Diagnostic Criteria

No formal diagnostic criteria for PHAII have been published.

Suggestive Findings

Pseudohypoaldosteronism type II (PHAII) should be suspected in individuals with the following clinical features, supportive laboratory findings, and family history. Clinical features. Hypertension (blood pressure 140/90 mm Hg) generally manifesting in adolescence or adulthood but also reported in children. Note: The absence of frank hypertension does not preclude the diagnosis.

Supportive laboratory findings

Source: GeneReviews — "Pseudohypoaldosteronism Type II"

Differential Diagnosis

Other causes of hyperkalemia. Hyperkalemia resulting from the following can generally be distinguished from hyperkalemia caused by PHAII on the basis of plasma renin levels, which are increased in the following conditions and suppressed in PHAII:

Source: GeneReviews — "Pseudohypoaldosteronism Type II"

Management Guidelines

Evaluations Following Initial Diagnosis

To establish the extent of disease and needs of an individual diagnosed with pseudohypoaldosteronism type II (PHAII), the following evaluations (if not performed as part of the diagnostic evaluation) are recommended:

• Serum electrolyte analysis

• Noninvasive blood pressure measurement

• Consultation with a clinical geneticist and/or genetic counselor

Treatment of Manifestations

Electrolyte and blood pressure abnormalities of PHAII are often corrected with thiazide diuretics. Metabolic abnormalities and hypertension generally improve within one week. Different thiazide diuretics exist, with different dosing regimens. In general dosing is titrated to normalization of blood pressure. It is possible that dosing will need to be increased over time or that additional anti-hypertensives will be required to adequately control blood pressure. There are no established guidelines regarding age at which treatment should begin for individuals with PHAII, but affected children who have hypertension are generally treated.

Prevention of Primary Manifestations

See .

Prevention of Secondary Complications

Control of blood pressure is important to reduce the risk for cardiovascular and renal disease and stroke.

Surveillance

Appropriate surveillance includes routine electrolyte and blood pressure measurements, monitored in the same manner as for any person treated with a thiazide diuretic.

Agents/Circumstances to Avoid

Untreated individuals with PHAII should avoid excessive...

Source: GeneReviews — "Pseudohypoaldosteronism Type II"

Clinical Guidance

Untreated individuals with PHAII should avoid excessive intake of foods high in salt and potassium as these may exacerbate hypertension and hyperkalemia.

Source: GeneReviews — "Pseudohypoaldosteronism Type II"

Therapies Under Investigation

Search ClinicalTrials.gov in the US and EU Clinical Trials Register in Europe for access to information on clinical studies for a wide range of diseases and conditions. Note: There may not be clinical trials for this disorder.

Source: GeneReviews — "Pseudohypoaldosteronism Type II"

Surveillance Guidelines

Appropriate surveillance includes routine electrolyte and blood pressure measurements, monitored in the same manner as for any person treated with a thiazide diuretic.

Source: GeneReviews — "Pseudohypoaldosteronism Type II"