Renal. Polycystic Flashcards

1
Q

PKD. how is characterized?

A

by the presence of progressive cystic dilation of renal tubules.

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2
Q

PKD. classification - AD ir AR

A

.

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3
Q

PKD. which type most common?

A

AD PKD.

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4
Q

PKD. ADPKD - symptoms?

A

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

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5
Q

PKD. ADPKD - age?

A

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

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6
Q

PKD. ADPKD in what other organs? 4

A

liver, pancreas, spleen, epididymis

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7
Q

PKD. ADPKD in what proc and when need dialysis?

A

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

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8
Q

PKD. ADPKD. what symptoms if not ERDS?

A

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

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9
Q

PKD. AR PKD. how common and severe?

A

less common than AD, but more severe

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10
Q

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

A

AD PKD

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11
Q

PKD. Affect liver, pancreas, spleen, epididymis?

A

AD PKD

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12
Q

PKD. AR PKD in what population?

A

infants and young children.

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13
Q

PKD. AR PKD manifestation?

A

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

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14
Q

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

A

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

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15
Q

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

A

AR PKD

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16
Q

PKD. Which in children?

A

AR PKD

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17
Q

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

A

AR PKD

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18
Q

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

A

AD PKD

suspect subarachnoid hemorrhage

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19
Q

PKD. AD PKD. symptoms?

A

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

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20
Q

PKD. AD PKD. symptoms - what additional?

A

polyuria and nocturia

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21
Q

PKD. AD PKD. symptoms - why pain? 3

A

due to rupture of cyst, infection or passage or stona

22
Q

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

A

AD PKD.

23
Q

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

A

AD PKD.

24
Q

PKD. polyuria and nocturia

A

AD PKD.

25
Q

PKD. AR PKD - symptoms?

A

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

26
Q

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

A

IDENTIFIED PRENATALLY.

27
Q

PKD. in both - what symptom?

A

may have large, palpable kidneys on abdominal examination.

28
Q

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

A

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

29
Q

PKD. Evaluation instrumental?2

A

UG (most common) or CT scan

30
Q

PKD. what is seen on instrumental?

A

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

31
Q

PKD. need genetic evaluation?

A

available but often not necessary.

AD PKD - PKD1 and PKD2 (called polycystins)
AR PKD - PKHD

32
Q

PKD. screening criteria?

A

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

33
Q

PKD. treatment main idea?

A

prevent complications and decr. rate of progression to ESRD.

34
Q

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

A

.

35
Q

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

A

.

36
Q

PKD. c) decr. sodium intake

A

.

37
Q

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

A

.

38
Q

PKD. when dialysis?

A

dialysis ir transplantation in ERDS

39
Q

PKD. high fluid why give?

A

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

40
Q

PKD. what medication may be used?

A

Vassopressin rec. antagonist are also been approved for use.

41
Q

UW. AD PKD. clinical presentation?

A

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

42
Q

UW. AD PKD. extrarenal presentation?

A

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

43
Q

UW. AD PKD. diagnosis?

A

UG shows multiple renal cysts

44
Q

UW. AD PKD. management?

A

aggressive control of risk factors for CV and CKD

ACEI for HTN preffered

Hemodialysis, renal transplan ESRD

45
Q

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

A

due to cystic rupture or nephrolithiasis

46
Q

UW. AD PKD. HTN mechanism?

A

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

47
Q

UW. AD PKD. why poliuria and nocturia?

A

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

48
Q

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

A

due to cystic progression

49
Q

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

A

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

50
Q

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

A

HTN also contributes by facilitating aneurysm progression and rupture