23. Polycystic Kidney Disease Flashcards Preview

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Flashcards in 23. Polycystic Kidney Disease Deck (15)
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1
Q

what is polycystic kidney disease (PKD)

A

genetic condition where the kidneys develop multiple fluid-filled cysts. Kidney function is also significantly impaired.
the cysts arise from the renal tubules

2
Q

which is the more common type of PKD

autosomal dominant or autosomal recessive

A

autosomal dominant type

3
Q

how do you diagnose PKD

A

ultrasound scan and genetic testing

4
Q

what are the pain autosomal dominant genes and what chromosome are they on

A
  • PKD-1: chromosome 16 (85% of cases)

* PKD-2: chromosome 4 (15% of cases)

5
Q

what extra renal manifestations can you get with PKD

A
  • Cerebral aneurysms
  • Hepatic, splenic, pancreatic, ovarian and prostatic cysts
  • Cardiac valve disease (mitral regurgitation)
  • Colonic diverticula
  • Aortic root dilatation
6
Q

what are the main complications with PKD

A
  • Chronic loin pain
  • Hypertension
  • Cardiovascular disease
  • Gross haematuria can occur with cyst rupture (this usually resolves within a few days
  • Renal stones are more common in patients with PKD
  • End-stage renal failure occurs at a mean age of 50 years
7
Q

autosomal receive PKD is rarer and more severe

when does it often present and how

A

presents in pregnancy with oligohydramnios as the fetus does not produce enough urine.

8
Q

what is the management of PKD to slow the development of cysts

A

Tolvaptan (a vasopressin receptor antagonist

9
Q

What are the other management options of PKD that are mainly supportive of the complications

A
  • Antihypertensives for hypertension.
  • Analgesia for renal colic related to stones or cysts.
  • Antibiotics for infection. Drainage of infected cysts may be required.
  • Dialysis for end-stage renal failure.
  • Renal transplant for end-stage renal failure.
10
Q

what advice can you give people who have PKD

A
  • Genetic counselling
  • Avoid contact sports due to the risk of cyst rupture
  • Avoid anti-inflammatory medications and anticoagulants
  • Regular ultrasound to monitor the cysts
  • Regular bloods to monitor renal function
  • Regular blood pressure to monitor for hypertension
  • MR angiogram can be used to diagnose intracranial aneurysms in symptomatic patients or those with a family history
11
Q

what is the diagnostic criteria called for diagnosing PKD

A

modified ravines criteria

12
Q

there are a lot of genetic causes of cystic renal disease , name some of them

A

see notes

13
Q

what is the most common cause of renal failure due to a cystic renal disease

A

ADPKD accounts for 10% of the dialysis population

14
Q

name some extra renal complications of ADPKD

A

• Extrarenal (berry aneurysms, liver, pancreas, spleen cysts, mitral valve prolapse,? diverticuli)

15
Q

why are people with PKD monitored from a young age

A

there is a high mortality secondary to CVD so BP control from childhood is essential