Lymphocytic sodium and potassium pump function in Bartter's syndrome.
Bartter's syndrome is characterized by chronic hypokalaemia, activation of the renin-angiotensin system and normal blood pressure. To investigate whether a generalized disturbance of sodium-potassium pump function might be of pathogenetic importance, lymphocytic sodium-potassium homeostasis was examined in 5 patients suffering from Bartter's syndrome. Two of the patients were treated with potassium chloride supplementation, the others were without medical treatment when studied. All were severely hypokalemic (serum potassium 2.8 +/- 0.24 mmol/l, mean +/- SEM). Lymphocyte sodium and potassium concentration (14.4 +/- 0.37 and 94.4 +/- 7.7 mmol/l, respectively), ouabain sensitive 22Na-efflux rate constant (2.68 +/- 0.25 h), and absolute ouabain sensitive efflux rate (38.16 +/- 4.2 mmol l-1 h) did not differ from matched controls. Ouabain binding capacity was 126 900 +/- 23 500 sites/cell in patients vs 50 400 +/- 17 900 in controls (p less than 0.05). In conclusion, patients with Bartter's syndrome may have an intrinsic abnormal pump function, characterized by an increased pump density and a low cation turn-over rate per pump unit.
Jest
P
P
Department of Clinical Chemistry, Odense University Hospital, Denmark.
Pedersen
K E
KE
Klitgaard
N A
NA
Thomsen
N
N
Kjaer
E
E
Simonsen
E
E
Jacobsen
J G
JG
D000328:Adult; D001477:Bartter Syndrome / Q000097:blood* / Q000503:physiopathology; D005260:Female; D006801:Humans; D006929:Hyperaldosteronism / Q000097:blood*; D008214:Lymphocytes / Q000378:metabolism*; D008297:Male; D011188:Potassium / Q000097:blood*; D011955:Receptors, Drug / Q000378:metabolism; D012964:Sodium / Q000097:blood*; D000254:Sodium-Potassium-Exchanging ATPase
D011955:Receptors, Drug; C028996:cardiac glycoside receptors; D012964:Sodium; D000254:Sodium-Potassium-Exchanging ATPase; D011188:Potassium