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Polycystic Kidney Disease

A common disease in the population, its responsible for 5-10%
of ESRD (end stage renal disease) and Dialysis. Its an
autosomal dominant genetic disease that insidiously converts
the renal parenchyma to cysts. These cysts have no orientation
and can be any size. Eventually, functional nephrons are
obliterated and replaced. Along the way the cysts can bleed
(producing pain and hematuria, commonly mistaken for stones),
get infected (pyelo), or actually form stones. These cysts also
retain the ability to activate the RAS and can produce malignant
hypertension. A symptomatic patient can be diagnosed with an
Ultrasound. Theres no treatment, but manage complications
then do dialysis / transplant when they finally fail. Whats
critical in this disease is to identify the Extrarenal
manifestations. Cysts can form in the liver (cirrhosis), pancreas
(pancreatitis) and in the cerebral vasculature; they predispose
the patient to subarachnoid hemorrhage.



SAH, Liver, Pancreas
Hematuria, Flank Pain, Infxn, Stones, HTN
Ultrasound or CT to see cysts
Radially aligned cysts at birth
Barely compatible with life (peds only)
Incidental finding do nothing
Biopsy to rule out malignancy
Smoking, ESRD, VHL
Flank Pain, Flank Mass, Hematuria
Ultrasound or CT scan to find it
Needle to biopsy it
Excision, Rads / Chemo available
Epo Paraneoplastic Syndrome or Anemia

Simple Cysts
Sometimes an ultrasound or CT will reveal a cyst. If its simple no echoes and just one continuous mass (like a smooth balloon)
theres no need to worry about it. If symptoms develop (see
below) biopsy and then excise.
Complex Cysts
If that ultrasound or CT reveals a large or septated cyst it must
be biopsied to rule out malignancy. Do a needle-guided biopsy
and treat if its a cancer or for symptomatology.
Renal Cell Carcinoma
A Renal Cell Carcinoma can be detected from the ultrasound or
CT, which is why the biopsys done for complex cysts. However,
if the classic triad of flank pain, hematuria, and a flank mass is
seen its almost guaranteed to be cancer (though it may not always
be present). Patients are at increased risk with smoking, ESRD,
and with Von Hippel-Lindau. If a hematuria comes up on a U/A,
go ahead and get an ultrasound or CT the flank to visualize the
kidneys. Biopsy the lesions and resect. Since the renal cell
carcinoma spreads hematogenously disseminated spread may
have already occurred. Renal vein thrombosis is a real problem
with this cancer. Finally, there can also be either anemia or
Polycythemia. Either the cancer is stealing the blood (anemia) or
its actually producing an epo paraneoplastic syndrome.

Polycystic Kidney Disease

Simple Cyst

Septated Complex Cyst

Renal Cell Carcinoma