Anti-immunosuppressive drugs including cyclosporineA and cyclophosphamide have been used for the management of frequency relapse nephrotic syndrome (FRNS). However, there is the limitation of the usage of such agents because of the cytotoxic advers effects. Moreover long-time use of the same immunosuppressive agent may induce the tachyphylaxis. The aim of this study is to determine the efficacy and safety of mizoribine, a relatively newly developed immunosuppressive agent in Japan for the management of FRNS. Single divided of 5 mg/kg of mizoribin (max. 150 mg/day) was given to the patients at morning and serum concentration of this drug was measured 2 hours after administration. 22 patients with FRNS (relapse:9 and remission 13) were enrolled in this study. Their mean age (years)/mean body weight(kg)/mean mizoribine dose(mg/kg/day)/mean mizoribine serum concentration (μg/ml) was 17.1/49.5/3.23/1.56 and 9.8/27.9/4.63/2.93, respectively. This study may suggested that mizoribine is effective for the suppression of the relapse in patients with FRNS, especially in children below than 10 years and 30kg of body weight.

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Cyclosporin (emulsion formulation) was administered before a meal (twice a day) to six males with steroid dependent nephrotic syndrome (five MCNS, one FSGS). As the target value of C₂ (concentration at two hour after drug administration), 400-600ng/ml had seemed a goal setting enough for the maintenance of remission. After altering from “twice a day” to “once a day” administration, two cases had relapsed, whereas, there was a patient who had maintained remission with 300ng/ml (or lower) of C₂. For two cases who entered the discontinuance period, “every other day” administration was attempted. One had relapsed one month later and the other has maintained remission with twice a week administration. It is presumed that the dependency for the cyclosporin, just as well as the steroid, is different in each case. So, it is expected that using the cyclosporin at long term or more becomes possible for patients who can set target of value low or who can expand dose interval.

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We evaluated the effect of initial steroid therapy according to ISKDC regimen for idiopathic nephritic syndrome. 19 patients with idiopathic nephrotic syndrome have been treated with steroid for the last 8 years. Four patients out of these 19 patients, whom we experienced recently, relapsed during the period of 1.3mg/kg alternate day prednisolone therapy. Three out of these 4 patients were under 3 years of age. The responses to steroid therapy were not faverable, and they need long time to get remission. Steroid was administered intravenously, because oral therapy was not effective. After relapse, in all four patients, the dosage of steroid were raised to initial daily therapy. After inducing remission, cyclosporine was added when initial daily therapy was changed to alternate day therapy. Then the dosage of steroid was tapered, and finaly stopped. The remission has been maintained with cyclosporine therapy alone. We have to investigate how to treat these childhood patients who were prone to steroid dependency.

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We report a 16-year-old girl with Frasier syndrome, who had suffered from steroid resistant nephrotic syndrome and developed end-stage renal failure (ESRF). In examining her primary amenorrhea, sex reversal (46XY) and a mutation in the WT1 gene were revealed. Since no signs of breasts enlargement at 13-year-old in Japanese girls is considered as one of the first manifestations of the delayed puberty, endcrinological examinations as well as genetic analysis, such as the WT1 gene, should be performed even in patients with ESRF manifesting delayed puberty.

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We report a case of end-stage renal disease(ESRD) with a colostomy. The patient is a male infant who was born with an imperforate anus, tetralogy of Fallot, duodenal stenosis, sacral defect, and bilateral hypo/dysplastic kidneys. He had a sigmoid colostomy in the left abdomen and became ESRD during the newborn period. To reduce the incidence of catheter-related infectious complications, peritoneal dialysis(PD) catheter was placed on day 11 of life, with its exit site located over the right chest wall. We drew upon the technique described by Twardoski et al. PD was performed successfully with no infectious complications. The chest wall PD catheter is safe and useful in infants with colostomy.

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We report a child case of IgA nephropathy associated with Takayasu's arteritis. A 12 year old girl tested positive to both microscopic hematuria and proteinuria after a school urinary screening program. Renal biopsy further revealed a case of mild IgA nephropathy. After the biopsy, the patient showed signs of intermittent pyrexia and had a high level of CRP. Pulsatile swelling of the left side of the neck was noted about seven weeks later, and ultrasound revealed an irregular widening of the left internal carotid artery. CT and MRI angiography also disclosed arterial lesions leading to the diagnosis of Takayasu's arteritis. Previously, we have not found any child case reports of IgA nephropathy associated with Takayasu's arteritis. The patient had an HLA locus, DQ4, which is frequently detected in patients with IgA nephropathy, but didn't have the loci associated with Takayasu's arteritis.

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We report a one-year-old girl with hemolytic uremic syndrome caused by enterohemorrhagic E. coli O157 infection and cerebral infarction. She had been transferred to our hospital due to bloody diarrhea,coma and oliguria in the eighth day. The hemolytic anemia, thrombocytopenia and acute renal failure developed, and the hemolytic uremic syndrome was diagnosed. In the tenth day, because of the progression of acute renal failure, peritoneal dialysis was applied. At the same day a right hemiconvulsion suddenly developed, followed by left hemiplegia. The head MR study revealed the right cerebral lacunar infarction. Her symptoms had resolved by the conservative therapy. We should do careful management so that the patient who has suffered from hemolytic uremic syndrome is not overhydration or dehydration.

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Galloway-Mowat syndrome (GMS) is characterized by congenital microcephaly, developmental delay, often hiatus hernia, and nephrotic syndrome. Most patients die before the age of six years by renal failure, infection and other complications. From the pathological point of view, this syndrome has been considered as a disorder of glomerular basement membrane formation and function. We describe two sibling GMS patients. The elder patient (case 1) who had microcephaly, developmental delay and nephrotic syndrome, died of renal failure at 8 years old. His renal biopsy specimens showed minimal change. The younger brother (case 2) showed microcephaly, developmental delay and mild proteinuria with normal renal function. Renal biopsy was performed at 15 years old; one of twenty glomeruli showed segmental sclerosis on light microscopy. Electron microscopy revealed some abnormal lysosomes in the podocytes. Renal biopsy specimens from case 1 and case 2 did not show typical findings of GMS. Case 1 died of renal failure at 8 years old, however, case 2 was alive at 18 years old. Recently, this syndrome is acknowledged to have a wide spectrum of clinical course and pathological findings. So we propose that they may be affected with mild or atypical form of GMS.

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[in Japanese]

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