In addition to the commonly reported characteristics of GS such as familial hyperkalemia, mild metabolic acidosis, and low PR activity with normal PA levels, our patients with the p.Glu630Gly variant of WNK1 showed hypertension (with superimposed pre-eclampsia in two cases), short stature and low weight in adults, and initial isolate hyperkalemia in one of the children. This evidence concerns the gene WNK1 and metabolic acidosis.