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Polycystic Kidney Disease

This is an inherited disorder and is characterised by multiple grape-like clusters of cysts that enlarge the kidney. There are two sorts: Infantile, and Adult.

 

Infantile

This may cause stillbirth or early neonatal death. However, many infants do survive, live for several years and then develop renal failure.

 

Adult

This is not usually obvious until the person is much older, around thirty to fifty. Progression may be slow even after signs of renal insufficiency occur. Not all adults will require dialysis.

 

Causes

The disease is transmitted genetically. The infantile type is recessive with the risk of the next infant being affected being 25%. The adult type is dominant. Here the risk of transmission to the next child is 50%. Both types affect males and females equally.

 

Signs and Symptoms

At birth, affected babies may have a distended abdomen. They may show signs of respiratory distress and heart failure.

Eventually they will develop uraemia and renal failure. If the renal failure is not treated with dialysis they may die. However, many infants are normal at birth and do not develop problems until later in life.

 

Diagnosis

A family history and a physical examination revealing large bilateral irregular masses in the flanks strongly suggests polycystic kidney disease.

 

Investigations

Intravenous or retrograde pyelography: This reveals enlarged kidneys with elongation of the pelvis, flattening of the calyces and indentations caused by cysts.

  • Ultrasound: This shows that the kidneys are enlarged and that cysts are present.
  • Tomography: This shows large areas of cystic damage.
  • Urinalysis and creatinine clearance: These show what the kidney function is doing and show if there is any kidney failure.

 

Diagnosis must also rule out the presence of renal tumours.

 

Treatment

Polycystic kidney disease cannot be cured.

 

Management

The management of the disorder consists of the treatment of associated complications:

  • Urinary infection.
  • Bleeding into cysts.
  • Hypertension.
  • High blood pressure must be treated. This will slow down the progression of the renal failure.
  • Kidney failure eventually develops in all children and most adults. They will need dialysis and kidney transplantation.

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