an uncommon entity in children. This prospective study was conducted to evaluate the aetiology, clinical spectrum and outcome in children with crescentic glomerulonephritis. The single-centre prospective study comprised of 22 children with biopsy proven CsGN who had been referred to our institute over the period January 2000 to December 2005. These patients were subjected to detailed clinical and biochemical examinations. The diagnosis of underlying renal disease was based on various criteria, including the clinical picture, serology and histopathology. The patients received intravenous methyl prednisolone, oral steroid treatment, and oral cyclophosphamide with or without plasmapheresis. All patients received supportive care, including control of hypertension and oedema and supportive management of renal insufficiency. During this 5-year period, CsGN accounted for 5.1% of all biopsies done in children. The mean age was 12.27 years (range 4 years to 18 years). There were eight girls and 14 boys. The mean duration of symptoms prior to referral was 2.47 months (range 5 days to 21 months). Aetiology was immune complex in 19 cases, anti-glomerular basement membrane (anti-GBM) antibody disease in two cases and pauci-immune (Wegener’s granulomatosis) in one case. The percentage of crescents ranged from 50% to 100% (mean 70.6%). Twenty-one out of 22 (95.5%) children in our series had hypertension at presentation that required treatment with antihypertensive medications. The serum creatinine level at presentation ranged from 1.5 mg/dl to 11.4 mg/dl (mean 5.5 mg/dl). Of the 22 children, two were lost to follow-up, while the mean follow-up period of the rest of the 20 children was 8.13 months (range 1 month to 43 months). At the last follow-up of the 22 children, ten had stage 5 chronic kidney disease (CKD) and three had stage 4 CKD, while seven children had a calculated glomerular filtration rate (GFR) of >60 ml/min per 1.73 m² body surface area. Persistent proteinuria was seen on follow-up in the majority [13/20 (65%)] of patients. The outcome of CsGN in children continues to be poor, in our experience, due to delayed referral and delayed diagnosis. This was correlated histologically by the presence of fibrocellular crescents in the majority of our patient. Thus CsGN should be treated as a renal emergency. A greater awareness of this disease needs to be created amongst the referring paediatricians in developing countries to facilitate early diagnosis and prompt treatment.     

End-stage renal disease in Kuwaiti children: an 8-year experience

BACKGROUND: Prior to the establishment of the pediatric nephrology service in Kuwait in 1995, no accurate registry of end-stage renal disease in children was available due to management by various adult nephrologists. In this study we analyzed our experience with renal replacement therapy in children, as the only center in the country offering this service for the past 8 years. SUBJECTS AND METHODS: The records included all children less than 16 years of age with end-stage renal disease treated in the pediatric nephrology unit over a period of 8 years (January 1995 to December 2002). RESULTS: Of the 48 children boys comprises 52% and the overall mean age at institution of dialysis was 94.4 months. Causes of renal disease included congenital structural anomalies in 52%, including obstructive uropathy in 16.6%, vesicoureteric reflux in 16.6%, and renal dysplasia/hypoplasia in 18.7%. Hereditary nephropathy was diagnosed in 35.4%, including primary hyperoxaluria in 10.4%, nephronophthisis in 2%, autosomal-recessive polycystic renal disease in 8%, and glomerulopathies in 14.5%. Other etiologies constituted 14%. Renal replacement therapy was necessary in 43 patients: 46% by peritoneal dialysis and 43% by hemodialysis. The mortality rate in the dialyzed group was 16%. Twenty-four patients received kidney transplants from, cadaveric donors in 19 cases. CONCLUSION: Genetic factors contributed to the high incidence of end-stage renal disease, which is most likely due to the common practice of consanguineous marriages in our country.     

Percutaneous nephrolithotomy for treating renal calculi in children

OBJECTIVE: To report our experience with the percutaneous management of renal stone disease in children. PATIENTS AND METHODS: The medical and radiological records of children up to 18 years old who were treated for renal calculi by percutaneous nephrolithotomy (PCNL) at our institution between March 1995 and April 2003 were reviewed. For stone removal a special paediatric 18 F access sheath was used. RESULTS: In all, 26 PCNLs were used in 23 patients (10 boys and 13 girls, aged 1.7-16.8 years). The presenting symptoms were urinary tract infection, abdominal pain and/or haematuria. Of the 23 patients, 17 (75%) had associated metabolic disease or underlying urological anatomical abnormalities. Urinary tract infections were found in 15 patients (65%). The mean (range) stone burden was 6.0 (0.5-18.2) cm2, and the operative duration 127 (50-260) min. The primary stone-free rate was 58%, which increased to 81% after treating residual fragments. One blood transfusion was required and one patient developed urosepsis after PCNL, which was treated with antibiotics. CONCLUSION: PCNL is an effective alternative for treating renal stones in children, and is the treatment of choice for stones refractory to extracorporeal shock wave lithotripsy.     

Asymptomatic isolated microhaematuria: natural history of 136 children

In a mass screening programme, 251 children with isolated microhaematuria were detected. Of these 251 children, 115 were excluded from the study because of microhaematuria, secondary to a specific cause. The remaining 136 children were diagnosed as having asymptomatic isolated microhaematuria (ASH). Of these 136 children, 23 had evidence of urinary abnormalities in their family members. Red blood cell casts were evident in 31 children at their initial visit or during the follow-up period. Ten children had one or more episodes of macrohaematuria during the study. Renal biopsy was performed in 19 children because of indications of glomerular discase, and 13 of these 19 children had mild to moderate glomerulonephritis. None of these 136 children developed hypertension or renal impairment after a mean period of 7.4 years (range 6–13 years). Thirty-five children had normal urinary findings within 6 years of their initial visit, and 100 have had persistent microhaematuria, without proteinuria throughout the follow-up period. The other child had microhaematuria with proteinuria greater than 1 g/m² per day at the end of the study. This study suggests that the prognosis of ASH is good and that renal biopsy is not indicated for children with ASH.     

Pre- and postcaptopril renal scintigraphy as a screening test for renovascular hypertension in children

We studied the ability of pre- and postcaptopril renal scintigraphy to predict renovascular disease (RVD) in children. Retrospective review of medical notes and radiology reports of all hypertensive children who had had both pre- and postcaptopril renal scintigraphy with [^99mTc] dimercaptosuccinic acid (DMSA) and/or [^99mTc] mercaptoacetyltriglycine (MAG3) and digital subtraction angiography (DSA). 81 children aged 1–18 (median 10) years were studied with 62% (51) having a diagnosis of RVD. Main renal artery disease, intrarenal disease, and both main and intrarenal artery disease were present in 25, 14, and 12 patients respectively. The isotope study accurately diagnosed RVD, confirmed by DSA, in 47% (24 of 51) children, with eight false positive studies. The sensitivity, specificity, and positive and negative predictive values of the isotope study to predict RVD were 48%, 73%, 76%, and 51%, respectively. Pre- and postcaptopril renal scintigraphy was unable to predict RVD in children.     

Renal transplantation in children

INTRODUCTION: Various immunological, metabolic, and technical factors render pediatric recipients with end-stage renal disease unique from their adult counterparts. In addition, the potential for complications after renal transplantation is far greater in children than in adults. In this study, we retrospectively analyzed 83 pediatric recipients who underwent kidney transplantation at our institution from 1975 to 2004. MATERIALS AND METHODS: From November 1975 to December 2004, 1523 renal transplantations were performed at our institution with 56 procedures in 83 pediatric patients (44 boys and 39 girls; age range, 7 to 17 years; mean age, 14.9 +/- 2.2 years). RESULTS: Long-term follow-up revealed the following morbidities in 14 (16.3%) recipients: lymphocele in 7 (8.1%) patients, perirenal hematoma in 2 (2.3%), graft renal artery stenosis in 2 (2.3%), ureteral stenosis in 2 (2.3%), and ureteral anastomotic leak in 1 (1.2%). Six (7.2%) recipients with a functioning graft died during follow-up (five deaths were infection related, and the cause of one death was unknown). Five grafts failed (four for immunological reasons and one as a result of recurrent disease). The 1-, 3-, 5-year patient and graft survival rates were 98%, 93%, 92% and 91%, 78%, 67% for living related transplantations versus 98%, 91%, 90% and 92%, 76%, 65% for cadaveric transplantations, respectively. DISCUSSION: Better outcomes for renal transplantation in children may be obtained by strict adherence to precise surgical techniques, better immunosuppressive management, and early diagnosis/effective treatment of complications.     

Anaphylactoid purpura: Characteristics of 16 patients who progressed to renal failure

Renal insufficiency occurs in at least 1.5% of children with anaphylactoid purpura (AP). We reviewed the records of 16 children who developed end-stage renal disease (ESRD group) secondary to AP and matched them for age, era of onset, renal histology, and clinical severity at onset with 16 children who has AP but whose creatinine clearance returned to and remained normal (recovery group). We reviewed creatinine clearances at 1, 3, 5, and 10 years after onset. A creatinine clearance >70 ml/min per 1.73 m² was present in 50% of the patients in the ESRD group at 3 years and in 25% at 5 years after onset. In contrast, all patients in the recovery group had a creatinine clearance >70 ml/min per 1.73 m² by 3 years (7 of 16 had a creatinine clearance >125 ml/min per 1.73 m²) and all were normal 95–125 ml/min per 1.73 m²) by 5 years. Thus, the presence of an increased creatinine clearance (>125 ml/min per 1.73 m²) at 3 years predicted recovery, while failure to reach a creatinine clearance of >70 ml/min per 1.73 m² at 3 years predicted progression to ESRD. There was no evidence of recurrent systemic AP or nephritis in the 14 patients who underwent renal allograft transplantation. We conclude that long-term evaluation of patients over many years is required to identify those who will progress to ESRD from AP and that recurrence of AP in the renal transplant is uncommon.     

Repeat nephron-sparing surgery for children with bilateral Wilms tumor

Background

Renal insufficiency is a significant complication of Wilms tumor treatment in the 5% with bilateral disease. Nephron-sparing surgery (NSS) is recommended after neoadjuvant chemotherapy initially. However, the role of NSS in recurrent disease is unknown. We reviewed our experience to assess the feasibility and oncologic and functional outcomes of repeat NSS for children with recurrent disease.

Methods

A retrospective review was performed of all children treated at our institution for bilateral, favorable histology (FH) Wilms tumor. Patients undergoing repeat NSS for locally recurrent disease were identified. The outcomes evaluated included tumor recurrence, renal function, and patient survival.

Results

Since 2001, 36 children with bilateral FH Wilms tumor have been treated at our institution. Eight patients (22%) underwent repeat NSS for locally recurrent disease. Two patients had a second local recurrence and underwent a third NSS. Six patients are alive without disease (75%) with an average follow-up of 4.5 years. Two patients have died, each with blastemal-predominant histology at repeat NSS. The surviving patients have normal renal function, although two patients require medical management of hypertension.

Conclusions

Our experience suggests that repeat NSS for local recurrence of FH bilateral Wilms tumor is feasible and affords acceptable oncologic outcome with preservation of renal function. However, more aggressive therapy may be required for patients whose recurrence has blastemal-predominant histology, given the poor outcome for these patients in our series.     

Management of sensitized pediatric patients prior to renal transplantation

Background

Data on renal allograft outcome in sensitized children are scarce. We report the clinical courses of four children who received desensitization therapy prior to renal transplantation in our institution.

Methods

Between 2009 and 2011, four pediatric patients with stage 5 chronic kidney disease received desensitization therapy due to: (1) positive donor-specific antibodies (DSA) and/or crossmatches with potential living donors, (2) more than three positive crossmatches with deceased donors or (3) high calculated panel-reactive antibody of >80 %. Desensitization with rituximab, intravenous immunoglobulin and bortezomib was performed in all patients. Induction therapy included combinations of plasmapheresis and/or alemtuzumab or anti-thymocyte globulin. Standard post-transplant medications included tacrolimus, mycophenolate mofetil and prednisolone.

Results

Post-transplant screening revealed DSA in three patients. Biopsy showed no evidence of rejection at 1 month in two patients, one of whom developed chronic active antibody-mediated rejection 4.5 years later. One patient developed borderline acute cellular rejection at 1 month, but the serum creatinine level was stable and DSA disappeared without treatment 1 month later, with stable long-term allograft function at 3 years. Estimated or measured glomerular filtration rate of the patients ranged between 30 and 75 ml/min/1.73 m² after 1 to 4.5 years.

Conclusions

The four sensitized patients reported here who received desensitization therapy had successful renal transplants with a low risk of immediate post-transplant rejection. Overall, long-term allograft functions and complications from immunosuppression were encouraging.

     

Childhood membranoproliferative glomerulonephritis type I: limited steroid therapy.

Nineteen patients with biopsy proven membranoproliferative glomerulonephritis type I (MPGN I) and a minimum of three years of follow-up (mean 6.5 +/- 0.7 years) have been treated with an uncontrolled regimen of limited corticosteroids. Initial therapy ranged from 20 mg per os (po) every other day to 30 mg/kg/day i.v. for three consecutive days, depending on clinical disease severity. Therapy was then decreased based on each patient's improving clinical status. At diagnosis creatinine clearance (CCr) was less than 80 ml/min/1.73 m2 in 12 patients and less than 50 in 2. All patients had hematuria and proteinuria, with 15 in the nephrotic range. Hypertension, present at diagnosis in 13, developed in five others following institution of prednisone, and was controlled medically. Renal biopsy was repeated after two years of therapy prior to cessation of treatment (mean total treatment duration 38 +/- 3 months). Follow-up biopsy revealed decreased glomerular inflammatory activity in 88% of patients. All patients have now been off prednisone for 40 +/- 9 months. The mean CCr is 126 +/- 5 ml/min/1.73 m2. Eight patients have normal urinalyses. These data suggest that early therapy with a limited course of corticosteroids, and control of associated hypertension, may forestall progressive renal insufficiency in children with MPGN type I.