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

A 26-year-old female with Bartter's syndrome associated with Graves' disease is reported. This patient had a history of Graves' disease from the age of 22 and anti-thyroid drug (Methimazole) had been administered for 2 years. Thyroid function returned to normal but general fatigue and polyuria continued. Hypokalemia was diagnosed at 25 years of age and she was referred to our hospital for evaluation. Blood pressure was normal and laboratory data revealed normal thyroid function, hypokalemic alkalosis, high plasma renin activity and high plasma aldosterone concentration. She showed normal pressor sensitivity to norepinephrine infusion, grossly diminished pressor sensitivity to exogenous angiotensin II infusion compared with the normal. A renal biopsy specimen showed juxtaglomerular cell hyperplasia. Electron microscopy confirmed lacis cell (agranular cell) proliferation.
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PMID:Bartter's syndrome--case report. 15 51

We describe a patient with Bartter's snyndrome. In addition to the well-known characteristic findings by light microscopy, electron micrograms confirmed the presence of juxtaglomerular cell hyperplasia with polymorphous renin secretory granules and dense multivesicular bodies. Volume expansion by albumin infusion decreased plasma renin activity and aldosterone excretion, and improved the pressor response to exogenous angiotensin, suggesting that the renin-angiotensin-aldosterone system was not autonomous but that a decreased extracellular volume might be a major defect in this patient. During hypotonic saline diuresis, moreover, fractional free water clearance per fractional distal sodium delivery, CH2O/CH2O + CNa, was markedly depressed in the patient when compared with the value in the controls. Evidence presented suggests that chronic extracellular volume depletion exists as a consequence of an impaired sodium transport in the ascending limb of Henle's loop.
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PMID:The pathogenesis of Bartter's syndrome. Functional and histologic studies. 90 Jan 49

Discussed here is a patient with normotension, hypokalemic alkalosis, hyperreninemia, hyperaldosteronism, juxtaglomerular cell hyperplasia and insensitivity to the pressor effects of angiotensin (Bartter's syndrome). The hyperreninemia and hyperaldosteronism were both suppressible with volume expansion. Hypokalemia was correctible both short-term with potassium chloride infusions and long-term with spironolactone. Nevertheless, the abnormal pressor response to infused angiotensin could not be corrected by these maeuvers, suggesting that this defect is likely to be of primary pathophysiologic significance. We found that potassium loading markedly stimulated aldosterone excretion. This may explain the inadequacy of potassium supplementation alone to correct the hypokalemia and the observed "escape" from the potassium conserving effects of spironolactone seen in patients with Bartter's syndrome. The administration of propranolol in large doses only partially suppressed the marked hyperreniemia of our patient and failed to prevent a subsequent rise in the renin level which was associated with spironolactone therapy. In contrast, suppression of the renin level to normal was demonstrated by sodium loading. It is suggested that patients with Bartter's syndrome be treated simultaneously with large doses of spironolactone and a high sodium intake.
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PMID:Bartter's syndrome. New insights into pathogenesis and treatment. 116 59

Microscopic analysis of over 2,000 renal biopsies taken at surgical sympathectomy operations has been correlated with blood pressures in essential hypertension. Statistically significant relationships exist between the average diastolic pressure and the degree of afferent renal arteriolar thickening and narrowing. Postoperative prognosis was favorable with all but the most severe and diffuse arteriolar sclerosis, and vascular fibrinoid necrosis did not affect survival. Juxtaglomerular cell hyperplasia and inferred renin hypersecretion were significantly involved in the kidney biopsies of essential hypertension. Diabetic juxtaglomerular cell atrophy and sclerosis reduced this component of hypertension. After age 80, kidneys at autopsy showed no evident relation between arteriolar lesions and hypertension.
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PMID:Renal pathology of essential hypertension. 169 71

A Japanese family in which a father, daughter and son had hypokalemia and hyperreninemia was investigated. Both father and daughter had reduced vascular sensitivity to angiotensin II; in addition, the daughter had juxtaglomerular cell hyperplasia and dwarf glomeruli. These features are consistent with Bartter's syndrome occurring in 2 successive generations in 1 family. The 12-year-old girl had growth retardation in spite of normal growth hormone secretion. No chromosomal abnormalities were found. Indomethacin administration to this patient in doses sufficient to reduce urinary prostaglandin excretion resulted in a marked improvement of polydipsia and polyuria, and an increase in serum sodium, potassium and chloride concentrations. Even though plasma aldosterone concentrations were reduced to within the normal range, serum potassium concentrations remained low, and plasma renin activity (PRA) remained elevated. Thus it is not likely that hypokalemia is induced only by aldosteronism. These results suggest that prostaglandins are the major determinant of polydipsia, polyuria and high plasma aldosterone levels and contribute to the hypokalemia observed in this patient. However, the failure of complete correction of the hypokalemia and the persistence of the raised PRA with a significant reduction of the prostaglandins suggest the possibility that additional factors are involved in the pathogenesis of Bartter's syndrome.
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PMID:Familial Bartter's syndrome and the effect of indomethacin in one family member. 708 75

Bartter's Syndrome is characterized by renal potassium wasting with hypokalemia, metabolic alkalosis, increased renin-angiotensin-aldosterone system, normal blood pressure, resistance to the pressor effects of angiotensin II and juxtaglomerular cell hyperplasia. Most of the cases have been noted in the pediatric age group and adult-onset cases are very rare. We report a case of adult-onset Bartter's syndrome.
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PMID:A case of adult-onset Bartter's syndrome. 762 59