180 children with Kawasaki disease were admitted to Kyoto City hospital from 1980 to 1996. We compared 78 patients whose serum sodium was below 135mEq/l and 102 patients whose serum sodium was above 135mEq/l. There was no significant difference in two groups with age, sex and the febrile period, but the occurrence of diarrhea was more frequent and the number of cases treated with gamma-globulin was significantly larger in the group with hyponatremia. Laboratory findings on admission showed that the neutrophil count, the hemoglobin, the total serum protein and CRP were significantly different between the two groups. Coronary artery lesions were recognized in 21 cases (27.3%) in the group with hyponatremia, 14 cases (14.4%) in the group without hyponatremia. There was the significant difference. One patient with the most severe hyponatremia had SIADH (Syndrome of Inappropriate Secretion of Antidiuretic Hormone).
  Hyponatremia in Kawasaki disease is not rare and appear in the case with sever inflammation. Moreover we suggest that hyponatremia is a sign which predicts coronary artery lesions.

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  We describe the clinicopathological characteristics of a patient with acute tubulointerstitial nephritis and uveitis syndrome (TINU) with mast cells infiltrating in the interstitium. Repeated biopsies were performed before and after treatment of prednisolone. The pathogenesis of TINU remains unknown, but the T cell-mediated immune response or autoantibodies is considered to be involved. Recently, mast cells have been postulated to play a role in renal interstitial fibrosis. We investigated the localization of mast cells in the biopsy sections by immunohistochemistry. First renal biopsy showed that the mast cells infiltrated into the interstitial fibrotic lesion. Second biopsies showed the number of mast cells is decreased with the improvement of clinical symptoms and pathological lesions. These suggested that mast cells play an important role in the development of renal inerstitial injury in TINU.

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  Cyclosporin A (CyA), a potent immunosuppressive agent, has recently been introduced for the treatment of nephrotic syndrome especially in the patients resistant to steroid. However, the use of cyclosporin A is associated with multiple side effects, nephrotoxity, hypertension and neurotoxity. Posterior reversible encephalopathy syndrome (PRES) is one of the neurotoxities of CyA and the risk factors for developing CyA neurotoxity are following; intensive chemotherapy, irradiation, drug interaction, hypocholesterolemia, hypomagnesaemia, and aluminium overload.
  We report a 9-year old boy with nephrotic syndrome developed headache, vomits and convulsions 11 days after CyA administration. FLAIR and T2-weighted magnetic resonance imaging (MRI) on day 2 showed high signal intensities in the cortical and subcortical regions of right parieto-occipital lobes. Clinical symptoms disappeared in the following days and the MRI on 21 days did not show any abnormal findings. He was diagnosed as PRES, and diastolic hypertension and hypomagnesaemia may have been associated with developing CyA neurotoxity.

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  We reported a boy with Denys-Drash syndrome who received allogenic renal transplantation at the age of 31 months. He had hypospadias and bilateral cryptorchidism at birth, and presented with nephrotic syndrome at the age of 18 months. 2 months later, he developed renal failure with severe hypertension and started on continuous ambulatory peritoneal dialysis (CAPD).
  The histological findings of a renal biopsy showed diffuse glomerulosclerosis and tubullar atrophy.
  Chromosomal analysis showed a normal make karyotype, but molecular genetic analysis revealed C to T transition missense mutation at amino acid 394 in exon 9 at one allele of WT 1 gene, and the diagosis was made.
  At the age of 2 year 7 months, he received allogenic renal transplantation with bilateral nephrectomy and the post-operative course was uneventful with good renal function on immunosuppresion consisting of methylpredonisolone, mizoribine and tacrolimus.

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  The prognosis for childhood nephrotic syndrome is generally quite positive. However, patients and their families always worry about relapses and the side effects of drugs. Three years ago, we established “Soramame no Kai”, a society for nephrotic syndrome patients and families, to help relieve the stress associated with the condition. Here we report the results of a survey of patients and families, affected by the disease.
  Results of the survey, show that the actual health condition of patients was not bad, and yet 27% of patients and 8.1% of families were suffering stress as a result of the disease. Stress scores were high in the following groups: younger age (<6 years old) patients, recently diagnosed patients (<2 years), recently relapsed patients (<6 months), and frequently relapsed (>5 times) patients. Their families were particularly stressed about the future for the family member suffering from the disease. This survey showed that families needed more detailed information about daily life management and treatment. Communication and the exchange of information among patients and families in “Soramame no Kai” would help with these problems.

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  We report a 4-year-old boy with acute obstruction of hydronephrosis due to incarceration with clotted blood in the ureter. The patient had undergone a right pyelo-ureteroplasty for congenital hydronephrosis at 4 month after birth. Just before the present admission, he had fallen down some stairs, causing a blow to the right abdomen. Gross hematuria was found at a few hours later. On admission, blood examination data showed no anemia, and blood chemistry parameters were within the normal ranges. Abdominal ultrasound showed only mild hydronephrosis in the right kidney. We started the conservative therapy with a drip infusion with hemostatic agents containing 1000mg/day tranexamic acid under a tentative diagnosis of renal injury (Ia). On day 3, gross hematuria disappeared and drip infusion therapy was withdrawn. However, a few hours later the patient complained of severe abdominal pain and vomited frequently. At that time, abdominal ultrasound and intravenous pyelograph showed severe right pelvic dilatation. The patient then voided a blood clot in the urine, and the abdominal pain and frequent vomiting promptly disappeared.
  The cause of intraureteral coagulation in this case would have been urinary stagnation due to hydronephrosis and excessive antifibrinolytic activity of tranexamic acid. Hydration and administration of hemostatic agents at an appropriate dose are important for prevention of clot formation in cases like the present one.

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  We report on a case of juvenile nephronophthisis (JN) who developed end-stage renal failure (ESRF) at the early infancy. The patient was a 3-month-old male infant who presented with poor feeding and dyspnea. He was diagnosed as having hepertrophic cardiomyopathy (HCM), and was treated with diuretics and cathecholamines. However, he developed ESRF following next 4 months. An open renal biopsy revealed compatible lesion to JN. Although it has been reported that overt renal impairment in most JN patients developed over 3 years of age, some cases showed a rapid progression to ESRF at the early infancy: so called infantile nephronophthisis (IN). To our knowledge, this is the 4 th report describing Japanese IN patient. Since he had been suffering from heart failure due to HCM prior to ESRF, HCM might be a complication of IN, and which has rarely been reported to date.

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  We reported a case of 3 year-old-boy Hurler syndrome, who developed nephrotic syndrome 7 months after the second allogenic bone marrow transplantation (BMT). After BMT he had been treated by immunosuppressive drug (Tacrolimus) for prophylaxis of graft-versus-host disease. He had been suffered from autoimmune hemolytic anemia and thrombocytopenic purpura at 5 months later from second BMT, which had been treated with steroid hormone. While the steroid hormone dosage was decreasing, he showed severe hypoproteinemia and oliguria abruptly.
  Renal biopsy revealed one cellular crescent and one glomerular thrombosis, several mild mesangial cell proliferation, increase of mesangial matrix and swelling of epithelial cells among 24 glomeruli. Vacuolization of medial smooth muscle cells of afferent arterioles was prominent. Electron microscopic findings showed severe swelling and degeneration of subendothelial cell and no electron dense deposits. These findings suggested thrombotic microangiopathy (TMA) after BMT.
  Because the arteriolopathy of this case was considered to be induced by cell toxicity of tacrolimus, this drug was discontinued. Instead, methylprednisolone pulse therapy, oral prednisolone with dipyridamole therapy were employed. Proteinuria disappeared in two weeks, and prednisolone therapy discontinued at 8 months later without any recurrence.
  The glomerulopathy is suspected to be attributed to not only tacrolimus but also total body irradiation and chronic graft-versus-host disease.

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