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Nephrotic and Nephritic Syndrome
Dr Claire Gibbons FY2
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Learning Objectives Understand and define nephrotic and nephritic syndromes. Describe the initial investigations and management of nephrotic and nephritic syndromes. Describe the complications of nephrotic and nephritic syndromes.
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Draw a nephron!
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Glomerulonephritis Glomerulus – capillary loop with basement membrane which allows passage of specific molecules into the nephron Glomerulonephritis – inflammation/damage of the glomerular basement membrane resulting in altered function. Relatively uncommon cause of kidney injury. Can present as nephrotic and/or nephritic syndrome.
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What is nephrotic syndrome?
Increased permeability of the glomerulus leading to loss of proteins into the tubules.
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Nephrotic Syndrome Triad of: And often:
MASSIVE Proteinuria >3g/24hours Or spot urine protein:creatinine ratio > mg/mmol Hypoalbuminaema <25g/L Oedema And often: Hypercholesterolaemia/dyslipidaemia (total cholesterol >10mmol/L)
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Presentation New-onset oedema Frothy urine
Initially periorbital or peripheral Later genitals, ascites, anasarca Frothy urine Generalised symptoms – lethargy, fatigue, reduced appetite
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Further possible presentations...
Oedema BP normal/raised Leukonychia Breathlessness: Pleural effusion, fluid overload, AKI DVT/PE/MI Eruptive xanthomata/ xanthalosmata
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You are a GP with the following patients...
Young, fit 24 year old male complaining of frothy urine. 10 year old boy with puffy eyes. 74 year old female with multiple co-morbidities and swollen ankles.
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Differential Diagnosis for Oedema
Congestive Cardiac Failure Raised JVP, pulmonary oedema, mild proteinuria Liver disease Hypoalbuminaemia, ascites/oedema What investigations can you do? You decide to send your patient to the renal clinic...
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Causes of Nephrotic Syndrome
Primary glomerulonephritis Minimal change disease (80% paeds cases) Focal segmental glomerulosclerosis (most common cause in adults) Membranous glomerulonephritis
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Systemic Causes Secondary glomerulonephritis Diabetic nephropathy
Sarcoidosis Autoimmune: SLE, Sjogrens Infection: Syphilis, hepatitis B, HIV Amyloidosis Multiple myeloma Vasculitis Cancer Drugs: gold, penicillamine, captopril, NSAIDs
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Investigations Urine dipstick and send to lab Urine microscopy
Bloods – the usual ones, plus renal screen Immunoglobulins, electrophoresis (myeloma screen), complement (C3, C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-GBM) Renal ultrasound Renal biopsy (all adults) Children generally trial of steroids first
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Management Conservative Medical Surgical
Monitor U&E, BP, fluid balance, weight Salt and fluid restriction Treat underlying cause Medical Diuretics ACE-inhibitors/ARBs Corticosteroids/immunosuppression Dialysis Anticoagulation Surgical Renal transplant
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Complications Increased susceptibility to infection Thromboembolism
20% adult cases Due to reduced serum IgG, reduced complement activity, reduced T cell function Thromboembolism 40% adult cases Partly due to increased clotting factors and platelet abnormalities Hyperlipidaemia due to hepatic lipoprotein synthesis to restore osmotic pressure
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Prognosis Varies With treatment, generally good prognosis
Especially minimal change disease (1% progress to ESRF) Without treatment, very poor prognosis Children under 5 or adults older than 30 = worse prognosis
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What is nephritic syndrome?
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Pathophysiology Thin glomerular basement membrane with pores that allow protein and blood into the tubule.
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Nephritic Syndrome Clinical syndrome defined by:
Haematuria/ red cell casts Hypertension (mild) Oliguria Uraemia Proteinuria (<3g/24 hours)
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Signs and Symptoms Haematuria (E.g. cola coloured) Proteinuria
Hypertension Oliguria Flank pain General systemic symptoms Post-infectious = 2-3 weeks after strep- throat/URTI
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What are your differentials?
Malignancy (older patients) UTI Trauma What bedside investigation would you like to do? You decide to refer to the renal clinic...
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Causes Post-infectious glomerulonephritis Primary
IgA Nephropathy (Berger's disease) Rapidly progressive glomerulonephritis Proliferative glomerulonephritis Secondary glomerulonephritis Henoch-Schonlein purpura Vasculitis
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Investigations Urine dipstick and send sample to lab
Urine microscopy – red cell casts Bloods – the usual plus renal screen Immunoglobulins, electrophoresis, complement (C3, C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-GBM); blood culture; ASOT (anti-streptolysin O titre) Renal ultrasound Renal biopsy
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Red Cell Casts
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Management Conservative Medical Surgical
Monitor U&E, BP, fluid balance, weight Salt and fluid restriction Treat underlying cause Medical Diuretics Treat hypertension Corticosteroids/immunosuppression Dialysis Surgical Renal transplant
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Prognosis Varies Post-infectious usually self-resolving (95% recover renal function) Others are a bit more nasty
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Example Case
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Summary Nephrotic syndrome = MASSIVE proteinuria
Nephritic syndrome = haematuria/red cell casts May be a mixed presentation New oedema? Dipstick that urine! Haematuria? Exclude malignancy!
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Any Questions?
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Sources Oxford Handbook of Clinical Medicine
Oxford Handbook for the Foundation Programme Essential Revision Notes for the MRCP
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