Cystic Kidney Diseases Flashcards
What are the different autosomal dominant cystic kidney diseases?
ADPKD
Tuberous sclerosis
Von Hippel Lindau Disease
Medullary Cystic Disease
What are the different autosomal recessive cystic kidney diseases?
ARPKD (childhood)
Juvenile onset Nephrophthisis
What are the different X linked cystic kidney diseases?
Orofacial digital syndrome Chromosomal disorders (trisomy 13/18/21)
What are the different developmental cystic disease?
Medullary sponge
Multicystic dysplastic
Pyelocalyceal cysts
What are the different acquired cystic diseases?
Simple cysts
Acquire cystic disease (ESRD)
Hypokalaemia related cysts
Where are simple cysts usually located?
Unilocular and in the cortex.
This does not cause kidneys to enlarge
How are simple cysts classified?
Bosniak classification to rule out cancer
Who most likely gets acquired renal cystic disease?
those who have been on dialysis
What symptoms do cysts cause?
Pain
Haematuria
How many people are affected by ADPKD?
1 in 800
What is the prognosis of ADPKD?
Approximately 50% reach ESRD by 60 years
What are extrarenal manifestations of ADPKD?
Berry aneurysms, SAH
Liver, pancreas, spleen cysts
MV prolapse
Diverticulitis
What questions do you need to ask someone who has PCKD?
FH - SAH, liver, pancreas, spleen, MV and diverticulitis
How do we manage ADPKD?
Monitory renal function and size Family screening Monitor BP and manage Vasopressin antagonists Nephrectomy if pain but otherwise no surgical role.
What is von Hippel-Lindau disease?
AD
Presents with lots of benign tumours in CNS, retina and kidneys. Haematuria
Onset in 20s and 30s.
