Table 1.

The three types of pseudohypoaldosteronism^a

Type	Inheritance	Clinical and Biochemical Characteristics	Molecular Pathology	OMIM
^a AR, autosomal recessive; AD, autosomal dominant; PAC, plasma aldosterone concentration; PRA, plasma renin activity.
^b Gordon syndrome or familial hyperkaliemic hypertension.
^c Transient PHA.
I	AR	Renal: salt wasting, hyponatremia, hyperkalemia, metabolic acidosis elevated PAC and PRA, treatment by salt supplementation for life	Mutations on the three genes coding for the epithelial sodium channel	264350
		Lung: chest congestion, cough and tachypnea
		Na⁺ and Cl⁻ elevated in sweat, saliva, and stool
	AD	Renal: salt wasting, hyponatremia, hyperkalemia, metabolic acidosis, elevated PAC and PRA, treatment by salt supplementation	Mutations on the gene coding for the mineralocorticoid receptor	177735
		Spontaneous remission over time.
II^b	AD	Hyperkalemia, hypertension, hyperchloremic acidosis, normal PAC, low PRA, treatment by thiazide diuretics	At least three subtypes:
			subtype a: associated with locus on chromosome 1q31–q42 (gene unknown)	145260
			subtype b: mutations on the gene coding for WNK4	601844
			subtype c: mutations on the gene coding for WNK1	605232
III^c	No	Hyperkalemia, acidosis, elevated PAC and PRA, low glomerular filtration rate.	Secondary to:
			excessive salt loss: intestine, sweat
			nephropathies: obstructive uropathy, sickle cell and lead nephropathy, amyloidosis, urinary tract infections.