Familial hyperkalemic hypertension is caused by pathogenic variants in genes of the CUL3
(cullin-3)-KLHL3 (kelch-like-family-member-3)-WNK (with no-lysine [K] kinase) pathway …

K+ during a NaHCO3 infusion. As will be discussed below, we now know that there are 5
levels of severity of PHAII so that the response to PRAN may differ between individual cases …

Pseudohypoaldosteronism type II (PHAII) is characterized by hyperkalemia despite normal
glomerular filtration rate (GFR) and frequently by hypertension. Other associated findings in …

(1) Background: Gordon syndrome (GS) or familial hyperkalemic hypertension is caused by
pathogenic variants in the genes WNK1, WNK4, KLHL3, and CUL3. Patients presented with …

The inherited tubulopathies are lifelong disorders and their clinical features and
complications may present quite different challenges in adulthood from those in childhood …

Background: Familial hyperkalemia and hypertension (FHHt) is a rare genetic disorder
manifested by hyperkalemia and early hypertension. Hypercalciuria is another …

Mutations in the novel serine/threonine WNK [W ith N o lysine (= K)] kinases WNK1 and
WNK4 cause PHAII (pseudohypoaldosteronism type II or Gordon's syndrome), a rare …

Introduction: Familial Hyperkalemic Hypertension (FHHt) is an inherited disease
characterized by hyperkalemia, hypertension and hyperchloremic acidosis. The primary …

Background and hypothesis Gordon syndrome (also pseudohypoaldosteronism type II
(PHAII) or Familial Hyperkalemia with Hypertension (FHHt)) is a genetic condition …

Abstract Introduction: Familial hyperkalemic hypertension (FHHt) is an inherited disease
characterized by hyperkalemia, hypertension, and hyperchloremic acidosis (HCA). The …