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Obstruction of urine flow at the level of the pelvo-ureteric junction (UPJO) and subsequent development of hydronephrosis is one of the most common congenital renal malformations. UPJO is associated with development of salt-sensitive hypertension, which is set by the obstructed kidney, and with a stimulated renin-angiotensin-aldosterone system (RAAS) in rodent models. This study aimed at investigating the hypothesis that 1) in pediatric patients with UPJO the RAAS is activated before surgical relief of the obstruction; 2) in rats with UPJO the RAAS activation is reflected by increased abundance of renal aldosterone-stimulated Na transporters; and 3) the injured UPJO kidney allows aberrant filtration of plasminogen, leading to proteolytic activation of the epithelial Na channel γ-subunit (γ-ENaC). Hydronephrosis resulting from UPJO in pediatric patients and rats was associated with increased urinary plasminogen-to-creatinine ratio. In pediatric patients, plasma renin, angiotensin II, urine and plasma aldosterone, and urine soluble prorenin receptor did not differ significantly before or after surgery, or compared with controls. Increased plasmin-to-plasminogen ratio was seen in UPJO rats. Intact γ-ENaC abundance was not changed in UPJO kidney, whereas low-molecular cleavage product abundance increased. The Na-Cl cotransporter displayed significantly lower abundance in the UPJO kidney compared with the nonobstructed contralateral kidney. The Na-K-ATPase α-subunit was unaltered. Treatment with an angiotensin-converting enzyme inhibitor (8 days, captopril) significantly lowered blood pressure in UPJO rats. It is concluded that the RAAS contributes to hypertension following partial obstruction of urine flow at the pelvo-ureteric junction with potential contribution from proteolytic activation of ENaC.

SY:48歳女.全身倦怠感を主訴とした.頻尿, 残尿感があり, 超音波検査で両側水腎症を認めたため入院した.血液検査で軽度の貧血と腎不全を認め, 尿検査で血尿を認めた.膀胱鏡検査で三角部に腫瘍を認めたため, 腫瘍生検を行った.又, 腹部CT, 胸部CT.骨シンチにより全身への転移を認めた.生検による病理所見より印環細胞癌と診断した.入院当初は膀胱への転移を疑い, 原発巣検索のため他科的検査を行ったが異常は見られず, 膀胱原発と診断した.全身に転移しており, 疼痛コントロールのみで退院した.良好な疼痛コントロールを得たが, 診断の約5ヵ月後死亡した.病理解剖の結果, 膀胱原発で全身に転移した印環細胞癌を確認した, We report a case of signet-ring cell carcinoma of the urinary bladder. A 48-year-old female was hospitalized because of general fatigue, pollakiuria and residual sensation. Renal ultrasonography disclosed bilateral hydronephrosis. The serum level of cretinine was 3.1 mg/dl, and we diagnosed the patient with post-renal failure. Cystoscopic examinations revealed non-papillary sessile tumors in the trigone. Histopathological findings of the biopsy specimen demonstrated signet-ring cell carcinoma. There was no evidence indicating bladder metastasis from the cancer in other organs. The patient died of cachexia 5 months after the diagnosis. She was autopsied, and the diagnosis of signet-ring cell carcinoma of the urinary bladder with metastases in various organs was confirmed. This disease is extremely rare and has a poor prognosis. We review the previous cases reported in the literature.

We report a case of signet-ring cell carcinoma of the urinary bladder. A 48-year-old female was hospitalized because of general fatigue, pollakiuria and residual sensation. Renal ultrasonography disclosed bilateral hydronephrosis. The serum level of cretinine was 3.1 mg/dl, and we diagnosed the patient with post-renal failure. Cystoscopic examinations revealed non-papillary sessile tumors in the trigone. Histopathological findings of the biopsy specimen demonstrated signet-ring cell carcinoma. There was no evidence indicating bladder metastasis from the cancer in other organs. The patient died of cachexia 5 months after the diagnosis. She was autopsied, and the diagnosis of signet-ring cell carcinoma of the urinary bladder with metastases in various organs was confirmed. This disease is extremely rare and has a poor prognosis. We review the previous cases reported in the literature.