[Arterial hypertension with hyperkalemia, tubular acidosis and normal renal function: Gordon syndrome and/or pseudohypoaldosteronism type II?].
Basic examination of Mr S., 45 years of age, short in stature and overweight (1.60 m, 76 kg), was carried out because of the mild hypertension (mean AP 125 mm Hg) from which he had suffered for 20 years. The results were as follows: (1) variable hyperkalemia: plasma potassium values were 5.3 to 6.9 mmol/l; (2) normal renal function: serum creatinine 91.5 mumol/l, clearance of inulin 136.6 ml/mn; (3) proximal tubular acidosis: plasma bicarbonate and chloride values were 18.4 and 109 mmol/l, respectively; urinary pH was 7.1 with negative H+ ions urinary excretion (-33 mumol/mn); when plasma bicarbonate level was raised to 26 mmol/l by acute loading, fractional excretion of bicarbonate increased to 19,5 p. 100 while plasma potassium value decreased to 4.2 mmol/l; (4) low PRA (0.29 ng/ml/h) and normal plasma aldosterone concentration (63 pg/ml) with a normal intake of sodium and in a recumbent position. Plasma atrial natriuretic factor (ANF) level was normal: 14 fmol/ml. Intravenous infusion of ANF for 2 h (1 microgram/mn) induced the expected increases in urinary flow rate, and sodium and potassium excretions (+226, +307 and +171 p. 100, respectively). Intravenous infusion of isotonic saline (2 l in 2 h) and oral administration of fludrocortisone acetate for 4 weeks (400 micrograms per day) resulted in a normal decrease in PRA and plasma aldosterone concentration, a normal rise in plasma ANF level (22 and 42 fmol/ml) while slightly increasing AP without improving bicarbonaturia and acidosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Ader
J L
JL
Service de néphrologie, CHU de Toulouse-Rangueil, France.
Waeber
B
B
Suc
J M
JM
Brunner
H R
HR
Tran-Van
T
T
Durand
D
D
Praddaude
F
F
D000141:Acidosis, Renal Tubular / Q000209:etiology*; D000450:Aldosterone / Q000097:blood; D009320:Atrial Natriuretic Factor / Q000502:physiology*; D006801:Humans; D006947:Hyperkalemia / Q000209:etiology*; D006973:Hypertension / Q000209:etiology* / Q000503:physiopathology; D007668:Kidney / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011546:Pseudohypoaldosteronism / Q000150:complications*; D015499:Renal Tubular Transport, Inborn Errors / Q000150:complications*; D012083:Renin / Q000097:blood; D013577:Syndrome
D000450:Aldosterone; D009320:Atrial Natriuretic Factor; D012083:Renin
Hypertension artérielle avec hyperkaliémie, acidose tubulaire et fonction rénale normale: syndrome de Gordon et/ou pseudo-hypo-aldostéronisme type II?