It is suggested that minimal change nephrotic syndrome (MCNS) is an immunomodulatory disorder, primarily involving peripheral blood lymphocytes. Most MCNS patients respond excellently to steroid therapy, while some patients show steroid resistance. The steroids act on target cells through the glucocorticoid receptor (GCR), exerting its biological effects, such as immunomodulation. Thus, we evaluated intra-nuclear GCR of peripheral blood mononuclear cells by electrophoretic mobility shift assay in children with idiopathic nephrotic syndrome (INS) to study if GCR may play a role in the pathogenesis of INS or steroid resistance. There was no marked difference in the intensity of GCR bands between INS patients and control group. On the other hand, increased intensity of GCR bands was observed after steroid therapy in INS patients. The increased intra-nuclear GCR after steroid therapy may play a role in the remission of INS.

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  Prenatal ultrasonography has revolutionized the approach of congenital anomalies of genitourinary tract. Detection of asymptomatic hydronephrosis during perinatal periods has raised new questions with regard to the need of management and the indications for surgical interventions. Most popular indications for pyeloplasty include high grade (SFU-grade 3 and 4) hydronephrosis on ultrasonography and/or obstructive washout pattern on diuretic renography or decrease of renal function. There still remains controversy whether or not pyeloplasty improves long-term renal function. Our surgical results on asymptomatic hydronephrosis suggest that, although pyeloplasty decreases ultrasonographic dilatation of hydronephrosis and improves excretory pattern of diuretic renogram, significant increase of differential renal function is expected in only one third of the involved kidneys.

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  We studied the insertion/deletion (I/D) polymorphism of the angiotensin converting enzyme (ACE) gene in 78 patients with primary vesicoureteral reflux (VUR), and examined the renal function comprised of dimercaptosuccinate (DMSA) renoscintigraphy and diethylenetriaminepentaacetic acid (DTPA) renogram in each genotype.
  Patients were classified in three genotypes according to the ACE gene I⁄ D polymorphisms: 32 in II genotype, 36 in ID and 10 in DD. The incidence of presumably congenital unilateral small kidneys were high in DD patients (70%). Glomerular filtration rate (GFR) obtained from DTPA renogram was 120.7±35.7ml/min (expressed as mean±SD) in II genotype, 111.7±33.3 in ID and 88.0±18.0 in DD. Quantitative DMSA tracer total uptake of both kidneys was also low in patients with the D allele.
  This study shows that the D allele of ACE gene is closely related with congenital small kidneys with refluxing ureters in patients with primary VUR, and in accordance with previous reports, this allele also relates to the progression of reflux nephropathy.

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  The cellular lesion is considered as one of the morphological variants of primary FSGS. The pathology of the cellular lesion is characterized by swollen, vacuolated, and proliferating glomerular epithelial cells throughout Bowman's space. In this review, pathological significance of the cellular lesion and clinical features and outcome in pediatric patients with the cellular lesion of primary FSFS were briefly summarized.

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  We reported a girl (14 years) with minimal change nephrotic syndrome who showed frequent reccurence during menstruation. The patient was first diagnosed as nephrotic syndrome at 4 years old. Thereafter, she experiensed reccurence of this syndrome frequently. Since she had steroid-dependent nephrotic syndrome, cyclosporin had been administreted to maintain a complete remission since 1996. Even though she was given a cyclosporin therapy, nephrotic syndrome was reccurent again during her first menstruation, but it disappeared spontaneously at the end of menstruation period. Thereafter, frequent relapse had been noticed concomitantly with her menstruation. Our observation may suggest the menstruation is one of the triggering factor for the reccurence of nephrotic syndrome.

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  We reported a case of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis which was seemed to be valuable to know the initial stage.
  A fifteen year-old-boy was pointed out proteinuria and hematuria by school urinalysis at seven years old. Abnormal urinalysis was disappeared when he was nine years old, but was pointed out again by school urinalysis when he was thirteen years old in April 1999. He was referred to our hospital. The first renal biopsy specimen showed segmental sclerosis in one of five glomeruli. Anti-platelet therapy was started. However, proteinuria increased and the levels of creatinine in serum elevated rapidly since April 2000. The second renal biopsy was performed in May 2000, because of elevated serum MPO-ANCA levels (304EU). Fibro-cellular crescent were present in seven of nine glomeruli in this specimen. Therefore, he was diagnosed as crescentic glomerulonephritis of the pauci-immune type. The levels of MPO-ANCA and creatinine in serum decreased after the methylprednisolone pulse therapy and subsequent oral prednisolone therapy was performed. The urinalysis was improved.
  In a pupil discovered proteinuria and hematuria by school urinalysis, we should measure ANCA positively.

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  We reported a case of Henoch-Schönlein purpura in child with severe and rare complications. 9-year-old girl was refered to our hospital because of purpura and arthritis on lower extremities. After admission, she had several severe and rare hemorragic complications. At first, she had bleeding of upper and lower gastro-intestinal tract and severe subcutaneous edema involving face, head and upper extremities. Next, she complained dyspnea becase of bilateral hemothorax. At last, many renal-ureteral stones with hydronephrosis were detected and nephritis progressed to nephrotic syndrome. Fourtunatly, these complications were improved by conservative treatments containing corticosteroid therapy. The histopathologic changes on renal biopsy was very mild, presentig no crescent formation. At present, about 2 years after admission, she enjoys her life without limitation. Her renal function is normal, renal-ureteral stones and proteinuria are disappeared, and only light microhematuria is remained.

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  We reported a girl followed for 29 years and biopsied four times. Her clinical symptoms were macrohematuria and nephrotic syndrome in 1972. Because of only light microscopy at first biopsy in 1976 she was not diagnosed as DDD. She was diagnosed as it with hypocomplemetemia by second biopsy in 1981 showed mesangialproliferation, double tracks, C3 granular stain and very strong intralaminal deposits. Then she was treated by methyprednisolone pulse therapy, prednisolone, cyclophophamide, dipyridamole and γ-globurine. Fourth Biopsy in 1992 showed decrease of mesangialproliferation and C3 stain. Intralaminal deposits were “dropping-off” and new membranes appeared. She has no symptoms and her urinalysis is normal from 1992.

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  A eleven-years old girl was detected as asymptomatic hematuria and proteinuria at the mass urine screening for school children. Biopsied renal pathology was IgA nephropathy showing diffuse mesangial proliferative glomerulonephritis on light microscopy. She received the following diffrent three therapies every two months; first therapy: combined therapy of intravenous methylpredonisolon pulse and heparin, second therapy: combined therapy of oral predonisolon, mizolibin and wafarin, third therapy: combined therapy of oral predonisolon, cyclosporin A and warfarin. The effect of reduced proteinuria was evaluated in these three different therapies using the ratio of proteinuria at the first morning urine⁄ urine creatinine and 24-hour urine protein excretion. A rapid decrease of proteinuria was obtained in the last therapy as compared to the other two therapies. Our results suggest in our patients that the decrease of proteinuria is due to the other effect of cyclosporin A against the hyperpermeability of protein in the glomerulus in addition to immunosppressive effect.

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  We report a case of Kawasaki disease in eighty six day-old-boy with grosshematuria and proteinuria after repeated treatment with a single 2g/kg dose of intravenous gamma globulin. He had no signs of renal insufficiency before repeated treatment of intravenous gamma globulin. Grosshematuria and proteinuria developed shortly after this treatment. Grosshematuria, proteinuria and microscopic hematuria disappeared 3 days, 2 weeks and 3 months after the treatment, respectively. Leukocyturia and mild proteinuria may be present in patients with Kawasaki disease, but grosshematuria and proteinuria has been rarely reported. There was no evidence of nephrotic syndrome on physical and blood examinations.We conclude that intravenous gamma globulin therapy in this patient with Kawasaki disease might have some effects on developing this renal symptoms.

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  Xanthogranulomatous pyelonephritis (XPN) is an atypical form of choronic pyelonephritis that predominantly affects middle-aged women and is rarely seen in children.
  We have recently seen 3 children with XPN (2 diffuse and 1 focal type) and report them with their radiological features.
  Although all of them had shown both clinical and laboratory findings similar to acute pyelonephritis, protracted course such as prolonged fever more than 2 weeks despite intravenous antibiotic therapy were observed. After 2 weeks from the onset of the disease, ultrasonography (US) of the abdomen revealed hypoechoic renal masses in all cases. Common renal masses in children, such as acute focal bacterial nephritis (AFBN) and Wilms tumor were considered as presumptive diagnoses. For differential diagnosis, diverse radiological examinations were helpful as following; (1) A CT delineates a tumor as a homogenous round low density mass in XPN while it shows wedge-shaped low density mass in AFBN and heterogenous low density mass in Wilms tumor. The finding of “rim-enhancement” by contrast-medium was only seen in XPN. (2) While the renal mass was visualized as high signal intensity in both XPN and Wilms tumor by MRI (T2-WI), it was seen as low signal intensity mass in AFBN. (3) As affected kidney in XPN has no function, DMSA reno-scintigraphy demonstrates complete defect of isotope in the affected kidney. In contrast, migration defect of the isotope was only seen in tumor lesions in AFBN and in Wilms tumor.
  Total nephrectomy was curable in two cases of diffuse type and resection of tumor was done in one focal type; the postoperative courses were uncomplicated.
  In conclusion, the diagnosis of XPN should be considered even in children if protracted clinical course is observed in acute pyelonephritis. While repeated US examinations might detect the renal masses, combined radiological examinations such as CT, MRI, and DMSA reno-scintigraphy would be helpful for the definite diagnosis of XPN.

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  Here we report a boy's case of idiopathic membranous nephropathy freshly found by screening urinalysis at school. Recently, some children with the disease have been found in the same way, although the disease itself is not so common at school age. The diagnosis was made by serological tests and renal histology. We started the treatment with dypiridamol, heparin, and predonisolone (60mg/m²/day), since the patient met the criteria of nephrotic syndrome. The amount of urinary protein began reducing in two weeks and returned to within normal range in four months. Second biopsy at the ninth month revealed that the glomerular lesion, evaluated by Ehrenreich & Churg's criteria for electron microscopic findings, progressed to stage IV from stage II. In adult cases, it usually takes about 1 or 2 years to progress to stage IV from stage I or II, and about 4 or 5 years to become normal. In this case, predonisolone may have hastened the recovery of glomerular basement membrane, although the histological findings are not yet normal. Concerning the risk of relapse, tapering and termination of predonisolone therapy should be carefully considered. Generally speaking, the prognosis of this disease in childhood is better than that in adulthood. Some patients show remission in natural course, but others show nephrotic syndrome or chronic renal failure. That's why the treatment of this disease has not yet been standardized. Therefore, we consider it is necessary to accumulate case reports including clinical course and histology, and try to do controlled clinical study in pediatric patients.

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  We reported a 14-year-old boy revealed poststreptococcal acute glomerulonephritis (PSAGN) with acute renal failure (ARF). He suffered from proteinuria (4.3g/day), hematuria, hypertention (190/110mmHg), edema, oliguria and erysipelas. The laboratory data showed the elevation of BUN (36.7mg/dl), creatinine (2.72mg/dl), and K (5.45mEq/L). The titer of ASO and ASK were elevated, 894 IU/ml and × 20480, respectively. CH50 and C3 serum levels were decreased. The bacterial cultures including blood, throat swab and skin were all negative. ARF due to overhydration, overexercise by judo on the acute phase, was improved by the restriction of salt and water intake, bed rest and treatment with furosemide and penicillin-G. Renal biopsy showed proliferative endocapillary glomerulonephritis on light microscopy, and fine granular pattern of C3 was detected by immunofluorescent studies. Electron microscopic findings showed “humps”, splitting of basement membrane and “apoptosis”. We consider that apoptosis play an important role in recovery process of PSAGN.

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