Systemic diseases

Question 1

Diabetic Nephropathy

Answer 1

Causes ~20% of ESRF

Advanced / ESRF occurs in 40% of T1 and T2 DM

Question 2

Pathology of diabetic nephropathy

Answer 2

Diabetic nephropathy describes conglomerate of lesions occurring concurrently.
Hyperglycaemia → renal hyperperfusion → hypertrophy and ↑ renal size
Hypertrophy and metabolic defects inc. ROS production → glomerulosclerosis and nephron loss
Nephron loss → RAS activation → HTN

Question 3

Clinical effects

Answer 3

Microalbuminuria (30-300mg/d or albumin:creatinine >3) Strong independent RF for CV disease
Progresses to proteinuria (albuminuria >300mg/d)
Diabetic retinopathy usually co-exists and HTN is
common

Question 4

Screening for diabetic nephropathy

Answer 4

T2DMs should be screened for microalbuminuria 6moly

Question 5

Histology of diabetic nephropathy

Answer 5

Damage to the glomerular basement membrane allows proteins in the blood to leak through, leading to accumulation in Bowman’s space - called Kimmelstiel–Wilson nodules

Question 6

Treatment of diabetic nephropathy

Answer 6

Good glycaemic control delays onset and progression
UKPDS: UK Prospective Diabetes Study
DCCT: Diabetes Control and Complications Trial
Control HTN: BP target 130/80
ACEi/ARB: even if normotensive
Stop smoking
Combined kidney pancreas Tx possible in selected pts

Question 7

Amyloidosis and features

Answer 7

Renal involvement usually caused by AL/AA amyloid
  Features:
  Proteinuria
  Nephrotic syndrome
  Progressive renal failure

Question 8

Differentiating features and investigations

Answer 8

Large kidneys on US

Biopsy

Question 9

Malignancy - direct

Answer 9

Renal infiltration: leukaemia, lymphoma Obstruction: pelvic tumour
Mets

Question 10

Malignancy - nephrotoxicity

Answer 10

Toxic chemo
Analgesics
Tumour lysis syndrome

Question 11

Effects of high PTH

Answer 11

High Ca2+

Question 12

Effects of sarcoidosis

Answer 12

High Ca2+ and TIN

Question 13

Myeloma pathology

Answer 13

Excess production of monoclonal Ab ± light chains (excreted and detected in 60% as urinary BJP).
Light chains block tubules and have direct toxic effects → ATN.
Myeloma also assoc. ̄c ↑↑Ca2+

Question 14

Myeloma presentation

Answer 14

ARF / CRF Amyloidosis

Question 15

Myeloma treatment

Answer 15

Ensure fluid intake of 3L/d to prevent further impairment Dialysis may be required in ARF

Question 16

RA

Answer 16

NSAIDs → ATN

Penicillamine and gold → membranous GN AA amyloidosis occurs in 15%

Question 17

SLE pathogenesis and treatment

Answer 17

Involves glomerulus in 40-60% → ARF/CRF
Immune complex deposition → T3 hypersensitivity
Typically membranous GN
Proteinuria and ↑BP common
Rx
Proteinuria: ACEi
Aggressive GN: immunosuppression

Question 18

Diffuse systemic sclerosis and treatment

Answer 18

Renal crisis: malignant HTN + ARF Commonest cause of death

Rx: ACEi if ↑BP or renal crisis

Question 19

Reno vascular disease causes

Answer 19

Cause
  Atherosclerosis in 80%
  Fibromuscular dysplasia
  Thromboembolism
  External mass compression

Question 20

Presentation of renovascular disease

Answer 20

Presentation
Refractory hypertension
Renal bruits
Worsening renal function after ACEi/ARB
Flash pulmonary oedema (no LV impairment on echo) Other signs of PVD

Question 21

Investigations of renovascular disease

Answer 21

US + doppler: small kidney + ↓ flow
CT/MR angio
Renal angiography: gold standard

Question 22

Treatment of renovascular disease

Answer 22

Rx medical CV risk factors Angioplasty and stenting

AVOID ACEi/ARB

Question 23

Hypertension effects on the kidney

Answer 23

HTN can be both the cause and effect of renal damage.
Renal diseases are commonest causes of 2O HTN
Activation of RAS
Retention of Na and water due to ↓ excretion

Question 24

What is haemolytic uraemia syndrome

Answer 24

E. coli O157:H7: verotoxin → endothelial dysfunction

Question 25

Features of HUS

Answer 25

You children eating undercooked meals e.g. burgers. Bloody diarrhoea and abode pain precedes 1. MAHA 2. Thrombocytopenia 3. Renal failure

Question 26

Investigation of HUS

Answer 26

Schistocytes, ↓ plats ↓Hb Normal clotting

Question 27

Treatment of HUS

Answer 27

Usually resolves spontaneously | Dialysis or plasma exchange may be needed

Question 28

What is Thrombotic thrombocytopenia pupura (TTP)

Answer 28

Genetic or acquired deficiency of ADAMTS13 → giant vWF multimers

Question 29

Features of Thrombotic thrombocytopenia pupura (TTP)

Answer 29

``` Adult females Pentad: Fever CNS signs: confusion, seizures MAHA Thrombocytopenia Renal failure ```

Question 30

Investigating TTP

Answer 30

↓ plats ↓Hb Normal clotting

Question 31

Treatment of TTP

Answer 31

Plasmapheresis, immunosuppression, splenectomy