Transcript Nephrotic syndsome

Nephrotic syndrome &
GN
Ebadur Rahman
FRCP (Edin),FASN, Specialty Certificate in Nephrology(UK)
MRCP(UK),DIM(UK),DNeph(UK),MmedSciNephrology(UK).
Consultant & clinical tutor
Department of Nephrology
Riyadh Armed Forces Hospital
Nephrotic syndrome
This is characterized by proteinuria (Typically >
3.5g/24h),
hypoalbuminemia ( less than 30g/dL ) and edema.
Hyperlipidaemia.
“nephrotic range” –nephrotic proteinuria
Presentation
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New-onset oedema
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Initially periorbital or peripheral
Later genitals, ascites, anasarca
Frothy urine
Generalised symptoms – lethargy, fatigue, reduced
appetite
Figure 1.
Nephrotic edema.
Heavy proteinuria (albuminuria)
Further possible presentations...
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Oedema
BP
Leukonychia
Breathlessness:
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Pleural effusion, fluid overload, AKI,PE,MI.
DVT
Eruptive xanthomata/ xanthalosmata
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Hemoconcentration
Immobility, especially in patients with anasarca
thrombocytosis, increased platelet activation,
decreased levels of
antithrombin,
 free protein S,
 and plasminogen (due to urinary losses),
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NEPHROTIC SYNDROME
Secondary
- Primary glomerular disease
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Primary glomerular disease
PRIMARY NEPHROTIC SYNDROME
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Minimal Change Disease- commonest in children
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Focal Segmental Glomerulosclerosis
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Membranous Nephropathy- commonest in adult
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Membranoproliferative Glomerulonephritis(MPGN)
Iga nephropathy RARE to cause nephrotic
syndrome but commonest GN
For exam
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Minimal change gn
Membranous gn
Amiloidosis
SLE
DM
Urine dipstick
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The urine sediment (or direct counting of RBC per
mL of uncentrifuged urine) is the gold standard for
the detection of microscopic hematuria.
Dipsticks for heme detect 1 to 2 RBCs per high
power field
false positive tests due to the
following:
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Semen is present in the urine after ejaculation and
may cause a positive heme reaction on the dipstick [
urine pH greater than 9 or contamination with
oxidizing agents used to clean the perineum
The presence of myoglobinuria
False negative tests
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have been reported in patients ingesting large
amounts of vitamin C
Isolated glomerular hematuria
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Renal biopsy is not usually performed
there is no specific therapy for these conditions
renal prognosis is excellent
management of these patients is not usually affected
by the biopsy
When renal biopsy is performed in such patients, the
most common findings-are a normal biopsy
IgA nephropathy,
 Thin basement membrane disease (benign familial
hematuria),
 mild nonspecific glomerular abnormalities,
 Alport syndrome
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All patients should have a urine
culture to exclude infection prior to
evaluation of hematuria.
Hyperlipidemia
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total cholesterol and low-density lipoprotein (LDL)
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Lipoprotein (a) [Lp(a)]
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high-density lipoprotein (HDL) cholesterol .
Renal biopsy
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Nephrotic syndrome
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Nephritic syndrome
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Unexplained renal impairement
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Vasculitis where kidneys are commonly involved
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Renal allograft dysfunction
Percutaneous renal biopsy is generally contraindicated
in the following settings:
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Uncorrectable bleeding diathesis
Small kidneys which are generally indicative of
chronic irreversible disease
Severe hypertension, which cannot be controlled
with antihypertensive medications
Multiple, bilateral cysts or a renal tumor
Hydronephrosis
Active renal or perirenal infection
An uncooperative patient
Table 4
In children- diagnosis of MCD is usually made based upon the clinical.
Almost all children with MCD respond to a short course of glucocorticoids.
it is considered steroid-resistant (SR-NS) when no remission is achieved after about two
months of full-dose steroid therapy.
Pediatricians usually restrict renal biopsy to individuals with SR-NS
The clinical findings at presentation in adults
Proteinuria – nephrotic range
Hematuria – 29 percent
Hypertension – 43 percent
Acute renal failure – 18 percent
PRIMARY NEPHROTIC SYNDROME
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Minimal Change Disease
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Focal Segmental Glomerulosclerosis
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Membranous Nephropathy
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Membranoproliferative
Glomerulonephritis(MPGN)
Figure 6. Light microscopic appearances in focal segmental glomerulosclerosis.
Segmental scars with capsular adhesions in otherwise normal glomeruli.
Table 5
PRIMARY NEPHROTIC SYNDROME
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Minimal Change Disease
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Focal Segmental Glomerulosclerosis
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Membranous Nephropathy
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Iga nephropathy
Membranoproliferative
Glomerulonephritis(MPGN)
Membranous nephropathy (MN) - one-third of biopsy diagnoses in adult nondiabetics
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diffuse thickening of the glomerular basement
membrane (GBM) on light microscopy, "spikes" on silver
stain, diffuse granular IgG and complement deposition
on immunofluorescence, and subepithelial dense deposits
on electron microscopy
Antibodies to the M-type phospholipase A2 receptor
(PLA2R ) are found in a high proportion of patients with
primary (idiopathic) MN.
Most patients with MN present with the nephrotic
syndrome with normal or near normal serum creatinine.
10% associated with malignancy
Table 6
Figure 7a. Early MN: a glomerulus from a patient with severe nephrotic syndrome and
early MN, exhibiting normal architecture and peripheral capillary basement membranes
of normal thickness (Silver–methenamine ×400).
Figure 7b
morphologically advanced MN
Figure 7c. Morphologically more advanced MN (same patient as in (b))
IgA nephropathy
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most common cause of primary glomerulonephritis. in
Asians and Caucasians.
There is a 2:1 male to female predominance
IgA nephropathy is characterized by prominent, globular
deposits of IgA in the mesangium on immunofluorescence
microscopy.
Electron microscopy shows dense deposits primarily in the
mesangium
Most patients with IgA nephropathy present with either
visible hematuria (single or recurrent), usually following an
upper respiratory infection, or invisible hematuria with or
without mild proteinuria incidentally detected on a routine
examination
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40 to 50 percent present with one or recurrent
episodes of visible hematuria, usually following an
upper respiratory infection
Another 30 to 40 percent have microscopic
hematuria and usually mild proteinuria, and are
incidentally detected on a routine examination
Less than 10 percent present with either nephrotic
syndrome or rapidly progressive glomerulonephritis
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A kidney biopsy only
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protein excretion above 0.5 to 1 g/day, elevated
serum creatinine concentration, or hypertension.
PRIMARY NEPHROTIC SYNDROME
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Membranoproliferative Glomerulonephritis(MPGN)
Figure 8.
Pathology of membranoproliferative glomerulonephritis type I.
(a) Light microscopy shows a hypercellular glomerulus with accentuated lobular
architecture and a small cellular crescent (methenamine silver).
Table 7
Diagnosis
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fasting blood sugar and glycosylated hemoglobin
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antinuclear antibody test
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serum complement,
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In selected patients, cryoglobulins, hepatitis B and C serology,
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anti-neutrophil cytoplasmic antibodies (ANCAS),
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anti GBM antibodies,
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renal biopsy to define the pattern of glomerular involvement.
Complications
 Infection
 Coagulation
disorders
 Protein malnutrition and dyslipidemia
 Acute renal failure
Treatment
General treatment
1.
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Relief edema
2.
Rx Htn
2. Symptomatic treatment
Treating dyslipidemias,
anticoagulate treatment,
3. Immunosupressive treatment