Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.23.15 (renin)
 35,795 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The possible point-mutational activation of the ras oncogene, which probably contributes to the evolution of aldosterone-producing adenomas and ectopic reninoma, was evaluated. Chromosomal DNA was extracted from six aldosterone-producing adenomas and one renin-secreting liver carcinoma, using the standard method with phenol:chloroform:isoamyl alcohol. One microgram of sample DNA, forward and reverse primers, Taq I polymerase and deoxyribonucleotide triphosphates (dNTPs) were mixed and the ras gene was amplified by the polymerase chain reaction. Point mutations at ras-12 or ras-61 sites of amplified DNA were tested by dot-blotting, using H-, N- and K-ras oligonucleotide probes with mutations at codons 12 or 61. However, there were no point mutations at amino acid codons 12 or 61 of c-Hi-ras, c-Ki-ras and N-ras oncogenes in all aldosterone-producing adenomas and the one ectopic reninoma. These results indicate that point-mutational activation of ras oncogenes does not contribute, at least in this series, towards the evolution of aldosterone-producing adenomas and the reninoma.
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PMID:Contribution of the activation of the ras oncogene to the evolution of aldosterone- and renin-secreting tumors. 184 28

The authors describe a Juxtaglomerular Cell Tumor (JGCT) in a hypertensive young man whose symptomatology disappeared when the JGCT was removed. The clinical diagnosis was made before surgery by selective determination of plasma renin activity level in both renal veins and in the draining vein of the tumor. This neoplasm is benign and usually occurs in young patients. The morphological, immunocytochemical and ultrastructural features are described. Other renin-secreting tumors and the theories about histogenesis of JGCT are discussed.
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PMID:Juxtaglomerular cell tumor of the kidney. Morphological, immunohistochemical and ultrastructural studies of a new case. 206 20

1. A 17 year old female presented with severe hypertension, hypokalaemia and elevated levels of plasma renin activity due to a renin-secreting tumour. 2. Renin was responsive to posture, low sodium diet, saline infusion and frusemide, but relatively unresponsive to raising or lowering circulating levels of angiotensin II. 3. Renal venous renin levels lateralized to the side of the tumour with good contralateral suppression when measured with control of posture and avoidance of prior stimulation, with and without angiotensin converting enzyme inhibition. 4. Levels of atrial natriuretic peptide were elevated and responsive to posture, saline infusion and angiotensin infusion. 5. The tumour was evident on computerized tomography, but not on intravenous pyelography or renal angiography. 6. Responsiveness of renin secretion to normal stimuli in reninoma may make diagnosis difficult, and renal vein sampling under controlled conditions is necessary.
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PMID:A renin-secreting tumour sensitive to changes in central blood volume (presumably via sympathetics) but not to circulating angiotensin II. 216 Mar 42

This case report of a reninoma or juxtaglomerular cell tumor illustrates that careful preoperative radiological investigation and preoperative frozen-section examination does not always lead to a correct diagnosis. The preoperative diagnosis of juxtaglomerular tumors should therefore primarily depend upon clinical suspicion, eventually followed by directed renal vein renin ratio blood sampling. The importance of early preoperative diagnosis and treatment is stressed.
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PMID:Juxtaglomerular cell tumor: importance of clinical suspicion. 760 63

Two women with spontaneous hypokalemia (1 normotensive, 1 hypertensive in the absence of renal artery stenosis), underwent unilateral nephrectomy because of angiographic and/or split renin-based suspicion of a reninoma. The normotensive patient clinically resembled Bartter syndrome but had some elements suggestive of a renin-secreting tumour, justifying surgical exploration and resection. The hypertensive patient presented clinically as a typical reninoma except for negative angiography. Surprisingly, the histology of the kidneys in both cases demonstrated juxtaglomerular hyperplasia without evidence of reninoma. The postoperative follow-up (8 and 19 yrs, respectively) has shown in the normotensive patient a considerable improvement in the hyper-reninism and previously uncontrollable hypokalaemia and in the hypertensive patient a complete normalisation of BP, renin and electrolyte status. Although the histological condition of the contralateral kidneys remains unknown in both patients the preoperative lateralisation of hyper-reninism to one kidney, the postoperative complete relief of the hyper-reninism in the hypertensive patient after uninephrectomy and its decrease, exceeding that corresponding to the removal of one kidney in the normotensive patient, suggest that the juxtaglomerular hyperplasia might have been unilateral or asymmetrical and that nephrectomy may, unexpectedly, relieve the hyper-reninism caused by juxtaglomerular hyperplasia. An increased unilateral susceptibility to trophic or renin-releasing factors or an asymmetrical abnormality in the macula densa-initiated mechanism of juxtaglomerular hyperplasia may be implicated in this disorder.
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PMID:Unilateral juxtaglomerular hyperplasia, hyperreninism and hypokalaemia relieved by nephrectomy. 845 May 25

Through a mechanism similar to renal artery stenosis, patients with reninoma and page kidney also suffered from renin mediated hypertension. Captopril renograms performed on our patients with the latter two conditions, however, did not yield diagnostic findings. Therefore, equivocal or negative captopril renography cannot serve to rule out conditions with elevated renin other than renal artery stenosis.
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PMID:Negative captopril renography on patients with renin mediated hypertension due to page kidney and reninoma. 1047 1

A 20-year-old man with no past medical history and no clinical symptoms was referred for examination because of severe hypertension. The blood chemistry studies showed elevated levels of peripheral renin with evidence of secondary aldosteronism. Sonography and computed tomography demonstrated a mass of the left kidney. After excluding all other possible causes for severe hypertension, excision of the tumor was performed. Histological studies confirmed the diagnosis of a juxtaglomerular cell tumor. Reninoma represents a rare but surgically curable cause of hypertension. The clinical suspicion of this tumor is very important because of the young age of the patients. If the diagnosis is confirmed a renal sparing surgery should be the treatment of choice.
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PMID:[Reninoma: a rare cause of hypertension. Case report, review of the literature and possibility for organ preserving operation]. 1095 78

A 14-year-old hypertensive boy was evaluated in our clinic. The physical examination was essentially normal except for his high blood pressure. Laboratory findings showed increased plasma renin activity. Abdominal ultrasonography detected a hypoechoic, 2-cm mass in right kidney. Contrast-enhanced computed tomography of the abdomen revealed a well-circumscribed, solid, hypoenhancing cortical lesion in the middle of the right kidney. Magnetic resonance angiography documented bilateral normal renal arteries. With a preoperative diagnosis of reninoma, the patient underwent nephron-sparing surgery. Intraoperative frozen section analysis revealed a benign lesion. Subsequently, histopathologic examination and electron microscopy confirmed the diagnosis of juxtaglomerular cell tumor. The patient remained normotensive in the postoperative period. Follow-up intravenous urography showed bilateral normally functioning kidneys.
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PMID:Reninoma treated with nephron-sparing surgery. 1280 18

We report a case of a renin secreting tumor, which is a very rare cause of secondary high blood pressure. A 22-year-old woman was hospitalised for exploration of high blood pressure (160/110 mmHg) with severe hypokaliemia (2,7 mmol/l) and secondary hyperaldosteronism. Physical examination was normal except the high blood pressure. Bioassays show increased kaliuresis (66 mmol/24h), plasma renin (89 pg/ml in clinostastism--108 pg/ml in orthostatism), pro-renin (1207 pg/ml in clinostastism--1412 pg/ml in orthostatism) and aldosterone (210 pg/ml in clinostastism--566 pg/ml in orthostatism). The rest of the endocrine tests were normal (cortisol and ACTH at 8:00 am, urinary free cortisol, overnight 1 mg dexamethasone suppression test). Doppler ultrasound method, performed by an experienced radiologist, did not show renal artery stenosis. Abdominal computerized tomography showed a nodular formation at the upper pole of the right kidney, isodense to renal medullary. The size tumor was 15 mm. The renal vein sampling shows high values of renin on both sides whereas, for the pro-renin, the values were higher on the tumor side. In spite of treatment with CEI (Converting Enzyme Inhibitors) and calcium antagonists, the blood pressure was not controlled. Hypokaliemia persisted (3 mmol/l) in spite of high daily potassium intake (64 mmol/l of potassium chloride). After tumor resection, reninoma was diagnosed by the pathology examination and blood pressure, plasma rennin, plasma aldosterone level returned to normal.
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PMID:[Reninoma: a rare but curable cause of high blood pressure, a case report]. 1291 61

Reninoma is a tumor of the renal juxtaglomerular cell apparatus that causes hypertension and hypokalemia via hypersecretion of renin. We describe a case of reninoma and provide a review of the literature, with a discussion emphasizing the diagnostic evaluation for such patients. The subject had persistent elevation of both plasma renin activity (PRA) and aldosterone. Imaging studies revealed the presence of a lesion in the renal cortex, which was further identified as a renin-producing lesion via selective venous catheterization following administration of an angiotensin-converting enzyme inhibitor (ACE-I). Following partial nephrectomy, the PRA and plasma aldosterone levels declined rapidly and the blood pressure and potassium supplementation requirements normalized. This case demonstrates the utility of both appropriate imaging studies and selective venous catheterization following provocative administration of an ACE-I for diagnosis.
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PMID:Reninoma: case report and literature review. 1819 52


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