In a study of 408 samples obtained from 137 children with renal disease, the protein/creatinine ratio (P/Cr) in a single early morning urine sample correlated closely with total daily protein excreation (Up). The linear regression analysis of Up(y) and P/Cr(x) was highly significant (r=0.822). The regression equation was y=0.57x+0.2. This equation was suitable when values of x were one and over. While analyzing 183 samples in which values of x were under 0.5, we obtained another regression equation that was y=0.78x+0.04 (r=0.71). It was suitable when values of x were under one.
  Furthermore the change of the creatinine consentration in a single urine sample and using steroid hormone influenced the regression equation.

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  We report a 5-year-old girl with complete C9 deficiency who developed membranous nephropathy. She presented with proteinuria (0.4-1.0g/day) and hematuria (RBC 15-20/HPF), while neither edema nor hypertension were noted. Laboratory data showed decreased CH 50 value: analysis of each complement component revealed complete deficiency of C9. The titer of ASO and ASK, and blood chemistry data were within normal range. Both anti nuclear antibody and anti DNA antibody were negative. The renal biopsy specimen showed membranous nephropathy (stage I). This is the first report of idiopathic membranous nephropathy in a patient with congenital C9 deficiency.

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  Although Sjoegren's syndrome (SjS) is occasionally complicated with tubulointerstitial nephritis as one of the extraglandular manifestations, the association of crescentic glomerulonephritis is not common. We report here a female patient with SjS complicated with myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies positive rapidly progressive glomerulonephritis. Renal biopsy revealed necrotizing and crescentic glomerulonephritis in addition to tubulointerstitial nephritis. She developed progressive renal failure, but this was improved by steroid therapy including methylpredonisolone pulse therapy.

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  To clarify the pathogenesis of male SLE, we compared clinical manifestation of male SLE with those of female SLE. We enrolled 23 patients who had been diagnosed with SLE. These patients were divided into two groups, 6 patients (Group A) were male and 17 (Group B) were female. The causative agents, clinical features, laboratory data, renal pathological findings, treatment and prognosis were investigated for the two groups. Proteinuria at onset was found in six patients (100%) of group A and in four patients (23.3%) of B (p<0.05). Eighty % of male-SLE patients and 76.5% of female showed type III or more of WHO classification. Patients with cardiac and pulmonary disorder were less seen in group A than in B. Clinical remission occurred in 4 patients (66.7%) of group A and in one patient (5.9%) of B (P<0.05). The relapse rate was higher in group B than in A (P<0.05). These findings suggest that male SLE children may have few complications except renal disorder and were good therapy-response, though male-SLE as same as female SLE have severe pathological findings.

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  Methylpredonisolone pulse therapy has been used widely on various diseases and pediatric kidney diseases. Renal blood flow analysis was done on 7 nephrotic patients and 3 lupus nephritis patients who were prescribed methylpredonisolone pulse therapy.
  On nephrotic patients, renal blood flow velocity was increased after methylpredonisolone pulse therapy. On the other hand, renal blood flow velocity was not changed remarkably on lupus nephritis patients. 14 year-old girl with lupus nephritis who had no response for methylpredonisolone pulse therapy, and renal blood flow velocity was decreased.
  This study shows that renal blood flow analysis is a useful examination for the efficacy of methylpredonisolone pulse therapy.

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  We report a case of congenital nephrogenic diabetes insipidus (CNDI) with a mutation in the vasopressin V2-receptor gene (AVPR2), found by failure to thrive. He was a-2-month-old infant, and admitted to our hospital because of failure to thrive. On admission, investigation showed hypernatremic and hypertonic dehydration with a serum sodium 161 mEq/L, chrolide 125 mEq/L, and osmolality 336 mOsm/L, whereas urinalysis revealed dilute urine with a urine specific gravity 1.005, osmolality 98 mOsm/L. Further examinations revealed the abnormal elevation of plasma arginine vasopressin level to 78.2 pg/ml. In spite of dehydration, urine was dilute and its volume amounted to about 350ml a day (1,600ml/m² a day), so we suspected the nephrogenic diabetes insipidus. Intravenous fluid infusion was given to correct hypernatremic and hypertonic dehydration. Nevertheless rehydration, it was difficult to decrease the serum sodium level and restore the free water loss. Since the initially given intravenous fluid was containing 90mEq/L Na, so we gradually decreased the concentration of Na in fluids to decline serum sodium level. But it was unable to decline serum sodium level, because the urine output increased along with infusion, and urine volume amounted to 4,300ml/m² a day. It was necessary to increase infusion exceeding urinary water loss for the decrease in serum sodium level and the restoration of free water loss. Hypernatremia and dehydration were corrected by the large amount of intravenous fluid infusion, finally, its water quotient amounted to about 300ml/kg a day and containing 30mEq/L Na. We suggested that the failure to correct hypernatremia and hypertonic dehydration was mainly caused by inappropriate fluid infusion, which was inadequate to supplement free water and increased urinary volume, rather than decreasing the concentration of Na in fluids. We reflected that, considering the patient had diabetes insipidus, it was significant to increase quantity of fluid infusion corresponding to urinary water loss, and to correct hypernatremic dehydration within 48 to 72 hours.

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  Acute focal bacterial nephritis (AFBN) refers to renal inflammatory disease without liquefaction. We report six cases of AFBN in which hyperleukocytosis and an elevated level of C-reactive protein were evident. However only three of the patients demonstrated abnormal findings upon initial urinalysis. Ultrasonography was useful for diagnosis of AFBN. The main findings were a focal hyperechoic area and a hypovascular area upon color doppler ultrasonography. The focal area showed low density with enhanced CT. Both enhanced CT and ultrasonography are indispensable for diagnosis of AFBN. We recommend that ultrasonography be performed for patients with fever of unknown origin.

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  Focal segmental glomerulosclerosis (FSGS) is steroid-resistant, and not sensitive to various immunosuppressive drugs as well. We report here a 12-year-old boy with drug-resistant nephrotic syndrome. He was treated with prednisolone (PSL) at the dosage of 2mg/kg/day at first, but his nephrosis continued. Then we tried methyl-prednisolone pulse therapy and many other drugs including cyclosporin (CyA), mizoribine, HMG-CoA reductase inhibitor, but the nephrotic syndrome was resistant to these drug therapies. Although the administration of CyA in addition to steroids reduced proteinuria temporarily, we forced to reduce the dosage of CyA, for leukopenia, a rare side effect of CyA, occurred. So we tried the treatment of LDL apheresis (LDL-A) combined with the drug therapy. It is well accepted that the administration of CyA and/or steroids during or immediately after LDL-A treatment can take a better effect due to the remission of hyperlipidemia by LDL-A. However, in this case, we tried to restart the administration of steroid two months after LDL-A therapy, the proteinuria became sensitive to steroid therapy. Since serum lipid increased again at that time point, we therefore speculated that it is not the remission of hyperlipidemia which leads to the effect of steroid sensitivity after LDL-A therapy. Furthermore, no relapse has been observed during a follow-up period of 3-month till now. Although the precise mechanism of LDL-A is still uncovered, it can be conjectured that apheresis of unknown substances except cholesterol or the down-regulation of hyperlipidemia might improve steroid sensitivity. In conclusion, LDL-A might be useful for ameliorating drug-resistant nephrotic syndrome.

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