Polycystic Kidney Disease

Polycystic Kidney Disease:  Characterized by multiple, bilateral, grape-like clusters of fluid filled cysts that grossly enlarge the kidneys, compressing and eventually replacing functioning renal tissue.  This disorder is inherited.  It appears in two distinct forms.  The infantile form causes stillbirth or early neonatal death.  Infants with this disease may survive for 2 years and then develop fatal renal, congestive heart, or respiratory failure.  Onset of the adult form is insidious but commonly becomes obvious between ages 30 and 50.  Rarely, polycystic kidney disease doesn't cause symptoms during an individual's life-time, and may only be found during autopsy.  Renal deterioration in the adult form of this disorder is slower than in the infantile form, but often leads to renal failure.

Cause:  The infantile form appears to be inherited as an autosomal recessive trait.  The adult form appears to be autosomal dominant trait.

Symptoms:

Infantile: 
Pronounced epicanthal folds (a vertical fold of skin on either side of the nose)
Pointed nose
Small chin
Floppy, low set ears
Huge bilateral masses on the flanks. 
Enlarged kidney

Adult : 
Hypertension 
Polyuria 
Signs and symptoms of urinary tract infection 
Lumbar pain 
Widening girth, and swollen or tender abdomen 
Hematuria 
Life threatening retroperitoneal bleeding 
Proteinuria 
Colicky abdominal pain
Enlarged kidney

Diagnostic test:

Retrograde pyelography reveals enlarged kidneys, with elongation of the pelvis, flattening of the calyces, and indentations caused by cysts.

IVP: neonate shows poor excretion of contrast medium

CT scan and ultrasound show kidney enlargement and the presence of cysts

Urinalysis and creatinine clearance tests indicate abnormalities.

Treatment:

Goal:  to preserve the renal parenchyma and avoid infection.

No known cure.  Careful management of associated urinary  tract infections and secondary hypertension may prolong life.

Antibiotic therapy 
Dialysis or kidney transplantation.

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