Misc. Renal Conditions Flashcards
What type of Hypersensitivity reaction is Goodpasture’s Disease?
Type 2
What is the pathophysiology of Goodpasture’s Disease? What are the classic symptoms?
Autoimmune condition, affecting the lungs (haemotpysis) and kidneys (haematuria)
IgG antibodies attack COL4A3 in basement membranes.
What are the clinical features of Alport’s Syndrome?
- Glomerulonephritis (Haematuria, renal insufficiency, hypertension)
- Ear problems: Sensorineural hearing loss
- Eye problems: Anterior Lenticonus
What collagen is affected in Alport’s Syndrome?
Collagen IV, specifically COL4A3, COL4A4, COL4A5
What is the main inheritence pattern of Alport’s
X-linked dominant
Granulomatosis with Polyangitis, aka Wegener’s is underpinned by what ANCA?
c-ANCA
Churg-Strauss syndrome is underpinned by what ANCA?
p-ANCA
Microscopic Polyangitis is underpinned by what ANCA?
p-ACNA
Granulomatosis with Polyangitis, Churg-Strauss syndrome, Microscopic Polyangitis and Henoch-Schonlein Pupura are all types of what?
Small vessel vasculitis diseases
What are the characteristic features of Henoch-Schonlein Pupura? 4 examples
- Palpable purpuric rash on buttocks and extensor surfaces of legs and arms
- Polyarthritis
- Abdominal pain
- IgA nephropathy (haematuria, proteinuria) - not always occurs
Which is more common, ADPKD or ARPKD?
ADPKD
ADPKD is caused by what two possible mutations? What is the onset of each? Which is more severe?
PKD1 - earlier onset, more severe
PKD2 - later onset, less severe
ADPKD has cysts which arise from which structure in the kidney?
Epithelial lined cysts arising from RENAL TUBULES
ARPKD has cysts which arise from which structure in the kidney?
Epithelial lined cysts arising from COLLECTING DUCTS
What are some other manifestations of ADPKD, other than cysts in the kidneys?
- Liver cysts and pancreatic cysts
- Berry aneurysm in Circle of Willus -> subarachnoid haemorrhage
- Aortic root dilatation
ARPKD is caused by what mutation?
PKHD1
ADPKD and ARPKD manifest when?
ADPKD - during adulthood
ARPKD - during infancy
Haemolytic Uraemic Syndrome is a triad of..?
- Acute renal failure
- Thrombocytopenia
- Haemolytic anaemia
What is the characteristic finding on blood film in a patient with Haemolytic Uraemic Syndrome?
Fragmented blood cells
What micro-organism commonly causes Haemolytic Uraemic Syndrome?
E. Coli 0157:H7
What population of patients does Haemolytic Uraemic Syndrome commonly affect?
Children
What is the mainstay management for Haemolytic Uraemic Syndrome
Supportive management, i.e Fluids, transfusions, dialysis. NO ANTIBIOTICS
Which vasculitic condition gives patients a classic “saddle shaped” nose deformity
Granulomatosis with Polyangitis (Wegener’s)
