A second gene responsible for polycystic kidney disease (PKD) has been identified recently, and an antisera (YCC2) against this gene's product, polycystin 2, has been generated. In the present study, we investigated the normal distribution of polycystin 2 in human adult kidneys and analyzed the expression of polycystin 2 in the cystic tubules of kidneys from patients with PKD and acquired cystic disease of the kidney (ACDK). The expression of polycystin 2 in normal regions of resected human kidneys, 4 cases of autosomal dominant polycystic kidney disease (ADPKD) and 4 cases of ACDK was examined by immunohistochemically staining the specimens with a polyclonal antibody specific to the C-terminal region of polycystin 2. This region is specific to polycystin 2 and does not crossreact with polycystin 1. In normal kidneys, prominent expression of polycystin 2 was observed in the distal tubules. A faint level of expression was detected in the proximal tubules, and the glomerulus and vessels were almost negative for expression. In the cystic kidneys of ADPKD patients, 68.7 % of the cystic tubules stained positively for YCC2, although partial staining was seen in 41.2 % of the positive cystic tubules. Although the genetic background of the samples is unknown, the co-existence of positive and negative cysts suggest that a "two-hit" hypothesis is feasible and that the mutations are likely to be missense or in frame changes. In ACDK cysts, YCC2-positive staining was prominent in small cysts (less than 0.5 mm in diameter), which were also positive for DBA, a marker for distal tubules. In contrast, larger cysts of more than 0.5 mm in diameter which stained positive for a proximal tubule marker, Lotus T, tended to be less positively stained for YCC2. Overall, 94.5 % of the cysts stained positive for YCC2, which is a much higher rate than that of PKD cysts. These results suggest that ACDK cysts may be generated by a different mechanism from that of PKD cysts.

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The main target organs of myeloperoxidase (MPO) antineutrophil cytoplasmic antibodies (ANCA) - related disease are the kidney and lung. This report describes a 71-year-old man with rapidly progressive glomerulonephritis (RPGN) and interstitial pneumonitis associated with MPO ANCA. The patient was admitted to our hospital because of bloody sputum, low grade fever and appetite loss on October, 1998. He was diagnosed as having interstitial pneumonitis from the findings of chest X-ray and CT examinations. Moderate proteinuria and hematuria, renal dysfunction (serum creatinine : 5.6 mg/dl, BUN : 58.0 mg/dl) and positivity for MPO ANCA were noted. He was negative for anti-glomerular antibody and PR3-ANCA. Renal biopsy was performed and revealed crescentic glomerulonephritis without deposition of immunoglobulins. Therefore, the diagnosis of pauci immune type RPGN was made. Pulse therapy with methylprednisolone (1.0g/day×× days) following oral administration of prednisolone (60 mg/day) found marked improvement of renal function maintenance and interstitial pneumonitis, respectively. However, he died because of lung tuberculosis and acute tuberculous pericarditis during treatment with prednisolone. In this case, MPO ANCA might have been directly associated with both RPGN and interstitial pneumonitis. Furthermore, chronic lung disease, such as interstitial pneumonitis, is important as a preceding disease of RPGN. MPO ANCA-relateddisease is more frequent in aged persons, therefore particular attention should be paid during their treatment with an immunosuppressant.

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A 38-year-old woman was admitted to our hospital on for evaluation of thirst, bilateral backache and a feeling of abdominal fullness. She had hypokalemia, normotension, hyperreninemia, hyperaldostronism and hyperplasia of the juxtaglomerular apparatus on renal iopsy. Ultrasonography, intravenous pyelography and computed tomography showed marked bilateral renal calcification. Considering her history of persistent soft stool caused by chronic laxative abuse for 15 to 16 years and past diuretic abuse for several years since 1986, we diagnosed her as pseudo-Bartter's syndrome with nephrocarcinosis. The value of urinary Ca excretion was in the normal range, and acidification disturbance in NH₄C1 loading test was revealed. In addition, she had taken analgesics for 2 to 3 years and interstitial nephritis on renal biopsy was seen. It is thus suggested that the cause of nephrocarcinosis in this case was the reduction of Ca solubility in the tubular cavity induced by incomplete renal tubular acidosis associated with analgesic nephropathy or interstitial nephritis caused by hypokalemia.

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We describe here two cases of chronic disseminated intravascular coagulation (DIC) secondary to aortic aneurysms. The patients were 78- and 84-year-old males, who visited our hospital to receive hemodialysis therapy for chronic renal failure probably due to nephrosclerosis. They had mild bleeding tendency and thrombocytopenia (<10×10μl). Coagulation test revealed the findings of chronic DIC in both patients, and computed tomography showed abdominal and thoracoabdominal aortic aneurysms with mural thrombi, respectively. In one patient, subcutaneous hemorrhage after vascular access surgery had continued for a month. However, the hemorrhage and swelling of the limb disappeared after continuous subcutaneous heparin infusion(CSHI) therapy in a daily dose of 10, 000-14, 000 unit. These findings suggest that chronic DIC secondary to aortic aneurysm should be considered when bleeding tendency and thrombocytopenia are observed in aged patients, and that CSHI is the choice of therapy for the bleeding tendency of chronic DIC.

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We present a case of familial juvenile gouty nephropathy which was associated with a right renal tumor that was found incidentally. The patient was a 27-year-old woman who initially presented with acute gouty arthritis at the age of twenty years. Her mother and her sister had been attacked with acute gouty arthritis in their early twenties. Progressive deterioration in the renal function was noted in the three family members who had experienced gouty attack. Her mother was maintained on hemodialysis. As image diagnoses could not rule out malignancy in her right renal tumor, in situ non-ischemic enucleation of the right renal tumor using a microwave tissue coagulator (Microtaze) and a renal biopsy were performed. The pathological diagnosis of the renal tumor was tubulopapillary adenoma, and the renal biopsy showed minimal change in the glomeruli and tubules. The post-operative course was uneventful. The serum creatinine and creatinine clearance before and three months after the operation were 2.4 mg/dl and 2.6 mg/dl, 36.7 ml/min and 32.5 ml/min, respectively. The renal biopsy findings that glomerular and tubular changes were minimal and there was no tissue precipitation of uric acid or sodium urate, and the fact that her renal function decreased progressively despite the treatment of hyperuricemia suggested strongly that renal function might have deteriorated due to unknown factors other than hyperuricemia.

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We report here a unique case of renovascular hypertension (RVH) showing spontaneously normalized blood pressure (BP) after progression of a stenosis of the renal arteries for 16 years. The patient was a 41-year-old man. At the age of 25, he developed drug-resistant hypertension with hypokalemia, hyperreninemia and severe stenosis of the right renal artery, and was diagnosed with RVH. He soon discontinued medication without medical advice. At age 40, he developed pons infarction and his aortogra phy showed progression of the stenosis to the bilateral renal arteries. However, his BP along with the levels of plasma potassium and renin activity were normalized and his renal function was preserved. Renal scintigraphy revealed a significant size difference between the bilateral kidneys. We assume that the hypotrophic changes in the ischemic kidneys counterbalanced the reduction in renal blood flow.

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