[Dwarfism, arterial hypertension and hyperkalemic acidosis corrected with thiazides. A case of type II pseudohypoaldosteronism].
BACKGROUND
Type II pseudohypoaldosteronism is a rare tubulopathy defined by abnormal renal potassium excretion.


METHODS
A 12 1/2 year-old girl, was admitted for dwarfism. Her parents were not consanguineous and her 5 living sibs were normal. At admission, she had moderate hypertension: systolic 130-150 mmHg; diastolic 80-100 mmHg and no pubertal development.


METHODS
pH (arterial): 7.34; bicarbonates: 18-20 mEq/l; chloride: 112-120 mEq/l; potassium: 5.6-7 mEq/l; aldosterone: 200-700 pg/ml (N < 60); plasma renin activity: 0.4 ng/ml/hr (N 2.2 +/- 0.2). The bone maturation was 8 1/2 years. All the other renal function tests were normal. The titratable acidity was 22 mEq/day (N 20-40) and the ammonia excretion 15.2 mEq/l (N 44-61). The fractional excretion of potassium was 6.5% (N 11.8 +/- 1.9). This girl was given polystyrene sulfonate resin followed by hydrochlorothiazide (1 to 3 mg/kg/day). There was a subsequent improvement in all data, a growth spurt and pubertal development.


CONCLUSIONS
This is the fifth case of type II pseudohypoaldosteronism reported in childhood and the first one with hypertension. The beneficial effect of hydrochlorothiazide is underlined.
Poujol
A
A
Service de Pédiatrie, CHG, Aix-en-Provence.
Rimet
Y
Y
Cournelle
M A
MA
Cornus
P
P
Frayssinet
R
R
Zarrouk
F
F
Brusquet
Y
Y
D000141:Acidosis, Renal Tubular / Q000188:drug therapy* / Q000209:etiology; D002648:Child; D004392:Dwarfism / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D006801:Humans; D006852:Hydrochlorothiazide / Q000627:therapeutic use*; D006947:Hyperkalemia / Q000188:drug therapy* / Q000209:etiology; D006973:Hypertension / Q000188:drug therapy* / Q000209:etiology; D011546:Pseudohypoaldosteronism / Q000150:complications*
D006852:Hydrochlorothiazide
Nanisme, hypertension artérielle et acidose hyperkaliémique corrigés par les thiazidiques. Un cas de pseudohypoaldostéronisme de type II.