Pathology:
Proteinuria > 3g/24 hours,
Hypoalbuminaemia < 30g/l and
Hyperlipidaemia
Oedema also an associated hypercholestrolaemia.
Aetiology: Primary: Minimal change disease, membranous nephropathy, focal segmental
glomerulonephritis
Secondary: Diabetes, SLE, amyloidosis, hypertension
Symptoms: Swollen legs, weight gain, lethargy, shortness of breath.
Signs: Marked pitting oedema, ascites and pleural effusions
Investigations: Bloods: FBC, U&E, LFT’s, lipid profile glucose, myeloma screen, ANA
MSU: Urine dipstick and protein: creatinine ratio
Ultrasound: Renal ultrasound to assess for structural abnormalities
Renal Biopsy: Diagnostic
Treatment: Medical: Loop diuretics, ACE inhibitors, statins
Complications: Hypercoagulability, hyperlipidaemia, infection
Prognosis: Dependent on underlying cause and response to treatments; persistent heavy proteinuria is associated with progressive renal failure and worse outcomes.
Figure 4.1 Classical signs/symptoms of Nephrotic Syndrome
Pathology: Nephrotic Syndrome with little changes seen on light microscopy. Most common cause of nephrotic syndrome in children.
Aetiology: Unknown aetiology
Symptoms: Swollen legs, weight gain, lethargy, shortness of breath.
Signs: Marked pitting oedema, ascites and pleural effusions
Investigations: Bloods: FBC, U&E, LFT’s, lipid profile glucose, myeloma screen, ANA
MSU: Urine dipstick and protein: creatinine ratio
Renal Biopsy: Light microscopy shows no changes but electron microscopy
show podocyte fusion
Treatment: Medical: Steroid therapy, immunosuppression
Complications: Renal failure
Prognosis: Children: 95% resolve with steroids – 33% have no further relapses with 33%
having regular relapses
Adults: 70% resolve with steroids but are more likely to relapse
Pathology: Slowly progression nephrotic syndrome caused by immune complex deposition and glomerular inflammation. Most common in adults.
Aetiology: Idiopathic
Infection: Streptococcus viridans, HBC, mumps, malaria, syphilis
Malignancy: Melanoma, lymphoma and lung cancer
Autoimmune: SLE, rheumatoid arthritis
Drugs: Gold, penicillamine, heroin
Symptoms: Swollen legs, weight gain, lethargy, shortness of breath.
Signs: Marked pitting oedema, ascites and pleural effusions
Investigations: Bloods: FBC, U&E, LFT’s, lipid profile glucose, myeloma screen, ANA
MSU: Urine dipstick and protein: creatinine ratio
Renal Biopsy: Thickened basement membrane with spikes detected on the
epithelial aspect using silver staining
Treatment: Medical: Steroids, immunosuppression and dialysis if renal failure evident.
Complications: Renal failure, renal vein thrombosis
Prognosis: Young female patients have the best prognosis.
Overall, 30% remission, 25% relapse, 25% slow progression, 20% end stage renal failure
Pathology: Nephrotic Syndrome in which only some of the glomeruli are affected in a region of the glomerulus leading to renal scarring.
Aetiology: Primary: Idiopathic
Secondary: IgA nephropathy, Obesity, Heroin use, HIV, ureteric reflux
Symptoms: Swollen legs, weight gain, lethargy, shortness of breath.
Signs: Marked pitting oedema, ascites and pleural effusions
Investigations: Bloods: FBC, U&E, LFT’s, lipid profile glucose, myeloma screen, ANA
MSU: Urine dipstick and protein: creatinine ratio
Renal Biopsy: Partially sclerosed glomeruli
Treatment: Medical: Steroid therapy, immunosuppression
Complications: Renal failure
Prognosis: Majority develop renal failure within 10 years.
Pathology: A group of disorders which cause inflammation of the glomerulus.
Aetiology: Post Group A β-haemolytic streptococcal infection, haemolytic uraemic syndrome, IgA nephropathy, Goodpasture Syndrome
Symptoms: May be asymptomatic. Oliguria, oedema, haematuria, arthralgia, myalgia, nausea
and vomiting
Signs: Hypertension and oedema
Investigations: Bloods: FBC, U&E, blood cultures, ANA, ANCA, anti-GBM, C3 and C4
MSU: Urine dipstick and protein: creatinine ratio
Ultrasound: Renal ultrasound to assess for structural abnormalities
Renal Biopsy: Diagnostic
Treatment: Medical: Salt restriction, fluid monitoring, blood pressure control,
thromboprophylaxis
Complications: Renal failure, pulmonary oedema and hypertensive encephalopathy
Prognosis: Adults: 60% resolve
Children: 90% resolve
Pathology: Immune complex deposition in the glomerulus following infection with Group A β-haemolytic streptococcal
Aetiology: In children after a streptococcal infection
Symptoms: Malaise, anorexia, abdominal pain, Oliguria, oedema, haematuria, arthralgia,
myalgia, nausea and vomiting
Signs: Gross haematuria, oedema, hypertension and fever
Investigations: Bloods: U&E (raised urea and creatinine), C3 (decreased), serum ASO titre to
confirm recent streptococcal infection
MSU: Dipstick for proteinuria and haematuria.
Treatment: Medical: Antibiotics for infection
Complications: Hypertension and renal failure
Prognosis: Very good prognosis in children; 60% of adults recover.