kidney and urinary tract disorders
Pseudohypoaldosteronism Type I
Introduction
Pseudohypoaldosteronism type I is a rare hereditary disorder in which the kidney tubules retain too much potassium and excrete too much sodium and water, leading to low blood pressure. (See also Introduction to Disorders of Kidney Tubules .) 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 hormonealdosterone, 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 (CF) , such as frequent respiratory tract infections. They may have Dehydration, possibly causing nausea and vomiting Fatigue Muscle weakness 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. 