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)

Serum concentrations of 17OH-progesterone were studied serially over 24 hours in 13 treated and untreated patients with the C21 hydroxylase form of congenital adrenal hyperplasia. The results were correlated with measurements of plasma renin activity, serum electrolytes, and urinary 17-ketosteroids and pregnanetriol. In 500 healthy subjects from birth to adult life, serum 17OH-pregesterone levels ranged from 5 to 315 ng/dl. In untreated CAH, serum 17OH-progesterone was markedly elevated (2,000 to 80,000 ng/dl). Treatment with cortisol (20 to 30 mg/m2/day in 3 doses) resulted in normal serum 17OH-progesterone levels in both non-salt-losing and salt-losing patients receiving adequate mineralocorticoid. Even slightly inadequate mineralocorticoid therapy (shown by high plasma renin activity with normal serum electrolytes) was associated with elevated 17OH-progesterone (to 65,000 ng/dl) in spite of usually effective doses of cortisol. Some patients showed isolated 17OH-progesterone elevations (usually early morning), a situation which requires only revision of the cortisol dosage schedule without an increase in total dosage. The data confirm the value of 17OH-progesterone assays in both the diagnosis and management of CAH. Taken together with determinations of plasma renin activity, serum 17OH-progesterone assays can permit more exact control of CAH without excessive doses of glucocorticoid.
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PMID:The application of a serum 17OH-progesterone radioimmunoassay to the diagnosis and management of congenital adrenal hyperplasia. 127 Nov 37

The renin substrate angiotensinogen (AGT) belongs to a supergene family of proteins that also includes alpha 1-antitrypsin (AAT) and alpha 1-antichymotrypsin (ACT), acute-phase reactants with known serine proteinase inhibitory (serpin) function. AGT lacks a known inhibitory function but is an acute-phase reactant. In this study we have compared the plasma levels, as analysed by electroimmunoassay, of AGT with AAT in patients with different types of chronic liver disease. AAT levels are regularly elevated in liver disease patients in contrast to AGT, which remains normal until late in the disease course. The AGT levels (mean +/- SD) were: in alcoholic cirrhosis (n = 19) 100 +/- 27.3%, in chronic active hepatitis (n = 14) 100 +/- 23.2%, in primary biliary cirrhosis (n = 18) 106 +/- 26.1% and in non-alcoholic cirrhosis (n = 15) 92 +/- 38.4%. Only occasionally were levels less than 50% of normal seen. In general, AGT levels were unrelated to sex and type of underlying liver disease and did not correlate with degree of hepatocellular impairment. Crossed immunoelectrophoresis showed no abnormal charge heterogeneity of AGT in patients with low levels. Our data are consistent with a dissociate expression of the homologous serpin genes in chronic liver disease. We speculate that the magnitude of the dissociated response is influenced by hormonal factors.
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PMID:Angiotensinogen in chronic liver disease. 159 89

The angiotensin II (AT II) levels in plasma were measured 68 times by using radio-immunoassay in 30 patients with viral hepatitis B (HB) and in 35 healthy persons as the control group. The results showed that the AT II levels of patients with HB were much higher than those of the control group (P less than 0.001). They were 219.25 +/- 91.31 ng/L and 60.70 +/- 10.73 ng/L, respectively. These indicated that the renin-angiotensin-aldosterone system (RAAS) of the patients was in exciting state. The levels of 8 cases of severe chronic active hepatitis (SCAH) (AT II = 270.40 +/- 106.55 ng/L, 6 cases of subacute fulminant hepatitis (SFH) (AT II = 332.80 +/- 140.12 ng/L), and 4 cases of hepatocirrhosis (HC) (AT II = 218.50 +/- 97.64 pg/ml) were all higher than those of 8 cases of chronic active hepatitis (CAH) (100.50 +/- 83.81 ng/L) and those of 4 cases of acute icteric hepatitis (A1H) (123.33 +/- 64.97 ng/L). These findings showed that the levels of AT II were directly related with the severity of the illness. The AT II levels of 8 cases of HRS (270.50 +/- 66.31 ng/L) were higher that those without HRS (174.50 +/- 78.48 ng/L). After treatment with captopril (CPT), the renal function of the patients returned to normal and the patients got better, while the AT II levels decreased greatly. The results suggested that high AT II levels in plasma may be one of the causes aggressing the HRS. The CPT may inhibit the produce of AT II and there are some therapeutic effects for the HRS.
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PMID:[The relationship between hepatitis B and renin-angiotensin-aldosterone system]. 174 16

The relationship between adrenocorticotropic hormone as well as renin and potassium activity and blood aldosterone secretion was examined in normal subjects and patients with chronic hepatic diseases. It is demonstrated that aldosterone stimulation is controlled by simultaneous renin-angiotensin (RA) and hypothalamo-adenopituitary effects in normal subjects and patients with chronic active hepatitis (CAH), the RA effects prevailing in normal subjects, and hypothalamo-adenopituitary ones, in CAH patients. Cirrhosis of the liver was associated with the greatest deviations in the stimulant-aldosterone relationship. Viral cirrhosis with ascites featured a considerable RA increase, affecting the adrenals, while the contribution of ACTH was reduced considerably. ACTH level was the highest in patients with alcoholic cirrhosis.
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PMID:[Hormonal changes in chronic diffuse diseases of the liver]. 255 Oct 48

The plasma levels of atrial natriuretic peptide were determined by radioimmunoassay in 24 patients with chronic liver disease, including three patients with alcoholic liver disease, four with chronic active hepatitis, 13 with liver cirrhosis, and four with hepatocellular carcinoma. When compared with normal subjects (180 +/- 12 pg/ml), the plasma levels of atrial natriuretic peptide in cirrhotic patients (349 +/- 64 pg/ml) were significantly elevated (p less than 0.001) but not in other disease groups. In patients with chronic liver disease the plasma levels of atrial natriuretic peptide were correlated significantly with plasma renin activity but not with plasma aldosterone, and furthermore showed a negative correlation with indocyanine green disappearance rate. These results suggest that the increased plasma levels of atrial natriuretic peptide, which appear to be associated with an increase in plasma renin activity and with hepatic dysfunction, may participate in maintaining homeostasis of sodium and fluid volume in patients with chronic liver disease.
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PMID:Plasma levels of atrial natriuretic peptide in patients with chronic liver disease. 303 81

Urinary kallikrein excretion was found as compared with 22 normal subjects (0.88 +/- 0.05 mumol/min/day) to be significantly reduced in 15 cirrhotics without ascites (0.42 +/- 0.04; p less than 0.01) and in 23 cirrhotics with ascites (0.15 +/- 0.02; p less than 0.01), and further, showed a significant difference between the two groups (p less than 0.01), but did not significantly change in 14 patients with chronic active hepatitis. Urinary kallikrein excretion in cirrhotics showed a positive correlation with serum albumin, indocyanine green disappearance rate, cholinesterase, and prothrombin, and an inverse correlation with bilirubin. After indomethacin administration to 13 cirrhotics with ascites, not only plasma renin activity and plasma aldosterone decreased significantly (p less than 0.01), but urinary kallikrein excretion also showed a small but statistically significant decrease (p less than 0.05). These results suggest that urinary kallikrein excretion decreases almost parallel to the severity of liver damage and is mediated via prostaglandins or the renin-angiotensin-aldosterone system, which may be involved in the reduction of renal blood flow in patients with liver cirrhosis.
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PMID:Urinary kallikrein excretion in chronic liver disease and effect of indomethacin. 351 May 29

In order to evaluate the pathogenesis of the systemic hyperdynamic circulation in portal hypertension, serum concentration of eight kinds of hormones including glucagon (Glu), aldosterone (Ald), renin (Ren), and epinephrine (Adr), and the hemodynamic parameters were measured in a series of 30 patients, of whom 23 were patients with liver cirrhosis, 3 were with Banti's disease, 2 with chronic active hepatitis, and 2 with pre-cirrhotic change. The average cardiac index was 4.6l/min, m2, with normal PCWP of 6.7 mmHg. CI. and SVR. showed significant inverse correlation of r = -0.767 (p less than 0.01), however, PCWP and CI did not have any significant correlation. Average serum concentrations of Glu, Ald, and Ren were 160 pg/ml, 139 pg/ml, and 5.4 ng/ml, respectively, all of which were increased up to 2.5 times above the normal values. Adr, norepinephrine, cortisol, estrone and estradiol were within normal limits. Of the eight hormones being measured, only Glu had significant correlation with both liver function tests and the cardiac index (r = 0.479, p less than 0.05). Neither Ald nor Adr had significant correlation with hemodynamic parameters.
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PMID:[Systemic hyperdynamic circulation and serum hormone concentrations in portal hypertension]. 352 13

The conventional treatment of CAH with hydrocortisone (16-19 mg/m2 per day) and 9 alpha-F-cortisol (just enough to normalise renin concentrations, started at 07:00 h) was ineffective in suppressing the early morning rise of 17-OH-progesterone and in turn androgens in about 20% of our patients. The present work explored the effect of a modified dosage regimen of the drug in five patients. The schedule was: 03:00 h F 33% + 9 alpha-F-F 33%; 07:00 h F 30%; 12:00 h F 22% + 9 alpha-F-F 33%; 17:30 h F 15% + 9 alpha-F-F 33%. Monitored levels of circulating 17-OH-progesterone, testosterone, and individual urinary 17-ketosteroids showed significant improvement, which was not achieved by giving higher or later evening doses. Menarche was induced in two girls (bone age 15 years). The modified dosage schedule offers on the one hand the possibility of better management of CAH, and on the other, cuts down the risk of enhanced Cushing-like effects, which in animal models have been related frequently to dosage schedules not corresponding to the circadian rhythm. The difficulty of administering the drugs at 03:00 h should be overcome by the development of a late-releasing preparation.
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PMID:Chronopharmacology of hydrocortisone and 9 alpha-fluorhydrocortisone in the treatment for congenital adrenal hyperplasia. 407 53

Patients with salt-wasting congenital adrenal hyperplasia (SW-CAH) most commonly carry an A-G transition at nucleotide 656 (nt 656 A-->G), causing abnormal splicing of exons 2 and 3 in CYP21, the gene encoding active steroid 21-hydroxylase. Affected infants are severely deficient in cortisol and aldosterone, and usually come to medical attention during the neonatal period. We report on 2 affected boys, homozygous for the nt 656 mutation, who thrived in early infancy, but suffered salt-wasting crises unusually late in infancy, at 3.5 and 5.5 months, respectively. Laboratory studies at presentation showed hyponatremia, hyperkalemia, dehydration, and acidosis; serum aldosterone was low in spite of markedly elevated plasma renin activity. Basal 17-hydroxyprogesterone levels were only moderately elevated, yet the stimulated levels were more typical of severe, classic CAH due to 21-hydroxylase deficiency. Genomic DNA from the patients was analyzed. Southern blot showed no major deletions or rearrangements. CYP21-specific amplification by polymerase chain reaction, coupled with allele-specific hybridization using wild-type and mutant probes at each of 9 sites for recognized disease-causing mutations, revealed a single, homozygous mutation in each patient: nt 656 A-->G. These results were confirmed by sequence analysis. We conclude that the common nt 656 A-->G mutation is sometimes associated with delayed phenotypic expression of SW-CAH. We speculate that variable splicing of the mutant CYP21 may modify the clinical manifestations of this disease.
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PMID:Splicing mutation in CYP21 associated with delayed presentation of salt-wasting congenital adrenal hyperplasia. 767 50

We report a child in whom DOC excess secondary to congenital adrenal hyperplasia (CAH, 11 beta-hydroxylase deficiency) caused malignant hypertension. Clinical and metabolic control could be achieved only by replacement of both glucocorticoid and mineralocorticoid, thus confirming in clinical practice the hypothesis that DOC is produced from both the zonae fasciculata and glomerulosa of the adrenal cortex under the independent control of the ACTH and renin-angiotensin systems respectively.
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PMID:Deoxycorticosterone, 11 beta-hydroxylase and the adrenal cortex. 837 Jan 38


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