[in Japanese]

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Extracorporeal ultrafiltration was performed to one-year-old boy with a marked hypercoagulability in order to improve respiratory distress including pulmonary edema. Cannulation of the femoral vein and the external jugular vein with single-lumen 4.2Fr silicon rubber catheter failed to allow enough blood flow rates. Therefore, we could not help performing continuous veno-venous hemofiltration at the blood flow rate and the ultrafiltration rate of 5ml/min and 50ml/hr, respectively. We contrived effective anticoagulation consisted of an intravenous loading dose of heparin at the initiation of hemofiltration after the administration of antithrombin III, followed by constant infusions of heparin and nafamostat mesilate, and bolus administration of heparin every 4hours to maintain activated clotting times of 200seconds. The anticagulation treatment we used may be one of the tools to perform dialysis in patients with marked hypercoagulatible state.

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The pharmacokinetic study of mizoribine was conducted in 8 patients with child-onset renal diseases. These patients were divided into two groups, which were administered respectively a low dose of mizoribine (less than 4mg/kg/day, less than 2mg/kg/dose) and a high dose of mizoribine (more than 4mg/kg/day, more than 2mg/kg/dose). Renal function was normal in these patients. In the group of low dose mizoribine, mean peak blood level (Cmax) and AUC_0−∞ were 0.64±0.15mg/mL and 3.64±1.20mg•h/mL, respectively. In the group of high dose mizoribine, Cmax and AUC_0−∞ were 1.46±0.95mg/mL and 8.28±5.93mg•h/mL, respectively. It has been reported that in adult rheumatoid arthritis, Cmax and AUC obtained under the administration of 1.00±0.13mg/kg/dose were 0.59±0.22mg/mL and 4.30±1.98mg•h/mL, respectively and Cmax and AUC obtained under the administration of 1.89±0.31mg/kg/dose were 1.48±0.36mg/mL and 9.84±1.72mg•h/mL, respectively. We suggest that to obtain the same Cmax and AUC as adult patients about twice the dose of mizoribine is required, when the dose is corrected for body weight. Therefore, we should adopt a different therapy regimen of mizoribine for pediatric patients from the one for adult patient.

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We analyzed 12 children (mean age:12.3years) with steroid-dependent nephrotic syndrome (SDNS) under long-term cyclosporine (CsA) treatment who had been treated with 6 month high-dose mizoribine (MZB) treatment (10.3mg/kg/day) for the purpose of reducing the dose of CsA. Mean CsA dosage was significantly reduced from 3.94 to 1.98mg/kg/day (P<0.01) by the introduction of MZB and CsA treatment was tapered off in 4 of 12 patients. During 6 months of MZB treatment, relapse rate was approximately one-third of that for 12 months before its use, and mean prednisolone (PSL) dosage remained the same throughout the treatment. From these findings, we suggest that this high-dose MZB treatment may be an effective alternative for CsA treatment for childhood SDNS with CsA nephrotoxicity.

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β₂-agonists are commonly used for the treatment of hyperkalemia in the USA and Europe due to their potassium lowering effect. However, only one report on the use of β₂-agonists for hyperkalemia has been published in Japan. We studied the effect of an inhalation of a β₂-agonist, salbutamol, on serum potassium in 1 adult and 4 pediatric patients with acute renal failure and hyperkalemia(≥5.5mEq/L). Each patient inhaled 100μg/kg salbutamol, and blood samples were obtained after 30 min and after 4h from 2 patients and after 4 h from 3 patients. From 30min to 2h after the inhalation, sodium polystyrene sulfonate was given to 4 patients, and insulin and glucose was administered to 1 patient. In 2 patients, 30min after inhalation, serum potassium levels decreased from 6.3 to 6.0mEq/L and from 6.9 to 6.0mEq/L, respectively. The median serum potassium of the 5 patients declined significantly from 6.3mEq/L (range 5.5-7.4) to 5.2mEq/L (range 4.6-7.0) 4h aadverse effects of salbutamol were observed. Salbutamol inhalation appears to be a safe and efficacious primary treatment for hyperkalemia in children.

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We assayed concentrations of the microemulsion formulation of Cyclosporine, and estimated the pharmacokinetic parameters using concentration time profiles and correlation of the parameters with the area under the concentration curve (AUC) and coefficient of variation (CV%) in 16 children with idiopathic nephrotic syndrome. C0 and C6 were well correlated with AUC based on Pk0-12. Most cases showed the Tmax was from 1 hour to 2 hours, some cases of Cmax were indistinct and Tmax varied widely. C2 is suitable as an index for monitoring more than C0 in adult transplant patients. However our results demonstrate that C0 and C6 have good correlation compared with C2, and they may provide a useful monitoring index. Furthermore, we need to conduct further investigations because of the peculiarity of some pharmacokinetic cases

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In this report, we described the case of a nineteen years-old-man with obesity-related focal segmental glomerulosclerosis. He was suffering from hypomelanosis of Ito, mental retardation and symptomatic localization-related epilepsy. Mild proteinuria was detected during a follow-up screening when his body weight was 69.0kg at twelve years old. At sixteen years old, the following were the clinical findings body weight, 98.0kg; height, 163.0cm; body mass index, BMI 36.9kg/m³;Blood pressure, 144/88mmHg, and urinary protein level, 1.5g/day. The administration of an angiotensin-converting enzyme (ACE) inhibitor was then started. For obesity, he was given guidance on a dietary regimen, but he still gained weight. The initial renal biopsy was performed when he was eighteen years old, weighing 107.7kg, 164.0cm tall, with a BMI of 40.0, and with a urinary protein level, 3.9g/day. Renal biopsy samples showed the swelling and segmental sclerosis of glomeruli on light microscopy;therefore, he was dig obesity-related focal segmental glomerulosclerosis. He was treated with the ACE inhibitor; to decrease his weight, he underwent a dietary regimen and exercise therapy. Obesity-related focal segmental glomerulosclerosis is rarely encountered in children, but obese children with proteinuria may be predisposed to this disease.

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A case of intractable cystitis induced by Tranilast (Rizaben^®) is presented. A 3 year-old boy was referred to our hospital for recurrent bladder irritability for 1 month which had not been relieved by antibiotic therapies. He had an episode of burn injury 2 months ago and had taken the tranilast since then. Repeated urinalysis showed sterile pyuria with hematuria and ultrasound examination revealed only edema of the bladder wall. From these findings, diagnosis of tranilast induced allergic cystitis was made and its administration was discontinued. The symptoms and the abnormal findings on urinalysis improved drastically in the following week. Cystoscopic examination was not performed. We reviewed the clinical characteristics of 29 reported cases with allergic cystitis in Japanese children: half of the children take Chinese herbal medicine, anti-allergic drugs and/or xanthine derivatives for allergic diseases. Therefore, we should have high index of suspicion for allergic cystitis if the child takes these drugs.

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We reported a 14 year old female patient with SLE. We diagnosed ISN/RPS 2003 classification of lupus nephritis class IV-S(A) by renal biopsy. And then we treated her by two intravenous methylprednisolone pulses therapy followed by 20 mg per day oral prednisone, and six monthly pulses of cyclophosphamido therapy. After six month treatments, her laboratory data were improving. But she had some side-effects, moon face and hairiness, by corticosteroid and she was depressed. To our knowledge, we need to do follow-up treatment chronic disease patient at puberty with psychosomatic counseling.

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We report two patients, 21 and 22 years old respectively, with childhood-onset minimal change nephrotic syndrome showing frequent relapses. Treatment with cyclosporin-A, cyclophosphamide, mizoribine did not prevent the appearance of new relapses and both patients showed moderate nephrotoxicity induced by cyclosporin-A. The introduction of mycophenolate mofetil (1000-1500mg/day) was followed by a sustained remission in both cases, without secondary side effects. Our observation suggests that mycophenolate mofetil may be useful in maintaining remission in nephrotic patients who manifest toxicity to standard immunosuppressive agents. A prospective controlled study should be performed to evaluate the efficacy of mycophenolate mofetil in the therapy of nephrotic syndrome.

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We report a 8-year-old girl with focal segmental sclerosis confirmed by repeated real biopsy. She had microhematuria and proteinuria. The initial renal biopsy specimens showed mild focal segmental mesangial proliferation resembling minor glomerular abnormalities. Immunofluorescence studies demonstrated no deposition of IgA. She was diagnosed as non-IgA nephropathy and treated with the combined therapy of prednisolone and dipyridamole. Proteinuria was reduced but 5 years after first renal biopsy edema was developed and proteinuria was elevated. We performed the second renal biopsy 7 years after initiation of hematuria. The second biopsy showed focal segmenal sclerosis. The case indicates that some cases of focal segmental sclerosis in early phase are difficult to diagnose.

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[in Japanese]

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Purpose:To evaluate our results of anti-reflux surgery and determine the best time for surgery in infants with vesicoureteral reflux (VUR).
Materials and methods: We report on 41 consecutive patients of VUR under one year of age (male 41, female 1), who underwent open anti-reflux surgery between August 1988 and March 2005. A retrospective evaluation was performed for clinical manifestations, relation of VUR grade to renal dysfunction, indication of surgical intervention and surgical outcomes.
Results: VUR was discovered during an evaluation for a urinary tract infection in 38 of 41 patients(93%). In 6 of them a breakthrough infection occurred. In two patients it was discovered during an evaluation for anal atresia, and in one for prenatal hydronephrosis. The higher the VUR grade was(usually over grade 4), the more severe the renal dysfunction was. The success rate of anti-reflux surgery was 97% in the first 6 months and became 100% in 2 years.
Conclusion: Anti-reflux surgery should be performed as soon as possible for infants with high grade VUR(usually over grade 4) and for infants with grade 3 VUR accompanied by breakthrough infection.

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[in Japanese]

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