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 causing IgG antibodies attack COL4A3 in basement membranes.
Leading to haemoptysis and haematuria
What is Alport Syndrome?
What are the clinical features of Alport’s Syndrome?
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
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 is Henoch Schonlein Purpura?
What are the characteristic features of Henoch-Schonlein Pupura? 4 examples
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)
Which is more common, ADPKD or ARPKD?
ADPKD
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 Willis -> subarachnoid haemorrhage
- Aortic root dilatation
- Colonic diverticula
Autosomal recessive PKD is caused by what mutation?
PKHD1, chromosome 6
ADPKD and ARPKD manifest when?
ADPKD - during adulthood
ARPKD - during infancy
Signs of ARPKD?
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
Diagnosis and management of PKD?
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)
What counselling would you offer a patient with PKD?
Avoid contact sports
Avoid NSAIDs and anticoags
MR angiography to screen for cerebral aneurysm
What is haemolytic uraemic syndrome?
Haemolytic Uraemic Syndrome is a triad of..?
Thrombosis formation in small blood vessels caused by Shiga toxin
- Acute kidney injury
- Thrombocytopenia
- Haemolytic anaemia
What is the characteristic finding on blood film in a patient with Haemolytic Uraemic Syndrome?
Schisocytes
What micro-organism commonly causes Haemolytic Uraemic Syndrome?
Shiga toxins from E. Coli 0157/shigella
What population of patients does Haemolytic Uraemic Syndrome commonly affect?
Children and after gastroenteritis
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)
What are the affected genes in AD PKD? Which one is more likely and which one is more severe?
PKD1 gene, chromosome 16
PKD2 gene, chromosome 4
PKD1 mutation = more likely + severe
Difference between nephrotic and nephritic syndrome in terms of proteinuria values?
Nephrotic > 3.5g/day
Nephritic < 3.5g/day
What is nephrotic syndrome
Damage to the basement membrane of glomerulus making it highly permeable to proteins
Clinical features of nephrotic syndrome?
Hypoalbuminaemia <25g/L
Peripheral oedema
Proteinuria >3.5g/day
Frothy urine
Hypercholestrolaemia
Clotting (loss of antithrombin III)
Infections (loss of IgG)
Most common cause of nephrotic syndrome in children?
Minimal change disease
Most common causes of nephrotic syndrome in adults?
Other causes?
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)
Features of nephritic syndrome?
Haematuria
Hypertension
Jugular venous distension
Oliguria
Mild oedema
Mild proteinuria
Azotaemia
Causes of nephritic syndrome?
‘Acute Crescents Indicate Aggression’
Acute post streptococcal glomerulonephritis (under 30yo)
Crescenteric glomerulonephritis (rapidly progressing GN)
IgA nephropathy
Alport syndrome
Causes of proteinuria?
Glomerular disease (Nephrotic/nephritic syndrome)
Tubular disease (Acute tubular necrosis, acute interstitial nephritis)
What disease typically presents as a patient in their 20s presenting with haematuria?
What is shown on histology?
IgA nephropathy
IgA deposits and mesangial proliferation
Which disease shows immune complex deposits and mesangial proliferation?
Membranoproliferative glomerulonephritis
What infections can acute post streptococcal glomerulonephritis follow?
tonsilitis
impetigo
What systemic diseases can cause glomerulonephritis?
SLE
Vasculitis (microscopic polyangiitis/ granulomatosis with polyangiitis)
Lupus nephritis
Which diseases are these antibodies associated with?
1. Anti GBM
2. p-ANCA/MPO antibodies
3. c-ANCA/PR3 antibodies
- Anti GBM - Goodpasture syndrome
- p-ANCA/MPO - microscopic polyangiitis
- c-ANCA/PR3 - granulomatosis with polyangiitis
How does Haemolytic uraemic syndrome lead to an AKI?
Formation of thrombi throughout blood vessels -> blocks blood getting to kidneys -> hypoperfusion of kidneys
How does Microangiopathic haemolytic anaemia occur with HUS?
What findings appear on histology of someone with haemolytic uraemic syndrome
As RBCs try to move past thrombi they get shredded leading to haemolysis
Schistocytes
