Renal Cystic Disease Flashcards Preview

Y4 Nephrology/Urology > Renal Cystic Disease > Flashcards

Flashcards in Renal Cystic Disease Deck (16)
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1

what adult age range is affected by polycystic kidneys ?

40-60yrs

2

what are the two types of PCK?

PKD1 and 2

3

what mutation is PKD1?

Chr16, polycystin 1

4

what mutation is PKD2?

Chr4, polycystin 2:

5

what structures are affected in PKD1?

cell-cell and cell- matrix membrane receptor

6

what structures are affected in PKD2?

Ca2+ channel that interacts ̄c polycystin 1

7

what is the presentation of polycystic kidney disease? MISSHAPES

Mass: abdo mass and flank pain
􏰀 Infected cyst
􏰀 Stones
􏰀 SBP↑
􏰀 Haematuria or haemorrhage into cyst
􏰀 Aneurysms: berry → SAH
􏰀 Polyuria + nocturia
􏰀 Extra-renal cysts: liver
􏰀 Systolic murmur: mitral valve prolapse

8

what medication is used to treat HTN in PKA?

ACEI

9

what general measures can be taken for PKD?

↑ water intake, ↓ Na, ↓ caffeine (may ↓ cyst formation) 􏰁 Monitor U+E and BP
􏰁 Genetic counselling
􏰁 MRA (MRI and angiography) screen for Berry aneurysms

10

what is the inheritance of PKD?

AD

11

what gender is medullary sponge kidney more common?

females

12

is renal function normal with medullary sponge kidney ?

yes

13

is renal function normal with PKD?

no
- 70% ESRF at 70yrs

14

what are some DD for renal enlargement?

Polycystic kidneys: ADPKD, ARPKD, TS

􏰀 Hypertrophy 2O to contralateral renal agenesis

􏰀 Obstruction (hydronephrosis)

􏰀 Neoplasia: RCC, myeloma, amyloidosis

􏰀 Occlusion (renal vein thrombosis)

􏰀 Systemic: early DM, amyloid

15

what is tuberous sclerosis ?

AD condition ̄c hamartomas in skin, brain, eye, kidney

16

what are three features of the skin in tuberous sclerosis ?

nasolabial adenoma sebaceum, ash-leaf macules, peri-ungual fibromas