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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
what are risks of Atherosclerotic Renovascular disease
risk for generalised atherosclerosis - rarely renal alone
26
what is the clinical presentation of Atherosclerotic Renovascular disease
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
27
Ix of ischaemic renal disease
US - renal size asymmetry, affected side is smaller CT/MRI angiography Angiography - gold standard booted after CT/MRI as it is invasive
28
Tx of ischaemic renal disease
Medical therapy - manage hypertension Surgical therapy - Angioplasty - Angioplasty + stenting - Stenting alone
29
when are ACEi contra-indicated
Bilateral Renal Artery Stenosis
30
what is multiple myeloma
cancer of the plasma cells
31
what is produced in multiple myelomas that can cause kidney problems
paraprotein
32
what are Sx of myeloma
Bone Pain Weakness Fatigue Weight Loss
33
what are signs of myeloma
``` Anaemia Hypercalcaemia Renal Failure Amyloidosis Recurrent infections ```
34
Ix for myeloma
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
what are renal manifestations of multiple myeloma
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
what is amyloidosis, how is it characterised and classified
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
what are renal amyloidosis
primary (AL) amyloid and secondary (AA) amyloid
38
how do renal amyloidosis present histologically
Positive Congo red staining showing apple-green birifringence under polarised light (BUZZWORD)
39
Tx of myeloma and ARF
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
what type of vasculitis more commonly affects the kidneys
small vessel vasculitis
41
what are examples of small vessel vasculitis
ANCA associated - Wegener's, Churg-Strauss, Microscopic polyangitis Immune complex associated - HSP, Behcets disease, anti-GBM disease e.g. Good pasture disease
42
what is Granulomatosis with Polyangiitis
(previously Wegeners) Necrotising granulomatous inflammation which most commonly affects the respiratory tract
43
Sx of Gw/P
Nasal crusting, sinusitis, persistent rhinorrhea, otitis media, oral/nasal ulcers, bloody nasal discharge. Evidence of bony/cartilage destruction (saddle nose)
44
what is Eosinophilic Granulomatosis with Polyangiitis
(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
what is microscopic polyangiitis
Small vessel vasculitis, no granulomatous infiltration
46
what do all 3 ANCA associated vasculitis have
pulmonary haemorrhage as a consequence of alveolar capillary involvement
47
what is seen in renal involvement of small vessel vasculitis
(more common in GPA and MPA) Haematuria and proteinuria AKI Renal biopsy- shows segmental necrotizing glomerulonephritis
48
Tx of small vessel vasculitis
Immunosuppressive therapy Plasma Exchange May require renal support
49
what is seen in SLE in relation to the kidneys
Lupus Nephritis - Proteinuria - number of other renal diseases
50
how can the renal disease affecting the kidney in SLE be differentiated
Renal Biopsy
51
Tx for SLE affecting Kidneys
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
what is the classification of kidney disease in SLE (Class I-VI)
``` Class I: Minimal mesangial Class II: Mesangial Proliferative Class III: Focal Proliferative Class IV: Diffuse Proliferative Class V: Membranous Class VI: Advanced sclerosing ```
53
what drugs can cause pre-renal failure
e.g. hypotension, hyopvoemia Diuretics > dehydration ACEi/ARBs > diarrhoea and vomiting
54
what drugs can renal failure
e.g. Acute Interstitial Nephritis/Tubular necrosis Gentamicin Sulphonamides Aspirin
55
what drugs can post-renal failure
e.g. Retroperitoneal fibrosis, crystaluria, urinary calculi Methysergide Chemotherapy
56
what can beta blockers cause if on for prolonged therapy
Diabetes
57
what can NSAIDs cause
hypertension through renal damage
58
what can abrupt stop of beta blockers cause
angina
59
what are the two forms of polycystic disease
Autosomal Recessive | Autosomal Dominant - more common
60
what are the two mutations seen in ADPKD and which is more common
PKD gene1 mutations; more common - located on chromosome 16 PKD2 mutations; less common - located on chromosome 4
61
who develops end stage kidney failure at an earlier stage
PKD1
62
renal features of ADPKD
Reduced urine concentration ability Chronic pain Hypertension-common, early(31 yrs-mean age) Haematuria- cyst rupture, cystitis, stones Cyst Infection Renal Failure
63
non-renal features of ADPKD
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
Ix of ADPKS
Ultrasound-presence of multiple bilateral cysts Renal enlargement seen. CT/MRI-when unclear on USS
65
in children, what suggests that it is ARPKD and not ADPKD
USS suggestion of Congenital Hepatic Fibrosis
66
if a person has ADPKD how likely is it that there child will have it
50%
67
Mx of ADPKD
Control Hypertension (target level 130/80 mmHg) e.g. ACEi best choice Tolvaptan - to reduce cyst volume and progression
68
how gets ARKD and what is it associated with
children | hepatic lesions
69
what is the mutation in ARKD
PKDH1 on chromosome 6
70
what is the renal involvement in ARKD
Renal involvement is bilateral and symmetrical Urinary tract is generally normal Histologically cysts are seen appearing from the collecting duct system
71
what is the clinical presentation of ARPKD
Kidneys always palpable Hypertension Recurrent Urinary Tract Infections Slow Decline in GFR -less than 1/3 reach dialysis
72
Ix of ARPKD
US CT scan MRI
73
what is Alports Syndrome and what is the genetic role in it
Hereditary Nephritis - Disorder of Type IV collagen matrix X-linked inheritance Mutation of COL4A5 leads to deficient collagenous matrix
74
Alports Syndrome manifestations
Sensorineural deafness Pyelonephritis Haematuria Renal failure
75
when should Alports be suspected
Haematuria and hearing kiss
76
Ix of Alports
Renal Biopsy- | Variable thickness GBM- characteristic feature
77
Tx for Alports
No specific treatment | Will need dialysis/transplantation
78
what is Anderson Fabrys disease
X linked disease lysosomal storage disease Leads to a deficiency of alpha-galactosidase A Affects kidneys, liver, lungs, erythrocytes Uncommon
79
Tx of Fabrys disease
Enzyme replacement-Fabryzyme Management of Complications
80
what is medullary cystic kidney
autosomal dominant inherited disroder causes tubular loss and medullary cyst formation See shrunken kidneys
81
Tx for medullary cystic kidney
Renal transplantation
82
what is medullary sponge kidney
Sporadic Inheritance Dilatation of collecting ducts Severe cases- medullary area appears like a sponge Cysts have calculi
83
Ix of Medullary sponge kidney
Excretion Urography

Renal SB (15 decks)

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