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SALMAN HARRISON'S > 309 - Polycystic Kidney Disease and other disorders of tubule growth and development > Flashcards

309 - Polycystic Kidney Disease and other disorders of tubule growth and development Flashcards

1
Q

PKD is usually __ in adults, and __ in children.

A

dominant

recessive

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2
Q

The main pathology in PKD is __

A

Ciliopathies

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3
Q

What are the 4 damage mechanism in PKD?

A

uncontrolled proliferation of cells
increased fluid excretion
decreased differentiation of cells
extra cellular matrix increase

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4
Q

PKD 1 is __ common than PKD 2

A

more

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5
Q

The pathophysiology of ADPKD is of:
__ decreased function -> __ signaling damage -> increased adenylyl cyclase function + decreased phosphodiesterase-> increase of __-> increase in __ -> promoting cell proliferation and fluid secretion -> __ formation.

A 
cilia
calcium
cAMP
protein kinase A
cyst
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6
Q

ADPKD is __ in the first stages.

A

asymptomatic

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7
Q

ADPKD symptoms include: 5

A 
BP increase
flank pain
abdominal mass
hematuria 
nephrolithiasis
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8
Q

In ADPKD the cyst develop __, starting with a small number at the age of __, increasing into the thousand during the _ decade.

A

bilaterally
30
4

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9
Q

The kidney can increase up to _ times in length, and _ times in weight in ADPKD patients.

A

4

20

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10
Q

More than half of the nephrolithiasis in ADPKD is made of __ acid.

A

uric

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11
Q

The 2nd most common mortality cause in ADPKD is __.

A

infection

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12
Q

How will you differentiate between malignancy and complex cyst in ADPKD patients?

A

MRI/CT

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13
Q

___uria is not a common finding in ADPKD.

A

protein

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14
Q

The most common extra renal symptoms in ADPKD is __ cysts; Usually in ___ women.

A

hepatic

Polyparous

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15
Q

What are the main risk factors for ESRD in ADPKD patients? 5

A 
young age
HTN
gross hematuria 
polyparous 
large kidney
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16
Q

__ morbidity increases the mortality of ADPKD patients.

A

cardiac

17
Q

RCC is a rare, uncommon complication, which in ADPKD patients tend to be more ___, __, __.

A

bilateral
multicentric
sarcomatoid

18
Q

ICA (__) is 4-5 times more common in ADPKD. 20-50% will experience warning __. Initial examination will be done by __, and diagnosis with __.

A

intracranial aneurysm
headache
CT
lumber puncture

19
Q

Diffuse arterial dolichoectasias can occur, leading to __

A

stroke

20
Q

Which heart valve is more likely to get involved in ADPKD?

A

MVP

21
Q

ADPKD increases the frequency of __ and __.

A

hernias

diverticula

22
Q

ADPKD diagnosis is usually based on __ and __ evidence.

A

family history

cyst

23
Q

Age criteria for ADPKD:
15-29: ___
30-59: __
>60: __

A

2 cyst in at least one kidney
2 cysts in each kidneys
4 cysts in each kidney

24
Q

Which imaging is used for the screening of in risk ADPKS population?

A

US

25
Q

ADPKD treatment is __ specific.

A

not

26
Q

What is the goal BP in ADPKD patients?

A

140/90

27
Q

Treating infected cysts is done by lipid soluble Abx: 3

A

quinolones
chloramphenicol
TMP SMX

28
Q

Over 50% of ADPKD will eventually require ___

A

dialysis

29
Q

ARPKD can be diagnosed intra __ or in the __ stage.

A

uterine

neonatal

30
Q

ARPKD diagnostic signs include: 4

A

renal hypertrophy
decreased fetal urine
oligohydramnios
pulmonary hypoplasia

31
Q

New born die usually __, 60% after _ month.

A

post partum

1

32
Q

What hepatic signs are possible in ARPKD? 5

A 
biliary dysgenesis
periportal fibrosis
congenital hepatic fibrosis
Caroli disease (dilatation of large intrahepatic ducts)
portal HTN
33
Q

ARPKD diagnosis is possible from the __ week of pregnancy.

A

24th

34
Q

For Caroli disease treatment includes __ shunt.

A

port systemic

35
Q

What other disease involve large renal cysts? 2

A

tuberous sclerosis

von Hippel- Lindau disease (VHL)

36
Q

What is the most common tuberous sclerosis finding?

A

renal angiomyolipoma

37
Q

In tuberous sclerosis there is a higher frequency of __, and cysts > _ cm will require __ surgery. The cyst tend to __ less.

A

RCC
4
prophylactic
bleed

38
Q

VHL is an __ RCC.

A

acquired

39
Q

Which genetic disease also involve tubules formation?

A 
AD tubulointerstitial kidney disease 
ADTKD (MCKD1)
ADTKD UMOD (MCKD2)
ADTKD-REN
nephronophthisis

SALMAN HARRISON'S (163 decks)

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