Hypertension in a patient with aldosterone deficiency.
OBJECTIVE
To describe a patient with aldosterone synthase deficiency, who presented with failure to thrive, hypovolemic hyponatremia, and the unexpected finding of hypertension.


METHODS
We present a case report, review the related literature, and outline a possible mechanism for the concomitant occurrence of high blood pressure and hyponatremia in this patient.


RESULTS
A 5-month-old infant with unambiguous female genitalia was admitted to our hospital with failure to thrive and hyponatremia. Her blood pressure was 115/88 mm Hg (>95% for age). The serum sodium concentration was 123 mEq/L (normal for age, >130), and the potassium level was 5.3 mEq/L (normal, 3.5 to 5.3). A direct renin measurement by immunochemiluminescence assay was 11,400 microU/mL (normal, <5), and the aldosterone level was 4 ng/dL (normal, 2 to 70). These findings indicated a diagnosis of aldosterone synthase deficiency. Treatment with fludrocortisone and sodium chloride was begun, but the hypertension worsened. Therapy with an angiotensin-converting enzyme inhibitor was transiently required.


CONCLUSIONS
Angiotensin II, a potent vasoconstrictor, is an intermediate in the renin-angiotensin system. We believe that this protein was the cause of the hypertension in the setting of aldosterone deficiency in our patient.
Touger
Leslie
L
Department of Endocrinology and Nephrology, Phoenix Children's Hospital, Phoenix, Arizona 85016-7710, USA.
Joseph
Mark
M
Hasan
Khalid S
KS
D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001794:Blood Pressure / Q000187:drug effects; D019405:Cytochrome P-450 CYP11B2 / Q000172:deficiency; D004359:Drug Therapy, Combination; D005183:Failure to Thrive; D005260:Female; D005438:Fludrocortisone / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D006973:Hypertension / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D006994:Hypoaldosteronism / Q000150:complications* / Q000209:etiology / Q000503:physiopathology; D007010:Hyponatremia / Q000209:etiology; D007223:Infant; D012965:Sodium Chloride / Q000009:adverse effects / Q000627:therapeutic use; D043202:Steroid Metabolism, Inborn Errors / Q000150:complications
D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D012965:Sodium Chloride; D019405:Cytochrome P-450 CYP11B2; D005438:Fludrocortisone