Professional Documents
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by Armando HF
Overview
Overview Nephrotic syndrome is a relatively rare but important manifestation of kidney
disease. Nephrotic syndrome classically presents with heavy proteinuria, minimal
hematuria, hypoalbuminemia, hypercholesterolemia, edema, and hypertension. In general, all
patients with hypercholesterolemia secondary to nephrotic syndrome should be treated with lipid-
lowering agents because they are at increased risk for cardiovascular disease. Nephrotic
syndrome has serious complications and must be part of the differential diagnosis for any patient
presenting with new onset edema. All patients should be referred to a nephrologist for further
investigation, which often include a renal biopsy.
Remember Classic tetrad of Nephrotic syndrome is proteinuria
(>3g/24hr), hypoalbuminemia, hypercholesterolemia an oedema.
Glomerulonephritis is inflammation of the glomerulus. There are two broad types Nephritic
glomerulonephritis and nephrotic glormerulonephritis
Remember Glomerulonephritis is divided into two broad groups:
Nephritic glomerulonephritis (Basement membrane/endothelium
injury causing Haematuria)
Nephrotic glomerulonephritis (Podocyte injury causing proteinuria)
Nephrotic Syndrome
Nephrotic Glomerulonephritis is a renal condition affecting the podocytes. This causes a leaky glomeruli
leading to proteinuria and subsequent hyperlipidemia, hypoalbuminemia and oedema
Classification
The page will focus on four common nephrotic syndromes towards the end:
The kidney is surrounding by an outer membrane called the renal capsule. The outer layer of the
kidney is the renal cortex where most of the nephrons are situated. The inner layer is the renal
medulla which contain renal pyramids. The renal pyramids drain urine into the minor – major
calyces before draining to the renal pelvis then the ureter.
The Nephrons are the functional units of the kidney. There are millions. The nephron is made up
of:
Bowman’s capsule – afferent arteriole enters the bowmans capsule and forms t and efferent exits
Proximal convoluted tubule
Loop of Henle (descending and ascending)
Distal convoluted tubule
Collecting ducts
The glomerulus is a network of blood vessels that are located within the bowman’s capsule. To
understand the cause, pathophyiology and effect of glomerulonephritis it is important to learn the
layers of the glomerulus + bowman’s capsule
Examination
Oedema
Periorbital oedema
Peripheral edema
Ascites
Hypoalbuminemia
Tiredness
Leuconychia
Dyslipidemia
Eruptive xanthomata
Xanthelasmata
Frothy Urine
Differential Diagnosis
This section will mainly focus on the Primary Aetiology + Diabetic Nephropathy
Investigations
General
Urine Dipstick
Mid-Stream Urine – microscopy
Full blood count
EUC
LFT
Plasma Ca2+
CRP
Glucose
Serum Immunoglobulins
Urine Immunoglobulins
Autoimmune screen (ANA, dsDNA, C3, C4)
Hepatititis B & C
HIV
Pathophysiology
Nephrotic Glomerulonephritis is a renal condition affecting the podocytes. This causes a leaky glomeruli
leading to proteinuria and subsequent hyperlipidemia, hypoalbuminemia and oedema
Pathophysiology – General
Minimal Change Disease
Overview Minimal change disease (MCD), sometimes known as nil lesion, causes 70–90% of
nephrotic syndrome in childhood but only 10–15% of nephrotic syndrome in adults. Minimal
change disease usually presents as a primary renal disease but can be associated with several
other conditions, including Hodgkin’s disease, allergies, or use of nonsteroidal anti-inflammatory
agents.
Clinical Presentation
Children
Anorexia
GI disturbance
Infections
Irritability
Oedema (peritorbital, genital)
Ascites
Oliguria
Minimal Change Disease The most common presentation of MCNS is
oedema. MCNS is characterised by oedema, selective proteinuria,
hypoalbuminaeia, hypercholesterolaemia, and a normal glomerular
filtration rate. Renal Biopsy: The only abnormality is diffuse effacement
and fusion of epithelial cell fot processes.
Renal Biopsy
Light Microscopy: nil
Immunoflourescence: occasional IgG in the mesangium
Electron microscopy: consistently demonstrates an effacement of 177 the foot process supporting the
epithelial podocytes with weakening of slit-pore membranes.
Management
Prednisone – First line
Cyclophosphamide – If steroid toxicity and relapsing nephrotic syndrome
Side note After initial treatment, proteinuria <0.3 g/day is defined as
complete remission; a reduction of baseline proteinuria by >50% with a
final value <3 g/day is defined as partial remission.
Management
ACE inhibitors
Angiotensin II receptor blockers
Membranous Glomerulonephritis
Membranous glomerulonephritis (MGN), or membranous nephropathy, accounts for
approximately 30% of cases of nephrotic syndrome in adults, with a peak incidence between the
ages of 30 and 50 years and a male to female ratio of 2:1.
Diabetic Nephropathy
Overview Type II Diabetes Mellitus is the leading cause of Chronic Kidney Disease. It is
classified as a secondary nephrotic syndrome. ~10% have nephropathy at diagnosis and up to
half will go on to develop it over the next 20yrs. 20% of people with Type II diabetes will develop
end stage kidney disease. Everyone with Diabetes should be screened yearly for
microalbuminuria.
Clinical features – Nephrotic Syndrome with signs and symptoms of diabetes (hyperglycemia)
Pathological features Diabetic nephropathy is defined by characteristic structural and functional
changes. The predominant structural changes include
Mesangial expansion
Glomerular basement membrane thickening
Glomerular sclerosis
Podocytopathy
Staging
Pathogenesis
Management and Prognosis Microalbuminuria is reversible if caught early and managed
vigorously.
Tight glycemic control
Right BP control with ACE/ARBs
Manage CV risk factors
More info on Chronic Kidney Disease
Complications Prognosis
Complications
Thromboembolism
Infection – due to loss of immunoglobulins and complement
Hyperlipidemia
Loss of vitamin D leading to bone disease
Acute renal failure
Chronic renal failure