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Year 3 > Case 23 - Glomerulonephritis And Polycystic Kidneys > Flashcards

Case 23 - Glomerulonephritis And Polycystic Kidneys Flashcards

1
Q

What are the main differences between nephrotic and nephritic syndrome?

A

Nephrotic - due to a loss of podocytes, leads to loss of protein and albumin (proteinuria, hypoalbuminaemia, oedema)

Nephritic - due to the formation of immune complexes in the glomeruli, leads to a loss of blood and protein (haemturia, proteinuria, oliguria, low GFR, hypertension)

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2
Q

What are the typical features of nephrotic syndrome?

A

Proteinuria
Hypoalbuminaemia
Odema
Hyperlipidema

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3
Q

What are the complications of nephrotic syndrome?

A

Thromboembolism (DVT, PE) - this is due to the loss of antithrombin III in the kidneys, resulting in hypercoagulation of the renal vein which could lead to renal vein thrombosis

Hyperlipidemia - low serum albumin causes the liver to componsate and produce more albumin, which has a side effect of lipid production

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4
Q

What are the main features of nephritic syndrome?

A 
Haematuria 
Proteinuria (small amounts)
Oliguira 
Low GFR
Hypertension
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5
Q

What are the primary causes of nephrotic syndrome?

A

Minimal change disease
Focal segmental glomerulosclerosis (FSGS) - scaring in glomerulus
Membranous glomerulonephritis - immune deposits on basement membrane
Mesangiocapillary GN (MCGN) - immune deposits on basement membrane and mesangial cells

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6
Q

What are the secondary causes of nephrotic syndrome?

A

Diabetic nephropathy
Hepatitis B/C - can cause membranous nephropathy or MCGN
Lupus
NSAIDS

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7
Q

How is nephrotic syndrome managed?

A

Furosemide to reduce oema
Fluid and salt restriction
ACEi/ARB - this help to reduce protein loss as they are renoprotective
Anticoagulants - to avoid PE/DVT risk caused by loss of antithrombin III

Ultimately - treat underlying cause e/g, hepatitis, systemic disease

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8
Q

What is the most common cause of glomerulonephritis?

A

IgA nephropathy

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9
Q

How is IgA nephropathy diagnosed?

A

Renal biopsy - finds IgA deposits in the mesangial cells

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10
Q

What is the pathophysiology of IgA nephropathy?

A

Increased IgA due to infection
IgA forms immune complexes and deposits in mesangial cells
Resulting inflammation causes glomerulonephritis
Damage to the filtration mechanism allows blood and protein to enter the urine

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11
Q

What is henoch-schonlein purpura?

A

It is a systemic varient of IgA nephropathy, causing small vessel vasculitis

More common in children

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12
Q

How is IgA nephropathy treated?

A

ACEi

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13
Q

What are the common secondary causes of glomerulonephritis?

A

Post Streptococcal GN
SLE (systemic lupus erthymatous)
Vasculitis

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14
Q

What is the most common causing bacteria that causes poststreptococcal glomerulonephritis and how does it do so?

A

Group A beta haemolytic streptococci

They carry M protein virulence factor which is considered to be nephritogenic
Streptococcal antigen is deposited on the glomerulus, causing type III hypersensitivity reaction and immune complex formation

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15
Q

What is rapidly progressive glomerulonephritis?

A

The most aggressive form of nephritic syndrome
Also known as crescentic glomerulonephritis due to crescent shaped cell proliferation that occurs during inflammation
Over time this crescent shape undergoes sclerosis and scaring
This causes damage to the filtration system

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16
Q

What are the causes of rapidly progressive glomerulonephritis?

A

Anti-GBM disease (goodpastures disease)
Immune complex disease (IgA, SLE, post streptococcal glomerulonephritis)
Pauci immune disease (vasculitis)(ANCA (anti neutrophilic cytoplasmic antibodies))

17
Q

What are the indications for renal biopsy?

A

Unexplained acute kidney injury
Chronic kidney diseae
Acute nephritic syndrome
Unexplained proteinuria and haematuria

18
Q

What is the difference between autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive Polycystic kidney disease (ARPKD)

A

ADPKD - presents with ESRF at the age of 60

ARPKD - presents in neonates/childhood

19
Q

What is the genetic pathology of ADPKD?

A

Mutations in PKD1 or PKD2
These genes act to inhibit cell proliferation
When they are absent then cells proliferate and express proteins that transport water into the lumen of the cyst

20
Q

What are the extra renal manifestations of polycystic kidney disease?

A

Liver cysts
Ovarian cysts
Intra-cranial aneurysms (berry aneurysms) - these can rupture causing a sub arachnoid haemorrhage
Diverticular disease

21
Q

What are the differentials for autosomal dominant polycystic kidney disease?

A

Acquired cystic kidney disease
Simple cyst
Tuberous culpritis complex
Von hippal-lindau syndrome

22
Q

What is the management of autosomal dominant polycystic kidney disease?

A 
Monitor U&E and kidney size 
Aggressive BP control - ACEi are best 
Painkillers (but avoid NSAIDS as can be nephrotoxic)
Kidney stone treatment 
Treat any cystic infections 
Transplantation for ESRF
23
Q

Why do you get increased BP in renal disease?

A

Renal diseases result in inappropriate retention of salt and water, leading to hypertension

24
Q

What blood tests would you do to investigate for a renal glomerular disease?

A

FBC, ESR, CRP
Renal profile - U&E, eGFR, Creatinine
Bone profile - calcium, phosphate, magnesium
HbA1c
Albumin
Myeloma screen
Hepatitis screen

25
Q

When would you send urine off for PCR (protein/creatinine ratio)?

A

When there is + protein on a urine dipstick

26
Q

Which patients are more likely to develop acquired renal cystic diseases?

A

Patients on long term dialysis

27
Q

How does kidney size differ in Acquired cystic kidney disease to inherited cystic kidney disease?

A

Acquired - kidneys appear small

Inherited - kidneys appear large

28
Q

In screening for ADPKD, how many cysts are required to make a diagnosis?

A

Age 15-29: 3 or more cysts (unilateral or bilateral)
Age 30-39: 3 or more cysts (unilateral or bilateral)
Age 40-59: 2 or more cysts in each kidney

29
Q

When a urine dipstick is positive, what is the first test you need to do?

A

Send urine for culture and sensitivity

30
Q

If a patient has a UTI, what are the parameters that may be positive on a urine dipstick?

A

Blood
Protein
Nitrates
Leucocytes

31
Q

If a patients urine dipstick is positive for invisible haematuria, which two specialties would you refer to and why?

A

Nephrology - as they may have glomerulonephritis

Urology - as they may have cancer

32
Q

What is the normal urine protein excretion in a day?

A

<150mg/24 hours

33
Q

What is the nephrotic protein range?

A

> 3.5g/24hrs

3500mg/24hrs

34
Q

What are the two types of ANCA (anti neutrophil cytoplasmic autoantibodies) associated vasculitis?

A

PANCA - peri nuclear staining, specific for myeloperoxidase enzyme

CANCA - diffuse cytoplasmic staining, specific for proteinase-3 enzyme

35
Q

What are the 3 types of vasculitis associated with ANCA (anti neutrophilic circulating antibodies)?

A 
Wegeners granulomatosis (GPA)
Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis polyangitis (EGPA)
36
Q

What is Von-hippel lindau syndrome?

A

A hereditary condition associated with tumours arising in multiple organs: renal cell carcinoma, pheochromocytoma, pancreatic neuroendocrine tumour

Also can present with kidney cysts

37
Q

What is pulmonary-renal syndrome?

A

Where there an autoimmune disease (most commonly ANCA associated vasculitis)

Can cause respiratory failure in the lungs, coughing up blood and glomerulonephritis in the kidneys

38
Q

What can cause pulmonary renal syndrome?

A

Autoimmune diseases:

ANCA associated vasculitis (granulomatosis with polyangitis)
Basement membrane disease (e.g, goodpastures syndrome)

39
Q

How would you treat rapidly progressive glomerulonephritis?

A

High dose steroids

Cyclophosphamide - supresses the immune system

Year 3 (24 decks)

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