Nephrotic syndrome Flashcards
What are the characteristics of nephrotic syndrome?
- Massive proteinuria (≥3.5g/d): frothy urine
- Hypoalbuminaemia (≤30g/L)
- Oedema
State three complications of nephrotic syndrome
- Infections
- AKI
- Thromboembolisms: urinary loss of antithrombotic factors
- Dyslipidaemia; hypercholesterolaemia; hypertriglyceridaemia
- Impaired clearence
- Abnormalities in coagulation/fibrinolysis
Request four investigations for nephrotic syndrome
- Urine dipstick
- Midstream urine for MCS: exclude UTI
- Early morning urinary protein:creatinine or ACR
- FBC; clotting
- U+Es; LFTs; ESR; CRP; lipid profile
- HbA1c; glucose
- Renal biopsy
Describe the general management of nephrotic syndrome
- Dietary sodium restriction + thiazide diuretic
- Furosemide if unresponsive
- Normal protein intake of 70g daily
List five causes of nephrotic syndrome
- Primary (idiopathic) glomerular disease:
- Minimal-change glomerular disease
- Focal segmental glomerulosclerosis
- Membranous glomerular disease
- Membranoproliferative glomerulonephritis
- Secondary glomerular disease:
- Infection eg. HIV; hepatits B and C; syphilis; malaria
- SLE; RA; HSP
- Amyloidosis; diabetic nephropathy
- Malignancy eg. myeloma; leukaemia; lymphoma
- Pre-eclampsia
Give three features of minimal change glomerular disease
Most often affects children aged 2-4
-
Highly selective proteinuria
- Usually loss of albumin without loss of immunoglobulins
- Oedema: predominantly around face
- Normal renal function; BP; and complement levels
- Increased risk of infections
- Especially UTIs and pneumococcal peritonitis
- Does not progress to CKD, but can relapse
Describe the prognosis of minimal change glomerular disease
Prognosis is overall good, although relapse is common:
- 1/3 one episode only
- 1/3 infrequent relapses
- 1/3 frequent relapses, which stop before adulthood
What is the management of minimal-change nephropathy
- General management of nephrotic syndrome
- Dietary sodium restriction + thiazide diuretic
- Furosemide if unresponsive
- Normal protein intake of 70g daily
- High-dose corticosteroids
- 80% are steroid-responsive
- Consider cyclophosphamide or ciclosporin
Define focal segmental glomerulosclerosis
Sclerotic glomerular lesion that affects some (not all) glomeruli, and some (not all) segments of each tuft
Describe the clinical features of focal segmental glomerulosclerosis
Common in children and young adults
- Massive proteinuria: usually non-selective
- May have haematuria; HTN; impaired renal function
- Can progress to CKD (50% within 10 years)
Outline the management of focal segmental glomerulosclerosis
-
High-dose prednisolone
- 50% response rate
- Consider ciclosporin; cyclophosphamide
Describe the clinical features of membranous glomerulopathy
More common in adults, especially men
- Asymptomatic proteinuria or frank nephrotic syndrome
- Microscopic haematuria; HTN
- 40% progress to CKD
What is the management of membranous glomeruler disease?
1/3 undergo spontaneous remission in 6-12 months
- ACEi at maximum tolerated dose
- Consider:
- Cyclophosphamide + prednisone
- Ciclosporin
Define amyloidosis
- Systemic acquired or inherited disorder of protein folding
- Extracellular deposition of abnormal insoluble fibrils
- Causes progressive organ dysfunction and death
Name three clinical manisfestations of amyloidosis
- Nephrotic syndrome
- Heart failure
- Autonomic and sensory neuropathies
- Carpal tunnel syndrome
