We introduced peritoneal dialysis (PD) to a 10 year old girl with MELAS, who had been suffering from end-stage renal disease. When she became critically ill with congestive heart failure due to renal dysfunction, hemodiafiltration using bicarbonate-buffered dialysate was performed. PD was consecutively started after the informed consent was obtained from her parents. Her systemic circulation improved almost completely with these procedures. Although we wondered about aggravation of mitochondrial respitatory status by loading lactate-buffered dialysate for a long period of time, unusual rise of lactate and pyruvate in her blood has not been observed at all. Her clinical condition is becoming fairly well after the continuance of PD for one year and nine months. The family has no trouble with manipulation of PD at home, and visits our outpatient department regularly.

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Acute renal failure is not an uncommon complication following stem cell transplantation (SCT) and requires blood purification therapy (BPT) to obtain space for transfusion and replace renal function. Twenty-three pediatric patients received allogeneic SCT in a recent 3-year-period at our hospital. Of these, we reviewed the records of 4 patients who underwent BPT for acute renal failure following SCT. The age of the patients was 8 years (7-12 years, median and range). The patients had ≥10% fluid overload after 30days (7-46days) of SCT. Days to BPT after SCT were 40days (34-80days), and BPT was performed for 6days (1-11days). During the procedure, 2.4L (1.2-39L) of fluids were removed and 2.4L (1.7-7.5L) of fluids were transfused. In all patients, hemoglobin and platelet count increased, and serum urea nitrogen and creatinine decreased. However, all patients died from multiorgan failure without termination of BPT. Although BPT for acute renal failure was useful in pediatric SCT patients to provid space for transfusion and replace renal function, aggressive management of fluid overload and earlier initiation of BPT for these patients were considered necessary to improve the outcome.

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We report on a 3 year old girl with idiopathic membranous nephropathy (IMN) who was found by mass screening program for renal disease and achieved complete remission by the combination therapy of angiotensin-converting enzyme inhibitor (ACE-I) and angiotensin receptor blocker (ARB). As IMN in Japanese children was reported to have favorable outcomes, we suggest that these patients with heavy proteinuria may be initially treated with inhibitor of the renin-angiotensin system prior to commencement of steroids.

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Contrast-induced nephropathy(CN) has clinically become an important cause of iatrogenic acute renal failure. Especially baseline renal dysfunction and diabetes mellitus increase the occurrence of CN. We report on a 10 year old girl with systemic-onset juvenile idiopathic arthritis in whom CN developing acute renal failure occurred without preexisting renal dysfunction. She accompanied sepsis, DIC and endotoxicemia. We suggest that even if baseline serum creatinine level is within normal range, contrast administration in cases of suspected renal impairment due to sepsis, DIC and endotoxicemia should be strictly limited.

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Serological response to influenza vaccine was studied in children with several renal diseases who received low- to middle-dose prednisolone combined with or without immunosuppressive agents. A total of 10 patients, aged 6-15 years who received low-dose prednisolone (0.2-0.3mg/kg per day): 7 with prednisolone alone and 3 with prednisolone combined with an immunosuppressive agent received '01-'02 or '02-'03 influenza vaccine. Eleven children without renal diseases in whom immunosuppressive therapy was not done, aged 2-16 years were served as control. Moreover, additional 4 patients, aged 5-14 years who received middle-dose prednisolone (0.6-1.0mg/kg every other day) were vaccinated with a '03-'04 influenza vaccine. The changes of serum antibody titers to influenza virus A and B before- and after- vaccination were evaluated. Serum titer of antibody to influenza A after vaccination significantly increased in all the study patients who received low-dose prednisolone combined with or without immunosuppressive agents. There was no significant difference between the study patients and the control. In the 4 patients who received middle-dose prednisolone, serum titer of antibody to influenza A also significantly increased following the vaccination. However, a poor response to influenza B was observed in all the study patients. No adverse reaction caused by vaccination was documented. Based on our results, enough serum antibody titer to influenza A could be obtained following the vaccination, even in children with renal diseases undergone immunosuppressive therapy. Influenza vaccine may be safe and effective for children with renal disease.

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We report here three patients with epilepsy who developed urolithiasis during zonisamide(ZNS) treatment. A one-year-old girl had periodic spasms and right single kidney. In addition to ZNS treatment, she received ACTH therapy. The patient developed urolithiasis with resultant acute renal failure and macrohematuria during ACTH therapy. The second patient was 14-year-old girl who had received steroid therapy for mixed connective tissue disease. Four months after the initiation of ZNS therapy, the patient developed urolithiasis. The third patient, who had benzodiazepines treatment for insomnia, suffered with urinary lithiasis during ZNS treatment. The urinalysis of the three patients revealed alkaline urine or hypercalciuria. Although their urinary lithiasis was resolved by discontinuation of ZNS and supportive therapy, routine examination of urine parameters such as pH and sediments, and ultrasonography are thought to be necessary during treatment with ZNS.

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Steroid-resistant nephrotic syndrome is sometimes unresponsive to not only oral prednisolone therapy, but also steroid-pulse therapy or various immunosuppressants. We report a case of steroid-resistant nephrotic syndrome who treated with the combination of intermittent steroid pulse therapy and oral cyclophosphamide successfully although she was resistant to either steroid pulse mono-therapy nor the combination of oral prednisolone and cyclosporine or cyclophosphamide. A three-years-old girl with nephrotic syndrome was refractory to oral prednisolone therapy of two weeks. Subsequent methyl-prednisolone pulse therapy of three weeks reduced her proteinuria but could not introduce into remission. Renal biopsy indicated minor glomerular abnormalities and there was no sclerotic lesion. On the immuno-fluorescence study, no deposition of immunoglobulin and complements was detected. Since her proteinuria increased and edema became severe after the pulse therapy, she required the intravenous supplementation of albumin almost every day. Her proteinuria was resistant also to cyclosporine and subsequent oral cyclophosphamide with oral predonisolone. So we tried the combination of methyl-prednisolone pulse therapy and oral cyclophosphamide. This combined therapy decreased her proteinuria, but did not introduced into the remission. Two weeks after the combined pulse therapy, thrice per week of steroid pulse therapy were performed again alternate weekly three times. During this intermittent steroid pulse therapy, her proteinuria had become negative. After the alternate weekly methyl-prednisolone pulse therapy, she is treated with the pulse therapy once a week at our out-patient clinic. Some authors reported the effectiveness of the combination of methyl-prednisolone pulse therapy and immunosuppressants such as cyclophosphamide or cyclosporine for the patients with focal segmental glomerular sclerosis, although international study of kidney disease in children denied the benefit of cyclophosphamide. In present case, even during steroid pulse therapy of three weeks with cyclophosphamide, proteinuria was reduced but not disappeared, and subsequent intermittent steroid pulse therapy had dissipated her proteinuria. We consider that the combined therapy of intermittent steroid pulse and cyclophosphamide is useful for the treatment of nephrotic syndrome resistant to various immunosupressive therapy.

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Renal lymphangiectasia is a very rare disorder in cystic renal disease, which occurs in childhood. We described a 1 year old boy with bilateral renal lymphangiectasia and polycythemia. Laboratory data were normal except for an elevated serum erythropoietin level. Abdominal ultrasound examinations showed high echogenicity and mild enlargement of the bilateral kidneys. Renal function was normal. To our knowledge, no previous case of renal lymphangiectasia associated with polycythemia has been reported.

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We reported a female patient with steroid resistant nephrotic syndrome of early onset (1 year) and rapid progression to end stage renal disease with mutation in the Wilms'tumor suppressor gene WT1. Analysis of WT1 in this patient presents the so-called hot spot mutation of exon9 (R 394 W). Which has been reported in many patients with Denys-Drash syndrome. It is suggested that she is incomplete Denys-Drash syndrome. It is proposed that patients with rapidly progressive nephrotic syndrome should be analysed of WT1 mutations. We think that we have to consider careful observation and preventive kidney extraction with renal transplant simultaneously when having confirmed mutation of WT1 because a risk of Wilms'tumor outbreak is high.

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I treated a patient of HSPN by CyA and Triple Blocker for Renin-Angiotensine-Aldosterone System. This Patient did not improve by Cocktail Therapy with Cyclophosphamide, Pulse Therapy with Steroid and Plasm Excange. With this treatment, this patient improved at proteinuria. She improved not only proteinuria but also Historogic change. So our treatment is good at Heavy HSPN.

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A 5 year old boy with chronic renal insufficiency was admitted for the purpose of undergoing operation for CAPD catheter retention and introduction of CAPD. He had been explained by his parents about hospitalization and operation. After admission, fever and mental instability Considered due to his insufficient psycnological preparedness were noticed, so the operation was put off. As such, a picture book on the CAPD introduction in which characters he likes appear was made before operation. He was explained about the CAPD introduction by this book. Using this book, the child was able to concretely image and mentally prepare himself about the operation and CAPD introduction which had been an unknown experience and a source of fear to him. Apprehensions of his parents were also mitigated.

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[in Japanese]

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