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Renal SB > Inherited and Systemic Diseases of the Kidney > Flashcards

Inherited and Systemic Diseases of the Kidney Flashcards

1
Q

what is overt diabetic nephropathy characterised by

A

persistent albuminuria

i.e. 300mg/24h on at least 2 occasions separated by 3-6 months

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2
Q

how can haemodynamic changes in diabetes cause nephropathy

A
  • afferent arteriolar vasodilatation mediated by range of vasoactive mediators e.g IGF-1
  • hyperfiltration
  • increased GFR
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3
Q

what cause nephropathy in diabetes

A 
haemodynamic changes 
Renal hypertrophy
Inflammation
Proteinuria
Tubulo-interstitial fibrosis
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4
Q

how is proteinuria caused in diabetic nephropathy

A

GBM thickening and podocyte dysfunction

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5
Q

how does renal hypertrophy come about in diabetic nephropathy

A

plasma glucose stimulates several growth factors within the kidney

Mesangial Expansion
Nodule Formation

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6
Q

what does nodule formation on the kidneys lead to

A

nodular diabetic glomerulosclerosis ( Kimmelstiel-Wilson lesion) and diffuse glomerulosclerosis

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7
Q

what is the relation ship of proteinuria and diabetic complications

A

The worse the proteinuria, the more likely the patient is to have diabetic complications

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8
Q

what is diagnostic for diabetic nephropathy

A

Levels of 24 hour and overnight urinary albumin excretion

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9
Q

what are the normal values of urinary albumin excretion

A

24-hr = > 30

Overnight = > 20

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10
Q

what are values of microalbuminuria in urinary albumin excretion

A

24hr = 30-300

Overnight = 20-200

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11
Q

what are values of overt nephropathy in urinary albumin excretion

A

24hr = > 300

Overnight = > 200

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12
Q

prevention/treatment of diabetic nephropathy

A

Maintain tight glycaemic control
ACE inhibitors AND ARBs
Lipid control

Diuretic can also be added

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13
Q

what is the blood pressure target in diabetes

A

130/85mmHg

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14
Q

when is dialysis normally started

A

when GFR is 10-12 ml/min

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15
Q

what are the possible renal replacement therapy for diabetic nephropathy

A

Simultaneous Kidney-Pancreas Transplant (Type 1 only)

Kidney transplant

Haemodialysis and Peritoneal Dialysis

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16
Q

what does microalbuminuria progress to

A

proteinuria and frank nephropathy

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17
Q

how can diabetic nephropathy be slowed down

A

if we slow down proteinuria

- can be done by ACEi or ARB

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18
Q

what is renovascular hypertension

A

secondary form of hypertension usually caused by renal artery stenosis

reduction in renal perfusion pressure activates a series of hormonal and neuronal responses that raise systemic arterial BP

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19
Q

what are causes of renovascular disease

A

Fibromuscular dysplasia

Atherosclerotic Renovascular disease

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20
Q

what is ischaemic nephropathy

A

Refers to the reduced GFR associated with reduced renal blood flow beyond the level of autoregulatory compensation

Over time can lead to renal atrophy and progressive CKD

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21
Q

what is Fibromuscular Dysplasia

A

non-atherosclerotic, non-inflammatory disease of the blood vessels that causes abnormal growth within the wall of an artery commonly the renal arteries

Causes renal artery stenosis

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22
Q

what is Fibromuscular dysplasia associated with

A

Ehlers-Danlos

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23
Q

how commonly gets Fibromuscular dysplasia

A

Females 15-50 years

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24
Q

what is Atherosclerotic Renovascular disease

A

combination of renal ischaemia and renal artery stenosis

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25
Q

what are risks of Atherosclerotic Renovascular disease

A

risk for generalised atherosclerosis - rarely renal alone

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26
Q

what is the clinical presentation of Atherosclerotic Renovascular disease

A

Renovascular hypertension

AKI after treatment of hypertension (usually with ACEi)

CKD in elderly with diffuse vascular disease

‘Flash’ pulmonary oedema
- sudden onset without LV impairment

Microscopic haematuria

Abdominal bruit

Atherosclerotic disease elsewhere

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27
Q

Ix of ischaemic renal disease

A

US - renal size asymmetry, affected side is smaller

CT/MRI angiography

Angiography - gold standard booted after CT/MRI as it is invasive

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28
Q

Tx of ischaemic renal disease

A

Medical therapy
- manage hypertension

Surgical therapy

  • Angioplasty
  • Angioplasty + stenting
  • Stenting alone
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29
Q

when are ACEi contra-indicated

A

Bilateral Renal Artery Stenosis

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30
Q

what is multiple myeloma

A

cancer of the plasma cells

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31
Q

what is produced in multiple myelomas that can cause kidney problems

A

paraprotein

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32
Q

what are Sx of myeloma

A

Bone Pain
Weakness
Fatigue
Weight Loss

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33
Q

what are signs of myeloma

A 
Anaemia
Hypercalcaemia
Renal Failure
Amyloidosis
Recurrent infections
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34
Q

Ix for myeloma

A

FBC

  • normocytic anaemia
  • Rouleaux formation
  • Raised CRP/PV
  • raised calcium

Renal impairment
- raised urea and creatinine

Protein electrophoresis
Bence-Jones protein in urine
Lytic lesions on skeletal survey
e.g. pepper pot skull

35
Q

what are renal manifestations of multiple myeloma

A

AKI secondary to hypercalcaemia

Monoclonal Immunoglobulin Deposition Disease

Cast nephropathy
- formation of plugs in the renal tubules from light chains leading to renal failure

Amyloidosis

36
Q

what is amyloidosis, how is it characterised and classified

A

Generic name for a family of diseases

Characterised by the deposition in extracellular spaces of a proteinaceous material

Classified by the type of precursor protein that makes up the main component of the fibrils

37
Q

what are renal amyloidosis

A

primary (AL) amyloid and secondary (AA) amyloid

38
Q

how do renal amyloidosis present histologically

A

Positive Congo red staining showing apple-green birifringence under polarised light
(BUZZWORD)

39
Q

Tx of myeloma and ARF

A

Stop Nephrotoxins;

  • NSAID’s
  • Diuretics in view of risk increasing cast formation

Treat hypercalcaemia;
IV NaCl to volume resuscitate
IV Pamidronate if required

Chemotherapy to reduce tumour load
- High dose dexamethasone may help reduce tumour load.

Plasma exchange
- to remove light chains

Dialysis
- to support AKI and CKD

40
Q

what type of vasculitis more commonly affects the kidneys

A

small vessel vasculitis

41
Q

what are examples of small vessel vasculitis

A

ANCA associated
- Wegener’s, Churg-Strauss, Microscopic polyangitis

Immune complex associated
- HSP, Behcets disease, anti-GBM disease e.g. Good pasture disease

42
Q

what is Granulomatosis with Polyangiitis

A

(previously Wegeners)

Necrotising granulomatous inflammation which most commonly affects the respiratory tract

43
Q

Sx of Gw/P

A

Nasal crusting, sinusitis, persistent rhinorrhea, otitis media, oral/nasal ulcers, bloody nasal discharge.

Evidence of bony/cartilage destruction (saddle nose)

44
Q

what is Eosinophilic Granulomatosis with Polyangiitis

A

(previously Churg-Strauss)
necrotising granulomatous inflammation

Asthma and eosinophilia
Lung most commonly involved (asthma in > 95%)

2/3 have skin involvement (palpable purpura to subcutaneous nodules)

45
Q

what is microscopic polyangiitis

A

Small vessel vasculitis, no granulomatous infiltration

46
Q

what do all 3 ANCA associated vasculitis have

A

pulmonary haemorrhage as a consequence of alveolar capillary involvement

47
Q

what is seen in renal involvement of small vessel vasculitis

A

(more common in GPA and MPA)

Haematuria and proteinuria

AKI

Renal biopsy- shows segmental necrotizing glomerulonephritis

48
Q

Tx of small vessel vasculitis

A

Immunosuppressive therapy
Plasma Exchange
May require renal support

49
Q

what is seen in SLE in relation to the kidneys

A

Lupus Nephritis

  • Proteinuria
  • number of other renal diseases
50
Q

how can the renal disease affecting the kidney in SLE be differentiated

A

Renal Biopsy

51
Q

Tx for SLE affecting Kidneys

A

Non-immunosuppressive:
ACE/ARB target BP 130/80

Immunosuppressive therapy:

1 - Induction;
High dose steroids
Cyclophosphamide/ MMF
Azathioprine, rituximab, tacrolimus

2 - Maintenance
Steroids
MMF/azathioprine

52
Q

what is the classification of kidney disease in SLE (Class I-VI)

A 
Class I: Minimal mesangial
Class II: Mesangial Proliferative
Class III: Focal Proliferative
Class IV: Diffuse Proliferative
Class V: Membranous
Class VI: Advanced sclerosing
53
Q

what drugs can cause pre-renal failure

A

e.g. hypotension, hyopvoemia

Diuretics > dehydration
ACEi/ARBs > diarrhoea and vomiting

54
Q

what drugs can renal failure

A

e.g. Acute Interstitial Nephritis/Tubular necrosis

Gentamicin
Sulphonamides
Aspirin

55
Q

what drugs can post-renal failure

A

e.g. Retroperitoneal fibrosis, crystaluria, urinary calculi

Methysergide
Chemotherapy

56
Q

what can beta blockers cause if on for prolonged therapy

A

Diabetes

57
Q

what can NSAIDs cause

A

hypertension through renal damage

58
Q

what can abrupt stop of beta blockers cause

A

angina

59
Q

what are the two forms of polycystic disease

A

Autosomal Recessive

Autosomal Dominant - more common

60
Q

what are the two mutations seen in ADPKD and which is more common

A

PKD gene1 mutations; more common
- located on chromosome 16

PKD2 mutations; less common
- located on chromosome 4

61
Q

who develops end stage kidney failure at an earlier stage

A

PKD1

62
Q

renal features of ADPKD

A

Reduced urine concentration ability

Chronic pain

Hypertension-common, early(31 yrs-mean age)

Haematuria- cyst rupture, cystitis, stones

Cyst Infection

Renal Failure

63
Q

non-renal features of ADPKD

A

Hepatic Cysts - very common :
Liver function generally preserved. Can result in SOB, Pain, Ankle swelling

Intra Cranial Aneurysms

Cardiac Disease
- Mitral/Aortic value prolapse

Diverticular disease
- Diverticulitis and colonic perforation

Hernias - Abdominal/Inguinal

64
Q

Ix of ADPKS

A

Ultrasound-presence of multiple bilateral cysts
Renal enlargement seen.
CT/MRI-when unclear on USS

65
Q

in children, what suggests that it is ARPKD and not ADPKD

A

USS suggestion of Congenital Hepatic Fibrosis

66
Q

if a person has ADPKD how likely is it that there child will have it

A

50%

67
Q

Mx of ADPKD

A

Control Hypertension (target level 130/80 mmHg) e.g. ACEi best choice

Tolvaptan - to reduce cyst volume and progression

68
Q

how gets ARKD and what is it associated with

A

children

hepatic lesions

69
Q

what is the mutation in ARKD

A

PKDH1 on chromosome 6

70
Q

what is the renal involvement in ARKD

A

Renal involvement is bilateral and symmetrical
Urinary tract is generally normal
Histologically cysts are seen appearing from the collecting duct system

71
Q

what is the clinical presentation of ARPKD

A

Kidneys always palpable

Hypertension
Recurrent Urinary Tract Infections

Slow Decline in GFR -less than 1/3 reach dialysis

72
Q

Ix of ARPKD

A

US
CT scan
MRI

73
Q

what is Alports Syndrome and what is the genetic role in it

A

Hereditary Nephritis - Disorder of Type IV collagen matrix
X-linked inheritance
Mutation of COL4A5 leads to deficient collagenous matrix

74
Q

Alports Syndrome manifestations

A

Sensorineural deafness
Pyelonephritis
Haematuria
Renal failure

75
Q

when should Alports be suspected

A

Haematuria and hearing kiss

76
Q

Ix of Alports

A

Renal Biopsy-

Variable thickness GBM- characteristic feature

77
Q

Tx for Alports

A

No specific treatment

Will need dialysis/transplantation

78
Q

what is Anderson Fabrys disease

A

X linked disease lysosomal storage disease
Leads to a deficiency of alpha-galactosidase A
Affects kidneys, liver, lungs, erythrocytes
Uncommon

79
Q

Tx of Fabrys disease

A

Enzyme replacement-Fabryzyme

Management of Complications

80
Q

what is medullary cystic kidney

A

autosomal dominant inherited disroder
causes tubular loss and medullary cyst formation
See shrunken kidneys

81
Q

Tx for medullary cystic kidney

A

Renal transplantation

82
Q

what is medullary sponge kidney

A

Sporadic Inheritance
Dilatation of collecting ducts
Severe cases- medullary area appears like a sponge
Cysts have calculi

83
Q

Ix of Medullary sponge kidney

A

Excretion Urography

Renal SB (15 decks)

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