Metabolic and Congenital Kidney Disorders

Kidney abnormalities can be anatomic or metabolic in origin. Many are hereditary and present at birth (congenital).
Renal Tubular Acidosis
Renal tubular acidosis is a disorder in which the kidney tubules can't adequately remove acid from the blood to excrete into the urine.
Normally, the kidneys remove acid from the blood and excrete it into the urine. In renal tubular acidosis, the kidney tubules don't function properly, and insufficient amounts of acid are excreted into the urine. As a result, acid builds up in the blood, a condition called metabolic acidosis, which leads to the following problems:
- Low potassium levels in the blood
- Calcium deposits in the kidney
- A tendency to become dehydrated
- Painful softening and bending of the bones (os-teomalacia or rickets)
Renal tubular acidosis may be hereditary or may be caused by drugs, heavy metal poisoning, or an autoimmune disease, such as systemic lu-pus erythematosus or Sjogren's syndrome.
Symptoms and Diagnosis
Three types of renal tubular acidosis exist. Each type produces slightly different symptoms. When blood potassium levels are low, neurologic problems may develop, including muscle weakness, diminished reflexes, and even paralysis. Kidney stones may develop, causing damage to kidney cells and leading to chronic kidney failure.
A doctor considers the diagnosis of renal tubular acidosis when a person has certain char-acteristic symptoms or when routine blood tests reveal high acid levels and low potassium levels. Special tests help determine the type of renal tubular acidosis.
Treatment
Treatment depends on the type. Types 1 and 2 are treated by drinking a solution of bicarbonate (baking soda) every day to neutralize the acid in the blood. This treatment relieves the symptoms and prevents kidney failure and bone disease or keeps these problems from becoming worse. Other specially prepared solutions are available, and potassium supplements may also be required. In type 4, the acidosis is so mild that bicarbonate may not be needed. High potassium levels can be kept in check by drinking plenty of fluids and taking diuretics.
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