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SM Renal > Congenital Abnormalities > Flashcards

Congenital Abnormalities Flashcards

1
Q

What is agenesis

A

Absence of one or both kidneys

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2
Q

What is hypoplasia

A

Small kidneys but normal development

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3
Q

What happens in horseshoe kidney

A

fusion at either pole - usually lower

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4
Q

What are the consequences of cystic disease

A

Normally no functional disturbance

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5
Q

What happens in infantile polycystic kidney disease

A

Terminal renal failure and less severe cases can survive for some months

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6
Q

How is polycystic disease in passed on

A

Autosomal recessive inheritence (ARPKD)

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7
Q

Describe the appearance of infantile polycystic kidney disease

A

Elongated cysts
Dilatation of medullary collecting ducts
Can survive for a few months but most patients die before this
Smooth

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8
Q

What conditions is infantile polycystic disease associated with

A

Congenital Hepatic Fibrosis

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9
Q

What is the least rare form of congenital cystic disease

A

Adult polycystic disease

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10
Q

How is adult polycystic kidney disease inherited

A

Autosomal dominant inheritance

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11
Q

What 2 chromosomes are involved in adult polycystic kidney disease

A

16 (90%) ADPKD1

and 4 APKD2

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12
Q

When do patients with adult polycystic disease present

A

usually middle adult life

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13
Q

What do patients with adult polycystic disease present with

A

abdominal mass
haematuria
hypertension
CRF

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14
Q

Where else in the body are cysts seen in adult polycystic disease

A

Liver
pancreas
lung

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15
Q

What happens to the size of the kidneys in adult polycystic disease

A

massive bilateral renal enlargement >1kg

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16
Q

What is adult polycystic disease associated with

A

Berry Aneurysms in circle of Willis > subarachnoid haemorrhage

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17
Q

Cystic lesions on the liver always alter function. True or false

A

False - usually don’t affect the function

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18
Q

What is the commonest benign renal tumour

A

Fibroma

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19
Q

Describe the appearance of a fibroma

A

Medullary origin
white nodules
few mm in size

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20
Q

Describe the appearance of a benign adenoma

A

Yellowish nodules

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21
Q

Describe the appearance of a benign Angiomyolipoma

A

Mixture of fat, smooth muscle and blood vessels

can be multople and bilateral

22
Q

What is a benign angiomyolopoma associated with

A

Tuberous sclerosis

23
Q

What does JGCT stand for

A

Juxtaglomelular apparatus tumour

24
Q

What do JGCT produce

A

renin > secondary hypertension

25
Q

What is the likelihood that an angiomyolipoma will become malignant

A

none - it has no malignant potential

26
Q

What is the commonest intra-abdominal tumour in children

A

Nephroblastoma (Wilm’s Tumour)

27
Q

What does a nephroblastoma arise from

A

residual primitive renal tissue

28
Q

Where are urothelial carcinomas found specifically

A

renal pelvis and calyces

29
Q

Where do renal cell carcinomas arise form

A

renal tubular epithelium

30
Q

What are 3 types of renal cell carcinomas

A

Clear cell Ca
hypernephroma
Grawitz tumour

31
Q

What is the commonest primary renal tumour in adults

A

Renal cell carcinoma

32
Q

Who is most likely to develop a renal cell carcinoma

A

55-60 years old

M:F 2:1

33
Q

How do renal cell carcinomas present

A

Abdominal mass
haematuria
localised flank pain
general features of malignant disease

34
Q

What can polycythaemia secrete

A

Erythropoeietic stimulating substance

35
Q

Describe the appearance of a renal cell carcinoma

A

Solid
cystic
necrotic
haemorrhagic areas

36
Q

What complication is common in renal cell carcinoma

A

renal vein extension- some may have stenosis in the vena cava and up to the right atrium

37
Q

How do renal cell carcinomas metastasise

A

Blood-borne metastatic spread to lung, bone

Lymphatic spread later

38
Q

What is the grading system used for renal cell carcinomas

A

Fuhrman

39
Q

Where do transition cell carcinomas arise

A

transitional epithelium - from pelvicalyceal system to urethra

40
Q

What are some risk factors for developing a transitional cell carcinoma

A

Aniline dyes
rubber industry
hydrocarbons
smoking

41
Q

What is the commonest presenting symptom of a transitional cell carcinoma

A

Haematuria

42
Q

Where do the majority of transitional cell carcinomas arise

A

in the region of trigone - ureteric obstruction

43
Q

What is meant by pTa, pT1

A

stromal invasion of a transitional cell carcinoma

44
Q

What is meant by a pT2

A

Muscle invasion of a transitional cell carcinoma

45
Q

where do transitional cell carcinomas spread to

A

local lymph nodes (obturator nodes in the pelvis), lung, liver

46
Q

How likely is it that a transitional cell carcinoma would recur

A

Very - tumours often progress to a higher grade / stage as well

47
Q

What is the commonest malignant bladder tumour in children

A

Embryonal Rhabdomyosarcoma

48
Q

Where are squamous carcinomas found in the kidneys

A

Calculi (squamous metaplasia)

49
Q

Where are adenocarcinomas found in the kidneys

A

extroversion (glandular metaplasia)

50
Q

What do squamous carcinomas often produce

A

keratin

SM Renal (23 decks)

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