Respect for others is the essence of humanism. Medical caregivers should treat all patients and their family with respect and regard for their worth and dignity. Honesty is the highest standards of behavior for medical caregivers or researchers. Withholding medical information from patients and their family without their knowledge or consent is ethically unacceptable. We should give them all information that we could know in many situations to obtain their informed consent so that they could make fair decisions. What kind of medical care to receive is a matter to be determined by patients themselves, because medicine has been resisting against the laws of nature historically. Therefore, communication is crucial to provide the best care for patients. Similarly, in medical research, dishonesty adversely affects investigators, clinicians, patients and their family in various situations. We must be aware that the goal of research is to develop new findings not for researchers nor for research itself but only for the patients. We have to accomplish faithful medical practice and clinical research with honesty.

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IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide both in children and adults. Japanese annual school urinary screening program can determine the onset time of IgAN within one year, and enables us to conduct clinical trials in patients with IgAN early during the course of disease. Based on the results of clinical trials, the Japanese Society for Pediatric Nephrology has developed “Guidelines for the treatment of childhood IgAN.” In these guidelines, the disease severity has been divided into two categories, i.e., mild and severe IgAN, and according to the severity, treatments were proposed. Treatment of children with severe IgAN showing diffuse mesangial proliferation with prednisolone, azathioprine, heparin-warfarin, and dipyridamole for 2 years early in the course of disease prevents immunologic renal injury and progression of the disease, and not only ameliorates the activity of the acute phase of nephritis but also improves the long-term outcome of severe childhood IgAN. Lisinopril for 2 years is recommended for mild cases with IgAN. As to the role of tonsillectomy in the treatment of IgAN employed mainly in adults, at present studies provide conflicting data. Therefore, it cannot be recommended for widespread use for the treatment of IgAN patients, especially for children with IgAN.

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Recently, it has been shown that a deficiency in the IgA1 hinge region sugar chain participates in the onset of IgA nephropathy. Activated B cells produce IgA1 carbohydrate chain deficient immune complex upon antigen stimulation and this is deposited in mesangial cells, activating complements, macrophages, and mesangial cells and resulting in inflammation. Treatment, stratified according to disease severity, is chosen to control these immunoresponses in patients. Multidrug therapy based on a combination of steroids, immunosuppressants and anticoagulant/fibrinolytic agents has been are used for severe cases and is shown to improve prognosis. On the other hand, tonsillectomy plus steroid pulse therapy does not require the use of immunosuppressants and has been found to be as effective as multidrug therapy when used in primary care, and has also been shown to be effective for steroid-resistant or recurrent cases as well as cases of recurrence after renal transplantation. In future, to establish a treatment that minimizes side effects in consideration of the developmental stage of the patient, it is necessary to undertake multicenter prospective comparative trials using multidrug therapy and tonsillectomy plus steroid pulse therapy.

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The management and treatment of Henoch-Schönlein purpura nephritis (HSPN) is still controversial. Some patients spontaneously resolve themselves without any treatment, on the other hand, some patients developed end-stage kidney disease, therefore, more cases have recently been treated aggressively, such as with immunodepressants. We strictly made treatment decision based on both clinical and pathological severities. Here we retrospectively examined the efficacy of treatment in HSPN. Severe group defined as more than ISKDC grade III or serum albumin less than 2.5 g/dl, received multiple combination therapy, and Moderately-severe group defined as less than ISKDC grade III or serum albumin more than 2.5 g/dl received angiotensin converting enzyme inhibitor (ACE-I) or angiotensin II receptor blocker (ARB). All patients resolved proteinuria without renal dysfunction during the observation periods. Our findings suggested it was desirable that the treatment strategy of HSPN should depend on both pathological and clinical severity. It is additionally necessary to emphasize that ACE-I/ARB was sufficient to treat with moderately-severe HSPN. However, in general, there is little evidence to show that ACE-I/ARB is the effective treatment of HSPN. And in some cases, ACE-I/ARB failed to decrease proteinuria. To understand the limitation of ACE-I/ARB therapy, we should make the best use of ACE-I/ARB for HSPN.

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Onset of acute kidney injury (AKI) in nephrotic syndrome (NS) with severe edema, namely NSAKI, is often experienced. Investigation of 4 cases of NSAKI of whom we recently experienced, revealed the clinical condition seemed to be caused by kidney ischemia with kidney parenchymal edema. Increased intravascular volume occurs as a result of the renin angiotensin system activated by kidney ischemia. Therefore, there is an unbalanced clinical condition where kidney ischemia and intravascular overflow water are present at the same time.

Acute tubular necrosis caused by NSAKI may be reversible, because the pathogenesis is partial ischemia even if kidney dysfunction has continued for several months.

Therefore, it is important to reduce kidney parenchymal edema in the treatment of NSAKI by albumin intravenous infusion and the combination of diuretics.

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Nearly 6.4% of children, 6 to 15 years of age, bother nocturnal enuresis, which is a second leading common chronic disorder next to allergies. Treatment for nocturnal enuresis is recommended for the children at the age of six or later. For the patients who are still enuretic after lifestyle advices, the active treatment is recommended. This comprises of desmopressin and alarm therapy or both. Other treatment including anti-cholinergics or tricyclic anti-depressants are options. We need to remind that tricyclic anti-depressants are cardiotoxic when used overdose. Japanese Society on Enuresis recently published the Treatment Guideline for Enuresis, which is the revised version of the one first come out in 2004. In this review article, we mention mainly the up-to-date treatment of monosymptomatic nocturnal enuresis followed by the new guideline.

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With the diagnosis of upper UTI, a radiographic workup is needed to identify which patients are susceptible to renal damage. However, there is incomplete agreement as to whether the emphasis should be on the presence of scarring versus the presence of vesicoureteral reflux (VUR). Historically, the study most commonly used to detect high risk patients was a voiding cystourethrography (VCUG). Collectively, this is now referred to as the bottom-up approach. This method relies on VCUG to identify lower urinary tract abnormalities and VUR. Patients diagnosed with VUR may undergo a ^99mTc-DMSA renal scan at a later date to assess scarring. Alternatively, the top-down approach targets the kidney with a ^99mTc-DMSA renal scan to diagnose acute renal parenchymal involvement at the time of the upper UTI. Patients with photon defects are subsequently referred for a VCUG to assess VUR in addition to a late ^99mTc-DMSA renal scan (6 months) to assess for permanent scarring. As each strategy carries advantages and disadvantages, it is difficult to declare the winner. Meanwhile, new approaches to diagnose renal scarring without invasive procedures, such as magnetic resonance urography or novel biomarkers are under investigation. They may help to clarify the interplay between VUR, renal scarring, and bladder and bowel dysfunction in the future.

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Glomerulopathy with fibronectin deposits (GFND) is a rare autosomal dominant disease showing chronic and progressive hereditary nephritis caused by mutations of fibronectin 1 gene (FN1) that encodes fibronectin. It is characterized by proteinuria, microscopic hematuria, hypertention and massive FN deposits in the mesangium and subendothelial space which lead to end-stage renal failure. Its pathogenesis has not been revealed. Anti-Fibronectin antibody immunostaining is helpful for diagnosis of GFND.

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Acute focal bacterial nephritis (AFBN), reported by Rosenfield et al., has many points of clinical deference between the pyelonephritis. Based on clinical features, we were investigated about mid-term outcomes and risk of the AFBN. Fever was found in all patients, pyuria was not observed in the four patients and urine culture was negative in three patients. Renal enlargement in four patients and tumor in 1 patient which was known as characteristics of AFBN, were pointed out by ultrasound sonography. 5/8 patients had a vesicourethral reflux, 4/5 patients had low renal uptake of ^99mTc-DMSA. Break through infection (BTI) was developed in two patients who had Bladder and bowel dysfunction (BBD), Hutch diverticulum and posterior urethral stricture. It was suggested to become risk to recurrent AFBN. In this examination, the contrast-enhanced CT was recognized as test necessary for a diagnosis because of ratio of false-negative in the ultrasound Sonography for AFBN was high with 45%. In the past reports, urinary tract abnormality and BBD were seen with 4–67% and BTI were seen with 13–67%. We concluded AFBN was good outcomes in the mid-term for reasons of 9/11 patients had no BTI and all patients had no renal dysfunction during three years.

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The 50–80% of tuberous sclerosis complex (as following, TSC) patients have kidney complications and it sometimes come to suffer from renal insufficiency. We retrospectively examined 95 TSC patients with renal complications at our hospital from April 2002 to March 2010 and found five patients (three males) with end-stage renal failure under renal replacement therapy at the age of 17–28 years old. These five patients were diagnosed as renal angiomyolipoma and two patients also diagnosed as polycystic kidneys. About their ADL, one patient was self-reliance, three patients were mental retardation, and one patient was bedridden. Three patients fell to end-stage renal failure due to bilateral nephrectomy for intra-tumor bleeding and another two patients decreased renal function slowly. Kidney transplantation, peritoneal dialysis and hemodialysis were performed in two, two and one patients, respectively. Patients with TSC should be followed with careful examination about kidney complications and adequate intervention for angiomyolipoma and their intra-tumor bleeding if necessary.

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Cyclosporine A (CsA) is an effective steroid-sparing agent for patients with steroid-dependent nephrotic syndrome (SDNS). In order to evaluate the efficacy and safety of once-daily CsA administration, we retrospectively analyzed 23 patients with SDNS due to minimal change disease. During 2-year one-daily CsA administration (mean dose, 2.6 mg/kg/day) , regression to SDNS-free survival probability and relapse-free survival probability was 65% and 35%, respectively. One patient had chronic CsA nephrotoxicity. Therefore, follow-up renal biopsy should be performed to detect CsA nephrotoxicity after the 2-year one-daily CsA administration. This study indicates that the 2-year once-daily CsA as well as twice-daily administration may be effective treatment for patients with SDNS.

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In Japan, children with idiopathic nephrotic syndrome usually have to be hospitalized for more than four weeks for initial treatment. However, there are no reports on the appropriate length of hospitalization. We retrospectively examined the medical records of 37 children with an initial episode of idiopathic nephrotic syndrome were discharged in less than four weeks. We analyzed the side effect of steroid, including hypertension and ocular hypertension, and body weight change. The median age was four years (range, 1–14 years), median days until remission was seven days (range, 4–15 days) and median length of stay was 16 days (range, 10–25 days). Only one patient needed antihypertensive agent for high blood pressure during hospitalization. 11 patients showed ocular hypertension and 3 of them showed after discharge, and continued treatment at the outpatient department. None of the patients was re-hospitalized because of complications. After 8 weeks of treatment, their body weight gained about 10 percent than the premorbid. We found that the length of initial treatment of idiopathic nephrotic syndrome can be less than four weeks if the patient can manage medicine, diet and infection and be frequently followed up at the outpatient department.

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We report a case of a 10-year-old girl with coexistence of anti-glomerular basement membrane antibody nephritis and myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). She initially complained of vomiting and loss of appetite. Serum creatinine (sCr) was at 2.80 mg/dl at the admission, and was elevated to 6.64 mg/dl 12 days later. Kidney biopsy revealed 90% sclerotic lesions and 67% fibrocellular crescentic glomeruli, as well as linear IgG depositions along the glomerular basement membrane (GBM). We diagnosed the patient as rapid progressive glomerular nephritis(RPGN) with anti-GBM glomerulonephritis. We opted for aggressive treatment because of the high percentage of cellular crescents. She received eight rounds of plasma exchange (PE) and six pulses of methylprednisolone (15 mg/kg) therapy, followed by prednisolone (1 mg/kg/day) and cyclophosphamide (2 mg/kg/day). Renal function slightly improved (sCr 2.0 mg/dl); however, it deteriorated after Pneumocystis jirovecci infection. Peritoneal dialysis could be avoided for four months. Her residual renal function was maintained for over 12 months with continuous ambulatory peritoneal dialysis performed twice daily. We should consider aggressive treatment with PE therapy, steroid pulse therapy and immunosuppressive therapy for anti-glomerular basement membrane antibody nephritis. Additionally, the possibility of infectious diseases should be noted.

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The comorbidity of non-infectious inflammatory nephropathy with horseshoe kidney is very rare. We herein report a case of an adolescent male with horseshoe kidney complicated by lupus nephritis. A 13-year-old boy was referred for an evaluation of proteinuria and hematuria. The onset of symptoms, which included fatigue, pallor, and anorexia, followed by fever occurred 2 months earlier. He was diagnosed with systemic lupus erythematosus based on physical examination and laboratory findings on admission. Abdominal ultrasonography and computed tomography showed horseshoe kidney and hydronephrosis in the left kidney. Computed tomography angiography with three-dimensional reconstruction (3D-CT) was performed in order to establish a safe puncture region and the best approach for renal biopsy. Retroperitoneal laparoscopic biopsy was performed without any complications such as massive hemorrhage. The histological diagnosis was lupus nephritis of ISN/RPS class IV-S. After induction therapies including intravenous methylprednisolone, intravenous cyclophosphamide, and oral prednisolone, he achieved remission. Thereafter, he has successfully remained in remission with combination therapy of mycophenolate mofetil and a small dose of prednisolone. This case was highlighted because preoperative 3D-CT of renal vessels provided very useful information for safely performing renal biopsy.

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