(See also Introduction to Disorders of Kidney Tubules Introduction to Disorders of Kidney Tubules The kidneys filter and cleanse the blood. They also maintain the body’s balance of water, electrolytes (such as sodium, potassium, bicarbonate, and chloride), and nutrients in the blood. The... read more .)
There are two types of pseudohypoaldosteronism:
Type I, which has 2 subtypes and is characterized by low blood pressure
Type II, which is very rare (not discussed here)
Two different gene mutations can cause pseudohypoaldosteronism type I. In both, the body does not respond to the hormone aldosterone, leading to the excretion of too much sodium and water. However, the two types differ. In one type, excess sodium is excreted only by the kidneys. In the other, more severe form, excess salt is also lost through sweat and from other organs.
Children with the severe form may have symptoms similar to those of cystic fibrosis Cystic Fibrosis (CF) Cystic fibrosis is a hereditary disease that causes certain glands to produce abnormally thick secretions, resulting in tissue and organ damage, especially in the lungs and the digestive tract... read more , such as frequent respiratory tract infections. They may have
Dehydration, possibly causing nausea and vomiting
Low blood pressure, sometimes with dizziness and fainting
Sodium loss through sweat may cause a rash of very small red bumps.
The less severe form may cause few symptoms other than those of low blood pressure. In the less severe form, the disorder may resolve as children age.
Diagnosis of Pseudohypoaldosteronism Type I
Blood pressure measurement
Blood and urine tests
In addition to measuring blood pressure, doctors measure the amount of sodium and potassium in the urine and blood. They may also do blood tests to detect levels of hormones that help regulate sodium levels in the blood and thus blood pressure (renin and aldosterone).
Genetic testing may also be done.
Treatment of Pseudohypoaldosteronism Type I
Eating a high-salt diet
Treatment is eating a diet that is high in salt (salt contains sodium). Sometimes people need to take fludrocortisone, a drug that helps the kidneys retain sodium and water.
The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): Insight into ongoing research, consumer health information in English and Spanish, a blog, and community health and outreach programs.
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