claire nephroticandnephritic syndrome (1)
TRANSCRIPT
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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: MASSIVE Proteinuria >3g/24hours
Or spot urine protein:creatinine ratio >300-350mg/mmol Hypoalbuminaema <25g/L Oedema
And often: Hypercholesterolaemia/dyslipidaemia (total
cholesterol >10mmol/L)
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Presentation
New-onset oedema 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
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 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
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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Sources
Oxford Handbook of Clinical Medicine Oxford Handbook for the Foundation
Programme Essential Revision Notes for the MRCP Www.almostadoctor.com Www.pathologystudent.com