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GI, liver, kidney > Nephritic syndrome > Flashcards

Nephritic syndrome Flashcards

1
Q

What is RPGN

A

Severe glomerulonephritis fulminant course with rapid detoriaration in renal function over days to weeks to months - type of nephritic syndrome

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2
Q

What is nephritic syndrome

A

Haematruia, proteinuria, renal impairment, HPTN

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3
Q

Cuases of nephritic syndrome

A

Immune complex deposition (AI, post strep, IgA)
AntiGBM deposition
Small vessel vasculitis (polyangitis)

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4
Q

Causes of immune nephritic syndrome

A

SLE
Post strep glomerulonephritis
IgA nephropathy
Cryoglobuniaemia

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5
Q

Antibodies ass with small vessel vasculitis

A

ANCA
anti-GBM -

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6
Q

ANCA realted vasculitis can cause

A

Microscopic polyangitis - MPA
Grnaulomatosis with polyangitis
Eosinophilic granulomatosis w polyangtitis

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7
Q

Anti GBm targets

A

GBM antibodies target type I collagen within basement membrane
Linear depositon of IgG in glomerular capillaries

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8
Q

Symptoms of nephritic syndrome

A

Lethargy
Recent infection - fever, sore throat, coryzal symptoms - post strep glom and IgA nephropathy
Haematuria
Oliguria
Oedema - peripheral, periorbital
SOB - fluid overload
Haemoptysis - due to pulmonary haemorrhage eg anti-GBM ANCA-vasculitis

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9
Q

Signs of nephritic syndrome

A

Haematuria
HPTN
Oedema - peripheral, periorbital
Fluid overload - raised JVP, bibasal crackles on auscultation, peripheral oedema, ascites
Reduced urine output

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10
Q

Extra renal manifestations

A

Skin - vasculitis rash - palpable purpura
ENT - rhinosinusitis, nasal discharge, polychondritis (cartliage inflammation)
Eyes - red, painful eyes (eg conjunctivitis, scleritis, uveitis)
Lungs - haemoptysis, pleuritic pain, wheeze
Heart - chest pain - pericardial or MI involvement
Nervous system - mononeuropathies

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11
Q

How is nephritic syndrome diagnosed

A

Haematuria, proteinuria, deteriorating renal function

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12
Q

Glomerular origin of haematuria evidence

A

Dysmorphic RNC
Red blood cell casts

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13
Q

What is a cast

A

Cluster of particles wrapped in uromodulin - secreted by epothelial cells lining loop of Henle, distal tube and collecting duct

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14
Q

Manaegment of nephritic syndrome

A

General supportive measures for AKI
Temporary RRT
Immunosupressive therapy

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15
Q

Complications nephritic syndrome

A

Can cause severe AKI and ass complications
Can lead to widespread organ dysfunction

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16
Q

What does post infective glomerulonephritis occur after

A

Group A beta haemolytic streptococcus

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17
Q

What population is PSGN most common in

A

Children

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18
Q

post exposure length after infection for PSGN

A

1-3 weeks

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19
Q

Diagnosis

A

Haematuria, proteinuria, decline renal function + recent strep infetion
Anti-streptolysin titre - ASOT - evidence for recent infection
Renal biopsy in adults

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20
Q

RSPN management

A

Managing complications
Resolution normally rpaid and outcome excellent in children
Adults more varibale
Active infection - antibiotic

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21
Q

Renal biopsy findings of post infective glomerulone[hritis

A

Diffuse proliferative and exudative glomerular hisotry
Dominant C3 staining and subepithelial humps

22
Q

SLE nephropathy what causes

A

Anti double stranded DNA deposit in glomeruli, other immune complexes, area of deposition can include mesangium, enothelium or epithelium

23
Q

Classiications of lupus nephritis

A

Class I - minimal mesangial
Class II - mesangial proliferative
Class III - focal
Class IV - diffuse
Class V - membranous
Class VI - advance scleorisis

24
Q

Most common and severe form of SLE nephritis

A

Class IV diffuse - nephrotic or nephritis syndrome

25
Q

How do class I, II and III of SLE nephritis present

A

I - asymptomatic
II - non visible haem and proteinuria
III - variabel, may have haematuria, proteinuria, renal impairment and/or nephrotic syndrome

26
Q

Class V SLE nephritis vs Class VI presentation

A

V - nephrotic syndrome similar to membranous nephropathy
VI - advance sclerosis - global sclerosis and slowly worsening renal function

27
Q

Management SLE nephritis

A

Class IV and above
High dose systemic immunosuppression require RRT

28
Q

What causes IgA nephropathy

A

IgA Deposition of IgA nephropathy in the glomeruli

29
Q

Treatment of IgA nephropathy

A

High dose prednisolone
Immunosupressive drugs Good BP control, largely supportive

30
Q

How does IgA nephropathy present

A

Nephritic syndrome 24-48 hours after URTI

31
Q

Investigations for IgA nephropathy

A

Increased mesangial cells
Increased matrix
IgA deposition in matric

32
Q

Prognosis of IgA nephropathy

A

20-30% -> renal failure

33
Q

Pathology of anti GBM disease

A

antiGBM attacks basement membrane in lungs and kidney -> glomerulo/pulm haemorrhage

34
Q

Treatment antiGBM

A

Potent immunosupression
Plasmapheresis

35
Q

What is membranoproliferative GN?

A

Glomerular basement membrane thickening
Proliferative changes on light microscopy

36
Q

What is membranoproliferative GN ass with

A

Hepatitis C
AI eg SLE

37
Q

Appearance of membranoproliferative disease on biospy

A

Thickened basement membrane
Thickened mesangium
Tram tracking appearance
Subendothelial deposition of IgG - on immunoflurescence

38
Q

Managmenet of membranoproliferative GN

A

Dipyridamole + aspirin
Kidney transplantation - end stage renal disease

39
Q

Managmenet of membranoproliferative GN in children with nephrotic range proteinuria

A

Corticosteroids

40
Q

What is RPGN

A

Acute nephritic syndrome with microscopic glomerular crescent formation

41
Q

What antibody do patients usually have in small vessel vasculitis

A

ANCA

42
Q

What is GPA

A

Wegners - extremely rare long term systemic disorder - formation of granulomas + inflammation of small and medium blood vessles
URTI, lungs, kidneys

43
Q

What is GPa caused by AB?

A

c-ANCA

44
Q

Biopsy of GPA shows

A

Leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation

45
Q

Management of GPA

A

Immunosupression - remission with rituximab or cyclophosphamide in combination with high dose corticosteroids

46
Q

What is microscopic polyangtitis

A

Systemic small vessel vasculitis

47
Q

Clinical features of microscopic polyangitis

A

weight loss, fevers, fatigue, renal failure

48
Q

Managemnet of microscopic polyangitis

A

Long term prednisolone
Cyclophosphamide therapy - cycled
Plasmapheresis

49
Q

What is goodpasteurs syndrome

A

When lung involvement with anti-GBM (haemoptysis)
IgG

50
Q

Management of anti-GBM disease

A

IV prednisolone
Cyclophophamide
Plasmapheresis

GI, liver, kidney (50 decks)

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