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Renal > 3.1: Congenital Abnormalities > Flashcards

3.1: Congenital Abnormalities Flashcards

1
Q

What is Agenesis of the kidneys?

A

Absence of one or both kidneys as they fail to develop during embryonic growth

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2
Q

What is hypoplasia of the kidneys?

A

Small kidneys

Develop normally

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3
Q

What is a horseshoe kidneys?

A

Fusion of the kidneys at either pole (usually lower pole)

Causes one horseshoe shaped kidney

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4
Q

What is duplex systems of the kidney?

A

This is two ureters

Can also be two renal pelvises

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5
Q

What does this photo show?

A

Kidney Agenesis

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6
Q

What does this photo show?

A

Kidney Hypoplasia

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7
Q

What does this photo show?

A

Horseshoe kidney

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8
Q

What does this photo show?

A

Duplex Systems (Duplex Ureter)

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9
Q

Describe simple cysts?

Cause?

A

These are very common

Can be small or large

Cause no functional disturbance
Secondary to other conditions (Eg: Native kidneys on long term dialysis)

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10
Q

What does this photo show?

A

Simple Kidney Cyst (Large)

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11
Q

What does this photo show?

A

Simple Cysts (Small)

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12
Q

Describe the infantile version of polcystic disease?

  • Rare or common?
  • Pattern of inheritance?
  • Signs?
A

Very Rare

AUTOSOMAL RECESSIVE (ARPKD - autosomal recessive polycystic kidney disease)

Causes uniform, bilateral renal enlargement. Elongated cysts (dilation of medullary collecting ducts)

No gross distortion of kidney

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13
Q

What is infantile polycystic kidney disease associated with?

What is the prognosis?

A

Associated with congenital hepatic fibrosis

Prognosis:

In perinatal group causes terminal renal failure

Less severe cases can survive for a few months

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14
Q

Describe adult polycystic disease?

A

Most common form of congenital cystic disease

Causes cyst formation and kidney enlargement

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15
Q

Describe the pattern of inheritance in adult polycystic kidney disease?

A

AUTOSOMAL DOMINANT

ADPKD 1 = Chromosome 16 defect

ADPKD 2 = Chromosome 4 defect

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16
Q

Describe the usual presentation of adult polycystic kidney disease?

A

Usually middle aged

  • Abdominal mass
  • Haematuria
  • Hypertension
  • Chronic Renal Failure
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17
Q

Describe the affect of polycystic kidney disease on the adult kidney?

A

Massive Renal Enlargment (>1kg)

Multiple cysts of varying size

1/3 of people there are also cysts in liver, pancreas, lung etc but these are non functional

18
Q

Describe a complication of adult polycystic kidney disease?

A

Association with Berry Aneurysms in the Circle of Willis

Can lead to subarachnoid haemorrhage

19
Q

What does this photo show?

A

Adult Polycystic Kidney Disease

20
Q

What is a Fibroma?

How is this found?

Symptoms?

Colour?

A

Benign Renal Tumour

Medullary Tumour

Often found incidentally

Asymptomatic

White

21
Q

Describe a renal adenoma?

A

Another benign renal tumour

Cortical tumour

Yellow

22
Q

Describe an angiomyolipoma?

Associated with?

A

Benign renal tumour

Mixture of fat, muscle and blood vessels

Associated with Tuberous Sclerosis

Can be multiple and bilateral

23
Q

Describe a JGCT?

Symptoms?

A

Juxtaglomerular Cell Tumour

Very rare

Causes extra production of renin leading to secondary hypertension

24
Q

What is the most common benign tumour of the kidneys?

A

Angiomyolipoma

25
Q

What does this photo show?

A

Angiomyolipoma

26
Q

What is the most common intra-abdominal tumour in children?

A

Nephroblastoma (Wilms’ Tumour)

27
Q

Describe nephroblastoma?

What does this arise from?

A

Also called Wilms’ tumour

Malignant Renal Tumour

Commonest intra-abdominal mass in children

Arises from residual primitive renal tissue

28
Q

Describe urothelial carcinomas?

A

Malignant Renal Tumour

Seen in renal pelvic and calyces

Most common type of bladder cancer/cancer of the ureter

29
Q

Describe renal cell carcinoma?

Arises from?

Presentation?

Most common type?

A

Malignant renal tumour

Arises from renal tubular epithelium

Presents with haematuria, flank pain and abdominal mass

Most common type is clear cell

30
Q

What is the most common primary renal tumour in adults?

A

Renal Cell Carcinoma

31
Q

Describe the presentation of renal cell carcinoma?

A

55-60 years

Male most common

Abdominal Mass

Flank Pain

Haematuria

May have hypercalcaemia

Polycythaemia

32
Q

Describe the appearance of a renal cell carcinoma?

A

Large, well circumscribed mass on renal cortex

Yellow colour

Solid cystic, necrotic and haemorrhagic areas

Renal vein extension is common

33
Q

What does this photo show?

A

Renal Cell Carcinoma

34
Q

Describe metastasis of renal cell carcinoma?

Most common type of renal cell carcinoma?

The tumour cells are rich in..?

What type of grading used?

A

Blood borne to lungs, liver, brain, etc.

Clear cell type is most common type

Tumour cells rich in glycogen and lipid

Fuhrman

35
Q

Describe transitional cell carcinoma?

Risk factors?

Most common symptom?

A

Transitional epithelium lines from pelvicalcanyeal system to the urethra

90% of bladder cancers are transitional carcinomas

Risk factors include dyes, rubber, analgesia, smoking

Most common symptom is haematuria

36
Q

Types of transitional cell carcinoma?

A

Papillary

Solid

37
Q

Where do 75% of TCCs occur?

What does this cause?

A

Trigone

Causes ureteric obstruction

38
Q

What does this photo show?

A

Transitional Cell Carcinoma

39
Q

What does this photo show?

A

Transitional Cell Carcinoma

40
Q

Commonest malignant bladder tumour in children?

A

Embryonal Rhabdomyosarcoma

Renal (34 decks)

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