Nephrotic Syndrome Flashcards

1
Q

Define nephrotic syndrome

A

Characterised by:
Proteinuria (>3g/24h)
Hypoalbuminaemia (<30g/L)
Peripheral oedema

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2
Q

What are the causes of nephrotic syndrome

A

Primary:
Steroid-sensitive: minimal change
Steroid-resistant:
- Focal segmental glomerulosclerosis
- Membranous glomerulonephritis/nephropathy
- Membranoproliferative glomerulonephritis (60% nephrotic)
Congenital nephrotic syndrome

Secondary:
Diabetes mellitus
Sickle cell disease
Amyloidosis
Malignancies
Drugs e.g. NSAIDs
Alport’s syndrome
HIV

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3
Q

What are the symptoms and signs of nephrotic syndrome

A

Swelling (face, abdomen, limbs, genitalia)
- Peri-orbital oedema, especially on waking
- Abdominal swelling → pain
Weight gain (oedema)
Muehrcke’s lines (white bands from hypoalbuminaemia)
Complications:
- pleural effusion → SOB
- Infections
- Renal vein thrombosis → loin pain, haematuria

+ symptoms of underlying cause

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4
Q

What are the features of steroid-sensitive nephrotic syndrome and which syndromes are steroid-sensitive

A

85-90% of children with nephrotic syndrome have treatment with corticosteroid therapy
Rarely progresses to CKD/ESRF
Often precipitated by respiratory infections
No macroscopic haematuria, normal BP, complement, renal function, biopsy on LM
Electron microscopy: Fusion of specialised epithelial cells that invest the glomerular capillaries (podocytes) on electron microscopy (minimal change disease)

Minimal change

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5
Q

Describe minimal change disease

A

Most common cause of nephrotic syndrome
Often after a viral infection (→ peri-orbital oedema, abdominal pain)
Steroid-sensitive

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6
Q

What will investigations show for minimal change disease

A

Complement: normal
Renal function: normal
Renal biopsy (not required for diagnosis):
- LM: normal
- EM: fusion of epithelial cells → loss of foot processes/podocytes in glomerular capillaries

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7
Q

What is the management for minimal change disease

A

First line: Steroids PO
- Prednisolone for 4 weeks (60mg/m^2)
- Reduce dose to 40 on alternative days for 4 weeks
- Wean and stop steroids

Second line: steroid-sparing Immunosuppressants
- Ciclosporin
- Mycophenolate
- tacrolimus
- Rituximab

+ Make sure up to date with immunisations
± prophylactic Abx (penicillin)

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8
Q

Describe focal segmental glomerulosclerosis (epidemiology, causes, appearance on microscopy, management)

A

Most common cause of nephrotic syndrome in adults, particularly Afro-Caribbeans and obese pts
May be primary or secondary (heroin, HIV, SCD)

LM: focal and glomerular consolidation → scarring, hyalinosis (NO immune complexes)
EM: loss of foot processes

Management: (1) steroids (2) calcineurin inhibitors

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9
Q

Describe membranoproliferative glomerulonephritis (association, types, investigation)

A

Can present as nephrotic (60%), nephritic (30%), or rapidly progressive glomerulonephritis
Elderly people
Associated with malignancy, SLE, hep B infection

Type I (90%): Primary or secondary (chronic infection e.g. abscess, IE, HIB )
Type II: Primary or secondary (partial lipodystrophy, factor H deficiency)

Haematuria + low complement

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10
Q

Describe congenital nephrotic syndrome

A

Presents in the first few months of life
Rare, usually recessive
More common in consanguineous families
Genetic testing recommended: >70% have a genetic defect
The albuminuria can be so severe that unilateral nephrectomy may be necessary

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11
Q

Describe membranous nephropathy (Cause, appearance on microscopy, management)

A

Primary or secondary (hepatitis B, SLE)
Antibody against Phospholipase A2r (PLA2R) → immune complex formation

LM: diffuse basement membrane thickening
EM: “spike and dome” subepithelial deposits of the entire GBM, loss of foot processes

Management:
ACEi or ARB (reduces proteinuria) ± anticoagulation
Severe: immunosuppression: steroids (but poor response) ± cyclophosphamide

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12
Q

What is the prognosis for membranous glomerulonephritis

A

1/3 - spontaneous remission
1/3 - remain proteinuric
1/3 - develop end-stage renal failure

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13
Q

What are the complications of nephrotic syndrome

A

Hypovolaemia (oedema → intravascular compartments become deplete)
thrombosis (loss of Antithrombin in urine)
Infection (loss of Ig in the urine → NHS bacterial infection)
Cholesterolaemia (albumin loss → reduced oncotic pressure → hepatic cholesterol synthesis)

Albumin given for hypovolaemia → fluid overload →
pulmonary oedema and HTN

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14
Q

What is the prognosis for the steroid-resistant nephrotic syndromes

A

Focal segmental: 30% progress to ESRF, 20% respond to immunosuppressants
Membranoproliferative: decline in renal function over many years, treat with ACEi or immunosuppressants
Membranous: most remit spontaneously within 5 years

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