Renal. Polycystic

Question 1

PKD. how is characterized?

Answer 1

by the presence of progressive cystic dilation of renal tubules.

Question 2

PKD. classification - AD ir AR

Answer 2

.

Question 3

PKD. which type most common?

Answer 3

AD PKD.

Question 4

PKD. ADPKD - symptoms?

Answer 4

usually asymtomatic until age 30 y/o (as cysts gradually enlarge with time)

Question 5

PKD. ADPKD - age?

Answer 5

symtoms occur after 30 y/o, about 10 proc. present in childhood

Question 6

PKD. ADPKD in what other organs? 4

Answer 6

liver, pancreas, spleen, epididymis

Question 7

PKD. ADPKD in what proc and when need dialysis?

Answer 7

in 50proc. of AD PKD patients ERDS to require dialysis by 60 y/o

Question 8

PKD. ADPKD. what symptoms if not ERDS?

Answer 8

in other 50 proc. have mildly reduced renal function and only require supportive care and BP control

Question 9

PKD. AR PKD. how common and severe?

Answer 9

less common than AD, but more severe

Question 10

PKD. usually asymtomatic until age 30 y/o (as cysts gradually enlarge with time)?

Answer 10

AD PKD

Question 11

PKD. Affect liver, pancreas, spleen, epididymis?

Answer 11

AD PKD

Question 12

PKD. AR PKD in what population?

Answer 12

infants and young children.

Question 13

PKD. AR PKD manifestation?

Answer 13

renal failure, liver fibrosis, portal hypertension. (IN CHILDREN)

Question 14

PKD. AR PKD. in what case can be death?

Answer 14

ARPKD can lead to death in the first few days of life if assoc. with IN UTERO OLIGURIA (oligohydramnios) leading to Potters sequence

Question 15

PKD. can lead to death in the first few days of life if assoc. with IN UTERO OLIGURIA (oligohydramnios) leading to Potters sequence?

Answer 15

AR PKD

Question 16

PKD. Which in children?

Answer 16

AR PKD

Question 17

PKD. which present as renal failure, liver fibrosis, portal hypertension?

Answer 17

AR PKD

Question 18

PKD. key fact. Patient has PKD and abruptly develops headache. in which type and what suspect?

Answer 18

AD PKD

suspect subarachnoid hemorrhage

Question 19

PKD. AD PKD. symptoms?

Answer 19

HTN, bilateral palpable masses, flank pain, history of UTI or gross hematuria.

Question 20

PKD. AD PKD. symptoms - what additional?

Answer 20

polyuria and nocturia

Question 21

PKD. AD PKD. symptoms - why pain? 3

Answer 21

due to rupture of cyst, infection or passage or stona

Question 22

PKD. HTN, bilateral palpable masses, flank pain, history of UTI or gross hematuria.?

Answer 22

AD PKD.

Question 23

PKD. pain due to rupture of cyst, infection or passage or stona

Answer 23

AD PKD.

Question 24

PKD. polyuria and nocturia

Answer 24

AD PKD.

Question 25

PKD. AR PKD - symptoms?

Answer 25

HTN, abdominal distension, flank masses - most commonly presenting findings. IDENTIFIED PRENATALLY.

Question 26

PKD. AR PKD - symptoms - in what age identified?

Answer 26

IDENTIFIED PRENATALLY.

Question 27

PKD. in both - what symptom?

Answer 27

may have large, palpable kidneys on abdominal examination.

Question 28

PKD. what finding does not suggest AD or AR PKD?

Answer 28

A single, simple renal cyst it does not require further evaluation

Question 29

PKD. Evaluation instrumental?2

Answer 29

UG (most common) or CT scan

Question 30

PKD. what is seen on instrumental?

Answer 30

multiple bilateral cysts will be present throughout the renal parenchyma and renal enlargement vill be visualised.

Question 31

PKD. need genetic evaluation?

Answer 31

available but often not necessary. AD PKD - PKD1 and PKD2 (called polycystins) AR PKD - PKHD

Question 32

PKD. screening criteria?

Answer 32

> 18 y/o + family history --> may be offered screening ultrasound

Question 33

PKD. treatment main idea?

Answer 33

prevent complications and decr. rate of progression to ESRD.

Question 34

PKD. a) early management of UTI to prevent renal cyst infection.

Answer 34

.

Question 35

PKD. b) BP control (ACEI, ARBs) - to reduce HTN induced renal damage and control proteinuria

Answer 35

.

Question 36

PKD. c) decr. sodium intake

Answer 36

.

Question 37

PKD. d) lipid control with satin - to decr. CV risk.

Answer 37

.

Question 38

PKD. when dialysis?

Answer 38

dialysis ir transplantation in ERDS

Question 39

PKD. high fluid why give?

Answer 39

High fluid intake needed to prevent kidney stones and may be helpful to prevent cyst development since ADH stimulate cyst growth.

Question 40

PKD. what medication may be used?

Answer 40

Vassopressin rec. antagonist are also been approved for use.

Question 41

UW. AD PKD. clinical presentation?

Answer 41

asymtomatic untill 30-40yo Flank pain, hematuria, HTN, palpable mases bilateral, CKD

Question 42

UW. AD PKD. extrarenal presentation?

Answer 42

cerebral aneurism, hepatic and renal cysts, mitral valve prolaps, aortic regurgitation, colonic diverticulosis, ventral and inguinal hernias

Question 43

UW. AD PKD. diagnosis?

Answer 43

UG shows multiple renal cysts

Question 44

UW. AD PKD. management?

Answer 44

aggressive control of risk factors for CV and CKD ACEI for HTN preffered Hemodialysis, renal transplan ESRD

Question 45

UW. AD PKD. flank pain - why may be?

Answer 45

due to cystic rupture or nephrolithiasis

Question 46

UW. AD PKD. HTN mechanism?

Answer 46

cyst enlargement --> localized renal ischemia --> incr. secretion of renin

Question 47

UW. AD PKD. why poliuria and nocturia?

Answer 47

due to cystic damage to nephron distal tubules with impaired sensitivity toward vasopressin signals --> urinary concentrating defect (ie mild nephorgenic DI)

Question 48

UW. AD PKD. renal dysfunction to CKD - why?

Answer 48

due to cystic progression

Question 49

UW. AD PKD. cerebral aneurysm. mechanism how occurs?

Answer 49

disruption of polycystins cause impaired vascular integrity, leading to cerebral aneurysm

Question 50

UW. AD PKD. cerebral aneurysm. what may contribute?

Answer 50

HTN also contributes by facilitating aneurysm progression and rupture