[in Japanese]

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  Reactive oxygen species (ROS) including O₂^·- produced by NADPH oxidase system have recently been recognized as cell signaling molecule in addition to classical mediator for tissue injury. We recently detected the expression of gp 91 phox in rat mesangioproliferative glomerulonephritis (GN) induced by injection of anti-Thy-1 monoclonal antibody 1-22-3 to uninephrectomized rats. Immunohistochemical study showed that gp 91 phox expression by mesangial cells (MCs) and ED-1-positive macrophages was increased during the course of GN, particularly more prominent when the number of total glomerular cells, PCNA-positive cells, and α-SMA intensity in glomeruli were markedly increased. The expression by podocytes was constitutively enhanced in GN. In contrast, the expression of interstitial gp 91 phox, which was predominantly espressed by macrophage, was increased in parallel with the numver of interstitial PCNA-positive cells and the degree of interstitial fibrosis during the late phase of GN. In situ O₂^·- and H₂O₂ detection assays showed that intracellular O₂^·- production was enhanced in MCs and macrophages during GN, while massive H₂O₂ was detected in infiltrating inflammatory cells. These results suggest that low level generation of ROS might act for MC activation in GN. On the other hand, considerable amount of ROS might play a role in glomerular and interstitial injury. Therefore, gp 91 phox-containing NADPH oxidase and ROS appear to be involved in the MC activation, glomerular injury, and the development of renal interstitial fibrosis.

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  We studied management and social acitivity of 46 patients (≤ 15 years of age on Dec 31 st, 1998) with chronic renal failure under medical treatment by councilors of Chugoku-Shikoku Society of Pediatric Nephrology. Sixteen patients were under conservative managment, 22 patients were undergoing dialysis (PD 21 and HD 1), and 8 patients received renal transplantation. Dietary cure was carried out in 14 patients with conservative mangament and 14 patients under dialyis. Drugs mainly given were vitamin D, alkalinizing medicine, growth hormone and erythropoietin in patients with conservative treatment. Phosphate binder, vitamin D, erythropoietin and growth hormone were given to the majority of patients under dialysis and immunosuppressnats were used in patients treated with renal transplantation. Among conservative group restriction of exercise was carried out in 5 patients. Howevre, no differences in patient's age and GFR between patietns treated with exercie limitaions and those treated without exercise limitations were found. Three years later 6 of 16 patients who had initially received conservative treatment were switched to dialysis and 8 of 22 patietns undergoing dialysis received renal allografts indicating encouraging to transplantation. Social activity including school adjustment was apparently fair. However, psychological problems of these patients remained to be investigated.

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  A9-year-old girl with acute renal failure associated with pyelonephritis caused by Salmonella O9 is reported. She started to have fever, left flank pain and gross hematuria and referred to us for further examination and treatment. Urine culture was positive for Salmonella O9 and leukocyturia was found. A diagnosis of left pyelonephritis was made. She also contracted acute renal failure (ARF) without oligouria which improved by conservative treatment. Urological examinations showed a vesicouretheral reflux (VUR) at the left ureter and a stenosis of extra urethral orifice. After urethrotomy VUR disappeared. A transient elevation of fractional excretion of sodium and prolonged proteinuria after recovery of ARF indicated a presence of renal causes as well as pre-renal causes. It is well known that Salmonella enterocolitis is occasionally complicated by pre-renal ARF. However, ARF induced by Salmonella infection without severe diarrhea is quite rare and ARF caused by acute pyelonephritis is also uncommon. We suppose that severe inflammation and/or diclofenac might be the risk factors of ARF in our patient.

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  We assessed the scores of disease-related stressors and stress responses in 59 adolescent patients with chronic renal diseases. Based on the characteristics or severity of disorders, patients were divided into 4 groups, such as nephrotic syndrome with frequent or infrequent recurrence, and chronic nephritis with or without receiving corticosteroid. Patients were requested to complete the appraisal scales of stressors for chronic renal diseases and stress response scales for adolescents. Both scores of disease-related stressors and stress responses were lower in chronic nephritis patients without corticosteroid administration than the other groups of patients. Therefore, the relationship between disease-related stressors and stress responses was analyzed among patients with nephrotic syndrome and those with chronic nephritis receiving corticosteroid. Multiple regression analysis showed that stressors, “disease-related daily discomfort” and “disease-related anxiety for future”, caused all of the stress responses, bad temper, apathy, depression, and physical distress. In patients with nephrotic syndrome some stress responses were particularly correlated with disease-related stressors. These results suggest that the management considering disease-related stressors and stress responses is necessary to reduce the psychological stress in adolescent patients with chronic renal diseases.

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  deroofing procedure is performed as a surgical treatment of tunnel infection and cuff infection in patients on chronic peritoneal dialysis using double-cuff Tenckhoff catheter.
  Our study assessed the usefulness of deroofing procedure in comparison of time interval until the onset of post-surgery tunnel infection, or catheter survival time, between those who underwent catheter replacement after tunnel infection and those who underwent deroofing surgery.
  The study included 36 patients (25 males aged 0.3 to 18.8 years and 11 females aged 0.4 to 20.4 years) who underwent catheter replacement or deroofing procedure for tunnel infection in the observation period from March 1, 1991 to May 1, 2001. These patients were divided into those who were operated on by deroofing (n=29; 35 catheters: deroofing group) and those who underwent catheter replacement surgery (n=19; 32 catheters: replacement group).
  For the purpose of evaluating the usefulness of deroofing procedure and investigating whether, or not, the time interval until post-surgery tunnel infection, or catheter survival time, was compared between the deroofing group and the replacement group, as well as between the detected causatives in the deroofing group.
  There was no significant inter-group difference with regard to catheter survival time (p=0.1464). The incidence of recurrence was no more than 11.4% in the deroofing group. The incidence of peritonitis due to post-surgery tunnel infection in the replacement group was 6.2% and 11.4% in the deroofing group (p=0.2). There was no significant variation in the time interval until postderoofing tunnel infection due to gram-negative rods or due to other causatives in the deroofing group.
  From the above results, deroofing procedure may be a procedure worth being considered as a treatment of first choice for tunnel infection.

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  We present an infant with idiopathic hypercalciuria (IH) who demonstrated gross hematuria due to microlithiasis in the renal pelvis. Although the patient was referred to us for evaluation of recurrent attack of gross hematuria, examinations including biochemistry, endocrinology, radiological survey for urinary tract revealed no abnormal findings except hypercalciuria in urinalysis. An abdominal computed tomography (CT), however, showed 2 pieces of urolithiasises (4 mm in diameter, each) in the right renal pelvis. From these findings, the diagnosis of IH complicated by microlithiasis was made.
  IH is one of the most common causes of gross hematuria in children: the incidence is reported to be 3∼4% and 35% in asymptomatic children and children with hematuria, respectively. Taken together, IH should be considered as a possible cause for gross hematuria even in infants. For detection of microlithiasis (diameter less than <5 mm), a plain CT for abdomen would be worthwhile.

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  We report a two-year-old boy who had undergone extirpation out of capsula lentis at 6 months of age because of his congenital catalact. On admission for examination of his mental retardation, his blood examination data showed elevated levels of serum alkaliphosphatase and creatine phosphokinase and high chloride iso-anion gap metabolic acidosis. His urine chemistry data showed hypercalcinuria, pan amino aciduria, and low molecular weitht proteinuria. A wrist X-ray examination showed the typical bone change of rickets on the distal side of the ulna. Although ^99mTc-DMSA scintigraphy revealed no morphologically abnormal accumulation, total accumulation of radioisotope in the kidneys were poor. We diagnosed the patient as having typical Lowe syndrome and started him on alkali treatment. No phosphatidylinositol 4,5-bisphosphate 5-phosphatase activity was detectable in his fibroblast. We identified A to C mutation at base 1783 in exon 15 of OCRL-1 cDNA (GenBank M 88162). This mutation was predicted to cause an amino acid change from serine to arginine. The predicted amino acid change was located in well conserved motifs of the human inositol polyphosphate 5-phosphatase molecule. Therefore, it was speculated that the predicted change of the amino acid sequence would greatly influence the activity of phosphatidylinositol 4,5-bisphosphate 5-phosphatase.

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  A 3 year-old girl was referred to our hospital for chance proteinuria. Proteinuria and microhematuria were positive. Blood examination revealed hypocomplementemia, positive anti-nuclear antibody, and positive anti-double stranded DNA antibody. Anti-streptolysin O was negative. She was diagnosed as lupus nephritis. Since she became febrile after hospitalization, initial renal biopsy was postponed. She was treated with four courses of methylprednisolone pulse therapy. Serum complements raised to normal after the third course of therapy. The renal biopsy performed after the forth pulse therapy demonstrated membranous glomerulonephritis with mild mesangial change (WHO classification of lupus nephritis: class Vb). Her proteinuria disappeared four months after treatment. She is in remission for three years. Low-dose oral prednisolone is still necessary as its reduction causes hypocomplementemia.

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  Recently, number of foreign residents is increasing in Japan; especially many Chinese are in the northeast part of Japan. We sometimes have troubles with foreign residents at medical consultation because we do not have sufficient information on their cultural and social context. We have difficulties not only in the treatment of acute and self-limited diseases, but also have more serious troubles in the treatment of chronic and/or life threatening diseases. It is very difficult for domestic doctors to take care of the patients by themselves.
  We had a 16-year-old Chinese boy who suffered from nephrotic syndrome before his migration. Against our medical advice, he refused medical examination, hospitalization and treatment, and took personally imported Chinese herb. Following four years, he fell into chronic renal failure. We mentioned about the medical problems of the patient. In addition, we discussed about three problems at the medical management for foreign residents based on our experience, (1) personal problems of the patient, (2) problems of the medical institution, and (3) problems of the social support system.

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  This report describes an 8-year-old girl with crescentic IgA nephropathy. Her father has also suffered from renal insufficiency due to IgA nephropathy and been treated with hemodialysis. Marked proteinuria and hematuria was pointed out by a follow-up screening. She was referred to our hospital for further evaluation of proteinuria and hematuria with red blood casts. First renal biopsy showed crescentic IgA nephropathy with mesangial cell proliferation and mesangial deposition of IgA. Intensive treatment including methylprednisolone pulse therapy and combination with oral prednisolone and cyclophosphamide ameliorated active pathological findings such as cellular crescents and mesangial cell proliferation as indicated by 2nd biopsy. Deterioration of renal function was not observed during the course of treatment. Proteinuria was reduced and disappeared by intensive treatment and combination therapy of angiotensin-converting enzyme inhibitor and angiotensin receptor blocker for 7 months. In conclusion, early start of intensive treatment is very important for the reverse of active pathological lesion of crescentic IgA nephropathy.

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  This report describes a case of relapsing Candida peritonitis complicating chronic peritoneal dialysis. The patient was a 16-year-old boy with cerebro-oculo-hepato-renal syndrome who was receiving nocturnal intermittent peritoneal dialysis for chronic renal failure. Fungal peritonitis with Candida albicans developed due to breakage of a connection tube. Since the patient was mentally retarded and unable to lie quietly, the dialysis catheter was replaced and antifungal medication was administered intraperitoneally, without changing to hemodialysis. Peritonitis subsequently improved rapidly, but relapsed twice within a short interval. The catheter was therefore removed and the patient maintained on hemodialysis with concomitant intravenous administration of antifungal agents for 4 weeks. A new dialysis catheter was then re-implanted. No new episodes of peritonitis developed after this treatment. The benefits of prophylactic antifungal treatment and early catheter removal should be elucidated in patients with fungal peritonitis receiving chronic peritoneal dialysis.

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  Therapeutic drug monitoring (TDM) is essential to maintain the efficacy of immunosuppressant drugs while minimizing their toxicity. The area under the concentration-time curve over the first 4 hours post-dose (AUC_0-4) has been reported to be useful for accurate TDM. However, AUC_0-4 is difficult for out-patients due to collecting blood five times. The estimated AUC 0-4 is method of estimate the AUC_0-4 with gathering blood three time-point. We examined the utility of estimated AUC 0-4 in pediatric kidney transplantation patients. The AUC_0-4 correlated better with estimated AUC_0-4 (cyclosporin: r2=0.874, tacrolimus: r2=0.967) than with concentration measurement 2 hour after administration (r2=0.648, 0.695). The estimated AUC_0-4 was very useful TDM for pediatric kidney transplantation patients.
  We also examined optimal starting dose of immunosuppressant drugs. The optimal dose of cyclosporine was 11 mg per kg regardless of patient's body weight. On the other hand, concerning tacrorimus, patients with lower body weight required a higher dosage.

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  Augmented carbonyl and oxidative stress may contribute to vascular complications in diabetes. The aim of the present study was to investigate whether carbonyl and oxidative stress is augmented in young patients with type 1 diabetes at early clinical stages of the disease. Urine samples of 38 patients with type 1 diabetes (mean age [±SD]: 12.8±4.5 years, diabetes duration: 5.7±4.3 years) and those of 60 age-matched healthy controls were assayed for advanced glycation end products (AGEs), pentosidine and pyrraline, and markers of oxidative stress, 8-hydroxy-2'-deoxyguanosine (8-OHdG) and acrolein-lysine. Of these four markers, urinary concentrations of pentosidine, 8-OHdG and acrolein-lysine were significantly higher in the diabetic patients than in the healthy controls. For the patient group, pentosidine correlated significantly with pyrraline, 8-OHdG and acrolein-lysine, and pyrraline correlated significantly with acrolein-lysine. Urinary pentosidine, 8-OHdG and acrolein-lysine, but not pyrraline, correlated significantly with urinary albumin excretion. Patients with microalbuminuria (≥15mg/g Cr) showed significantly higher levels of all the four markers than did normoalbuminuric patients and control subjects. The present study indicates that augmentation of carbonyl stress, which is closely linked to oxidative stress, and resultant endothelial dysfunction may start early in the course of type 1 diabetes.

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