A 14-year-old girl with myohemoglobinuria caused by “Kendo” exercise, Japanese fencing, was evaluated. The causes of myohemoglobinuria were intravascular hemolysis by “Fumikomi”, the motion of repeating strenuous steps barefoot, and exertional rhabdomyolysis. During an exercise test, the concentration of urinary β2-microgloburin increased and creatinine clearance decreased transiently. It was supposed that the failure of glomerular and tubular function was caused by hemoglobin and myoglobin. Temporal renal dysfunction did not occur when a supporter was used as a cushion for the foot during exercise. Although severe renal damage induced by exertional myoglobinuria and/or hemoglobinuria caused by “Kendo” training has not been reported, the usage of a foot supporter should be considered in order to prevent the exposure to nephrotoxic substances (i. e. hemoglobin and/or myoglobin) when this phenomenon is repeatedly observed.

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  We analyzed the questionnaires from 84 Japanese pediatric nephrologists to clarify the issues in the training of pediatric nephrologists and postgraduate education for them in Japan. Over 90% of them believed that specialized knowledges and skills for renal diagnosis and treatment in children are essential. 80% of them appreciated the training programs they received in Japan. However, 79.4% of them have a claim for establishing and distributing the training hospitals with pediatric nephrology unit throughout Japan, 71.8% for accrediting the board of pediatric nephrologists, and 95% for promoting the postgraduate educationprograms. Established pediatric nephrology units offer a full range of renal diagnostic facilities and facilities for treatment of acute and chronic renal failure in children. They are also provided with pediatric urologist, pediatric surgeon, transplant surgeon, pathologist and psychologist to consult and collaborate with the majority of other pediatric disciplines. We find it necessary that the Japanese pediatric nephrology association will have recommended training programs for Japanese pediatric nephrologists and set out postgraduate education programs for them in the near future.

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  We investigated 17 growth-retarded children with chronic renal failure (CRF) who had a structural abnormality as a primary renal disease. Seven patients with CRF were treated with recombinant human growth hormone (rhGH), and the mean standardized height of these patients were improved from -3.5±0.3 at baseline to -2.6±1.5 (P<0.05). The maximal growth velocity occurred during the first year of rhGH treatment. Six children were treated with rhGH for more than five years, and 4 of these children reached a height within the normal range (within 2 SD of normal height). In contrast, the mean standardized height of the untreated 10 children was 3.6±1.1 SD below normal, and 7 of these children had reached an adult height below the normal range. The mean calculated Ccr decreased from 31.1±16.5ml/min/1.73m² at baseline to 22.5±22.5ml/min/1.73m² at five years in long-term treated patients, which would be consistent with the natural history of CRF in children. The clinically significant adverse events potentially related to rhGH were absent. We conclude that long-term rhGH treatment is very effective in improving retarded growth in children with CRF..

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  Glomerulocystic kidney disease (GCKD) is an uncommon type of renal cystic disease characterized by cystic dilatation of Bowman's space and atrofy of the glomerular tuft. We report a 15-year-old boy who had asymptomatic choronic stable renal failure (CCr: 66.3-70.7ml/min/1.73m²). His renal biopsy specimens showed cystic dilatation of Bowman's space and atrofy of the glomerular tuft. His kidneys were small (right: 8.1×3.5cm, left: 7.8×3.9cm) without any other malformation of the urinary tract. His 42-year-old mother had small kidneys and asymptomatic chronic renal failure (CCr: 57.8ml/min/1.73m²). As our patients' findings satisfied the cliteria defined by Rizzoni et. al., we diagnosed this case as familial hypoplastic GCKD. This is the first report of familial hypoplastic GCKD in a Japanese family.

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  In July 1997, an outbreak of food poisoning due to the pathogenic Escherichia Coli (E. Coli) O-157 occurred in Sakai, Japan. We analyzed the longitudinal urine abnormalities of the E. Coli O-157 infected patients without hemolytic uremic syndrome. The 400 patients who were achieved urinary examination within one month after the onset are analyzed. Among the cases who were detected hematuria more than 1± within one month, 20% and 16.3% patients showed hematuria after one and two years later, respectively. Similarly, 25.5% and 16.2% patients showed proteinuria after one and two years later, respectively. These results suggested that, E. Coli O-157 infection might cause the long term urine abnormalities even without occurrence of hemolytic uremic syndrome.

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Backgroued: Evaluation of renal pelvis using ultrasound technique (US) is essential to detect congenital urinary tract anomalies, such as hydronephrosis. While the dilatation in renal pelvis has long been assessed by the maximum diameter in renal central echo complex (CEC-system) using US, SFU-grading system introduced by the Society for Fetal Urology in 1988 has recently been widely applied for this purpose.
Aims: This study was conducted to confirm whether the assessment of the dilatation in renal pelvis using US by SFU-grading is more objective than that by CEC-system.
Methods & Results: Thirty-one kidneys of 17 children (male 15, female 2, mean age 21 month old) with congenital pelviureteral junction obstruction were evaluated ultrasonographically by medical students and pediatric nephrologists. The dilatation in renal pelvis in each kidney was assessed both by CEC-system and SFU-grading. Results disclosed the US examination of the dilatation in renal pelvis assessed by SFU-grading agreed well with that assessed by CEC-system. The rate of agreement of the grade assessed by SFU-grading between medical students and pediatric nephrologists was higher than that assessed by CEC-system (74.2% and 71.0%, respectively). In addition, the agreement within one grade in SFU-grading was also superior to that in CEC-system (100% and 97%, respectively).
Conclusion: US evaluation of renal pelvis should be changed from CEC-system to SFU-grading because of its good reproducibility.

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  The report of clinical overview of UTI in children is rare in Japan. Single center experience with regard to culture-documented UTI in children is analized for searching future diretction of optimal care.
  Between Jan. 1995 and Dec. 1999, 427 children with culture-documented UTI are reviewed retrospectively about age, sex, symptoms, causative organisms, and subsequent radiographic studies.
  Characteristic findings include nonspecifics synptoms (apnea, grunting, and cyanosis) common in neonates, lack of pyuria in 21%, Eshericia coli as a most common agent (73.8%), vesicoureteric reflux (VUR) in 31%, and distinct correlation inbetween the degree of VUR and renal scarring.
  Diagnosis of UTI must be confirmed by the culture of clean-catched urine and radiographic study is to be followed consecutively. A controlled, prospective multicenter study for management of primary VUR is readily needed.

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  Transtubular potassium concentration gradient (TTKG) is a useful index of the excretion of potassium in the urine and renal aldosterone bio-activity in the cortical collecting ducts (CCD). Some reports have discussed with respect to TTKG in infant and children, but there have been no previous reports which have evaluated the relation between TTKG and plasma aldosterone concentration (PAC) in neonates. Our objective was to investigate the relation between TTKG and PAC in neonates. We studied TTKG and PAC in premature neonates (n=10) and full-term neonates (n=30 admitted to our hospital from April 1993 to March 1996. In full-term neonates TTKG gradually reached highest level from day 14 to month 1 of age, and reached to the level in adult from 3 to 5 months of age. PAC reached its highest in day 3, and was tendency to decrease gradually after day 3. In premature-neonates TTKG was significantly (p<0.001) higher in day 14 than in day 3, 7, respectively. PAC was high level continuously during 2 weeks of age. Low level TTKG in early neonates shows low sensitivity for renal aldosterone activity, suggests immature function of CCD. Therefore, in infant over 6 months of age TTKG is an easy and sensitive method for estimation of renal aldosteron bio-activity and useful index of function in the CCD.

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  Cyclosporine (CyA) is a immunosuppressive agent which has been used to a large number of children with nephrotic syndrome and transplantation. Therapeutic monitoring of this drug is essential because it has a narrow therapeutic window and inter-and intra-patient variability.
  The new micro-emulsion pre-concentrate (MEPC) drug reduce variability, and therapeutic drug monitoring and target level of its concentration are reconsidered. A trough concentration (C0) has been commonly used for drug monitoring. However, in cases of renal transplantation, some reports suggest that the concentration of CyA 2hr after dosing (C2) or C3 correlates with area under the blood concentration curve. Our results also suggest that C2 monitoring may be a better method in frequently relapsing children with steroid-sensitive nephrotic syndrome. Our observation should be extent to a large population study.

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  Blood purification therapy, such as plasmapheresis (mainly plasma exchange) and continuous hemodiafiltration has become a very important part in critical care of pediatric as well as adult patients, and is indicated for improvement of serious conditions in various diseases.
  From April 2000, we, pediatric nephrologists, have performed blood purification therapy for 14 pediatric patients, including four patients with systemic inflamatory response syndrome, three with acute exacerbation in autoimmune disease, two with severe hepatic failure, and a patient with acute exacerbation in chronic renal failure, a patient with near drowning, a patient with hemolytic uremic syndrome, a patient with thrombotic thrombocytopenic purpura, or a recipient of ABO incompatible live related liver transplantation.
  Our experience as above suggests that blood purification on extracorporeal circulation can be safely performed and effective even for pediatric patients whose weight is below 10kg. A suitable vascular access, reasonable priming of extracorporeal circuit, and careful monitoring of vital signs are mandatory for successful blood purification therapy in children.
  However, it is very stressful for a limited number of staffs to provide the all-day maintenance of extracorporeal circulation and continuous monitoring of patients. ICU setting will be necessary and urgent for staffs and patients who need such critical care medicine.

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  A two-month old male with fever was treated with antipyretic drugs, acetaminophen and mefenamic acid at a general clinic in March 2001. After receiving the drugs, right hemiconvulsion and drowsiness were appeared, going into shock soon afterwars. Multi organ dysfunction syndrome occurred and treated by plasma exchange. The damaged organs recovered in several days. Only minor neurological disturbance was remained. As a result of this child's response to antipyretic drugs, we concluded that caution should be used when considering the use of these drugs for newborns and infants.

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  We report a case of secondary amylodosis in a girl who had been treated for systemic type of juvenile rheumatoid arthritis since she was 3.9 years old. She had intestinal amylodosis and renal amyloidosis when she was 12.2 years old and 13.5 years old. Intestinal amyloidosis was improved with increased corticosteroid, but renal amylodosis exacerbated after the control of JRA. She showed massive proteinuria, microscopic hematuria, and hypertension. Renal biopsy revealed the accumulation of amyloid protein to the glomeruli, tubulointerstitium, and vascular wall. There was mild fibrosis in the interstitium. With the use of the angiotensin converting enzyme inhibitor, massive proteinuria was decreased, and she became normotensive. However, because the increased level of her serum creatinine, together with the finding of the renal biopsy, it was thought that the renal prognosis would be poor.

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  During past 10 years, 357 children with several renal diseases were hospitalized for examination at our institution. Of these, 14 children progressed to chronic renal failure (CRF) and/or endstage renal disease (ESRD). Four of the 14 children were found via urine mass screening system for school children. In order to see whether the urine mass screening was of benefit to the 4 children (GroupA), we evaluated serum creatinine levels at presentation and duration from presentation to ESRD between them and the other 10 children (Group B). There was no significant deference tetween 2 groups. However, 2 children in Group A achieved improved quality of life under close
observation thereafter, suggesting efficacy of the urine mass screening system in these children.

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  One Japanese girl with microscopic hematuria discovered by mass screening urinalysis was diagosed as having mild IgA nephropathy. One year after the diagnosis she noted macroscopic hematuria without acute illness. Follow-up renal biopsy specimen showed crescentic glomerulonephritis. Clinical characteristics of the patients with early exacerbation of IgA nephropathy in the literature, similar with our reported case, are early teenaged at diagnosis and relatively short duration for progression.
Careful observation must be needed in children with IgA nephropathy, especially during one or two years after diagnosis.

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  We did a nation-wide survey on patients less than 20 years of age with pediatric chronic end-stage renal disease (ESRD) in Japan for the year 1999. Patients newly introduced to renal replacement therapy in 1999 were 102 (male/female:67/35) and the incidence rate was 4 per million population (aged 0-19 years) per year. The major disease causing ESRD was renal hypoplasia/dysplasia (24.5%). Peritoneal dialysis was used more frequently than hemodialysis under 15 years of age (78-89% and 26%). Transplant rate for the year 1999 was 7 per 100 dialysis patient-years (aged 0-19years) and all was living kidney donor. There were no deaths. Transplant rate for the year 1999 of patients newly introduced to renal replacement therapy in 1998, was 10 per 100 dialysis patient-years (aged 0-19 years) and all was living kidney donor, and the death rate was 22 per 1000 dialysis patient-years (aged 0-19 years). The number of ESRD patients already on treatment before 1999 were 628 (male/female: 363/265). 41% were on peritoneal dialysis, 17% on hemodialysis, and 41% on renal transplantation in as of January 1, 1999. The major causes of ESRD were cystic/hereditary/congenital renal diseases as new patients. The number of renal transplant was 53 (living/cadaveric: 49/4), with a transplant rate was 11 per 100 dialysis patients-year (aged 0-19 years), living kidney donors was 10, and the death rate was 9 per 1000 dialysis patient-years (aged 0-19 years) of all ESRD patients (730) for the year 1999. Key words: chronic end-stage renal failure, children, Japan.

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