The role of chloride transport in the thick ascending limb in the pathogenesis of Bartter's syndrome

1982 ◽  
Vol 60 (19) ◽  
pp. 1212-1214 ◽  
Author(s):  
J. R. Gill
Keyword(s):  
Chloride Transport ◽  
Thick Ascending Limb ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome

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.

The Role of Arachidonic Acid Metabolites in the Pathophysiology of Bartter’s Syndrome

1983 ◽  
pp. 353-364 ◽  
Author(s):  
Jeffrey S. Stoff ◽  
David M. Clive ◽  
Diane Leone ◽  
D. Euan MacIntyre ◽  
Robert S. Brown ◽  
...  
Keyword(s):  
Arachidonic Acid ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Arachidonic Acid Metabolites ◽  
Acid Metabolites

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 Role of 20-HETE in Elevating Chloride Transport in the Thick Ascending Limb of Dahl SS/Jr Rats

Hypertension ◽  
1999 ◽  
Vol 33 (1) ◽  
pp. 419-423 ◽  
Author(s):  
Osamu Ito ◽  
Richard J. Roman
Keyword(s):  
Chloride Transport ◽  
Thick Ascending Limb

Role of prostaglandins in the pathogenesis of Bartter's syndrome

1976 ◽  
Vol 60 (6) ◽  
pp. 785-797 ◽  
Author(s):  
M.P. Fichman ◽  
N. Telfer ◽  
P. Zia ◽  
P. Speckart ◽  
M. Golub ◽  
...  
Keyword(s):  
Bartter’S Syndrome ◽  
Bartter's Syndrome

On the pathogenetic role of prostaglandins in Bartter's syndrome.

2009 ◽  
Vol 205 (S625) ◽  
pp. 135-140 ◽  
Author(s):  
Ingolf Nielsen ◽  
Birger Hesse ◽  
Poul Christensen.
Keyword(s):  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Pathogenetic Role

scholarly journals Pathophysiological Role of Magnesium in Familial Bartter's Syndrome.

1994 ◽  
Vol 33 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Motoki SANO ◽  
Masayoshi SHICHIRI ◽  
Takashi IDA ◽  
Sei SASAKI ◽  
Hiroshi TSUKAGOSHI
Keyword(s):  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Pathophysiological Role

scholarly journals Effect of Indomethacin in a Patient with Bartter's Syndrome. Evidence against a Primary Role of Prostaglandin in This Disorder

1980 ◽  
Vol 14 (12) ◽  
pp. 1395-1397 ◽  
Author(s):  
A Etzioni ◽  
C Chaimovitz ◽  
Y Levi ◽  
A Benderli ◽  
O S Better
Keyword(s):  
Primary Role ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome
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