Gordon Syndrome
It all begins with an idea.
Pseudohypoaldosteronism type 2 (i.e. Gordon Syndrome) is a disorder that is bound to show up on nephrology tests. The combination of the peculiar presentation that the fact that this presentation can be boiled down to abnormalities in two proteins make it a classic board question choice.
This disorder is due to abnormalities in the serine/threonine protein kinases WNK1 and WNK4. Of note, WNK stands for “with no lysine.” Mutations affecting these proteins cause the unusual combination of hyperkalemia, a non-anion gap metabolic acidosis, and hypertension. If you’re reading a question vignette and find what appears to be an odd conglomeration of a Type 4 RTA with hypertension, consider Gordon Syndrome in your answer choices.