During pregnancy, when progesterone levels increase 100-fold, the MCR becomes hyperactive and leads to sodium reabsorption and potassium secretion via the principal cell of the distal collecting tubule (Figure above). The avid sodium retention and volume expansion appropriately suppress renin and aldosterone levels. This condition is not associated with proteinuria, edema, or neurologic changes, which distinguishes it from pre-eclampsia.
Given the low levels of aldosterone, this condition is refractory to standard medical therapy aimed at reducing aldosterone levels through RAAS blockade. In fact, mineralocorticoid receptor antagonists can actually exacerbate hypertension in this condition! Delivery of the fetus may be necessary to treat severe, refractory hypertension during pregnancy.
Essential hypertension results from a complex interplay of both genetic and environmental influences. Uncommonly, an isolated genetic mutation can engender hypertension, like in this case. These monogenic forms of hypertension affect either electrolyte transport in the distal nephron, or the synthesis and/or activity of mineralocorticoids, leading to a common final pathway of increased distal tubular reabsorption of sodium and chloride, volume expansion, and hypertension.
- Familial Hyperaldosteronism Type 1 (aka, glucocorticoid-remediable aldosteronism)
- Familial Hyperaldosteronism Type II
- Syndrome of apparent mineralocorticoid excess
- Liddle syndrome
- Pseudohypoaldosteronism type II (aka, Gordon syndrome)
- Congenital Adrenal Hyperplasia
They provide excellent fodder for any number of board-style questions!
Michael Lattanzio DO
although Liddle syndrome would cause the same biochemical profile and clinical phenotype as the activating mutation of MCR, only the later would be expected to stabilize post-partum (once progesterone levels normalized), therefore it is the best answer.
Other than the age of the patient, any reason why this scenario cannot be from Liddles