Medullary sponge kidney

The medullary sponge kidney is a congenital disease, usually of both kidneys. They may consist of many small cysts in the renal medulla, which are connected to the manifolds. There is often increased calcium and decreased acid secretion, which can lead to stone disease and renal tubular acidosis. Sufferers should drink water to avoid the formation.

Pathogenesis

Cause of the disease are congenital abnormalities of the collecting ducts into the medullary pyramids and papillae. The change has an autosomal dominant inheritance, but occur spontaneously as a rule. It can be unilateral or bilateral, either only part of the kidney or the entire kidney be affected.

In the terminal manifolds there is an extension, near the papilla can also form cysts that contain many Calziumoxalatkonkremente. While the kidneys retain their normal shape, but have a spongy appearance and are enlarged. In the kidney caused obstructions, which alter the secondary parenchyma without causing insufficiency.

Clinic

Even if the disease is congenital, it remains a very long time without symptoms. Initial symptoms occur either in adolescence or after the 5th decade of life. Common symptoms are:

• Recurrent renal colic
• Urinary tract infections
• Hematuria

Diagnostics

The diagnosis is made by X-ray. It show multiple, bilateral calcifications in the papillary. In the iv Pyelographhie to first fill the voids in the papillae and then the areas of the calices.

Therapy

Treatment is usually symptomatic. In hypercalciuria, thiazides should be given. Dehydration should be avoided.

The life expectancy is not usually limited.

Swell

• Piper (2007) Internal Medicine. Springer Verlag