NEPHROTIC SYNDROME

The nephrotic syndrome is clinical complex characterized by

number of renal and extra renal feature .the most prominent of
which are proteinuria of >3.5g per 1.7m per 24 hour;
hypoalbuminemia; edema, hyperlipidemia, lipiduria, and
hypercoagulability.
Proteinuria results from altered permeability of the glomerular
filtration barrier for protein.
PATHOPHYSIOLOGY
-hypoalbuminemia result from proteinuria and increased
renal catabolism and inadequate hepatic synthesis of
albumin.
-oedema formation in nephrotic syndrome may be duo to
underfilling hypothesis postulate that hypoalbuminemia
result in decreased intravascular oncotic pressure leading to
leakage of extra cellular fluid from blood to the interstial,
intravascular volume falls, there by stimulating activation of
the renin-angiotesin-aldesteron axis and the sympathetic
nervous system and release of vasopressin(antiduretic
hormone)and suppressing atrial natriuretic peptide release.
These neural and hormonal responses promote renal salt and
water retention.
-hyperlipidemia is believed to consequence of increased
hepatic lipoprotein synthesis that is triggered by reduce
oncotic pressure and may be compounded by increase
urinary loss of protein that regulate lipid homeostasis.
Low-density lipoprotein and cholesterol are increased in
the majority of patient. Where as very low-density
lipoprotein and triglycerides tend to raise in-patient with
sever disease, hyperlipidemia may accelerate
atherosclerosis and progression of renal disease.
Hypercoagulablity is probably multifactor in origin and is
caused by increased urinary loss of anti thrombin Ш, altered
level and or activity of protein C and S, hyperfibrogeneima
duo to increase hepatic synthesis, impaired fibrinolysis and
increase platelate aggregability. As consequence of these
effects, patient can develop spontaneous peripheral arterial
or venous thrombosis, renal vein thrombosis and pulmonary
embolism. Clinical features that suggest acute renal vein
thrombosis include sudden onset of flank or abdominal pain,
gross hematuria , Lt .side varcocele , increase proteinuria
and acute decline GFR.
other complication;
Protein malnutrition
Iron resistance microcytic anemia duo to transferrin loss
hypocalcaemia and hyperparathyroidism can occur as
consequence of vit.D deficiency duo to enhance urinary
excretion of cholecalciferol binding.
-depressed thyroxin level duo to loss of thyroxin binding
globulin.
-an increase susceptibility to infection that result from
urinary loss of IgG.
secondary nephrotic synderome.
-dibetes mellitus.
-systemic lupus erythromatosis and other collagen
disease
-amyloidosis.
-vasculitic-imunological disease(mixed cryglobulinemia,
wegeners granulomatosis, rapidly progressive
glomerulnephritis, polyarteits, henoch-schonlen purpra,
sarcoidosis.
-infectious;
A-baterial(post-streptococcal,congenital and secondary
syphilis, subacute bacterial endocarditis,shunt nephritis).
B-viral(hepatitsB and hepatits C ), HIV infection ,infectious
mononucleosis, cytomegalovirus infection)
C-parasitic(malaria,toxoplasmosis, schistmiasis,filariasis)
medication related;
Gold, mercury, and heavy metal, nonsteroid anti-inflammetry
drugs ,pencillamine, lithium, paramethadione, trimethadione
captopril, street heroin, probencid, rifampin and tolbutamide.

-allergens,venom and immunization

-associated with neoplasma.
Hodgkin's lymphoma and leukemia (with minimal change
lesion)
Solid tumor (with memberonus nephropathy)
-hereditary and metabolic disease.
Alports syndrome, fabrys disease, sickle cell disease,
congenital nephrotic syndrome, nail-patella syndrome, partial
lipodystrophy
-other
Pregnancy, transplant rejection, serum sickness, unilateral
renal artery stenosis, massive obesity, refluxes nephropathy.
In occasional patient with evidence of hypovolemia,
intravenous salt poor albumin infusion may help to establish
adiuresis. Over diuresis risk for secondary impairment of
renal function through hypovolaemia.
- Venous thromboembolism is guarded against by
anticoagulation and there is case for routine anticoagulation
in all patient with chronic or sever nephrotic syndrome.
-hypercholesterolemia is common and treated with lipid-
lowering drug e.g. HMG- COA reductase inhibiter(statin)
- the risk of infection with pneumococci is especially high in
children who should be offered immunization.
Acute nephritic syndrome
classically present with
1-hypertension
2-hematuria
3-red blood cast
4-pyuria
5-mild to moderate proteinuria
6-renal impairment produce uremic symptoms with salt and
water retention
Poststreptococcal glomerulonephritis
the incidence of this decreased in developed countries
location is typically sporadic, epidemic less common
usually affects children between the age 2---14 year
p. glomerulonephritis duo to impetigo develops 2—6 weeks
after skin infection and 1—3 weeks after streptococcal
pharyngitis
The classic presentation is an acute nephritic picture with
hematuria, pyuria red blood cell cast, edema, hypertension
and oliguric renal failure
systemic symptom of headache , malaise, anorexia and
flank pain duo to swelling of renal capsule
Diagnosis
1-general urine examination reveal proteinuria less than 3g
per 24 hour, hematuria, granular cast
2-elveated blood urea and creatinine
3-decrease serum albumin
4-electrolyte disturbance
Treatment
supportive with control blood pressure, edema, antibiotic for
sreptococcal infection, may be need dialysis
there is no role for immunosuppresive therapy
recurrent is rare in poststreptococcal glomerulonephritis
prognosis is good in children with permanent renal failure less
than 1%
while in elderly is worse with high incidence of azotemia up to
60%