Percutaneous ultrasound-guided renal biopsy in children - safety, efficacy, indications and renal pathology findings: 14-year Brazilian university hospital experience.

INTRODUCTION: Pediatric percutaneous renal biopsy (Bx) is a routine procedure in pediatric nephrology to obtain renal tissues for histological study. We evaluated the safety, efficacy, indications and renal findings of this procedure at a tertiary care pediatric university hospital and compared our findings with the literature. METHODS: Retrospective study based on medical records from January 1993 to June 2006. RESULTS: In the study period, 305 Bx were performed in 262 patients, 127 (48.5%) male, aged 9.8 A+/- 4.2 years. A 16-gauge needle was utilized in 56/305 Bx, an 18-gauge needle in 252/305 Bx (82.6%). 56.1% Bx were performed under sedation plus local anesthesia, 43.9% under general anesthesia. The number of punctures per Bx was 3.1 A+/- 1.3. Minor complications occurred in 8.6% procedures. The 16-gauge needle caused a higher frequency of renal hematomas (p = 0.05). The number of glomeruli per puncture was >or= 5 in 96.7% and >or= 7 in 92%. Glomeruli number per puncture and frequency of complications were not different according to the type of anesthesia used. A renal pathology diagnosis was achieved in 93.1% Bx. The main indications of Bx were nephrotic syndrome (NS), lupus nephritis (LN) and hematuria (HE). The diagnosis of minimal change disease (MCD) (61.3%), class V (35.6%) and IgA nephropathy (26.3%) predominated in NS, LN and HE patients, respectively. CONCLUSION: Pediatric real-time ultrasound-guided percutaneous renal biopsy was safe and effective. The main clinical indications for Bx were NS and LN, the predominant renal pathology diagnoses were MCD and class V LN.

Comparative study of IgA nephropathy with and without crescents

Glomerular crescents were analyzed as a prognostic factor in retrospectively reviewed data from 144 patients with biopsy-proven IgA nephropathy. Crescents were found in 26 (18 percent) patients, and detected in 2 to 100 percent of glomeruli in each specimen. In 5 percent of the patients more than 50 percent of the glomeruli were affected. Thirty patients with IgA nephropathy without crescents were studied as a control group. Mean age was 30.3 ± 9.4 and 30.2 ± 12.0 years for the patients with and without crescents, respectively, and males prevailed in both groups. The length of follow-up was 23.2 ± 41.6 months for patients with crescents and 29.3 ± 35.3 months for patients without crescents. Eighty percent of the patients with crescents were hypertensive, compared to 27 percent of the non-crescent control group (P < 0.05). Mean serum creatinine at the time of diagnosis was 3.9 ± 2.9 and 1.9 ± 2.1 mg/dl for the patients with and without crescents, respectively. Initial urinary protein excretion was higher in patients with crescents (4.6 ± 3.5 vs 1.2 ± 0.9 g/day; P < 0.05). At the end of follow-up 17 patients (77.3 percent) from the crescent group and 3 (11.1 percent) from the non-crescent group had end-stage renal disease (P < 0.0001). The presence of crescents was associated with higher levels of initial serum creatinine and urinary protein excretion, and a higher frequency of hypertension and progression to end-stage renal disease.

Comparative study of IgA nephropathy with and without crescents.

Glomerular crescents were analyzed as a prognostic factor in retrospectively reviewed data from 144 patients with biopsy-proven IgA nephropathy. Crescents were found in 26 (18%) patients, and detected in 2 to 100% of glomeruli in each specimen. In 5% of the patients more than 50% of the glomeruli were affected. Thirty patients with IgA nephropathy without crescents were studied as a control group. Mean age was 30.3 +/- 9.4 and 30.2 +/- 12.0 years for the patients with and without crescents, respectively, and males prevailed in both groups. The length of follow-up was 23.2 +/- 41.6 months for patients with crescents and 29.3 +/- 35.3 months for patients without crescents. Eighty percent of the patients with crescents were hypertensive, compared to 27% of the non-crescent control group (P < 0.05). Mean serum creatinine at the time of diagnosis was 3.9 +/- 2.9 and 1.9 +/- 2.1 mg/dl for the patients with and without crescents, respectively. Initial urinary protein excretion was higher in patients with crescents (4.6 +/- 3.5 vs 1.2 +/- 0.9 g/day; P < 0.05). At the end of follow-up 17 patients (77.3%) from the crescent group and 3 (11.1%) from the non-crescent group had end-stage renal disease (P < 0.0001). The presence of crescents was associated with higher levels of initial serum creatinine and urinary protein excretion, and a higher frequency of hypertension and progression to end-stage renal disease.

Primary glomerular diseases in Brazil (1979-1999): is the frequency of focal and segmental glomerulosclerosis increasing?

AIMS: Different patterns of glomerulonephritis (GN) are reported from all over the world and the occurrence of primary GN is changing in the course of time. We report the frequencies of primary GN in a major teaching hospital in Brazil, from 1979-1999. METHODS: The case files of renal biopsies of primary GN were reviewed. The included patients were > 14 years of age, with native kidneys, and the specimens were examined with at least light and immunofluorescence microscopy. We excluded biopsy results of patients with any kind of known secondary glomerular involvement. Differences in proportions of diagnoses between the periods over time were evaluated using Chi-square test for trend. RESULTS: We considered 943 patients for the analysis. Focal and segmental glomerulosclerosis (FSGS) was the most common lesion (n = 279), followed by membranous GN (n = 140), membranoproliferative type I GN (n = 109) and IgA nephropathy (n = 109). FSGS (32.1%) was the most frequent diagnosis among nephrotic patients whereas IgAN (29.4%) predominated in non-nephrotic ones. The occurrence of FSGS increased from the earlier to the later periods: 22.3% (1979-1983), 23.7% (1984-1988), 35.7% (1989-1993), 33.9% (1994-1999), p < 0.05. The increase in frequency of FSGS was proportionally higher in non-nephrotic patients and FSGS became as common as IgA nephropathy in this group (31.6% and 28.0%, respectively) from 1994-1999. CONCLUSIONS: FSGS was the most common pattern of primary glomerulonephritis and its relative frequency seems to be increasing in biopsied patients over time. The reasons for this behavior are unclear and warrant further investigations.

Comparison of palpation-guided and ultrasound-guided biopsies in transplanted kidneys.

UNLABELLED: Biopsy is the gold standard for the diagnosis of conditions affecting the function of renal allografts. Obtaining representative tissue in biopsies is critical but these procedures are associated with up to 9% of complications and 20% of inadequate material. Although ultrasound guidance allows perfect control of depth and location of the graft, there is controversy regarding the cost-benefit of its use and reports of unsuitable material in ultrasound-guided biopsies are still high. PURPOSE: To compare ultrasound with the palpation method to guide biopsies in order to see if there is any difference between both methods and which one is better. PATIENTS AND METHODS: The casuistic consisted of 82 renal transplant patients (32 female and 50 male patients, age ranging between 5 and 64 yr; m=31.2 yr) randomized into two groups: GI, palpation-guided; GII, ultrasound-guided. Fifty-six biopsies were performed in GI and 66 in GII. RESULTS: Number of glomeruli, arcuate, and interlobar arteries and arterioles were compared in the two groups and were 503 (m=10) vs. 801 (m=12.9), 24 (m=0.5) vs. 38 (m=0.6), 104 (m=2.1) vs. 154 (m=2.5), and 174 (m=3.5) vs. 264 (4.3), respectively (p<0.05). Inadequate material for analysis in GI and GII was 7.1 and 7.6%, respectively (p=0.72). CONCLUSIONS: Although ultrasound guidance improves the number of glomeruli, arcuate, and interlobar arteries, as well as arterioles, compared with palpation-guided biopsies, there is no difference in the rate of adequate material between the two methods.

Hypertensive nephrosclerosis as a relevant cause of chronic renal failure.

It is currently unclear whether hypertensive nephrosclerosis (HN), usually diagnosed solely on clinical grounds, is a relevant cause of end-stage renal disease. We biopsied 81 hypertensive outpatients (blood pressure >/=160/95 mm Hg) with moderate renal insufficiency, who were referred to our service from 1988 to 1998. Patients with known causes of hypertension, systemic disorders, rheumatic disease, or nephrotic syndrome were excluded. In 65% of patients, HN was the sole histological abnormality associated with renal dysfunction. Benign nephrosclerosis (BN), defined as isolated arteriolar hyalinosis and/or intimal fibrosis, was found in 18 HN patients (22%), whereas malignant nephrosclerosis (MN), denoted mainly by myointimal cell proliferation, appeared in 35 HN patients (43%). Previously undiagnosed primary nephritis (PN) was found in 13 patients (16%), whereas focal and segmental glomerulosclerosis, which might be either primary or secondary to hypertension, appeared in 15 patients (19%). These findings suggest that HN, in both its BN and MN forms, can be a definite cause of chronic renal insufficiency and that a substantial fraction of patients with renal insufficiency and clinical diagnosis of HN may actually have PN.

Multicentric pheochromocytoma and involvement of the inferior vena cava.

CONTEXT: Extension of pheochromocytomas to the inferior vena cava is rare. Multicentric tumors are rare as well, being present in up to 10% of cases. Surgery is the treatment of choice because of the long-term survival free of disease. DESIGN: Case report. CASE REPORT: We report on a case of right adrenal pheochromocytoma with extension to the supra-diaphragmatic vena cava, which underwent surgical excision through thoracophrenic laparotomy without the need for cardiopulmonary bypass. In a 6-year follow-up, another pheochromocytoma was found in the infra-renal Zuckerkandl's organ. Complete surgical excision of the tumor was performed by a median laparotomy and complete retroperitoneal dissection. In both cases, the total removal of the pheochromocytoma has been guaranteed by having margins free of tumor and a normal post-operative level of catecholamines. The pathological study revealed a malignant pheochromocytoma with margins free of neoplasia in both specimens.

Histological outcome of acute cellular rejection in kidney transplantation after treatment with methylprednisolone.

BACKGROUND: Several studies comparing the response of acute cellular rejection (ACR) episodes to different corticosteroid regimens have been conducted. However, in most of them, the histological evaluation of the infiltrate and its correlation with clinical response was not studied. The clinical and histological outcomes of 37 episodes of ACR treated with methylprednisolone (MP) were studied, with the aim to determine how long the infiltrate takes to be cleared after therapy. METHODS: A total of 37 patients with biopsy-proven ACR were treated with 8 or 16 mg of MP/kg/day. Allograft biopsies were repeated at 5 and 10 days after the end of corticotherapy. Clinical and histological outcomes were compared. RESULTS: Six patients were excluded; 15 (48.4%) patients responded to therapy; the mean serum creatinine of these patients reached normal levels in the 2 weeks that followed treatment. Nine patients (60%) of this group had signs of ACR on biopsies done 5 days after corticotherapy, and four (26.7%) maintained them on the 10th day. Among 16 patients with no clinical response, none reached normal serum creatinine levels; 15 (93.7%) had signs of rejection 5 days after treatment and maintained them on the 10th day. Histological signs of ACR disappeared in 73.3% of patients with clinical response 10 days after therapy, but in only 6.3% of patients with no response (P=0.001). CONCLUSIONS: Biopsies performed 5 days after treatment show a high incidence of features of ACR; such features take on average 10 days to disappear in nearly 75% of cases with successful therapy with MP.

Renal transplantation in systemic lupus erythematosus. A case control study of 45 patients.

BACKGROUND: Outcome and the issue of recurrence of disease in systemic lupus erythematosus (SLE) renal transplant recipients is still a matter of controversy. There is a lack of comparative studies with non-SLE patients. The aim of this paper is to compare renal transplantation in lupus patients with a similar matched non-SLE group. METHODS: Forty-five patients with systemic lupus erythematosus subjected to 48 kidney transplants were studied. For comparative purposes, a case-control population was selected, matched for gender, race, type of donor, age, and time of transplantation. Patients with non-glomerulonephritis diseases were excluded. RESULTS: No differences in acute episodes of rejection, causes of kidney loss or patient death were observed. General as well as infectious complications were similar. Pregnancy rates and outcomes were similar with no deleterious effect on patients or grafts. Actuarial 1- and 5-year patient survivals (97.7 and 91.1% for SLE and 95.4 and 87% for controls, respectively) and graft survivals (93.1 and 80.7% for SLE and 88.8 and 70.2% for controls, respectively) were similar. Long-term renal function expressed by serum creatinine was the same. No differences in immunosuppressive drug (azathioprine, prednisone, and cyclosporin) requirements were found. Clinical SLE recurrence was suspected only once (a patient with thrombocytopenia, hypocomplementaemia with low complement levels and positive antiplatelet antibodies). Two SLE patients showed mesangial proliferative glomerulonephritis compatible with recurrence. Both grafts were lost. Two further patients showed membranous glomerulonephritis with an immunofluorescence pattern compatible with recurrence. A fifth patient had necrotizing arteritis which recovered after treatment with cyclophosphamide and another patient showed focal and segmental glomerulosclerosis. Histology of biopsies from five patients in the control group showed signs compatible with recurrence of focal and segmental glomerulosclerosis and membranous glomerulonephritis. There was a wide variation in serum levels of antinuclear antibodies. A wide variation in complement levels was also observed, but with a tendency towards low C4 levels. CONCLUSIONS: The safety of renal transplantation in SLE patients is equivalent to a matched case-control group with a similar rate of recurrence of disease.

Glycosuria in glomerular diseases: histopathology and clinical correlations.

There are doubts about the presence of glycosuria and the progress of glomerular disease. Some reports suggest that glycosuria could be an index of a more severe tubulointerstitial lesion. We investigated the presence of glycosuria in 60 patients with primary glomerular diseases: 17 patients (28%) had glycosuria and 43 patients (72%) were glycosuria free. The two groups were similar in age, arterial pressure and sex. Serum creatinine was higher in patients with glycosuria (2.0 +/- 1.7 vs 1.3 +/- 0.9 mg/dl, P < 0.05). The protein excretion rate was 7.5 +/- 3.7 vs 5.3 +/- 4.2 g/day (P > 0.05) in patients with and without glycosuria, respectively, while serum albumin was lower in patients with glycosuria (1.7 +/- 0.6 vs 2.7 +/- 1.0 g/dl, P < 0.05). Several histological forms were present in the group with glycosuria, with membraneous glomerulonephritis being the most frequent. Histological evidence of tubular atrophy and interstitial fibrosis prevailed in patients with glycosuria, suggesting a poor prognosis for these patients. We may conclude that the presence of glycosuria in patients with glomerular disease is associated with more pronounced tubular atrophy and interstitial fibrosis and therefore imply a poorer prognosis.

[Comparison of clinical and histological diagnosis in kidney post-transplantation period]. / Comparação entre diagnósticos clínicos e histológicos no pós-transplante renal.

PURPOSE: To assess the agreement between clinical and histopathological diagnosis in a renal transplantation center, 40 episodes of acute renal failure were studied. METHODS: Kidney biopsies were performed at the moment that a clinical diagnosis was made by the staff. RESULTS: Nineteen episodes of acute tubular necrosis (ATN), eighteen episodes of acute cellular rejection (ACR), 2 humoral rejections and 1 acute cyclosporin nephrotoxicity episodes were diagnosed. ATN episodes were confirmed by renal biopsy in 84.21%, ACR episodes in 83.33%, humoral rejections in 100%. Renal biopsy showed ATN in the occurrence of clinical cyclosporin nephrotoxicity. Total agreement was 82.5%. CONCLUSION: There is a good relationship between clinical and histopathological diagnosis in the post-transplantation period. Diagnostic mistakes occurred mainly when oliguria was present.

Acute vascular rejection: a clinical and morphological study.

We analyzed one special type of acute vascular rejection (AVR), defined as fibrous thickening of the arterial intimal layer that leads to early renal failure. Twenty-one patients who presented this histological pattern were studied among 339 transplanted over 4 years. Patients were separated into two groups. Thirteen patients have restained their kidneys (Group A, 61.9%) and 8 have lost their grafts (Group B, 38%). Diagnosis was made on average 430. POD in GA and at 49 degrees POD in GB on the 43rd postoperative day in group A and on the 49th postoperative day in group B (NS). In group A, mean serum creatinine is 2.2 mg/dl and follow-up time is 29 months. Oliguria was much more frequent in group B (75% versus 15.3%, P = 0.01). These patients were submitted to 91 renal biopsies always because of non-function. Typical vascular lesions began at arcuate arteries and progressed, as seen in sequential biopsies, to interlobular arteries and arterioles. When only arcuate arteries were affected, 22.5% of renal losses were seen, but when arcuate plus interlobular arteries were compromised, 72.2% of patients lost their kidneys (P = 0.006). We did not identify any difference in immunofluorescent staining from biopsies with or without vascular rejection, or between groups A and B. We concluded that about 2.3% of our patients lost their kidneys because of this kind of AVR, diagnosed near the 43rd postoperative day. The only clinical predictive sign of poor reversibility was oliguria. The attack on arcuate plus interlobular arteries meant a poor prognosis. Immunofluorescent staining did not have a prognostic value.

Contribution of the expression of ICAM-1, HLA-DR and IL-2R to the diagnosis of acute rejection in renal allograft aspirative cytology.

Acute rejection is associated with a poor long-term prognosis for renal allografts. Sequential fine-needle aspiration cytology (FNAC) has been used to monitor rejection. However, FNAC diagnoses rejection only when the infiltrating cells are already damaging the graft and, in some borderline cases with a low increment of inflammatory cells in the graft, FNAC lacks the specificity to diagnose rejection. In these cases, the number of inflammatory cells within the graft can decline, stabilize or increase with time. In this study, we sought to determine whether the analysis of the expression of ICAM-I, HLA-DR and IL-2R along with borderline FNAC results increases the specificity to diagnose rejection. Of 117 FNAC samples taken from 24 patients after renal transplantation, 85 (72%) were considered suitable for cytological analysis. Of these patients, 9 (37%) did not suffer an acute cellular rejection (ACR) episode and 15 (63%) had at least one ACR episode. ICAM-1 and IL-2R were studied using an immune-peroxidase technique. The ICAM-1 results are expressed as the percentage of tubular cells in the aspirate stained with this marker and the IL-2R results are expressed as the absolute number of positively stained lymphocytes in the whole cytopreparation. With a total corrected increment (TCI) of > 3 there was a sharp increase in the specificity index for rejection that reached almost 100% at a TCI of > or = 4. Sensitivity for rejection at this level was only 20%. Between a TCI of 2.5 and 2.9 the sensitivity increased to 75%, with specificity for rejection around 75%. There was an upregulation of ICAM-1 and IL-2R when FNAC diagnosed rejection but with a large overlap of the results when compared either to normal graft or acute tubular neurosis. The mean TCI during the week preceding the rejection episode was 2.5 and the TCI reached a mean value of > or = 3 only during rejection. The peak ICAM-1 and IL-2R expression occurred during the week preceding the clinically evident rejection episode. The expression of ICAM-1 by > or = 70% of the tubular cells increased the specificity for rejection of a TCI of > or = 2.5 to 100%. In the same way, the specificity for rejection increased up to 90% when eight to ten IL-2R-positive lymphocytes were seen along with a TCI of > or = 2.5. There was no further increase in specificity after that. A specificity index of 100% for rejection could be obtained for moderate levels of both ICAM-1 (70% or more tubular cells) and IL-2R (eight or more lymphocytes). ICAM-1 expression in 70% or more tubular cells and/or IL-2R expression in eight or more lymphocytes was found in 58% of the FNAC aspirates with a TCI between 2.5 and 2.9. In conclusion, the expression of IL-2R in lymphoid cells and ICAM-1 in tubular cells was upregulated during rejection episodes and the upregulation preceded both the clinical and the routine FNAC diagnosis of rejection by 1 week. The ddition of these markers to the FNAC increased substantially the specificity of the FNAC to diagnose rejection.

Post-transplant neutrophilic interstitial nephritis--an important cause of graft dysfunction.

Post-transplant neutrophilic interstitial nephritis (NIN) is characterized by an interstitial infiltrate consisting of polymorphonuclear cells that leads frequently to acute graft dysfunction. In 220 graft biopsies performed because of renal dysfunction over 2 years in our unit, 11 (5%) diagnoses of NIN were made. Only two patients had chronic pyelonephritis as original disease. Four patients had urological problems before transplantation. After transplantation, five patients had urinary tract infection, one had urethral stenosis, two had vesicourethral reflux and one patient had a perinephritic abscess. Seven patients had fever (63%). Only in six patients did urine culture lead to microorganism isolation. After 6 months, only two patients had a serum creatinine level < 1.4 mg/dl, five patients had abnormal function, three had lost their grafts, and one patient had died with sepsis. We conclude that 5% of the biopsies performed in our center disclosed NIN, an entity that causes graft dysfunction and progresses frequently to chronic renal failure. In some cases, no infectious etiology could be detected.

Comparaçäo entre diagnósticos clínicos e histológicos no pós-transplante renal / Comparison between clinical and histological diagnosis in kidney post-transplantation period

Objetivo. Para determinar o acerto obtido pelos diagnósticos efetuados em uma unidade de transplante renal, foram analisados 40 episódios de disfunçao renal aguda que ocorreram no período pós-transplante. Métodos. Os pacientes foram submetidos a biópsia renal por ocasiao do episódio de insuficiência renal ao mesmo tempo em que o diagnóstico clínico era realizado pelos membros da equipe. Resultados. Foram realizados 19 diagnósticos de necrose tubular aguda (NTA), 18 de rejeiçao celular aguda (RCA), dois de rejeiçao humoral (RH) e um de defrotoxicidade (NTX) pela ciclosporina A (CyA). O diagnóstico de NTA foi confirmado pela histologia em 84,21 por cento, o de RCA, em 83,33 por cento, o de RH em 100 por cento e o único diagnóstico de NTX realizado se apresentou como NTA à biópsia. No total, a clínica foi concordante com a histologia em 82,5 por cento das vezes. Conclusao. Os autores concluíram que esxiste uma boa acurácia nos diagnósticos clínicos de RCA, NTA e RH realizados em um centro experiente em transplante renal.

Glycosuria in glomerular diseases: histopathology and clinical correlations

There are doubts about the presence of glycosuria and the progress of glomerular disease. Some reports suggest that glycosuria could be an index of a more severe tubulointerstitial lesion. We investigated the presence of glycosuria in 60 patients with primary glomerular diseases: 17 patients (28 percent) and glycosuria and 43 patients (72 percent) were glycosuria free. The two groups were similar in age, arterial pressure and sex. Serum creatinine was higher in patients with glycosuria (2.0 + 1.7 vs 1.3 + 0.9 mg/dl, P<0.05). The protein excretion rate was 7.5 + 3.7 vs 5.3 + 4.2g/day (P>0.05) in patients with and without glycosuria, respectively, while serum albumin was lower in patients with glycosuria (1.7 + 0.6 vs 2.7 + 1.0 g/dl, P<0.05). Several histological forms were present in the group with glycosuria, with membranous glomerulonephritis being the most frequent. Histological evidence of tubular atrophy and interstitial fibrosis prevailed in patients with glycosuria, suggesting a poor prognosis for these patients. We may conclude that the presence of glycosuria in patients with glomerular disease is associated with more pronounced tubular atrophy and interstitial fibrosis and therefore imply a poorer prognosis.

Fetal renal biopsies in obstructive uropathy: feasibility and clinical correlations--preliminary results.

Final assessment on the outcome of fetal obstructive uropathy is a challenging matter. Ultrasonography, fetal urine electrolytes, and beta 2 microglobulin are postulated as being useful in many cases. For cases in which renal function remains unclear, ultrasound-guided fetal kidney biopsy may be used in order to detect histologic features distinctive of renal dysplasia. We present preliminary results aimed at studying the feasibility and possible risks. Biopsies were initially performed in 11 severely malformed fetuses, three of them with associated renal abnormalities. The success rate in obtaining renal material was 63.6 per cent with no maternal complications. In the next phase of this study, ten biopsies and urine collections were performed in fetuses with bilateral obstructive uropathy. The success rate was 50 per cent with no complications. Normal fetal renal histology was seen in 80 per cent of cases. In one case, although electrolytes were normal, a histologic diagnosis of renal dysplasia was made, showing a good correlation with outcome. In conclusion, fetal kidney biopsies for obstructive uropathy are feasible and further studies are needed to show their clinical relevance and risks.

Acute bilateral renal artery occlusion due to granulomatous and necrotizing vasculitis.

A case of anuric acute renal failure due to bilateral renal artery obstruction in a 42 year-old man is presented. The obstruction was caused by bilateral thrombosis secondary to arteritis. Autopsy showed granulomatous and necrotizing vasculitis in both main renal arteries. Scarring and also necrotizing vasculitis were found from interlobular to arcuate renal arteries. The present case indicates that vasculitis should be considered when there is renal artery obstruction in young patients.

Recurrence of focal segmental glomerulosclerosis associated with Kimura's disease after kidney transplantation.

A 13-year-old Brazilian boy with Kimura's disease (eosinophylic lymphoid granuloma) and nephrotic syndrome is reported. Native kidney biopsy showed focal segmental glomerulosclerosis (FSGS). Treatment with prednisolone resulted in partial remission of proteinuria, and he had a progressive loss in renal function, requiring initiation of chronic dialysis, which he underwent for 46 months. After kidney transplantation, the patient developed proteinuria. A renal biopsy showed recurrence of focal segmental glomerulosclerosis, and subsequently he developed renal insufficiency.