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GI, liver, kidney > ADPKD > Flashcards

ADPKD Flashcards

1
Q

What is Autosomal dominant polycystic kidney disease

A

Inherited disease causing Renal cycsts and extrarenal manifestations incl hepatic cysts, intracranial aneurysms and aortic root dialatiation

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2
Q

When do patietns with ADPKD typically present

A

30-60 - renal cysts start affecting kidney function

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3
Q

Presentitng symptoms ADPKD

A

Haematuria
Loin pain
HPTN
Recurrent UTIs
Kindye stones
Headache

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4
Q

Cause of ADPKD

A

Mutation in PKD1 or PKD2 coding for polycystin 1 or 2 (membran proteins)

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5
Q

What do renal cysts cause

A

Increased kidney volume
Compression of normal renal architecture
Intersitial fibrosis
Tubular atrophy
Progressive renal impairemtn

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6
Q

Which ADPKD type is more severe

A

Type 1 - more severe symptoms. 85% cases. Chromosome 16 rather than 4 in type 2

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7
Q

What can a headahce in ADPKD be a sign of

A

Ruptured berry aneurysm and subsequent SA haemorrhage

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8
Q

Signs of ADPKD on examination

A

Palpable kidneys/abdominal mass
hepatomegaly due to hepatic cysts
Abdominal wall hernias
Cardiac murmur

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9
Q

What cardiac complications can ADPKD cause

A

mitral valve prolapse, mitral regurgitation, aortic regurgitation and dilated aortic root
HPTN -> CHD, stroke, MI

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9
Q

Symptoms of renal filure in ADPKD

A

Develop on 50s
pERIPHERAL OEDEMA
sob
POOR appetitie and weight loss
Polyuria
Pruritis
Nausea

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10
Q

First line investigation ADPKD

A

Renal US scan

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11
Q

Why is creatinine and eGFR not first line in ADPKD

A

Nomral in early stgaes - not sensitive test

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12
Q

What criteria is used for diagnosis of ADPKD

A

Ravines

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13
Q

Ravines criteria for 15-29 years ADPKD

A

Presence of 3 or more renal cysts - uni or bilateral - 94.3% sensitivity for type 1 ADPKD 69.5% typ2 2 ADPKD

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14
Q

Ravines criteria 30-39

A

2 or more rena cysts 96.6% type 1 sensitivtiy + 94 type II

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15
Q

Ravines critiera 40-59 years

A

presence of 2 or more cysts in each kidney, which has a sensitivity of 92.6% for type 1 ADPKD and 88.8% for type 2 ADPKD

16
Q

What is diagnostic criteria for patients with no FH of ADPKD

A

> 10 cysts in each kidney

17
Q

What is the differnece between ADPKD and ARPKD

A

Autosomal recessive PKD presents soon anfter birth and may be no FH

18
Q

What is diffenrece between acquired CKD and ADPKD

A

Acquired a=occurs in patients with pre-existing renal disease and kidneys are usually smaller than ADPKD

19
Q

Tuberous scleroisis vs ADPKD

A

TS - other features present such as facial angiofibromas, ash leaf spots, shagreen patches, subungal fibromas, giant cell astrocytomas and ass with epilepsy

20
Q

Von hippel lindaru syndrome vs ADPKD

A

Both genetic
Renal cell carcinoma strongly ass w VHL but rare in ADPKD

21
Q

Meducllary cystic renal disease vs ADPKD

A

Kidneys smaller in MCR #Reucrrent gout is a common feature

22
Q

Mangement of ADPKD

A

Refer to nephrology
BP control
Renal pain management
Treatment UTIs (7-14 day ABS)
Treat kidney stones

23
Q

Managing ESR in ADPKD

A

Renal transplantation
Dialysis is second line - haemodialysis is preferred

24
Q

Medication in ADPKD

A

Tolvaptan

25
Q

What does tolvaptan do

A
  • slow cyst progression and renal insufficiency in adults with ADPKD
26
Q

When can use tolvaptan

A

IF - CKD stage 2 or 3 when start treat
Evidence rapidly progression

27
Q

Side effects of tolvaptan

A

vasopressin V2 receptor antagonist
thirst, polyuria and nocturia

28
Q

What can hepatic cysts cause

A

heartburn, nausea, early satiety and an increased abdominal circumference

29
Q

How prevent SAH in ADPKD

A

Offer MR angiography 1-5 year intervals for IC aneurysms
Can treat surically to avid rupture

30
Q

Pregnancy complications

A

Incidence of gestational HPTN and preeclampsia higher
HPTN - adveerse outcomes

31
Q

Factors indicating poor prognosis in ADPKD

A

Earlier age at diagnosis
Male sex
Type 1 ADPKD (mutation in PKD1)
Rapidly increasing kidney size
Hypertension

32
Q
A

GI, liver, kidney (50 decks)

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