Recently, dramatic breakthroughs in the management of end stage renal disease in children have improved the prognoses. However, when introducing renal replacement therapy to patients in end stage renal disease with mental retardation or other anomalous conditions, we have to carefully evaluate their level of disability and consider family compliance.
 We recently introduced hemodialysis in three adult patients in end stage renal disease with mental retardation. All cases have been received medical care in our department because of their families offer. We needed to change the treatments in line with families wishes and their mental growth during the therapeutic process. Improvements in their quality of life and clinical condition were observed after starting hemodialysis.
 In the treatment of end stage renal disease, coordination with internists, urologists, nurses, and other medical staff is very important. Therefore, to develop a positive relationship between medical staff and family is also desirable.
 It isn't easy to provide renal replacement therapy for the patients with mentalretardation. Besides, there are no quick and easy methods of rendering decisions on treatment. Factors such as the patient's clinical condition, prognosis and family compliance must be considered and communicated to family members to allow full discussion of treatment options. The decision to begin renal replacement therapy must involve patients and their families, and medical staff must also respect family decisions to alter therapy.

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 Membranous Nephropathy (MN) is classified as idiopathic or secondary, with the known causes of secondary MN including the following:infections, autoimmune diseases, drug reactions and malignancies. Idiopathic membranous nephropathy (IMN) is more frequently than secondary in Japanese children. We retrospectively reviewed 27 children (20 boys, 7 girls) with membranous nephropathy in our hospital for the period of 1985 to 2005, aged 8.1 +-3.9 years at onset. 17 patients are idiopathic membranous Nephropathy (IMN), 10 patients are hepatitis B virus related membranous nephropathy (HBMN) that is the most causes of secondary MN. In result, urinary abnormalities were detected in 16 patients through school urinary screening, and in other 11 children through chance urinary abnormalities. Six patients had nephrotic syndrome at onset. Electron microscopic staging according to the classification by Ehrenreich and Churg was performed in all patients. IMN consists of stage I in 4, II in 9, and III in 4, while HBMN consist of stage I in 3, II in 6, and III in 4. Oral corticosteroid was given to 17 patients, immunosuppressive therapy with cyclosporin was administered to only one patients. In spite of treatment with corticosteroid or not, almost IMN patients showed complete remission of proteinuria at the follow-up of 10 years, and only one patient had isolated mild hematuria. From these results, we suggest that IMN and HBMN in Japanese children might take a better course and outcome than IMN in adults and non-Japanese children.

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 This study investigated the side effects of steroid treatment on childhood IgA nephropathy.
 Forty-eight children with IgA nephropathy (IgAN) were retrospectively examined, and their heights, weights, and bone mineral densities before and 2 years after steroid treatment were compared. Mean patient age at the beginning of the treatment was 11.8y.
 The SD of height was significantly decreased from an average of 0.229±1.124 to -0.116±1.109 (p<0.05), and the obesity index was significantly increased from -0.38±14.54 to 5.94±21.81 (p<0.05). The SD of bone mineral density was decreased from -0.357±1.596 to -0.853±1.328, although the difference was not significant.
 In the group that started treatment during the growth phase, the average SD of their final height was significantly lower than that of pretreatment height. Bone densities of some patients decreased during the 2-year treatment period and were not completely recovered after the treatment.
 These findings suggest that steroid treatment could cause a high incidence of growth disturbance in children;thus, it is desirable to improve the standard treatment regimen for childhood IgAN.

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Purpose: Among the side effects of steroids, low growth is a problem of particular concern for children in the growth phase. Steroids are currently the first choice for treatment of nephrotic syndrome, but recently it has become possible to reduce or discontinue steroids in a growing number of steroid-dependent patients by combining with cyclosporine or various other immunosuppressants. At our hospital, combined use of immunosuppressants is currently adopted in cases of high steroid dependence with recurrence after repeated administration of Predonisolone (PSL) at 1 mg/kg every other day; however, we wanted to clarify whether or not this standard was appropriate from considerations of growth.
Methods: The relationship between height growth rate and total steroid dose over one year was investigated in children with nephrosis in our hospital. The subjects were 54 children (including 5 confirmed cases of FSGS) for whom we had growth records for the past year, from among the 82 children with idiopathic nephrotic syndrome under management at our hospital as of August 2008. A retrospective investigation was conducted of the relation between rate of increase in height and total steroid dose for the one year from August 1, 2007 to July 31, 2008.
Conclusion: The rate of increase in height tended to decline as the total steroid dose increased. However, the possibility was indicated that when the dose is small there is little effect on the height increase rate. At our hospital today, we consider a PSL dose of 1 mg/kg ADT (=0.5mg/kg/day) or more to be reasonable as a standard for combined use of an immunosuppressant.

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 Batter syndrome (BS) and Gitelman syndorome (GS) are genetic tubulopathy, and BS is classified into four types by genetic studies. But genetic studies are pensive and time-consuming. In addition to that, it is reported that some cases of BS type III show similar symptoms and biochemical abnormalities as GS. In this case, it is difficult to diagnosis only by symptoms and biochemical abnormalities. The diuretic loading test is useful to investigate to examine the main part of dysfunction in tubles. BS type I is caused by the dysfunction of NKCC2. NKCC2 is expressed in the assemble Henle's loop (TAL). BS type II is caused by the dysfunction of ROMK, and ROMK is also expressed in TAL. It is expected that cases with BS type I and type II have no response to furosemide loading. GS is caused by the dysfunction of NCCT, and NCCT is expressed in the part of the distal convoluted tubule (DCT). And it has already proved that cases with GS will have no response to thiazide loading. And BS type III is caused the dysfunction of ClC-Kb, and ClC-Kb is expressed in not only TAL but also DCT. It has been considered that BS type III has NKCC2 dysfunction secondary. But there are few studies that make sure this fact examining with patients with genetically defined by the diuretic loading test. We need to confirm the response to the diuretic loading in a large number of patients who are supported by genetic diagnosis, and the diuretic loading test will easily allow to diagnosis the genetic tubulopathy.

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 Imaging in children with urinary tract infections (UTIs) or prenatally detected urinary tract dilatation (hydronephrosis) is extremly important to permit identification, treatment and evaluation of the children who are at risk for renal damage. A thoughtful diagnostic strategy is necessary to proceed with appropriate management at minimal cost and morbidity to the patient. Ultrasound, voiding cystourethrography (VCUG) and radionuclide study (both static and dynamic scan) currently dominate the radiographic imaging of children with urologic problem. Ultrasound is ideally suited as a screening study and for following patients with known abnormalities because it is non-invasive, inexpensive and safe. Vesicoureteral reflux is the most common abnormality detected in imaging studies and has strong relationship with recurrent UTI. At present contrast VCUG is more frequently performed comparing to other imaging modalities such as radionuclide cystography in Japan. For morphological and functional evaluation of individual kidney, radionuclide study is useful and definitive for assessment of prenatally detected asymptomatic hydronephrosis.

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 We report two cases of idiopathic nephrotic syndrome following rh-GH treatment. Two cases were introduced to rh-GH therapy due to Turner syndrome and GH deficiency disease. Two cases present nephrotic syndrome 2 weeks and 3 years after initiation of GH treatment, respectively. Both two cases showed favorable clinical course with conventional corticosteroid treatment for nephrotic syndrome. Until now we are not recognized relapse with nephrotic syndrome in two cases. To date there is few clinical reports concerning nephrotic syndrome and rh-GH treatment, while it is suggested that GH could change a size selective filtration barrier formed by fenestrated endothelium, glomerular podocytes, and glomerular basement membrane, leading to the appearance of proteinuria. Including above mentioned results, it is clinically important to follow up these children and investigate the relationship between rh-GH and proteinuria.

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 We report a pediatric case of severe IgA nephropathy who showed rapid progression to end-stage renal failure. Since age 11 years, the patient was detected to have proteinuria and hematuria by a school urinary screening (SUS) system. However, she did not visit any clinics for further examinations. Two years later, she visited a pediatric clinic with complaints of prominent edema and oliguria. At the first visit to our hospital, the patient showed severe hypertension and renal dysfunction with massive proteinuria. Renal biopsy showed IgA nephropathy with significant numbers of sclerotic glomeruli and severe tubulointerstitial damage. Within 3 months since the first visit to us, the patient progressed to end-stage renal failure. Renal transplantation was performed following short-term dialysis. To achieve better performance of Japanese SUS system and to reduce patients with chronic kidney disease, care should be taken that further investigation will be carried out.

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 We report the case of an 11-year-old girl who developed lupus nephritis after remission of membranoproliferative glomerulonephritis (MPGN). She developed nephrotic syndrome with renal dysfunction and hypocomplementemia at 7 years old. Renal biopsy showed mesangial and endocapillary proliferation with severe lobulation and double-contoured basement membranes, indicating MPGN. Two years steroid pulse therapy and an alternate-day regimen of PSL promoted temporarily improvement of symptoms. This was also demonstrated by microscopic finding mild improvement of proliferative change. However, Proteinuria, hematuria and hypocomplementemia reappeared with appearance of butterfly rash at 11 years old. It was also noted positive antinuclear antibody, a high titer of anti-dsDNA antibody, and lupus nephritis (ISN/RPS classification class IV-G(A/C)) by renal biopsy. These findings diagnosed systemic lupus erythematosus (SLE). Steroid pulse therapy and intravenous low-dose cyclophosphamide therapy initiated to induce remission. Serial renal immunofluorescence microscopic finding revealed the shift to full-house nephropathy. Thus, this case warrants initial presentation of MPGN pattern may develop SLE later. The possibility of SLE must be considered in cases presenting with the MPGN pattern with variegated depositions of complement and immunoglobulin.

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