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Flashcards in 51. Renal cyst disorders Deck (32):
1

Renal cyst disorders

1. Autosomal dominant polycystic kidney disease ( ADPKD)
2. Autosomal recessive polycystic kidney disease ( ARPKD)
3. Medullary cystic disease
4. Simple renal cysts
5. Complex renal cysts

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Autosomal dominant polycystic kidney disease - gross apperance

Numerous cysts in cortex and medulla --> bilateral enlarged kidneys ultimately destroy kidney parenchyma

3

Autosomal dominant polycystic kidney disease - present with ( clinical presentation)

1. flank pain
2. hematuria
3. hypertension
4. urinary infenction
4. urinary infenction
5. Progressive renal failure (50%)

4

Autosomal dominant polycystic kidney disease - mechanism

PKD1 mutation (85% - ch 16)
PKD2 mutation ( 15% - ch 14 )

5

Autosomal dominant polycystic kidney disease - death from

complications of chronic kidney disease or hypertension

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Autosomal dominant polycystic kidney disease - mechanism of hypertension

high renin production

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Autosomal dominant polycystic kidney disease is associated with

1. berry aneurysm
2. mitral valve prolapse
3. benign hepatic cysts

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Autosomal dominant polycystic kidney disease - treatment

ACE inhibitors or ARBs

9

Autosomal dominant polycystic kidney disease - gross apperance and age

Cystic dilation of collecting ducts often present in infancy

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Autosomal dominant polycystic kidney disease is associated with

1. congenital hepatic fibrosis
2. Significant oliguric renal failure in utero --> Potter sequence

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Autosomal dominant polycystic kidney disease - concer beyond neonatal period include

1. Hypertension
2. progressive renal insufficiency
3. portal hypertension ( from congenital hepatic fibrosis)

12

Medullary cystic disease - histology and mechanism

inherited disease causing tubulointestitial fibrosis and progressive renal insufficiency with inability to concetrate urine

13

Medullary cystic disease -gross

Medullary cysts usually not visualised shrunken kidneys on ultrasoun

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Medullary cystic disease - US

shrunken kidneys

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Medullary cystic disease - prognosis / complication

poor --> progressive renal insufficiency with inability to concetrate urine

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The majority of all renal masses

simple renal cysts

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SImple cysts are fileed with ? presentation?

filled with ultrafiltrate ( anechoic on US)
found incidentally and typically asymptomatic

18

Complex cysts -

Complex cysts, inducing these that are septrated , enhanced , or have solid components on imaging requires follow - up or removal due to risk of renal cell carcinoma

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Renal cyst disorder with increased renin secretion

Autosomal dominant polycystic kidney disease

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Renal tubular acidosis- definirion

disorder of the renal tubules that leads to normal annion gap ( hyperchloremic ) metabolic acidosis )

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Renal tubular acidosis types

.

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• Name the autosomal recessive disease that presents with multiple bilateral kidney cysts and renal failure in infancy.

Autosomal Recessive polycystic kidney disease (ARPKD) (infantile polycystic kidney disease)

23

• What is autosomal dominant polycystic kidney disease (ADPKD)?

ADPKD is Autosomal Dominant and characterized by multiple bilateral cysts that destroy the kidney parenchyma during adulthood

24

• A man has flank pain, hematuria, hypertension, and azotemia. His father and brother have the same issues. What might brain imaging reveal?

This is likely Autosomal Dominant polycystic kidney disease (ADPKD), associated with berry aneurysms

25

• A man has multiple liver and kidney cysts. Echocardiogram shows mitral valve prolapse. Do you expect the kidney cysts to be bilateral?

Yes (autosomal dominant polycystic kidney disease presents with bilateral renal cysts)

26

• A patient presents with renal medullary cystic disease. What are the hallmark characteristics that you expect to find on workup?

Small kidneys, fibrosis, progressive renal insufficiency, and urine-concentrating defects

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• What liver disease is associated with autosomal recessive polycystic kidney disease?

Congenital hepatic fibrosis leading to portal hypertension and liver cirrhosis

28

• What are the complications of autosomal recessive polycystic kidney disease in utero and after the neonatal period?

In utero: renal failure leading to Potter sequence; neonatal: hypertension, portal hypertension, progressive renal and liver insufficiency

29

• A man on dialysis is found to have multiple cortical and medullary cysts, all simple. Are these of concern to the nephrologist?

No (these are likely to be simple cysts that are not of clinical concern)

30

• An elderly patient is found to have several cortical renal cysts that are thin and filled with ultrafiltrate. Are these lesions malignant?

Unlikely (they are more likely to be simple cysts, which are a benign, asymptomatic, incidental finding in older adults)

31

• Renal biopsy of a patient with kidney failure shows tubulointerstitial fibrosis. Ultrasound fails to visualize medullary cysts. Prognosis?

The prognosis is poor (this is likely medullary cystic disease)

32

• A patient's CT scan incidentally shows septated, enhancing renal cysts. He is healthy and asymptomatic. Is follow-up imaging recommended?

Yes; these cysts have complex features (e.g., septation, enhancement, solid parts) and must be monitored (renal cell carcinoma risk)

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