DEAR DR. GOTT: I have read that Caroli first described this disease in 1958 but my great uncle William died in 1944 and the cause of death was Caroli’s renal disease. Can this be correct? I can forward a copy of his death certificate if required. Thank you.
DEAR READER: I have written about Caroli’s disease/syndrome before and also found that Jacques Caroli first described the two conditions (Caroli’s disease is more common, whereas Caroli’s syndrome is more complex and rare) in 1958 while in France. This, however, still leaves us with how your great uncle was purported to have died of the condition 14 years before the gentleman credited with its discovery reported it.
For this reason, I would question then authenticity of the death certificate. If it is indeed real, then I have no explanation for the discrepancy. Perhaps there are medical historians out there interested in trying to solve this mystery.
In the meantime, Caroli disease is a rare, inherited disorder that affects the dilatation of the bile ducts from the liver. This form is considered isolated/more simplistic. Caroli syndrome is even more rare and more complex. It is associated with high blood pressure, congenital hepatic fibrosis, liver failure and polycystic kidney disease.
If your family (especially the children, grandchildren, etc., of your great uncle) has a history of polycystic kidney disease or liver disorders, perhaps further testing to determine if either form of Caroli’s is present is in order.
You can read my prior article on Caroli here: http://askdrgottmd.com/patient-symptom-free-with-rare-liver-disorder/.
Readers who are interested in learning more about kidney disorders can order my Health Report “Kidney Disorders” by sending a self-addressed, stamped number 10 envelope and a $2 US check or money order to Dr. Peter Gott, PO Box 433, Lakeville, CT 06039. Be sure to mention the title or print an order form from my website, www.AskDrGottMD.com.
