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This is a Kidney and Bladder condition.

Diffuse Cystic Renal Dysplasia and Hepatic Fibrosis

What is Diffuse Cystic Renal Dysplasia and Hepatic Fibrosis?

Diffuse Cystic Renal Dysplasia and Hepatic Fibrosis belongs to a group of diseases called ciliopathies. In ciliopathies, the hair-like structures of varying organs are either missing or misshapen leading to organ dysfunction. Normal ciliary function is also crucial for fetal organ development. This variant leads to kidneys that contain many small fluid-filled cysts and liver fibrosis. Having this variant can cause death within a few days of birth.

What are the signs & symptoms that develop in affected dogs?

Affected dogs may show abdominal pain and distention as the cysts grow. Affected dogs may also develop abnormal fluid in parts of the body, underdeveloped lungs, a cleft palate, and diaphragmatic abnormalities.

When do signs and symptoms develop?

Affected puppies may die shortly after birth.

How do vets diagnose this condition?

Diagnosis is made based on clinical history, genetic testing, and possibly ultrasonography (although the cysts may be too small to image clearly). Microscopic tissue examination may be needed for a definitive diagnosis.

How is this condition treated?

There is no cure for Diffuse Cystic Renal Dysplasia and Hepatic Fibrosis; all currently available treatments are symptomatic or supportive in nature. Frank discussions about the quality of life are warranted. Most affected puppies die shortly after birth.

What actions should I take if my dog is affected?

  • Keeping your affected dog comfortable at home is the best therapy you can provide.
  • Current therapies for other forms of kidney disease will likely not change the outcome.
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