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A 77-year-old man with a 13-year history of systemic sclerosis (SSc) was admitted to our hospital with fever, appetite loss, and disorientation. The patient was well 2 days prior to the admission and had been taking a low dose of a steroid and vasodilators over the previous 10 years. In regular clinic visits, his blood pressure was normotensive and serum creatinine (Cr) was within the normal range. On admission, hypertension (blood pressure 214/105 mmHg), proteinuria, and hypercreatinemia (3.6 mg/dL) led to the diagnosis of sclerotic renal crisis (SRC). Thrombocytopenia (5.4 × 10(4)/μL), erythrocyte fragmentation, and elevated lactate dehydrogenase were suggestive of thrombotic microangiopathy (TMA). The immediate initiation of angiotensin-converting enzyme inhibitor therapy and plasma exchange (PE) rapidly improved the disorientation and thrombocytopenia. It is notable that SRC might occur in patients with a 13-year history of SSc. PE should be considered as a treatment option for SRC complicated by TMA.

A 58-year-old woman was admitted to our institute. Aortitis syndrome was diagnosed when she was 39 years old. She developed intractable hypertension several years prior, and a computed tomography (CT) scan showed severe calcified stenosis of the descending aorta. The ankle brachial pressure index of the right leg was 0.70 and that of the left leg was 0.63. Plasma renin activity was 4.8 ng/ml/h. Ascending aorta-abdominal aorta bypass grafting was scheduled. The operation was successfully performed under partial cardiopulmonary bypass to control arterial blood pressure. An implanted graft was 14 mm in diameter. The postoperative ankle brachial pressure index of the right leg was 1.07 and that of the left leg was 1.03. Postoperative plasma renin activity was 0.2 ng/ml/h, and three-dimensional CT revealed that the implanted graft was patent. Postoperatively, hypertension was well controlled without medication.

The aims of treating hypertension in patients with chronic kidney disease including diabetes are to prevent both cardiovascular events and end stage renal failure, and goal of blood pressure is less than 130/80 mmHg. The first choice is angiotensin II receptor blocker (ARB) or angiotensin converting enzyme inhibitor. If the patient is salt-sensitive and hypervolemic, the second choice is diuretic. When the patient has some risk factors for cardiovascular events, the second choice is calcium channel blocker. In treating patient whose serum creatinine more than 2 mg/dL, we should start the ARB with lower dose and examine serum creatinine and potassium every 2 week.

A 17-year-old man was admitted to our hospital with a chief complaint of right flank pain. Both computed tomography and magnetic resonance imaging demonstrated a tumor hemorrhage in a mass located at the upper pole of the right kidney. He had a high blood pressure of 220/100 mmHg associated with high serum renin activity (36.2 ng/ml/hr). Partial nephrectomy was performed and histopathological examination revealed a juxtaglomerular cell tumor. Postoperatively, his blood pressure was markedly improved and the serum renin activity immediately fell into the normal range. Previously, 32 cases of juxtaglomerular cell tumor have been reported in the Japanese literature, and to our knowledge, this is the first case was found with a tumor hemorrhage.

Renovascular hypertension and renal hypertension are two major secondary forms of hypertension due to renal disease. Diagnosis of renovascular hypertension is important because revascularization therapy is an effective treatment for patients with renal artery stenosis. Doppler echocardiogaphy is a useful noninvasive test to detect renal artery stenosis. There are two Doppler echocardiographic methods to detect renal artery stenosis. The first is to measure flow velocity at the stenotic area. Pulse-Doppler and/or continuous Doppler methods are used to measure flow velocity. Significant stenosis is diagnosed by maximum flow velocity (Vmax) at the stenotic area: > 1.5m/sec or > 1.8-2.0m/sec. Flow waves of segmental and interlober arteries are assessed by the pulse-Doppler method. Decreased Vmax, early systolic acceleration, resistive index and pulsatility index indicate decreased renal artery blood flow and hence, renal artery stenosis.

Autosomal dominant polycystic kidney disease(ADPKD) is rarely observed in the neonatal period. We report 2 cases of ADPKD who showed bilateral enlarged, hyperechoic kidneys and severe hypertension. It is difficult to differentiate ADPKD from autosomal recessive polycystic kidney disease (ARPKD) based on the initial clinical presentations in this period. In both cases, bilateral enlarged kidneys and severe hypertension were detected without oligohydramnion and respiratory distress. The mother of case 1 has polycystic kidneys. The father of case 2 was diagnosed as ADPKD. Case 2 had heart failure due to hypertension. Angiotensin converting enzyme inhibitor (ACE-I) was administered to both patients and resulted in good control of blood pressure. ADPKD in the neonatal and very early infantile period has diverse clinical courses. In general, although severe cases are rare, some cases have renal failure and/or hypertension as we reported. We emphasize that both the prompt diagnosis of ADPKD and the start of medication are of great importance in the neonatal and very early infantile period. We recommend that neonates and infants with a family history of ADPKD undergo screening including physical examinations, blood pressure measurements and urinalysis.

Renal disease constitutes an important determinant of cardiovascular disease. Although the mechanisms for the progression of renal impairment remain fully undetermined, available evidence indicate that renal glomerular hypertension is responsible in part for the development of renal injury. In renal disease, afferent arteriolar tone is reported to be reduced, while the augmented intrarenal angiotensin II serves to act as an efferent arteriolar constrictor, both of which result in an increase in glomerular capillary pressure. Angiotensin converting enzyme inhibitors (ACE-I) are established as the agent possessing both antihypertensive and renoprotective actions, which exert vasodilator action on efferent arterioles. Calcium antagonists are also reported to have salutary effect on renal disease, although their beneficial action varies depending on the antagonists used and the underlying disease. The use of calcium antagonists, however, is mandatory particularly under the circumstance where renal failure moderately to severely progresses and the ACE-I cannot be used.

A case of renal cell carcinoma of the right native kidney following cadaveric renal transplantation is reported. A 44-year old male underwent cadaveric renal transplantation in 1993 and had stable graft function, but he had suffered from hypertension before the renal transplantation and multiple antihypertensive medications were not effective. Abdominal computed tomography demonstrated bilateral contracted kidneys, but we could not rule out renal cell carcinoma of the right kidney, completely. Angiography of the graft artery revealed no stenosis and venous sampling suggested that plasma renin activity was increased in the left renal vein. Thus we performed bilateral native nephrectomy. Histology of the right kidney was renal cell carcinoma, clear cell subtype, grade 1, pT2, and the left kidney was end stage of renal disease. Because of high incidence of malignant neoplasia after renal transplantation, routine careful examination is quite important.

The renal mechanisms of hypertension were reviewed, and new way to classify hypertension based on renal mechanisms was proposed. Theoretically, the pressurenatriuresis relationship can be affected in two different ways; either a parallel shift along the blood pressure axis toward a higher blood pressure level or a depression in the slope. The parallel shift of the pressure-natriuresis relationship induces non-sodium sensitive hypertension, while the depressed slope induces sodium sensitive hypertension. Since the degree of the shift of the pressure-natriuresis relationship is determined by the preglomerular vascular resistance from heart to glomeruli, non-sodium sensitive hypertension should be based on the increased preglomerular vascular resistance. On the other hand, the slope of the pressure-natriuresis relationship is determined by glomerulo-tubular balance of sodium, which is mainly controlled by the difference between the whole kidney ultrafiltration coefficient and the rate of tubule sodium reabsorption. Therefore, sodium sensitivity of blood pressure is based on a decrease in the whole kidney ultrafiltration coefficient (due to a decrease in either filtration surface area per glomerulus, hydraulic permeability of glomerular filtration barrier or the number of glomeruli) and/or an increase in tubule sodium reabsorption. In sodium sensitive states, whether whole kidney ultrafiltration coefficient is reduced or tubule sodium reabsorption is enhanced, the glomerular capillary hydraulic pressure rises to compensate for the impairments in sodium excretory capability. If there is a linkage between sodium sensitivity of blood pressure and glomerular hypertension as proposed, renal failure may be expected to be more common in sodium sensitive types of hypertension than in non-sodium sensitive types.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of Ask-Upmark kidney is presented. An 18-year-old male patient referred to this facility presented with symptoms of hypertension, microscopic hematuria and proteinuria. A hormonal study revealed a high plasma renin activity level. Intravenous pyelography and abdominal computed tomography revealed thinning of the cortex with calyceal dilatation. Arteriography revealed a deep cortical groove in the middle portion of the kidney without renal arterial stenosis. Plasma renin activity of the left renal vein was significantly higher than that of the right renal vein. A left simple nephrectomy was performed under the diagnosis of Ask-Upmark kidney. Postoperatively, plasma renin activity returned to the normal range and a decrease in blood pressure was noted. Recent reports have suggested Ask-Upmark kidney to be a consequence of vesicoureteral reflux rather than a true congenital malformation. Our case indicated no evidence of vesicoureteral reflux and suggests that the lesion was congenital rather than acquired.

We reported a case of juxtaglomerular cell tumor, which excessively produced renin, resulting in secondary hypertension. A 25-year-old woman complained of headache and nausea. Hypertension and elevation of plasma renin activity were found by physical and laboratory examination. US and CT showed a space occupying lesion at upper pole of the right kidney. Angiography showed a hypovascular area at the corresponding area of the right kidney. Renin-secreting renal tumor of the right kidney was diagnosed and right nephrectomy was performed. Postoperatively, blood pressure and plasma renin activity became normalized and symptoms ameliorated. The juxtaglomerular cell tumor was pathologically confirmed and localization renin in the tumor cells was shown by immunohistochemical study. Forty one cases of juxtaglomerular cell tumor have been reported, since Robertson reported the first case. We discussed the clinical and pathological characteristics of the disease in this report.

The renal impairments were studied clinicopathologically in 57 patients with progressive systemic sclerosis (scleroderma). Proteinuria, hematuria, azotemia and hypertension, used as markers for renal involvements, were observed in 3 (5.3%), 4 (7.0%), 2 (3.5%) and 6 patients (10.5%) respectively, at the initial examination. Hypertension was increased 2.6 times at the last observation, although the incidence of other three markers have not changed during the follow-up period. Finally, 17 out of 57 patients (29.8) revealed more than one of these clinical markers throughout the study. The decrease of GFR (CThio) was noticed in 3 out of 36 cases (8.3%), however that of RPF (CPAH) in 11 of 36 patients (30.6%), including 5 without abnormal clinical markers. Histological studies were performed in 12 patients. One showed crescentic glomerulonephritis, two membranous nephropathy, and the remaining 9 minor glomerular abnormalities. On the other hand, the vascular changes such as intimal proliferation of interlobular arteries were frequently observed. The frequency of pulmonary involvements, skin ulcer and gastro-intestinal involvement in the patients with renal lesions were not significantly different from that of the non-renal group. The level of RPF was significantly lower in the patients with skin ulcer than that of those without skin ulcer. No significant difference was noticed in the frequency of renal involvements between the patients with or without anti-Scl-70 antibody.

A 35-year-old female patient was hospitalized for a headache. She was referred to our department for the evaluation of right hydronephrosis noted on the excretory urogram which was performed as part of a hypertensive diagnostic study. Endocrine examination revealed renal hypertension. Excretory urogram and antegrade pyelography showed obstruction of the lower part of the right ureter. On surgical exploration, the lower part of the right ureter was surrounded by brown tissue. Complete hysterectomy and ureterovesiconeostomy were performed. Histologically, the brown tissue around the ureter was diagnosed as endometriosis. One year after the operation, excretory urogram showed normal urinary tract and the blood pressure was 130/80 mmHg. Endometriosis is a common gynecological disease but ureteral endometriosis is relatively rare. Review of the Japanese literature disclosed 16 previous cases of ureteral endometriosis and we report the 17th case with a review of the literature.