Neo-Mull-Soy Metabolic Alkalosis: A Model of Bartter's Syndrome?

PEDIATRICS ◽  
1980 ◽  
Vol 66 (5) ◽  
pp. 784-786
Author(s):  
Vivian M. Reznik ◽  
William R. Griswold ◽  
Stanley A. Mendoza ◽  
Richard M. McNeal
Keyword(s):  
Metabolic Alkalosis ◽  
Chloride Transport ◽  
Chloride Concentration ◽  
Thick Ascending Limb ◽  
Loop Of Henle ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Hypokalemic Alkalosis

A case of Neo-Mull-Soy-induced metabolic alkalosis occurred in an 8-month-old child. This child had hypochloremic hypokalemic alkalosis as well as hyperreninemia. Initially, a diagnosis of Bartter's syndrome was made and treatment consisted of KCl replacement, indomethacin, and aspirin. In retrospect, the diagnosis of Neo-Mull-Soy induced metabolic alkalosis could have been suspected on the basis of the low chloride concentration in his urine. Proposed mechanisms for the etiology of Bartter's syndrome are reviewed. Neo-Mull-Soy induced metabolic alkalosis simulates Bartter's syndrome and supports the concept that the primary abnormality in Bartter's syndrome is chloride deficiency. The chloride deficiency in Bartter's syndrome results from a defect in active chloride transport in the thick ascending limb of the loop of Henle.

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.

Effect of potassium depletion on chloride transport in the loop of Henle in the rat

1985 ◽  
Vol 248 (5) ◽  
pp. F682-F687 ◽  
Author(s):  
R. G. Luke ◽  
B. B. Booker ◽  
J. H. Galla
Keyword(s):  
Chloride Transport ◽  
Chloride Concentration ◽  
Distal Tubule ◽  
Normal Diet ◽  
Potassium Depletion ◽  
Thick Ascending Limb ◽  
Loop Of Henle ◽  
Chloride Absorption ◽  
Altered Response ◽  
And Control

Microperfusion of the superficial loop segment (latest proximal to earliest distal tubule) was performed in potassium-depleted and control rats. Potassium depletion was confirmed by analysis of muscle content (control 45 +/- 2, potassium depletion 33.5 +/- 0.9 meq/100 g dry solids). During perfusion at 20 nl/min net chloride absorption was decreased (66 +/- 3 vs. 77 +/- 2%, P less than 0.01) and early distal chloride concentration increased (70 +/- 5 vs. 50 +/- 4 meq/liter, P less than 0.01) in the potassium-depleted rats. In separate paired experiments in potassium-depleted rats, indomethacin infusion increased net chloride absorption (P less than 0.05) and lowered early distal chloride concentration (P less than 0.05) toward, but not to, normal. A similar effect of indomethacin to decrease early distal chloride concentration was seen in rats ingesting a normal diet and in control rats. We conclude that in potassium-depleted rats there is impaired net chloride absorption in the loop segment, most likely in the thick ascending limb, and that this effect is not produced by an altered response to prostaglandins. This defect in chloride transport may be responsible, at least in part, for the impaired concentrating capacity seen in potassium-depleted rats.

Effect of Na and Cl infusion on loop function and plasma renin activity in rats

1990 ◽  
Vol 258 (5) ◽  
pp. F1328-F1335 ◽  
Author(s):  
J. N. Lorenz ◽  
T. A. Kotchen ◽  
C. E. Ott
Keyword(s):  
Plasma Renin Activity ◽  
Chloride Transport ◽  
Chloride Concentration ◽  
Specific Effect ◽  
Distal Tubule ◽  
Plasma Renin ◽  
Renin Activity ◽  
Thick Ascending Limb ◽  
Loop Of Henle ◽  
Before And After

Inhibition of plasma renin activity (PRA) by saline has been shown to be related to a specific effect of chloride. The purpose of this study was to test the hypothesis that inhibition of renin release by selective chloride infusion in the rat is related to increased chloride transport in the thick ascending limb of the loop of Henle (TALH). Measurements of loop of Henle function were obtained by micropuncture before and after a 5% body wt infusion of solutions containing either 0.15 mol/l NaCl, 0.15 mol/l lysine monohydrochloride (LysCl), or 0.15 mol/l Na-assorted anions (NaAA). Both NaCl and LysCl infusion lowered PRA (60.8 +/- 11.9 to 22.6 +/- 3.7 ng angiotensin I (ANG I).ml-1.h-1 and 53.3 +/- 6.8 to 34.5 +/- 4.6 ng ANG I.ml-1.h-1; P less than 0.05), whereas NaAA infusion had no effect on PRA (66.7 +/- 15.1 to 59.1 +/- 12.4 ng ANG I.ml-1.h-1). Analysis of late proximal and early distal fluid showed that chloride transport in the TALH was significantly elevated by infusion in all three groups, and there were no differences among the groups after infusion. Distal chloride concentration increased in the NaCl and LysCl groups (26 +/- 2 to 37 +/- 1 meq/l and 26 +/- 2 to 36 +/- 2 meq/l; P less than 0.05), but distal chloride concentration decreased in the NaAA group (28 +/- 2 to 22 +/- 1 meq/l; P less than 0.05). There was no correlation between PRA and fluid flow rate or chloride delivery to the distal tubule.(ABSTRACT TRUNCATED AT 250 WORDS)

scholarly journals Mouse model of type II Bartter's syndrome. II. Altered expression of renal sodium- and water-transporting proteins

2008 ◽  
Vol 294 (6) ◽  
pp. F1373-F1380 ◽  
Author(s):  
Carsten A. Wagner ◽  
Dominique Loffing-Cueni ◽  
Qingshang Yan ◽  
Nicole Schulz ◽  
Panagiotis Fakitsas ◽  
...  
Keyword(s):  
Collecting Duct ◽  
Statistical Significance ◽  
Salt Transport ◽  
Postnatal Life ◽  
Thick Ascending Limb ◽  
Type Ii ◽  
Bartter’S Syndrome ◽  
Wild Type ◽  
Bartter's Syndrome ◽  
Altered Expression

Bartter's syndrome represents a group of hereditary salt- and water-losing renal tubulopathies caused by loss-of-function mutations in proteins mediating or regulating salt transport in the thick ascending limb (TAL) of Henle's loop. Mutations in the ROMK channel cause type II antenatal Bartter's syndrome that presents with maternal polyhydramnios and postnatal life-threatening volume depletion. We have developed a colony of Romk null mice showing a Bartter-like phenotype and with increased survival to adulthood, suggesting the activation of compensatory mechanisms. To test the hypothesis that upregulation of Na+-transporting proteins in segments distal to the TAL contributes to compensation, we studied expression of salt-transporting proteins in ROMK-deficient ( Romk−/−) mice. Plasma aldosterone was 40% higher and urinary PGE2 excretion was 1.5-fold higher in Romk−/− compared with wild-type littermates. Semiquantitative immunoblotting of kidney homogenates revealed decreased abundances of proximal tubule Na+/H+ exchanger (NHE3) and Na+-Pi cotransporter (NaPi-IIa) and TAL-specific Na+-K+-2Cl−-cotransporter (NKCC2/BSC1) in Romk−/− mice, while the distal convoluted tubule (DCT)-specific Na+-Cl− cotransporter (NCC/TSC) was markedly increased. The abundance of the α-,β-, and γ-subunits of the epithelial Na+ channel (ENaC) was slightly increased, although only differences for γ-ENaC reached statistical significance. Morphometry revealed a fourfold increase in the fractional volume of DCT but not of connecting tubule (CNT) and collecting duct (CCD). Consistently, CNT and CD of Romk−/− mice revealed no apparent increase in the luminal abundance of the ENaC compared with those of wild-type mice. These data suggest that the loss of ROMK-dependent Na+ absorption in the TAL is compensated predominately by upregulation of Na+ transport in downstream DCT cells. These adaptive changes in Romk−/− mice may help to limit renal Na+ loss, and thereby, contribute to survival of these mice.

scholarly journals Case of Pseudo-Bartter's Syndrome: An atypical presentation of cystic fibrosis

2011 ◽  
Vol 31 (2) ◽  
pp. 121-123 ◽  
Author(s):  
Enayatollah Nemat Khorasani
Keyword(s):  
Cystic Fibrosis ◽  
Key Words ◽  
Metabolic Alkalosis ◽  
Atypical Presentation ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
In The Beginning

A three months infant who in the beginning had disease cystic fibrosis was diagonosed with pseudo-bartter's syndrome. The disease began with coughing, diarrhoea, vomiting and weakness. Investigation revealed; electrolytes showin hyponatremia (110 mmol/L) and hypokalemic (2.6 mmol/L) and hypochloremic (63 mmol/L) metabolic alkalosis (HCO3=43 mmol/L). Key words: Pseudo Bartter's Syndrome; Cystic fibrosis; Metabolic alkalosis. DOI: 10.3126/jnps.v31i2.3911 J Nep Paedtr Soc 2010;31(2):121-123

Variant of Bartter’s Syndrome with a Distal Tubular rather than Loop of Henle Defect

Nephron ◽  
1988 ◽  
Vol 50 (3) ◽  
pp. 205-211 ◽  
Author(s):  
Jules B. Puschett ◽  
Arthur Greenberg ◽  
Robert Mitro ◽  
Beth Piraino ◽  
Rajni Wallia
Keyword(s):  
Loop Of Henle ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome

scholarly journals Variant of Bartter’s Syndrome with a Distal Tubular Rather than Loop of Henle Defect

Nephron ◽  
1989 ◽  
Vol 53 (2) ◽  
pp. 164-165 ◽  
Author(s):  
H.A. Koomans ◽  
R.J. Hené ◽  
E.J. Dorhout Mees ◽  
W.H. Boer
Keyword(s):  
Loop Of Henle ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome

Effects of NaCl on renin and aldosterone responses to potassium depletion

1983 ◽  
Vol 244 (2) ◽  
pp. E164-E169 ◽  
Author(s):  
T. A. Kotchen ◽  
G. P. Guthrie ◽  
J. H. Galla ◽  
R. G. Luke ◽  
W. J. Welch
Keyword(s):  
Plasma Renin Activity ◽  
Volume Expansion ◽  
Chloride Transport ◽  
Plasma Renin ◽  
Plasma Aldosterone ◽  
Renin Secretion ◽  
Renin Activity ◽  
Potassium Depletion ◽  
Thick Ascending Limb ◽  
Loop Of Henle

We have previously suggested that renin secretion is inversely related to the magnitude of absorptive chloride transport in the thick ascending limb of the loop of Henle. Potassium depletion inhibits chloride transport at this site in the nephron. Consequently, we studied the effects of varying sodium and chloride intakes on the renin and aldosterone responses to potassium depletion. Potassium depletion prevented suppression of plasma renin activity (PRA) by dietary NaCl loading and augmented the PRA response to NaCl deprivation. PRA was stimulated (P less than 0.01) by selective chloride (without sodium) deprivation, and potassium depletion did not augment this response. Potassium depletion did not interfere with suppression of PRA by albumin-induced volume expansion. Plasma aldosterone was suppressed by potassium depletion, and the effect of potassium depletion on aldosterone was augmented by NaCl deprivation. In conclusion, the magnitude of PRA stimulation and aldosterone suppression by potassium depletion is modulated by dietary NaCl intake. The results are consistent with the hypothesis that potassium depletion stimulates renin release by inhibiting chloride transport in the loop of Henle.

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