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Renal > Renal vascular disease > Flashcards

Renal vascular disease Flashcards

1
Q

What is renovascular disease defined as?

A

Stenosis of the renal artery or one of its branches

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2
Q

Causes of renal artery stenosis

A

Normally atherosclerosis - older patients - can progress and cause complete occlusion
Fibromuscular dysplasia in younger patients - doesn’t usually cause occlusion

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3
Q

Why hypertension in renal artery stenosis

A

Because decreased renal perfusion stimulates JG apparatus to release renin - vasoconstriction and salt and water retention

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4
Q

Renal impairment in renal artery stenosis

A

Angiotensin II constricts efferents more than afferents therefore maintains glomerular filtration rate - if give ARB or ACEi - decreasing renal function

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5
Q

Oedema in renal artery disease

A

Angiotensin II causes increased glomerular pore size therefore mild proteinuria can occur

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6
Q

Rarer causes of renal artery stenosis

A

Takayasu’s inflammatory artertitis, neurofibromatosis, pressure from renal artery aneurysms and extrinsic pressure

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7
Q

What biochem finding in renal artery stenosis

A

Hypokalaemia - aldosterone promotes K+ secretion

Therefore hypokalaemia unless there is renal impairment

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8
Q

DX of renal artery stenosis

A

MR angiography and USS

CT

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9
Q

Rx of renal artery stenosis

A

Transluminal angioplasty
Stenting
Revascularisation surgery

HTN treatment - ARB or ACEi need careful monitoring

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10
Q

What is haemolytic uraemic syndrome?

A

Microangiopathic haemolytic anaemia
Endothelial damage in microvasculature - usually caused by E.Coli (in outbreaks in children after eating uncooked meat)
Endothelial damage triggers thrombosis, platelet consumption and fibrin deposition

Causes mechanical destruction of RBCs

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11
Q

What does HUS cause?

A

Thrombcytopenia and AKI

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12
Q

Signs of HUS

A

Abdominal pain, bloody diarrhoea and AKI

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13
Q

Rx of HUS

A

Dialysis may be needed for AKI

Plasma exchange for severe cases

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14
Q

Other features present in Thrombotic Thrombocytopenia

A

CNS signs (seizures, hemiparesis, decreased consciousness and fever)

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15
Q

Management of kidneys in diabetes

A

Screening for microalbuminuria - as soon as detected started on ACE i (or ARB) even if not HTN

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16
Q

What is renal tubular acidosis?

A

Metabolic acidosis due to impaired acid secretion by the kidney
Hypercholeraemic acidosis and normal anion gap

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17
Q

Types of RTA

A 
4 types 
1 - distal 
2- proximal 
3 - rare combination of 1 + 2 
4 - hyperkalaemic
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18
Q

What causes type 1 RTA

A

Failure to excrete H+ in distal tubule - even when metabolic acidosis
- Inherited disorders but also acquired with SLE, and drug related

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19
Q

Features of type 1 RTA

A

Rickets, osteomalacia - because buffer H+ with calcium in bone

Nephrocalcinosis with renal calculi - recurrent UTIs (due to hypercalciuria, decreased urinary citrate (reabsorbed as buffer for H+) and alkaline urine - all favour calcium phosphate stone formation

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20
Q

Treatment of type 1 RTA

A

Oral sodium bicarbonate or citrate

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21
Q

What causes type 2 RTA

A

Bicarbonate leak from proximal tubule

Normally associated with more general tubular defect - Fanconi’s

22
Q

What biochem in type 2 RTA

A

Hypokalaemia because of osmotic diuretic effect of decreased bicarb reabsorption causing increased flow rate and increased K+ excretion

23
Q

DX of type 2 RTA

A

Sodium bicarb load and there is high fractional excretion of bicarb

24
Q

RX of type 2 RTA

A

High dose bicarb - patients find it intolerable

25
Q

What causes type 4 RTA?

A

Hyporeninaemic hypoaldosteronism

Addisons
Diabetic nephropathy
Interstitial nephritis

Hypoaldosteronism causes hyperkalaemia and acidosis

26
Q

Rx of type 4 RTA

A

treat underlying cause

furosemide to hyperkalaemia

27
Q

What is Fanconi syndrome?

A

Proximal tubular dysfunction leading to loss of amino acids, phosphate, bicarb and glucose

28
Q

What does Fanconi syndrome cause

A

Dehydration
Osteomalacia
Metabolic acidosis
Electrolyte abnormalities

29
Q

Congenital causes of Fanconi syndrome

A

Congenital - idiopathic, cystinosis, Wilsons, tyrosinemia, Lowe syndrome

30
Q

Acquired causes of Fanconi syndrome

A

heavy metal poisoning
drugs (gent, cisplatin, ifosfamide)
light chains (myeloma, amyloid)

31
Q

Rx of Fanconi syndrome

A

Treat cause
replace K+, bicarb, phosphate
Vit D supplements

32
Q

What is cystinosis and features

A

Congenital accumulation of cystine
Causes Fanconi syndrome, visual impairment, myopathy, hypothyroidism
Progression to ESRF

33
Q

Rx of Cystinosis

A

Can give oral cysteamine - poorly tolerated

Renal transplant - fanconis does not reoccur but extra-renal disease progresses

34
Q

What is Bartter’s syndrome?

A

Inherited mutation in the co-transporter targeted by LOOP diuretics
Therefore alkalosis, hypokalaemia and hypercalciuria

Usually presents in infancy - polyuria, polydipsia, tendency to dehydration

35
Q

Rx of Bartters syndrome?

A

Replace K+, consider DOMETHACIN - prostaglandin synthesis inhibitor - resolves abnormalities

36
Q

What is Gitelman syndrome?

A

Inherited mutation in co-transporter targeted by thiazide diuretics therefore

Low K+, metabolic alkalosis, hypocalciuria and low mg2+

37
Q

Presentation and Rx of Gitelman syndrome?

A

Usually present later than Bartters- with muscle cramps, weakness and low BP

Replace Mg2+
K+ supplementation

38
Q

Prevalence of autosomal dominant polycystic kidney disease (APKD)

A

1 in 1000

39
Q

2 types of autosomal dominant polycystic kidney disease

A

PKD1 mutation - 85% - reach ESRF by 50s

PKD2 mutation - 15% - reach ESRF by 70s

40
Q

Renal signs of PCKD

A 
Renal enlargement with cysts 
Abdominal pain (stretching of capsule)
Haematuria - haemorrhage into a cyst (pain also)
Cyst infection 
Renal calculi 
HTN 
Progressive renal failure
41
Q

Extrarenal signs of PCKD

A 
Liver cysts
Intra-cranial berry aneurysms - SAH 
Mitral valve prolapse 
Ovarian cysts 
Diverticular disease
42
Q

Treatment of PCKD

A 
Low target BP 
Treat infections
Dialysis or transplantation 
Genetic counselling 
High water intake and decrease salt intake 
Can do cyst removal or nephrectomy
43
Q

Screening for SAH in PCKD

A

Magnetic resonance angiography
Done in 1st degree relatives of those with SAH + ADPKD
Or if certain occupations - pilot

44
Q

Prevalence of autosomal recessive PCKD, presentation and treatment

A

1 in 40,000
Present in infancy with multiple renal cysts and congenital hepatic fibrosis
No current therapy

45
Q

What is medullary cystic disease

A

Inherited disorder with tubular loss and medullary cyst formation
Juvenile (recessive) form accounts for 10-20% of all ESRF in children
Adult form (dominant) is rare

46
Q

Signs of medullary cystic disease

A

Shrunken kidneys, cysts only in medulla

Salt wasting, polyuria and polydipsia, enuresis (can’t concentrate urine) failure to thrive and renal impairment

47
Q

Extrarenal signs of medullary cystic disease

A 
Retinal degeneration 
Retinitis pigmentosa
Skeletal changes 
Cerebellar ataxia 
Liver fibrosis
48
Q

What is Von-Hippel Lindau syndrome?

A

Chief cause of inherited renal cancers - mutations in VHL tumour-suppressor gene

49
Q

What is Alport syndrome?

A

Mutation in gene coding type 4 collagen - GBM splitting - collagen targeted in GP therefore risk of Goodpastures post-renal transplant

50
Q

Signs of Alport

A

Haematuria, proteinuria and progressive renal insufficiency

Often systemic manifestations too

Renal (9 decks)

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