Nephrotic nephritic

Question 1

classical features of nephrotic syndrome

Answer 1

1. Proteinuria (>3g in 24h)
2. Hypoalbuminemia (<25g/L)
3. Oedema

Question 2

additional features of nephrotic syndrome

Answer 2

Severe hyperlipidaemia

●↑Susceptibility to infection: (cellulitis, Streptococcus infections SBP in up to 20% of adult patients because of ↓serum IgG, ↓complement activity, and ↓T cell function (due in part to loss of immunoglobulin in urine and also to immunosuppressive treatments).

●Thromboembolism: (up to 40%): DVT/PE, renal vein thrombosis. This hyper-coagulable state is partly due to ↑clotting factors & platelet abnormalities.

Question 3

Features of nephritic syndrome

Answer 3

1. Haematuria with RBC casts ± dysmorphic RBCs
2. Mild-moderate proteinuria
3. ↑BP
4. Progressive oliguria and renal impairment → Uraemia (symptoms of this include lethargy, pruiritis, nausea and vomiting)
5. Oedema (often periorbital, as well as dependent e.g. ankle and sacral)

Question 4

GLOMERULONEPHRITIS is what?

Answer 4

GN is a common cause of ESRF in adults, along with diabetes and hypertension. T

hey are a group of disorders where damage to the glomerular filtrating apparatus causes a leak of protein ± blood into the urine, depending on the disease

Question 5

GNs causing nephrotic synderome – podocyte injury, no inflammation:

Answer 5

1⁰ due to autoimmune → membranous GN

2⁰ due to:

• Drugs (NSAIDS, gold, penicillamine),
• Malignancy,
• HBV, HCV;
• SLE → ANA dsDNA (antinuclear antibodies to dsDNA)
• Sarcoidosis → high serum ACE
• Amyloidosis
• Bacterial/protozoal infections
• Sjogren syndrome
• Myeloma: Bence-Jones protein in urine(free immunoglobulin lght chain) or monoclonal band in plasma

Question 6

NG causing nephritic syndrome – inflammation, cell proliferation

Answer 6

• IgA nephropathy (Berger’s disease)
• Anti-GBM disease (goodpasture’s) + pulm Haemorrhage (↑transfer factor)
• ANCA-associated vasculitis (anti neutrophil cytoplasmic antibodes)
• Post-strep GN
• SLE

Question 7

Minimal change GN prevalence

Answer 7

90% of nephrotic syndrome n children, 20% in adults

Question 8

cause of minimal change GN

Answer 8

usually idiopathic, can be URTI, NSAIDS

Question 9

Light and electron microscopy of minical change GN

Answer 9

light microscopy normal, immunostaining normal

electron micro: fusion of podocyte foot processes

Question 10

tx of minimal change GN

Answer 10

steroids, ciclosporin

Question 11

prevalence of focal segmental glomerulosclerosis FSGS

Answer 11

15% o f adult nephrotic syndrome

Question 12

features and pathology of FDGS

Answer 12

• Some glomeruli have scarring of certain segments (ie focal sclerosis).

(immunofluorescence): IgM and C3 deposits in affected areas.

●electron microscopy → fusion of podocyte foot processes

Question 13

presentation of FSGS

Answer 13

: Usually nephrotic syndrome or proteinuria. ~50% have impaired renal function. •

may be primary (idiopathic) or secondary (vesicoureteric reflux, IgA nephropathy, Alprot’s syndrome , vasculitis, sickle-cell disease, heroin use).

o HIV is associated with a subtype.

Question 14

Tx of FSGS

Answer 14

steroids, if resistant: ciclosporin, cyclophosphamide

Question 15

prognosis of FSGS

Answer 15

30–50% → ESFR. It recurs in 20–50% of transplanted kidneys, which may respond to plasma exchange.

Question 16

membranous GN prevalnece

Answer 16

most common cause of nephrotic syndromne in the elderly,

20-30% of nephrotic syndrome in adults

Question 17

membranous GN pathology and features

Answer 17

thickening of glomerular basement membrane + subepithelial deposits

Question 18

presentation of membranous GN

Answer 18

●Presentation: nephrotic syndrome, DVTs

Question 19

aetiology of membranous G

Answer 19

• malignancy,
• HBV,
• SLE, RA,
• gold, captopril or penicillamine drugs,
• malignancy in lungs, colon,
• CLL, lymphoma

Question 20

tx of membranous GN

Answer 20

steroids, chlorambucil, cyclophosphamide

Question 21

prognosis of membranous GN

Answer 21

40% will have spontaneous rmission

Question 22

Membranoproliferative GN frequency and patho

Answer 22

uncommon, mainly in the young ●

mesangial cell proliferation, excess matrix, thickening of glomerular basement membrane ●immune deposits, C3, C5 complement depletion

Question 23

Membranoproliferative GN: presnetation

Answer 23

nephrotic syndrome, ~30% nephritic syndrome

Question 24

Membranoproliferative GN: biopsy findings

Answer 24

• Biopsy shows large glomeruli: mesangial proliferation and thickened capillary walls → ‘tramline’ appearance of a double bm.

Question 25

Membranoproliferative GN tx and prognosis

Answer 25

• Tx: None proven of benefit so far; steroids in children, anti-CD20 (Rituximab). Prognosis: 50% develop esrf. It can recur in transplants.

Question 26

Proliferative GN is classified histologically

Answer 26

: focal, diffuse, or mesangiocapillary GN. The chief cause is post-streptococcal GN (a diffuse proliferative GN), occurring 1–12 weeks after a sore throat or skin infection. A streptococcal antigen is deposited on the glomerulus causing a host reaction and immune complex formation.

Question 27

IgA GN features and etiology

Answer 27

Berger’s disease/Mesangioproliferative

most common primary glomerular disease

●typically: young ♂ with macroscopic hematuria 1-2d after URTI ●↑mesangial cell & matrix, IgA deposition ●no Tx ●ESRF

Question 28

Antiglomerular basement membrane disease (Goodpasture’s syndrome

Answer 28

antibodies agains type 4 collagen in the GBM and alveolar BM in lung → inflammation → acute renal failure, lung haemorrhage

●Tx: plasma exchange to remove antibodies, immunosuppression with steroids and cyclophosphamide

Question 29

Alprot’s syndrome features

Answer 29

Features: bilateral sensorineural deafness (may be mild), persistent microscopic haematuria, proteinuria (defect in GBM), CRF, may have ocular abnormalities.

85% of cases are X-linked dominant.

Defect in gene encoding type IV collagen