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A History of Polycystic Kidney Disease

By Conor Underwood

PKD affects just about all ethnic groups. This suggests that the initial PKD mutation/s first occurred early in our evolutionary history in an ancestor common to each ethnic group. Therefore, PKD has probably inflicted humans for a very long time, and no doubt has influenced the history and culture of mankind.

While some of the first anatomical dissections in the 16th century describe cystic kidneys, the first known case of PKD is that of a Polish king, Stefan Bathory, who ruled from 1576 till his death at 53 years of age in 1586. On a hunting trip the king developed fatigue and chest pain with increasing intensity for five days before repeatedly losing consciousness until shortly afterwards his heart slowed and eventually stopped. At the time, the cause of the king’s death was a mystery. Although the autopsy described the king’s kidneys, they were remarked as having a rather king-like quality “as being large, like those of a bull, with an uneven and bumpy surface”. Certainly, the king’s doctors did not believe that kidney failure was responsible for the death – in fact, the idea that changes in organ structure can cause disease was still new at time. It wasn’t until about 350 years after the king’s death, after PKD had been defined by the medical community, that historians and physicians first established the most likely cause of the king’s death: the build-up of toxins in the blood (uraemia) due to autosomal dominant PKD.

Following the definition PKD and its inheritance patterns in the early 20th century, scientific research into PKD increased exponentially. This is clearly illustrated by plotting the number of scientific articles published each year over time. The cumulative effect of all of this research lead to several major discoveries in the late 20th century (e.g. discovery of the genes that cause PKD) and 21st century (e.g. the harmful role of the hormone vasopressin). Now in 2018 tolvaptan (a drug that blocks vasopressin) has been approved by most major regulatory bodies as the first treatment for PKD. However, the story of PKD does not end with tolvaptan. Ongoing clinical and laboratory studies will be fundamental to determine whether novel therapies such as high-water intake can derail the progression of PKD and the development of related conditions. The story of PKD will end with a cure.

Reference and images: Torres, VE & Watson, ML. Polycystic kidney disease: antiquity to the 20th century. Nephrol Dial Transplant 2009; 13: 2690-6



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