Table 4. Familial forms of primary aldosteronism.
Type I | Type III | Type III | |
---|---|---|---|
Cause | Hybrid CYP11B1/CYP11B2 | Unknown | Germline KCNJ5 |
Transmission | Autosomal dominant | Autosomal dominant | Autosomal dominant |
Genetic diagnosis | Long PCR | No | KCNJ5 sequencing |
Hypertension onset | Very often < 20 years | Adulthood | Very often < 10 years |
Hypertension severity | Severe to resistant hypertension (normal BP is rare) | Stage 1 to resistant hypertension (normal BP is not often) | Stage 3 to resistant hypertension |
Hypokalemia | Rare | Not often | Very often |
Aldosterone after dexamethasone | < 4 ng/dL | > 4 ng/dL | > 4 ng/dL |
Adrenal CT | Normal | Unilateral or bilateral lesions | Bilateral macronodular hyperplasia |
Treatment | Dexamethasone or mineralocorticoid antagonist | Unilateral adrenalectomy or mineralocorticoid antagonist | Bilateral adrenalectomy or mineralocorticoid antagonist |
BP: blood pressure. CT: computed tomography.