We have already reported that decreased renal medullary blood flow and retention of sodium play an important role in the development of hypertension during chronic systemic L-NAME (N^G-nitro-Larginine methyl ester) administration, despite the absence of measurable changes in renal cortical blood flow as determined from fibers implanted for laser-Doppler flowmetry. In the present experiment, to investigate the interaction between nitric oxide and angiotensin II on renal hemodynamics, the effect of 1chronic intravenous infusion of L-NAME on blood pressure, sodium and water retention, and intrarenal blood flow distribution were studied in a separate group of rats pretreated with captopril and in which optical fiber for laser-Doppler flowmetry had been implanted. Captopril completely prevented sustained hypertension, sodium retention, and decreased medullary blood flow during chronic L-NAME infusion. These date indicated that angiotensin plays an important role in the regulation of renal medullary blood flow and sodium excretion by modulating the effects of nitric oxide in renal medullary blood flow.

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Amyloid osteoarthropathy has been seen frequently in long-term hemodialysis (HD) patients, in which the bone X-ray examination reveals characteristic cystic radiolucency (CRL) of the carpal bone, shoulder joint, hip joint and knee joint, and destructive spondylarthropathy (DSA) of cervical vertebrae. To clarify the clinico-pathological significance of CRL and DSA in HD-related amyloidosis, we investigated the grade and frequency of CRL or DSA and these relationship with age, HD duration, primary diseases, osteoarticular symptoms and blood analysis in 817 HD patients (492 male and 325 female, age : 52.6+15.5 years, dialysis duration : 6.8 + 5.4 years) . The number of cases with osteoarticular symptoms increased with the prolongation of HD duration. CRL and DSA were observed even in patients without osteoar- ticular symptoms : 26.7 % for carpal bone, 26.2 % for shoulder joint, 17.3 % for hip joint and 22.2 % for DSA. The grade and frequency of CRL and DSA also increased in accordance with age and HD duration. In patients with CRL of the carpal bone, shoulder CRL was noticed in 39.7 %, hip CRL in 25.8 %, and DSA in 14.3 % of cases, respectively, and these frequencies increased with the prolongation of HD duration. In the carpal CRL negative group, shoulder CRL was noticed in 14.6 %, hip CRL in 7.5 %, and DSA in 6.0 %, respectively. Although there was no relationship between CRL or DSA and serum β₂-MG level in any of the cases, the serum β₂-MG level was lower in patients with HD showing shoulder CRL (_??_) and DSA ( + ) for more than 16 years. No significant relationship was noticed between CRL or DSA and serum C-PTH and aluminium level. These results suggested that aging was related to CRL or DSA formation in dialysis-related amyloidosis. The findings also suggested that systemic bone X-ray examination should be considered in patients with carpal bone CRL, high-age patients and long-term HD patients even without osteoarticular symptoms.

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The most appropriate initial treatment for children with steroid-responsive nephrotic syndrome is controversial. Initial treatment with 18-week prednisolone and the Chinese herbal medicine, Sairei-to, may prevent subsequent relapse. To determine whether similar results can be obtained with a combination of just initial 8-week prednisolone and Sairei-to, we compared the effects of such treatment with those of treatment with 18week prednisolone and Sairei-to in 196 children with steroid responsive nephrotic syndrome. The patients were randomly assigned to receive 8-week (group 1) or 18-week (group 2) prednisolone for the initial therapy. All patients received Sairei-to for 2 years in addition to prednisolone . Eighty-eight of the 98 patients in group 1 and 83 of the 98 patients in group 2 completed their trial . At entry, the two groups of patients did not differ in their clinical and laboratory findings. During the 2-year trial, 62 group 1 patients (70 %) and 54 group 2 patients (65 %) had relapses, and 19 group 1 patients (21%) and 20 group 2 patients (24 %) had frequent relapses. The present study demonstrates that a combination of initial 8-week prednisolone and 2-year Saireito is effective in children with steroid-responsive nephrotic syndrome.

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A 68-year-old woman was admitted to because of progressive renal failure. She had been well until two months before admission. Laboratory data were as follows : serum creatinine 4.1 mg/dl, BUN 69 mg/dl, MPO-ANCA 33 EU, anti-glomerular basement membrane antibodies (AGBMA)118 U. Histological findings showed cellular and fibrocellular crescents in many glomeruli. Therefore, we diagnosed rapidly progressive glomerulonephritis (RPGN) due to MPO-ANCA and anti-GBM associated renal disease. The patient was started on prednisolone and double filtration plasmapheresis (DFPP) therapy. Subsequently, the values of MPO-ANCA and AGBMA decreased. However, the patient's condition suddenly worsened and she died of interstitial pneumonia. Autopsy examinationrevealed crescentic glomerulonephritis and alveolar hemorrhage with linear deposition of IgG along the glomerular and alveolar capillary walls by immunofluorescence studies. We considered this to be a rare case of Goodpasture's syndrome associated with not only anti-GBM antibodies, but also MPO-ANCA.

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A 41-year-old female was admitted to our hospital with acute renal failure. The renal biopsy showed focal necrotizing crescentic glomerulonephritis, and her serology revealed a high titer of MPO-ANCA. The diagnosis of MPO-ANCA-associated microscopic polyarteritis was made, and her basic illness proved to be systemic lupus erythematosus (SLE) because of renal injury, polyarteritis, pancytopenia, anti-double strand DNA antibody, and anti nuclear antibodies. After treatment with prednisolone (PSL) and cyclophosphamide, her renal function improved and her MOP-ANCA titer was lowered. After 2 years, she was readmitted due to dyspnea. Chest X-ray and echocardiography revealed pericarditis and bilateral pleural effusion. Renal biopsy was carried out again bacause of an increase in MPO-ANCA, which showed minor abnormalities. PSL therapy led to remission of pericarditis, resulting in a decrease in MPO-ANCA. In this case, MPO-ANCA is likely to be associated not with the activity of polyarteritis, but with that of SLE, and care should be taken in evaluating the clinical usefulness of MPO-ANCA in cases with SLE.

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We report a rare case of glomerulocystic kidney disease (GCKD) with congenital hypothyroidism. A gigantic abdominal mass was noted at birth. There was no family history of renal cystic disease. Ultrasonography revealed diffuse granular cysts in the markedly enlarged kidneys. Blood examination showed moderate renal failure and hypothyroidism. Bilateral nephrectomy was conducted at 47 days of age to relieve respiratory failure and severe abdominal distention caused by the growing cystic kidneys. Histological findings of the kidney showed numerous glomerular cysts without renal dysplasia. There were no other malformations. These findings were compatible with GCKD.

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A 30-year-old man had been treated for malignant rheumatoid arthritis from with a nonsteroidal anti-inflammatory drug, then bucillamine for six months and prednisolone. Mild proteinuria appeared in 4 years after bucillamine therapy was conducted. The patient was admitted to our hospital for a renal biopsy in The specimen revealed secondary amyloidosis and membranous nephropathy (MGN). These findings suggest that MGN unrelated to bucillamine therapy might have occured with secondary amyloidosis in rheumatoid arthritis.

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We treated a 13-year-old girl who developed myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) -related crescentic glomerulonephritis (GN) during propylthiouracil (PTU) treatment for Graves'disease. MPO-ANCA-related crescentic GN during PTU therapy has been described previously in only one recent report of 2 children. We report this case here and describe 15 (13 adult cases) more patients with MPO-ANCA-related GN associated with PTU found in a literature review. The mean age at onset was 41.3 years, and the length of PTU administration ranged from 2 weeks to 6 years (mean 3.5 years). Clinical signs and symptoms were hematuria (100%), proteinuria (100%), arthralgia (7 of 16 cases ; 43.8%), fever (4 cases ; 20.0%), purpura (2 cases ; 12.5 %), skin ulcer (1 case ; 6.3%) and dyspnea (1 case ; 6.3%) . These patients were treated with steroid (15 cases ; 93.8%), cyclophosphamide (8 cases ; 50.0%), steroid pulse therapy (4 cases ; 25.0%), or plasma exchange (1 case ; 6.3%), or were not treated (1 case ; 6.3%) . Most patients revealed crescentic GN (15 cases ; 93.8%) on renal biopsy, while one exhibited mesangial proliferative GN (6.3%) . For 2 of the 16 patients (12.5%) irreversible renal dysfunction persisted and hemodialysis was started. Patients with Graves' disease treated with PTU should be observed carefully by urinalysis and monitoring of the serum creatinine level.

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