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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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Is Caroli's Syndrome Genetic?

hi, our 3 month old son has been diagnosed wit Carolis syndrome,my partners dad passed away from liver problems and we know that it is genetic,my partner is been checked for the same thing but we want to know if our son is going to suffer the same path as my partners dad?
Thu, 31 Mar 2016
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General & Family Physician,  's  Response
Hi I can understand your concern...

Yes, caroli disease is genetic disease.
It may be simple dilatation of bile duct or it is associated with hypertension and fibrosis known as caroli syndrome.....

Let me know you condition is dangerous .you can consult surgeon for treatment....
Ursodiol can be used.
If limited to one lobe then lobectomy might done.
Liver transplantation is also another option...
Patient usually die by complications like repeated cholangitis....
Death usually within 5 to 7 year according to various literature...

Take care...

Dr.Parth....
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Is Caroli's Syndrome Genetic?

Hi I can understand your concern... Yes, caroli disease is genetic disease. It may be simple dilatation of bile duct or it is associated with hypertension and fibrosis known as caroli syndrome..... Let me know you condition is dangerous .you can consult surgeon for treatment.... Ursodiol can be used. If limited to one lobe then lobectomy might done. Liver transplantation is also another option... Patient usually die by complications like repeated cholangitis.... Death usually within 5 to 7 year according to various literature... Take care... Dr.Parth....