Presentation of glomerular
diseases
1.Nephrotic syndrome
2. Nephritic syndrome.
3. Rapidly progressive glomerulonephritis. ( RPGN).
4. Asymptomatic proteinuria.
5. asymptomatic hematuria.
6.Macroscopic hematuria.
7.Chronic glomerulonephritis.
Nephrotic syndrome
pathognomonic of glomerular disease
Definition five criteria
1. ( proteinuria) > 3.5 g protein in 24 h urine = frothy urine.
Normal protein in urine 150 mg/24 hour.
2. Edema = puffy eyes in the morning decreasing gradually
during the day, leg swelling, increasing body weight. Is due
to accumulation of fluid in the interstitial space . The most
acceptable mechanism is the
under fill theory . (The protein loss in urine lead to decrease
albumin in plasma and decreased effective vascular volume
with resultant activation of the aldosterone leading to
retention of fluid ).the other less acceptable is
the over fill theory which is that the primary glomerular
pathology leads to retention due to inflammation and
activation of the RAAS .
3- hyperlipidemia increase serum lipid because the
liver compensate for proteinuria by protien synthesis
and one of these are lipoproteins.
Lipid abnormality ( increase LDL, IDL, VLDL ,(
increase hepatic synthesis), increase total cholesterol
( increase hepatic synthesis), decrease HDL levels (
increase hepatic synthesis and increase urinary
clearances), increase atherogenisity due to increase in
lipoprotein level and increased oxidized form of the
LDL levels.
4. hypoalbuminemia : serum albumin < 3.5 g/dl (
normal 3.5-6 ) = white nails
5- Lipiduria appearance of lipid substances in urine
during microscopic exam.
•Differential diagnosis of generalized
oedema
1.Heart failure ( increase jugular venous pressure + EF
<55%)
2.Hepatic cirrhosis ( stigmata of chronic liver disease and
usually started as ascites)
3.Malnutrition
4.Protein losing enteropathy
5.Iatrogenic
6.Cyclical edema
7.Static edema
Complications
I.
Hypercoagulability = abnormal spontaneous thrombosis of the
blood.
(increase platelets aggregation, immobility, hypovolemia, increase
fibrinogen, factors V, VII, von willibrands and ά 1 macroglobulin,
increase urinary clearances of anti thrombin III) this leads to venous
thrombosis and with accelerated atherosclerosis to arterial thrombosis
the later is more in children with minimal change disease. These usually
happened when albumin level decrease to less than 2 g/dl.
Dysfibrinogenemia leads to increase ESR even more than 100. rena;l
vein thrombosis can ocuur ( clinically in 8% ) but when looking for it
present in 50 % and if bilateral can leads to AKI ( rare) and when present
it present as flank pain and hematuria. Pulmonary embolism
II.
malnutrition
III. Hyperlipidemia and lipidurea
IV. decreased Vit D2 level with normal free Vit D level ( due to loss of
binding protien ) so frank osteomalacia is rare in nephrotics.
V. Iron, zink, copper deficiency ( binding protiens lost in urine)
VI. Decrease total T-4 but free T-4 & TSH are normal and
no clinical consequences.
VII.. Infection : due to oedema fluid which is a good
culture media, loss of complement and IgG in urine,
fragile skin in nephrotics, dilution of local humoral
immunity factors by fluids , impaired neutrophil
phagocytosis, loss of transferrin and zink in urine
which are required for lymphocytes function
VIII. Acute kidney injury : due to volume depletion,
sepsis , transformation of the disease, bilateral renal vein
thrombosis, drugs susceptibility ( NSAIDs, ACEIs, ARBs),
increased risk of interstitial nephritis due to the drugs
including diuretics, nephrosarca ( intra renal oedema with
compression of the tubules)
IX. Chronic kidney disease : especially if protienuria > 5
gs /24 h but not usually in minimal change disease.
•Management of nephrotic syndrome
1.Non specific management:
❖Diuretics for edema (loop diuretic +/- thiazides ).
❖Lipid lowering for hyperlipidemia ( statin)
❖ACEIs + ARBs with CCBs and spironolacton if
needed for proteinuria
• 2-Specific treatment according to the cause (
coming lectures)
3-Treatment of complications as antibiotics for
infections and anticoagulants for thrombotic events.
causes of nephrotic
1. minimal change disease usually in children ( no changes in
LM,
2. membranous GN( thikining and spikes in GBM with IgG
deposition in the glomeruli only,
3. focal segmental glomeruloseclerosis( fibrosis and
adhesion or synechiae of the bowman capsule and now
regarded the most common cause of nephrotic syndrone
in adults)
4. diabetes mellitus ( diabetic nephropathy, nodular
seclerosis )
5. amyloidosis ( deposition of abnormal protien)
6. Membranoproliferative GN ( type I.II, cryoglobulinemic)
nephritic syndrome
Definition:
1. hypertension ( normal blood pressure in nephrotic),
2. less oedema than nephrotic,
3. less protienuria, i.e non nephrotic range that is less than 3.5 g / 24
hour
4. very rapid onset ( usually insidious in nephrotics except in minimal),
5. Hematuria with RBCs cast ( for academic purposes we will regard as
not present in nephrotic)
RBC cast = proteinatious substances embedded in it RBCs and these
RBCs are usually destructed RBCs ( dysmorphic RBCs) coming up from
the inflamed glomerulus . (hematuria + proteinuria ) is called active
urine sediment ( absent in nephrotic in which urine sediment is called
bland),
1. Oliguria ( may happened in nephrotics with severe oliguria )
2. normal to slightly reduced serum albumin ( severe in nephrotic),
3. elevated jugular venous pressure ( normal or low in nephrotic)
Causes of nephritic syndrome.
1. Post streptococcal GN ( typically 2 weeks after pharyngitis,
3 weeks after impetigo .), elevated ASOT , tea color urine ,
decrease urine output = oliguria= < 300ml/24 h or anuria =
<100 ml /day , hypertension )
2. IgAN typically few days… 3-5 days after URTI or
Gastroenteritis
3. Systemic lupus ( SLE) = (lupus nephritis). SLE is a
connective tissue disease of unknown etiology (4 of 11
diagnostic criteria) with positive antinuclear Ab, Anti
double strand DNA Ab, decreasesd C3 & C4 complement
level
4. After endocarditis, abcesses. In both blood culture is
helpful in diagnosis.
5. Vasculitis ( small vessel vasculitis ( wegners
granulomatosis = granulomatous polyangitis) , microscopic
polyangitis , churg – strauss syndrome.
Rapidly progressive glomerulonephritis
Days to weeks renal function will deteriorate with features of nephritic
But Usually blood pressure is normal, may have vasculitic features as
petichial rash in skin,
Causes
1. Goodpasture syndrome ( renal failure + pulmonary hemorrhage ..
Hemoptysis positive anti glomerular basement membrane Abs.
2. Vasculitis;
wegner ( upper.. sinusitis & lower RT…. lung nodules, hemoptysis,
lung cavitation with renal failure and positive C- ANCA)
microscopic polyangitis.. Multisystem involvement with positive P
ANCA
pauci immue crescentic GN .. Renal involvement only with positive P
ANCA
3. PSCGN
4. IgAN/ Henoch Schonlein purpura
5. endocarditis
Asymptomatic Protienuria (150 mg- 3000mg / 24 hours)
Asymptomatic Hematuria > 2 RBCS/ HPF or > 10 milion / liter
and are usually dysmorphic RBCs
Macroscopic hematuria
Brown red painless no clots coincide with infection
And between attacks there is microscopic hematuria +/-
protienuria
Chronic glomerulonephritis
Presents as Renal insuffiecncy ( increase blood urea and
serum creatinine) , hypertension, protienuria often > 3
grams, shrunken smooth kidney by U/S < 9 cm length and
renal biopsy is contraindicated.
•oliguria < 500ml in 24 hour
•Anuria < 100 ml/ 24 hour