Week 3 - D - Pathology 2 - PKD, Kidney&Bladder carcinoma Flashcards

1
Q

What is the absence of one of the kidneys known as?

A

Kidney agenesis

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2
Q

What level is the right and left kidneys located between?

A

Right kidney - vertebra L1-L3

Left kidney - vertebra T12-L2

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3
Q

Why does the right kidney sit lower than the left?

A

Due to the presence of the liver the right kidney sits lower

The spleen is small and does not take up as much space as the liver

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4
Q

fusion at either pole of the kidney, Kidneys usually lie lower ie beneath inferior mesenteric artery WHat is this?
What is a duplex system?

A

This is a horseshoe kidney

A duplex system means there are two ureters coming from the same kidney and inserting into the bladder

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5
Q

What is the most common polycsytic disease in people?

A

Autosomal dominant polycystic kidney disease (ADPKD)

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6
Q

What is the most common polycystic disease typically diagnosed in children and infants ?

A

This autosomal recessive polycystic kidney disease (ARPKD)

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7
Q

ARPKD - how does it present?

A

Present at birth, first year.

Bilateral flanks masses.

Dilated distal tubes, collecting ducts, enlarged kidneys at birth.

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8
Q

What can ARPKD lead to?

A

This can lead to kidney failure in children which can result in death

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9
Q

What can result from the poor kidney function in autosomal recessive polycystic kidney disease? It gives rise to a child with poor lung function and deformity of the face and ears

A

This can lead to oligohydroaminos as kidney function does not work and this causes a syndrome cause Potter’s syndrome

Potter’s syndrome classically occurs due to bilateral renal agenesis but can be seen in ARPKD

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10
Q

How are the kidneys involved in olioghydramnios that can lead to Potter’s syndrome?

A

Fetal urine production begins in early gestation and comprises the majority of the amniotic fluid in the second and third trimesters of pregnancy - therefore need working kidneys

Oligohydramnios occurs if the volume of amniotic fluid is less than normal for the corresponding period of gestation.

The fetal urine is critical to the proper development of the lungs

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11
Q

Adult Polycystic Disease is of autoosmal inheritance What genes/chromosomes are there mutations?

A

Chromosome 16 in 90% (ADPKD 1), others
Chromosome 4 (ADPKD 2).

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12
Q

How does adult polycystic disease usually present?

A

Usually presents in adult life with bilateral abdominal mass, haematuria and chronic renal failure

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13
Q

What are the 4Bs of adult polycystic renal disease?

A

Big - abdominal mass

Bilateral

Bleeding - haematuria

Berry Aneurysms in the Circle of Willis

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14
Q

What can the Berry Aneurysms lead to?

A

Berry aneursyms are small weak vessels that cause aneusyms to form and can lead to rupture causing subarachnoid haemorrhage

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15
Q

Due to the chronic renal failure seen in ADPKD, how can this lead to left ventricular hypertrophy?

A

This causes secondary hypertension which will lead to left ventricular hypertrophy when trying to pump blood around the body

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16
Q

What will ultrasound of the kidneys show? Can the cysts spread? (do they have a functional effect)

A

Ultrasound shows the large cystic kidneys

In 1/3 – cysts in liver, pancreas, lung.

No functional effect.

17
Q

Which chromosome’s can cause ADPKD? (which is most common) What are the symptoms/signs?

A

ADPKD1 on chromosme 16 accounts for 90%

ADPKD2 on chromosme 4 is the other 10%

Bilateral abdominal swelling and Berry aneusyms

Secondary hypertension due to chronic renal failure

Cystic kidneys on US

Haematuria

Abdminal pain

18
Q

What is the best treatment option for patients with ADPKD?

A

ACE inhibitors

19
Q

There are other causes of benign renal tumours What is a renal fibroma?

A

Tumour of medullary origin that is white nodules

20
Q

Adenoma – yellowish nodules usually less than 2cm in size and are cortical in the kidney
What is the tumour of fat, muscle and blood vessels known as?

A

This is known as an Angiomyolipoma

21
Q

What genetic condition are angiomyolipomas associated with?

A

Associated with tuberous sclerosis

22
Q

Where are the mutations in tuberous sclerosis? What are the presenting symptoms of tuberous sclerosis?

A

TSC1 mutation on chromosome 9 and TSC2 mutation on chromosome 16

Ash leaf macule

Infantile seizures

Periungual fibroma

Longitudinal ridging

Know also Angiomyolipoma

23
Q

What is the renin producing tumour in the kidneys that can lead to secondary hypertension?

A

Juxtaglomerular cell tumour (JGCT)

24
Q

commonest intra-abdominal tumour in children. Abdominal mass. Arises from residual primitive renal tissue. What is this?

A

This is a nephroblastoma - aka Wilm’s tumour

25
Q

How can a nephroblastoma present in children?

A

Can present with abdominal mass and pain, vomiting

26
Q

What is the most common type of cancer of the collecting system in urinary tract? (from renal pelvis to proximal urethra)

A

This is a transitional cell carcinoma

27
Q

What is the common type of renal tumour in adults? It is known as clear cell carcinoma due to its histological appearance

A

This is renal cell carcinoma

Known as clear cell as the cells are clear on hisstology

28
Q

How does renal cell carcinoma present?

A

Presents with abdominal mass, haematuria, flank pain, general features of malignant disease.

29
Q

What are 2 paraneoplastic features of renal cell carcinoma?

A

Increased erythropoetin (EPO) production - causing polycythaemia

PTH production causing hypercalcaemia (also associated with renin and ACTH production)

30
Q

Yellow colour, with solid, cystic, necrotic and haemorrhagic areas is how the kidney looks when taken out in renal cell carcinoma What can he renal cell carcinoma extend into? what condition is it associated with?

A

Can be assoicated with von Hippel-Lindau syndrome

31
Q

Transitional epithelium - from pelvicalyceal system to urethra. What percentage of kidney cancers do renal cell carcinoma account for? What percentage of bladder cancers do transitional cell carcinoma account for?

A

Kidney cancers due to RCC is around 90%

Bladder cancers due to TCC is around 90%

32
Q

if there is painless haematuria, what do you think this probably is?

A

Bladder cancer

33
Q

What is transitional cell carcinoma risk factors?

A

Linked with smoking, and working with dyes and rubber

34
Q

What is the first line treatment of small vessel vasculitis and which drug used here can also predispose to transitional cell carcinoma?

A

IV steroids + cyclophosphamide

TCC risk factors

* Linked with smoking * Working with dyes * Working with rubber * Cyclophosphamide

35
Q

Where do the majority of TCC occur? It is found at the base of the bladder

A

Trigone

36
Q

What is squamous cell carcinomas of the urinary tract linked to?

A

Linked to the parasitic infection via Schistosomiasis found in egypt and other african countries

Can see the schistosomiasis eggs here

Also linked to smoking

37
Q

Commonest malignant bladder tumour in children?

A

Embryonal Rhabdomyosarcoma