Cyst in Time
Not So Hipp
ABC of PCKD
Potpourri
100
Enlarged kidneys
Cystic kidney
Hepatic fibrosis
What is autosomal RECESSIVE polycystic kidney disease?
100
Part of the regulatory cascade for EPO synthesis and a risk factor for renal cell carcinoma.
What is the von Hippel-Lindau tumor suppressor gene?
100
CHROMOSOME 16
CHROMOSOME 4
What are the gene locations for ADPCKD?
PKD-1 gene, chromosome 16 = 90%
PKD-2 gene, chromosome 4 = 10%
100
RISK OF MALIGNANCY.
What is ALMOST ZERO?
200
What is a simple renal cyst?
200
German ophthalmologist, 1904
Swedish pathologist, 1927
Who are EUGEN VON HIPPEL and ARVID LINDAU?
von Hippel = described ocular angiomas
Lindau = described angiomas of cerebellum and spine
200
Only about 5%.
What is the risk of a BERRY ANEURYSM in the setting of ADPCKD?
Routine MRI imaging for ADPCKD looking for aneurysms is controversial due to the low incidence of the aneurysm formation.
200
Ectatic collecting system
Recurrent calcium kidney stones
What is MEDULLARY SPONGE KIDNEY?
The renal pyramids are lined with calcification due to the ectatic collecting ducts and probable acid imbalance.
300
WOSNIAK
BOSNIAK
Who is the co-founder of Apple Computers (circa 1976)?
What is the classification system for renal cysts?
Dr. Morton Bosniak;; NYU Medical School
Renal cysts with SEPTATIONS and MICROCALCIFICATION with increasing size have increasing malignant potential.
300
What is ADENOMA SEBACEUM?
Adenoma sebaceum are angiofibromas of the skin typically seen in patients with TUBEROUS SCLEROSIS. Other features include HAMARTOMAS and ANGIOMYOLIPOMA of the KIDNEYS.
It is AUTOSOMAL DOMINANT.
300
KIDNEY
LIVER
PANCREAS
What are the common locations for cyst development in ADPCKD?
300
Genetic markers:
ANGIOMYOLIPOMA, RENAL CELL CARCINOMA
What are TUBEROUS SCLEROSIS (TSC gene) and VON-HIPPEL-LINDAU (VHL gene)?
400
Major complication in a very small percentage of ADPCKD patients?
What is a SACCULAR ANEURYSM? (BERRY ANEURYSM)
400
Neuro-ocular-cutaneous syndrome.
What is a PHAKOMATOSIS?
Phakomatosis is a constellation of abnormalities involving the eyes, CNS, and skin -- all are ectodermal origin embryologically.
Tuberous sclerosis is one such example.
400
What is a cerebellar hemangioblastoma?
400
What is one of Founding Dean Tomkowiak's favorite Star Wars figurines?
500
What is the first thing Andy seeks when he is imprisoned in "The Shawshank Redemption"?
What is a rock hammer?