CASE STUDIES OF SIBLINGS WITH JUXTAGLOMERULAR HYPERPLASIA AND SECONDARY ALDOSTERONISM ASSOCIATED WITH SEVERE AZOTEMIA AND RENAL RICKETS—BARTTER'S SYNDROME OR DISEASE?

PEDIATRICS ◽  
1970 ◽  
Vol 46 (3) ◽  
pp. 344-361
Author(s):  
B. S. Arant ◽  
N. C. Brackett ◽  
R. B. Young ◽  
W. J. S. Still
Keyword(s):  
Renal Function ◽  
Normal Renal Function ◽  
Sodium Intake ◽  
Plasma Renin ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Cell Hyperplasia ◽  
Severe Renal Failure ◽  
Excretion Rates ◽  
Disease Present

In 1962 two cases of juxtaglomerular (J-G) hyperplasia and secondary aldosteronism without hypertension with otherwise near normal renal function were presented. We have followed, since birth, two male siblings (F.T., age 6; J.T., age 3), with many features of "Bartter's syndrome," but who progressed to severe renal failure (F.T. and J.T.) and in one child (F.T.) overt renal rickets. Metabolic alkalosis, high levels of plasma renin activity (PRA) not suppressed by Na loading were found, but renal biopsy (J.T.) and autopsy (F.T.) revealed a severe glomerular and interstitial nephritis with mild J-G hyperplasia (not the typical "invasive" J-G cell hyperplasia seen in Bartter's cases). Balance studies demonstrated renal sodium wasting in both J.T. and F.T. PRA and aldosterone excretion rates were inappropriately high for sodium intake. Severe bone disease present in F.T. was associated with an increase in the total hydroxyproline (HP): Cr ratio to 1.77 (N = .13). Free HP excretion was increased in J.T. in whom bones appeared normal. It is suggested that either the typical cases with "invasive" J-G hyperplasia and near normal renal function represent a distinct entity (?Bartter's disease) or that "Bartter's syndrome" must be extended to include uremia and renal osteodystrophy.

Intrarenal renin inhibition increases renal function by an angiotensin II-dependent mechanism

1988 ◽  
Vol 255 (4) ◽  
pp. F749-F754 ◽  
Author(s):  
H. M. Siragy ◽  
N. E. Lamb ◽  
C. E. Rose ◽  
M. J. Peach ◽  
R. M. Carey
Keyword(s):  
Renal Function ◽  
Angiotensin Ii ◽  
Sodium Intake ◽  
Renal Plasma Flow ◽  
Plasma Renin ◽  
Competitive Inhibitor ◽  
Urinary Flow ◽  
Renin Substrate ◽  
Plasma Aldosterone Concentration ◽  
Renin Inhibition

ACRIP is a competitive inhibitor of renin in which an analogue of statine, (3R,4S)-4-amino-3-hydroxy-6-methylheptanoic acid, is incorporated into analogues of porcine renin substrate. ACRIP inhibits the enzymatic activity of renin, thus blocking the initiation of the angiotensin cascade. We studied the intrarenal action of ACRIP in small quantities without measurable systemic effects on renal function. In the first experiment, ACRIP was administered intrarenally at 0.02, 0.2, and 2 micrograms.kg-1.min-1 to uninephrectomized conscious dogs (n = 6) in metabolic balance at sodium intake of 10 meq/day. ACRIP, in doses of 0.02 and 0.2 micrograms.kg-1.min-1, markedly increased urine sodium excretion (UNaV) from 5.8 +/- 1.4 to 15.1 +/- 5.1 and 19.9 +/- 3.2 mu eq/min, respectively. Urinary flow rate (UV) underwent a similar increase and glomerular filtration rate (GFR) increased from 25.7 +/- 2.5 to 35.6 +/- 2.5 at 0.02 micrograms.kg-1.min-1 of ACRIP. Renal plasma flow (RPF), plasma renin activity (PRA), and plasma aldosterone concentration (PAC) were not affected. At 2 micrograms.kg-1.min-1, ACRIP traversed the kidney in quantities large enough to produce a reduction in systemic PRA and mean arterial pressure and caused natriuresis, diuresis, and increased GFR. In a second experiment, ACRIP was administered intrarenally at 0.2 micrograms.kg-1.min-1 in a separate group (n = 4) under identical conditions. ACRIP-induced increases in UV and UNaV were completely blocked by concurrent intrarenal administration of angiotensin II. The results indicate that intrarenal angiotensin II acts as a physiological regulator of renal sodium and fluid homeostasis.

Renin-Angiotensin-Aldosterone System of a Woman with Bartter’s Syndrome: Juxtaglomerular Cell Hyperplasia without Hypertension

1974 ◽  
Vol 112 (3) ◽  
pp. 293-298 ◽  
Author(s):  
Teruhiro Nakada ◽  
Goichi Momose ◽  
Toyohiko Yoshida ◽  
Yukio Tateno ◽  
Hidekazu Shigematsu ◽  
...  
Keyword(s):  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Cell Hyperplasia ◽  
Renin Angiotensin Aldosterone System ◽  
Renin Angiotensin ◽  
Juxtaglomerular Cell

scholarly journals Atypical presentation of cystic fibrosis: Obese adolescent with hypertension and pseudo-Bartter’s syndrome

2012 ◽  
Vol 69 (4) ◽  
pp. 367-369 ◽  
Author(s):  
Aleksandar Sovtic ◽  
Predrag Minic ◽  
Radovan Bogdanovic ◽  
Natasa Stajic ◽  
Milan Rodic ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Metabolic Alkalosis ◽  
Caloric Intake ◽  
Chloride Concentration ◽  
Plasma Renin ◽  
Holter Monitoring ◽  
Atypical Presentation ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Obese Adolescent

Introduction. Infants with cystic fibrosis may fail to thrive despite recommended caloric intake because of electrolyte disurbances caused by salt depletion resulting in hypochloremic metabolic alkalosis or pseudo-Bartter's syndrome. In most patients reported symptoms began in infancy, but it may be an initial presentation of disease in a previously healthy adolescent. Case report. A 15-year-old boy was admitted for evaluation of recurrent episodes of malaise associated with dehydration and acute renal insufficiency. Laboratory analysis showed hypochloremic metabolic alkalosis with hyponatremia and hypokalemia. On admission the boy was obese, with body weight of 95.5 kg (> P97), height 174 cm (> P75), and body mass index of 31.2 kg/m2 (> P95). Physical examination was inconclusive. Blood pressure holter monitoring proved significant systolic hypertension. Routine urinalysis, protein and electrolyte levels in urine were normal. Plasma renin and aldosteron were normal. Sweat chloride concentration was 63 mmol/L. Genetic testing confirmed the diagnosis of cystic fibrosis. Conclusion. To our knowledge, this is the first reported case of atypical presentation of cystic fibrosis in an adolescent presented with pseudo-Bartter's syndrome and signs of obesity and hypertension. We suggest that every patient with hypochloremic metabolic alkalosis should be evaluated for cystic fibrosis.

Long-Term Use of Propranolol, Ibuprofen, and Spironolactone in the Management of Bartter's Syndrome

PEDIATRICS ◽  
1979 ◽  
Vol 63 (5) ◽  
pp. 754-756
Author(s):  
Robert J. Cunningham ◽  
Ben H. Brouhard ◽  
Michael Berger ◽  
Tom Petrusick ◽  
Luther B. Travis
Keyword(s):  
Plasma Renin Activity ◽  
Linear Growth ◽  
Plasma Renin ◽  
Renin Activity ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Prostaglandin Synthetase ◽  
Catch Up

This report concerns two patients wter'sth Barter's syndrome who were treated with propranolol, spironolactone, and potassium supplements. When ibuprofen was added to this regimen, potassium supplements were no longer required. In both patients, plasma renin activity decreased, plasma volumes increased, and a "catch-up" in linear growth ensued. This report confirms others that indicate prostaglandin synthetase inhibitors are a useful adjunct in the therapy of Bartter's syndrome.

The effect of methyldopa on plasma renin activity in a child with Bartter's syndrome

1970 ◽  
Vol 77 (6) ◽  
pp. 1071-1074 ◽  
Author(s):  
Ronald G. Strauss ◽  
Shakil Mohammed ◽  
Jennifer M.H. Loggie ◽  
William K. Schubert ◽  
Alfred F. Fasola ◽  
...  
Keyword(s):  
Plasma Renin Activity ◽  
Plasma Renin ◽  
Renin Activity ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome

Neurogenic control of renal function in response to graded nonhypotensive hemorrhage in conscious dogs

1993 ◽  
Vol 264 (4) ◽  
pp. R661-R667 ◽  
Author(s):  
L. D. Nelson ◽  
J. L. Osborn
Keyword(s):  
Renal Function ◽  
Blood Volume ◽  
Sodium Intake ◽  
Extracellular Fluid ◽  
Plasma Renin ◽  
Urinary Sodium Excretion ◽  
Volume Depletion ◽  
Total Blood ◽  
Before And After ◽  
Chronic Study

The reflex control of plasma renin activity (PRA) and urinary sodium excretion (UNaV) was evaluated in 13 dogs instrumented for chronic study and maintained on a normal sodium intake (40 meq/day). Graded blood volume depletion of 14 (BVD1) and 21% (BVD2) of the estimated total blood volume was used to activate renal sympathetic nerve activity (RSNA), and experiments were conducted before and after bilateral renal denervation (DNX). In dogs with innervated kidneys, nonhypotensive BVD1 increased RSNA 40.9 +/- 10.9% (P < 0.05) above control. Blood volume depletion increased PRA from 1.95 +/- 0.52 to 3.5 +/- 0.57 ng.ml-1 x h-1 and decreased UNaV from 58.2 +/- 10.1 to 35.5 +/- 4.3 mu eq/min without changing renal blood flow or glomerular filtration rate. BVD2 failed to further activate RSNA (52.0 +/- 16.7%) but did increase PRA to 4.85 +/- 0.83 ng.ml-1 x h-1 and decreased UNaV to 17.9 +/- 2.7 mu eq/min. Renal DNX (n = 13) abolished both the PRA and antinatriuretic responses to BVD1 and BVD2. Thus volume-invoked reflex activation of RSNA, but not altered renal hemodynamics, mediates, activation of PRA and antinatriuresis. This neurogenic control of renal function may be critical to the rapid regulation of extracellular fluid volume, via alterations in urinary excretion.

Evidence that intrarenal bradykinin plays a role in regulation of renal function

1993 ◽  
Vol 265 (4) ◽  
pp. E648-E654 ◽  
Author(s):  
H. M. Siragy
Keyword(s):  
Renal Function ◽  
Sodium Excretion ◽  
Sodium Intake ◽  
Renal Plasma Flow ◽  
Plasma Renin ◽  
Urinary Sodium Excretion ◽  
Sodium Balance ◽  
Kinin System ◽  
Plasma Aldosterone Concentration ◽  
Low Sodium

Bradykinin (BK) is produced by the kidney, but the role of the renal kallikrein-kinin system (KKS) in the control of renal function is not understood. We studied the effects of intrarenal infusion of the BK antagonist, D-Arg-Arg-Pro-Hyp-Gly-Thi-Ser-D-Phe-Thi-Arg-trifluoroacetic acid (BKA, n = 5) and BK (n = 4) alone or combined with antagonist (BKA 0.025 ng.kg-1 x min-1 + BK 0.25 ng.kg-1 x min-1, n = 4) in uninephrectomized conscious dogs in sodium balance at 10 and 80 meq/day. During low sodium intake, administration of BKA (infusions from 0.025 to 2.5 ng.kg-1 x min-1) caused a significant antidiuresis (P < 0.0001) and antinatriuresis (P < 0.0001) and a decrease in fractional sodium excretion (P < 0.0001). There were no changes in estimated renal plasma flow (RPF) or glomerular filtration rate during intrarenal administration of BKA at 0.025 and 0.25 ng.kg-1 x min-1. A dose of 2.5 ng.kg-1 x min-1 BKA caused a significant decrease in RPF. There were no changes in plasma aldosterone concentration, plasma renin activity, or systemic arterial pressure during intrarenal BKA administration. At 80 meq/day sodium balance (n = 5), intrarenal administration of BKA did not cause any systemic or renal effects. Intrarenal administration of BK at 0.25 ng.kg-1 x min-1 during low sodium balance caused an increase in urine flow rate and urinary sodium excretion. Coinfusion of BK with BKA completely abrogated the renal excretory changes induced by BKA. These data suggest that intrarenal KKS plays a role in control of renal function largely by a tubular mechanism during low sodium intake.

Blood pressure and renal function during chronic changes in sodium intake: role of angiotensin

1980 ◽  
Vol 239 (3) ◽  
pp. F271-F280 ◽  
Author(s):  
J. E. Hall ◽  
A. C. Guyton ◽  
M. J. Smith ◽  
T. G. Coleman
Keyword(s):  
Blood Pressure ◽  
Renal Function ◽  
Sodium Intake ◽  
Renin Angiotensin System ◽  
Plasma Renin ◽  
Plasma Aldosterone ◽  
Filtration Fraction ◽  
Plasma Aldosterone Concentration ◽  
Angiotensin System

The present study was designed to quantitate the role of the renin-angiotensin system (RAS) in determining the chronic relationships between arterial pressure (AP), renal hemodynamics, and Na excretion. In six control dogs, Na balance was achieved during chronic step increases in Na intake from 5 to 500 meq/day with small increases in AP (<7 mmHg), moderate increases in GFR (19%), and decreases in filtration fraction (FF) and plasma renin activity. Similar increases in Na intake in six dogs with angiotensin II (AII) fixed, due to constant intravenous infusion of 5 ng . kg-1 . min-1 AII, caused large increases in AP (42%), GFR (31%) FF, and calculated renal Na reabsorption (TNa) above control. In six dogs with AII formation blocked with SQ 14,225, Na balance at intakes of 5-80 meq/day occurred at reduced AP, GFR, FF, and TNa, although plasma aldosterone concentration (PAC) was not substantially different from that in control dogs. At Na intakes above 240 meq/day, AP was not altered by SQ 14,225. These data indicate that during chronic changes in Na intake the RAS plays a major role, independent of changes in PAC, in allowing Na balance without large changes in GFR or AP. The mechanism whereby AII conserves Na chronically is through increased TNa, since steady-state TNa was increased by AII and decreased by SQ 14,225.

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