Nephrotic syndrome (Complete)

Question 1

Define nephrotic syndrome

Answer 1

Presence of proteinuria (>3.5 g/24 hours), hypoalbuminaemia (<30 g/L), and peripheral oedema

Question 2

List some causes of nephrotic syndrome (7)

Answer 2

Minimal change disease (Type of kidney disease characterised by large loss of protein in the urine due to damage to the glomeruli often on a level not detcted by normal microscopes)

Congenital nephrotic syndrome

Focal segmental nephroscleorsis (disease in which scar tissue develops on the glomeruli)

Membranous nephropathy (damage or thickening of the glomeruli)

Diabetic nephropathy and Non-diabetic renal disease

Membranorpoliferative glomerulonephritis (auto-immune damage to glomeruli)

Malignant hypertension (High BP that comes on suddenly and quickly).

Amyloidosis

Systemic lupus erythematosus

Diabetes

Question 3

What 3 causes of nephrotic syndrome tend to present mainly in children?

Answer 3

Minimal change disease

Congenital nephrotic syndrome

Myeloproliferative glomerulonephritis

Question 4

What is the most common cause of nephrotic syndrome in children?

Answer 4

Minimal change disease

Question 5

What is the most common cause of nephrotic syndrome in adults?

Answer 5

Membraneous glomerulonephritis

Question 6

List 4 examples of infections which can increase risk of developing nephrotic syndrome.

Answer 6

Hepatitis B and C

HIV

Malaria

Untreated strep infection

Question 7

List examples of medications that increase the likelihood of developing nephrotic syndrome.

Answer 7

NSAIDs

Pamidronate (Osteoporosis)

Lithium (e.g. BPD)

Gold (Rheumatoid arthiritis)

Penicillamine (Wilson’s disease)

Question 8

List examples of over-the-counter or herbal remedies that can increase likelihood of developing nephrotic syndrome

Answer 8

Chinese herbal remedies

Mercurial skin-lightening creams

Question 9

In general, the evaluation of nephrotic syndrome should be done promptly, but it is not a medical emergency. In which circumstance would you consider hospital admission however? (2)

Answer 9

Rapid deterioration of renal function (indicated rapidly progessing glomerulonephritis)

Severe nephrotic syndrome in which a patient may need to be admitted for intravenous diuresis especially if there is an AKI.

Question 10

What are the 2 main clincal signs/symtpoms of nephrotic syndrome?

Answer 10

Odema predominantly in the legs (Can progress into anasarca in extreme cases)

Foamy urine

Question 11

List examples of associated signs/symptoms that may present alongside nephrotic syndrome.

Answer 11

Signs of malignancy: Cough, weight loss, night sweats, a change in bowel habits, gatrointestinal blood loss

Signs of SLE: Rash, mouth ulcers, or arthralgias

Signs of Fabry’s disease (e.g., painful neuropathy)

Question 12

List examples of physical signs that may present in patients with nephrotic syndrome. (4)

Answer 12

Mild oedema of the legs
Anasarca (Peri-oribital and facial swelling more likely to be nephrotic versus other causes e.g. cardiac)

White banding of the nails (due to hypoalbuminaemia aka Muehercke lines[https://en.wikipedia.org/wiki/Muehrcke%27s_nails]

Lean body mass (in heavy proteinuria however may be concealed by weight gain of odemea)

Xanthelasmata (hypercholesteroaemia)

N.B. Can present with other signs of underlying pathology such as rash in SLE, easy bruising and neuropathy with amyloidosis ect).

Question 13

Patients with nephrotic syndrome are at risk of which 2 complications?

Answer 13

Chronic kidney disease
End-stage renal disease (last stage of CKD)

Question 14

What common investigations are used to diganose nephrotic syndrome and their typical findings?

Answer 14

Bedside:

Urine dipstick: Proteinuria

Urinalysis: Cellular casts, raised urine albumin-creatinine ratio

Bloods:

FBC

Creatinine and U&Es: Assess renal function

Lipid profile: Show elevated total and LDL cholesterol

LFT and serum albumin: Check for hypoalbuminaemia

Other tests depending on if secondary cause is suspected: e.g. Serum free light chains and urine protein electrophoresis, HIV test

Invasive/imaging:

Renal biopsy: Indicated in all adults

Question 15

Why are renal biopsies rarely used when determining the cause for nephrotic syndrome in children?

Answer 15

The majority of causes of nephrotic syndrome in children are due to minimal change disease so they should be treated first and then consider a biopsy if treatement is inneffective to determine rarer causes.

Question 16

List 7 contraindications for renal biopsy

Answer 16

Bleeding diathesis (increased tendency to bruise and bleed)

Multiple, bilateral renal cysts

Renal tumour

Hydronephrosis (swelling of kidneys due to buildup of urine)

Active renal infection

Small kidneys secondary to chronic irreversible disease

Severe and resistant hypertension.

Question 17

What is the management plan for nephrotic syndrome?

Answer 17

Must treat the underlying cause which varies.
Main treatment goals should then be to:

Manage BP (e.g. ACE, ARBs)

Lower cholesterol (e.g. cholseterol lowering medications such as statins)

Reduce swelling (e.g. diuretics)

Medicine:

High dose steroids which are then tappered to clincial response.

Question 18

What is the main treatment option for nephrotic syndrome in children?

Answer 18

Steroids (e.g. prednisolone)

Question 19

List examples of complications of nephrotic syndrome (6)

Answer 19

Protein malnutrition

Hypovolemia due to over-diuresis

Hyperlipidaemia : Due to increased hepatic production of lipids to restore the serum oncotic pressure)

Hypercoagulability (causing arterial and venous thrombosis.)

Immunocompromised state and susceptible to
infections

Question 20

Why does nephrotic syndrome cause venous thromboembolism?

Answer 20

Due to urinary loss of antithrombin III

Question 21

Why does nephrotic syndrome increase susceptibility to infection?

Answer 21

Due to urinary loss of immunoglobulins

Question 22

Why does nephrotic syndrome cause hyperlipidaemia?

Answer 22

Due to increased hepatic production to compensate for low serum oncotic pressure