2. Nephrotic syndrome
Nephrotic syndrome is a clinical complex
charecterized by a number of renal and extrarenal
features :
1- protien urea of > 3.5 gram /24 hr.
2- hypoalbominaemia (usually <30 gm/L ).
3- oedema .
4- hyperlipidemia , hyperlipiduria .
5- hypercoagulability .
3. The key point for nephrotic syndrom is proteinuria
(>3.5 gm/24 hr ) .
The proteinuria result from altered permiability of
the glumerular filtration barrier for protein (namely
the GBM & podocytes )
The other components & metabolic complications of
nephrotic syndrome are all secondary to protein loss
in urine .
4. Consequences & Complications of NS :
1- edema
NS. Cause Hypoalbominaemia , & this results in
decrease intravascular oncotic pressure, leading
to leakage of extracellular fluid from the blood to
the interstitium .
The oedema accumulate predominantely in the
lower limbs in adults. Extending to the genitalia
& lower abdomen as it becomes more sever .
In the morning the upper limbs & face may be
more affected .
In children : ascitis occurs early & the oedema is
often seen in the face .
5. 2-Hyperlipidemia
– It is due to the increased hepatic lipoprotein
synthesis that is triggered by reduced oncotic
pressure .
– LDL & cholestrol are increase in majority of
patiens .
– VLDL & triglyceride are increase in patients with
sever disease .
– Hyperlipidemia may accelerate atherosclerosis &
progression of renal disease .
6. 3-hypercoagulability
It is multifactorial in origin & caused by :
1-Increased urinary loss of antithrombin 3 .
2-Altered level &lor activity of protein C & S .
3-Hyperfibrinogenemia (due to increased hepatic
synthesis ) .
4-Impaired fibrinolysis .
5-Increased platelet aggregability .
The patient may develop peripheral arterial or
venous thrombosis , renal vein thrombosis or
pulmonary embolism .
Venous thrombosis is common & some time fatal.
7. 4-Increased susceptability to Infection
It is due to the low level of IgG ( due to the
urinary loss of IgG ) .
Usually susceptable to infection by Pneumococci .
8. 5-Other metabolic complications of
nephrotic syndrome
A- Protien malnutrition .
B- Iron resistance hypochromic microcytic anaemia
due to transferrin loss.
C-Hypocalcaemia & secondary hyperparathyroidism
as a consequence of Vitamine D deficiency due to
the increased urinary excreation of cholecalciferol
– binding protein .
D- Depressed thyroxine level .
due to the loss of thyroxine – binding globulin .
9. Causes of nephrotic syndrome(types)
A- Nephrotic range proteinuria(NS.) with
bland urine sidement (non-inflammatory)
(Pure nephrotic )
1- Primary glomerular disease :
- minimal change NS.
- membranous glomerulopathy.
- focal glomerulosclerosis .
2- Secondary glomerular disease :
- diabetic nephropathy .
- amyloidosis .
11. Management of nephrotic syndrome
Management of NS. Has 4 elements :
1- Establish the cause .
2- Treat the cause (if possible ) .
3- Measures to control proteinuria &
treatment of complications.
4- Prevent complications.
12. Renal biopsy
Renal biopsy in adults with NS. Is a valuable tool
for establishing definitive diagnosis , guiding
therapy & estimating prognosis .
Renal biopsy is not required in the majority of
children with NS. As most cases are due to
minimal change disease &respond to emperic
treatment with glucocorticoid .
In NS. Renal biopsy is indicated in :
1- adult patient .
2-children not respond to steroid .
13. Lines of management of NS
A- Treatment of proteinuria:
non specific measure that may reduce
proteinuria is Angiotensin-converting enzyme
inhibitors like lisinopril .
ACE-inhibitors reduce proteinuria & slow the rate
of progression of renal failure by lowering
intraglomerular pressure & preventing the
development of hemodynamically mediated focal
segmental glomerulosclerosis .
ACE-inhibitors are renoprotective in diabetic
nephropathy.
14. B-Treatment of complications of NS
1-Oedema
-Moderate salt restriction ( 1-2 g/day)
-Diuretics : usually we use loop diuretic (frusemide) . But
in sever NS & sever oedema combination of diuretics can
be used acting on different parts of the nephron
(frusemide , thiazide , amiloride )
We should not remove > 1 Kg. of the oedema per day ,
as more aggressive diuretics may precipitate
intravascular volume depletion & pre-renal azotemia .
15. If the patient have sever hypoalbuminaemia , the
oedema may be resistant to diuretics & may
need to give IV albumine by infusion.
Giving albumine in NS. Is some times harmful ,
because the more albumine you give the more
will be loss in the urine & this may cause more
glomerular damage .
17. 3- Thromboembolism
Anticoagulation is indicated for patients with
NS. developed DVT , arterial thrombosis or
pulmonary embolism.
Also anticoagulation indicated routinly in
all patients with chronic or sever NS.
There may be a resistance to heparin
because of antithrombine III deficiency .
19. 5- Vit. D deficiency
Vitamin D supplementation is advisable in
patients with clinical or biochemical
evidence of vit. D deficiency .
20. C-Steroid & other immunosupressant
drugs
In adults :
renal biopsy should be done before giving steroid &
other immunosupressant drugs ( as cyclophosphamide ).
giving steroid & other immunosupressant drugs depend
on the result of renal biopsy
In children :
we can start steroid without renal biops because most
cases are minimal change type which have a good
response to steroid .
renal biopsy is indicated if the child with NS. have no
response to steroid.
