GU Flashcards
Describe the pathophysiology of nephrotic syndrome
Podocyte injury/scarring
What is the main difference between nephrotic and nephritic syndrome?
Nephrotic: proteinuria >3.5g/day
Nephritic: haematuria
Describe the symptoms of nephrotic syndrome
Proteinuria: >3.5g/day
Hypoalbuminaemia->oedema
Hyperlipidaemia
Hypogammaglobuniaemia
Hypercoagulability
How does nephrotic syndrome result in hyperlipidemia?
Liver increases synthesis of lipids in response to low albumin
How does nephrotic syndrome result in hypogammaglobulinemia?
Loss of Ig’s in urine
How does nephrotic syndrome result in hypercoagulability?
Loss of antithrombin 3 and proteins C and S in urine
How is nephrotic syndrome diagnosed?
Biopsy
Light/electron microscopy
What are the 3 main conditions that result in nephrotic syndrome?
Minimal change disease
Focal segmental glomerulosclerosis
Membranous nephropathy
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
What is the most common cause of nephrotic syndrome in adults?
Focal segmental glomerulosclerosis
What is the most common cause of nephrotic syndrome in the elderly?
Membranous nephropathy
What would you see in light microscopy in a patient with minimal change disease?
Nothing
What would you see in electron microscopy in a patient with minimal change disease?
Podocyte effacement and fusion
What would you see in light microscopy in a patient with focal segmental glomerulosclerosis?
Segmental sclerosis
What would you see in electron microscopy in a patient with focal segmental glomerulosclerosis?
Podocyte effacement
What would you see with light microscopy in a patient with membranous nephropathy?
Thickened glomerular basement membrane
What would you see in electron microscopy in a patient with membranous nephropathy?
Subpodocyte immune complex deposition
Spike and dome appearance
Describe the pathophysiology of nephritic syndromes
T3 hypersensitivity reactions
Apart from Goopasture’s-Type 3
GBM breaks: inflammation and Bowman crescents
Describe the symptoms of nephritic syndrome
Haematuria
Hypertension
Oedema
Oliguria
Name the conditions that commonly cause nephritic syndrome
IgA nephropathy
Post-strep glomerulonephritis
Goodpasture’s syndrome
SLE nephropathy
(Haemolytic uremic syndrome)
What is IgA nephropathy also called?
Berger’s syndrome
Name 2 conditions that can present as both nephrotic and nephritic
Diffuse proliferative glomerulonephritis
Membrano-proliferative glomerulonephritis
Name 2 risk factors for developing IgA nephropathy
Asian(greater incidence in Asian populations)
HIV
How do patients with IgA nephropathy usually present?
Visible haematuria(Ribena/coke) 1-2 days post viral infection (URTI-pharyngitis/tonsilitis/gastroenteritis)
How is IgA nephropathy diagnosed?
Biopsy:
Immunofluorescence microscopy shows IgA complex deposition in mesangium(supportive cells of Bowman’s capsule)
Describe the treatment for IgA nephropathy
Non-curative: 30% progress to ESRF
BP control: ACE-i 1st line control
Name a differential diagnosis for a patient presenting with IgA complex deposition in the kidneys
Could be :
IgA nephropathy
Henoch Schonlein purpura
What is Henoch Schonleun purpura?
AKA IgA vasculitis
Alos shows IgA complex deposition in kidneys
How can you distinguish between Henoch Schonlein purpura and IgA nephropathy?
IgA nephropathy: IgA deposition only in kidneys
Henoch Schonlein: systemic deposition-kidneys, skin ,liver
Describe how patients with post-strep glomerulonephritis commonly present
Visible haematuria (ribena/coke)
2 weeks after pharyngitis from group A,Beta haemolytic strep (S pyogenes)
Which bacteria is responsible for post-strep glomerulonephritis?
S pyogenes
How is post-strep glomerulonephritis diagnosed?
Biopsy then light/electron/immunofluorescence microscopy
What would you find in a patient with post strep glomerulonephritis with light microscopy?
Hypercellular glomeruli
What would you find in a patient with post-strep glomerulonephritis with electron microscopy?
Subendothelial immune complex deposition
What would you find in a patient with post strep glomerulonephritis with immunofluorescence microscopy?
Starry sky appearance
IgG, IgM and C3 deposition along GBM and mesangium
Describe the treatment for post strep glomerulonephritis
Usually self limiting
Can progress to RPGN(rapidly progressing glomerulonephritis)
Describe the cause of SLE nephropathy
ANA desposition in endothelium
How is SLE nephropathy diagnosed?
ANA positive
anti dsDNA positive
Describe the treatment for SLE nephropathy
Steroids
Hydroxychloroquine
Immunosuppressants: cyclophosphamide
Describe the pathophysiology of Goodpasture’s
Autoantibodies: anti-GBM that cause pulmonary+alveolar haemorrhage and glomerulonephritis
How is Goodpasture’s syndrome diagnosed?
Immunofluorescence: Linear deposition of IgG along glomerular capillaries
Describe the treatment for Goodpasture’s syndrome
Steroids and plasma exchange
Which nephritic syndrome can lead to rapidly progressing glomerulonephritis?
Post strep glomerulonephritis
How do patients with haemolytic uremic syndrome commonly present?
5 days post infection with shiga toxin
E coli, shigella
Name 2 micro bacteria that can cause haemolytic uremic syndrome
E coli, shigella
Name the consequences of haemolytic uremic syndrome
Haemolytic anaemia
AKI(glomerulonephritis) and uremia
Thrombocytopenia
How is haemolytic anaemia treated?
Mostly self-limiting
Present as a medical emergency-supportive fluids and antibiotics
What is rapidly progressing glomerulonephritis
Subtype of glomerulonephritis that progresses to ESRF very quickly-weeks to months
How is rapidly progressing glomerulonephritis diagnosed?
Inflammatory crescents in Bowman’s space
Name the causes of rapidly progressing glomerulonephritis
Wegener’s granulomatosis
MPA
Good pastures
Name 2 conditions that can present as both nephritic and nephrotic?
Diffuse proliferative glomerulonephritis
Membranoproliferative glomerulonephritis
What is polycystic kidney disease?
Cyst formation throughout renal parenchyma leading to bilateral enlargement and damage
How is polycystic kidney disease inherited?
Familial
Can be autosomal dominant-most common
Or autosomal recessive
Which gene mutations are involved in autosomal dominant PCKD?
85%: PKD1
15%: PKD2
What groups of people do autosomal dominant PCKD tend to affect?
Males
Present at 20-30 years
Is autosomal recessive or dominant PCKD more common?
Autosomal dominant more common
When do patients with autosomal recessive PCKD commonly present?
Disease of infancy or prebirth
High mortality
Name some congenital abnormalities associated with autosomal recessive PCKD?
Potter’s sequence: flattened nose, clubbed feet
Which kind of PCKD inheritance is associated with congenital abnormalities?
Autosomal recessive
Describe the normal physiology of PKD1+2 genes
Code for polycystin (Ca2+ channel)
When filtrate passes cilia of nephron, they move and polycystin(Ca2+ channels)open
Ca2+ influx->inhibits excessive growth of cilia
Describe the pathophysiology of PCKD
PKD mutations lead to decreased Ca2+ influx so excessive growth of cilia->cysts
Many cysts-polycystic
Describe the common presentation of a patient with polycystic kidney disease
Bilateral flank/back/ abdo pain
Can have hypertension and haematuria
Can also cause extra-renal cysts which can rupture and cause haemorrhage
Name a common location of extra renal cysts
Circle of Willis: Berry aneurysm
Name a complication that can arise from a ruptured berry aneurysm
Subarachnoid haemorrhage
How is PCKD diagnosed?
Kidney US
Genetic testing and fHx of PCKD