Misc. Renal Conditions

Question 1

What type of Hypersensitivity reaction is Goodpasture’s Disease?

Answer 1

Type 2

Question 2

What is the pathophysiology of Goodpasture’s Disease? What are the classic symptoms?

Answer 2

Autoimmune condition causing IgG antibodies attack COL4A3 in basement membranes.

Leading to haemoptysis and haematuria

Question 3

What is Alport Syndrome?

What are the clinical features of Alport’s Syndrome?

Answer 3

Rare inherited disease caused by Type IV collagen mutation causing defects in ears, kidneys eyes.

🩸Glomerulonephritis (Haematuria, renal insufficiency, hypertension)
👂Sensorineural hearing loss
👁️Eye problems: Anterior Lenticonus

Question 4

What collagen is affected in Alport’s Syndrome?

Answer 4

Collagen IV, specifically COL4A3, COL4A4, COL4A5

Question 5

What is the main inheritence pattern of Alport’s

Answer 5

X-linked dominant

Question 6

Granulomatosis with Polyangitis, aka Wegener’s is underpinned by what ANCA?

Answer 6

c-ANCA

Question 7

Churg-Strauss syndrome is underpinned by what ANCA?

Answer 7

p-ANCA

Question 8

Microscopic Polyangitis is underpinned by what ANCA?

Answer 8

p-ACNA

Question 9

Granulomatosis with Polyangitis, Churg-Strauss syndrome, Microscopic Polyangitis and Henoch-Schonlein Pupura are all types of what?

Answer 9

Small vessel vasculitis diseases

Question 10

What is Henoch Schonlein Purpura?

What are the characteristic features of Henoch-Schonlein Pupura? 4 examples

Answer 10

IgA vasculitis - condition mediated by IgA deposition in blood vessels causing inflammation and leakage from vessels

Palpable purpuric rash on buttocks and extensor surfaces of legs and arms
- Polyarthritis
- Abdominal pain
- IgA nephropathy (haematuria, proteinuria)

Question 11

Which is more common, ADPKD or ARPKD?

Answer 11

ADPKD

Question 12

ADPKD has cysts which arise from which structure in the kidney?

Answer 12

Epithelial lined cysts arising from RENAL TUBULES

Question 13

ARPKD has cysts which arise from which structure in the kidney?

Answer 13

Epithelial lined cysts arising from collecting ducts

Question 14

What are some other manifestations of ADPKD, other than cysts in the kidneys?

Answer 14

• Liver cysts and pancreatic cysts
• Berry aneurysm in Circle of Willis -> subarachnoid haemorrhage
• Aortic root dilatation
• Colonic diverticula

Question 15

Autosomal recessive PKD is caused by what mutation?

Answer 15

PKHD1, chromosome 6

Question 16

ADPKD and ARPKD manifest when?

Answer 16

ADPKD - during adulthood

ARPKD - during infancy

Question 17

Signs of ARPKD?

Answer 17

Oligohydramnios found during antenatal scan (means baby is affected)

Respiratory failure and haemodialysis within first few days of birth

Underdeveloped ear cartilage, low-set ears, flat nasal bridge

End stage renal failure by adulthood

Question 18

Diagnosis and management of PKD?

Answer 18

Ultrasound scan
Genetic testing

Managament: Tolvaptan
Manage hypertension
Analgesia for acute pain
Abx for UTI/cyst infections
Drainage via aspiration/surgery
Dialysis and/or renal transplant (if end stage renal failure)

Question 19

What counselling would you offer a patient with PKD?

Answer 19

Avoid contact sports
Avoid NSAIDs and anticoags
MR angiography to screen for cerebral aneurysm

Question 20

What is haemolytic uraemic syndrome?

Haemolytic Uraemic Syndrome is a triad of..?

Answer 20

Thrombosis formation in small blood vessels caused by Shiga toxin

• Acute kidney injury
• Thrombocytopenia
• Haemolytic anaemia

Question 21

What is the characteristic finding on blood film in a patient with Haemolytic Uraemic Syndrome?

Answer 21

Schisocytes

Question 22

What micro-organism commonly causes Haemolytic Uraemic Syndrome?

Answer 22

Shiga toxins from E. Coli 0157/shigella

Question 23

What population of patients does Haemolytic Uraemic Syndrome commonly affect?

Answer 23

Children and after gastroenteritis

Question 24

What is the mainstay management for Haemolytic Uraemic Syndrome

Answer 24

Supportive management, i.e Fluids, transfusions, dialysis. NO ANTIBIOTICS

Question 25

Which vasculitic condition gives patients a classic "saddle shaped" nose deformity

Answer 25

Granulomatosis with Polyangitis (Wegener's)

Question 26

What are the affected genes in AD PKD? Which one is more likely and which one is more severe?

Answer 26

PKD1 gene, chromosome 16 PKD2 gene, chromosome 4 PKD1 mutation = more likely + severe

Question 27

Difference between nephrotic and nephritic syndrome in terms of proteinuria values?

Answer 27

Nephrotic > 3.5g/day Nephritic < 3.5g/day

Question 28

What is nephrotic syndrome

Answer 28

Damage to the basement membrane of glomerulus making it highly permeable to proteins

Question 29

Clinical features of nephrotic syndrome?

Answer 29

Hypoalbuminaemia <25g/L Peripheral oedema Proteinuria >3.5g/day Frothy urine Hypercholestrolaemia Clotting (loss of antithrombin III) Infections (loss of IgG)

Question 30

Most common cause of nephrotic syndrome in children?

Answer 30

Minimal change disease

Question 31

Most common causes of nephrotic syndrome in adults? Other causes?

Answer 31

Mnemonic: Makes More Filtrate Motherfucking Heavy Dense and Impaired Membranous nephropathy Minimal charge disease Focal segmental glomerulosclerosis Membranoproliferative glomerulonephritis Henoch-Schönlein purpura (HSP) Diabetes Infection (e.g., HIV)

Question 32

Features of nephritic syndrome?

Answer 32

Haematuria Hypertension Jugular venous distension Oliguria Mild oedema Mild proteinuria Azotaemia

Question 33

Causes of nephritic syndrome?

Answer 33

'Acute Crescents Indicate Aggression' Acute post streptococcal glomerulonephritis (under 30yo) Crescenteric glomerulonephritis (rapidly progressing GN) IgA nephropathy Alport syndrome

Question 34

Causes of proteinuria?

Answer 34

Glomerular disease (Nephrotic/nephritic syndrome) Tubular disease (Acute tubular necrosis, acute interstitial nephritis)

Question 35

What disease typically presents as a patient in their 20s presenting with haematuria? What is shown on histology?

Answer 35

IgA nephropathy IgA deposits and mesangial proliferation

Question 36

Which disease shows immune complex deposits and mesangial proliferation?

Answer 36

Membranoproliferative glomerulonephritis

Question 37

What infections can acute post streptococcal glomerulonephritis follow?

Answer 37

tonsilitis impetigo

Question 39

What systemic diseases can cause glomerulonephritis?

Answer 39

SLE Vasculitis (microscopic polyangiitis/ granulomatosis with polyangiitis) Lupus nephritis

Question 40

Which diseases are these antibodies associated with? 1. Anti GBM 2. p-ANCA/MPO antibodies 3. c-ANCA/PR3 antibodies

Answer 40

1. Anti GBM - Goodpasture syndrome 2. p-ANCA/MPO - microscopic polyangiitis 3. c-ANCA/PR3 - granulomatosis with polyangiitis

Question 46

How does Haemolytic uraemic syndrome lead to an AKI?

Answer 46

Formation of thrombi throughout blood vessels -> blocks blood getting to kidneys -> hypoperfusion of kidneys

Question 47

How does Microangiopathic haemolytic anaemia occur with HUS? What findings appear on histology of someone with haemolytic uraemic syndrome

Answer 47

As RBCs try to move past thrombi they get shredded leading to haemolysis Schistocytes