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Prepared by:-
Mohammad Ali Al-shehri
..
Supervised by :
Dr.
Nephrotic Syndrome..(NS)
ephrotic Syndrome..(NS)
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Introduction
Definition of NS
Etiology of NS
Pathology of NS
Pathophysiology of NS
Clinical Manifestation of NS
Complication NS
Laboratory Data
Diagnosis
Treatment
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Nephrotic syndrome
ephrotic syndrome
Nephrotic syndrome (NS) results from
increased permeability of Glomeulrar
basement membrane ( GBM ) to plasma
protein.
It is clinical and laboratory syndrome
characterized by massive proteinuria, which
lead to hypoproteinemia ( hypo-
albuminemia), hyperlipidemia and pitting
edema.
(4-increase, 1-decrease).
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Nephrotic Criteria:-
*Massive proteinuria:
qualitative proteinuria: 3+ or 4+,
quantitative proteinuria : more than 40
mg/m2/hr in children selective).
*Hypo-proteinemia :
total plasma proteins < 5.5g/dl and serum
albumin : < 2.5g/dl.
*Hyperlipidemia :
serum cholesterol : > 5.7mmol/L
*Edema : pitting edema in different degree
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Nephr i tic Criteria
-Hematuria: RBC in urine (gross hematuria)
-Hypertension:
130/90 mmHg in school-age children
120/80 mmHg in preschool-age children
110/70 mmHg in infant and toddlers children
-Azotemia
renal insufficiency
:
Increased level of serum BUN Cr
-Hypo-complementemia:
Decreased level of serum c3
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Classification:
A-Primary Idiopathic NS (INS): majority
The cause is still unclear up to now. Recent 10 years
,increasing evidence has suggested that INS may
result from a primary disorder of T cell function.
Accounting for 90 of NS in child. mainly discussed .
B-Secondary NS:
NS resulted from systemic diseases, such as
anaphylactoid purpura , systemic lupus
erythematosus, HBV infection.
C-Congenital NS: rare
*1st 3monthe of life ,only treatment renal
transplantation
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Secondary NS
Drug,Toxic,Allegy : mercury, snake venom, vaccine,
pellicillamine, Heroin, gold, NSAID, captopril, probenecid,
volatile hydrocarbons
Infection : APSGN, HBV, HIV, shunt nephropathy, reflux
nephropathy, leprosy, syphilis, Schistosomiasis, hydatid
disease
Autoimmune or collagen-vascular diseases : SLE,
Hashimotos thyroiditis,, HSP, Vasculitis Metabolic disease : Diabetes mellitus
Neoplasma : Hodgkins disease, carcinoma ( renal cell, lung,
neuroblastoma, breast, and etc)
Genetic Disease
: Alport syn, Sickle cell disease,Am loidosis Con enital ne hro ath
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Idiopathic NS (INS):Pathology:
athology:
Minimal Change Nephropathy (MCN):
40mg/m2/hr for children
c- volume :oliguria (during stage of edema formation)
d-Microscopically:-
microscopic hematuria 20 , large number of hyaline
cast
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Investigations:-
2-Blood:
A-serum protein: decrease >5.5gm/dL , Albumin levels
are low (
2.5gm/dL).
B-Serum cholesterol and triglycerides:
Cholesterol
5.7mmol/L (220mg/dl).
C-- ESR100mm/hr during activity phase
.
3.Serum complemen : Vary with clinical type.
4.Renal function
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Kidney Biopsy:-
Considered in:
1-Secondary N.S
2-Frequent relapsing N.S
3-Steroid resistant N.S
4- Hematuria
5-Hypertension
6- Low GFR
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Differential Diagnosis of NS:
D.D of generalized edema:-
1-Protein losing enteropathy
2-Hepatic Failure.
3-HF
4-Protein energy malnutrition
5-Acute and chronic GN
6-urticaria? Angio edema
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Complications of NS:-
1-Infections :Infections is a major complication in children
with NS. It frequently trigger relapses.
Nephrotic pt are liable to infection because :
A-loss of immunoglobins in urine.
B-the edema fluid act as a culture medium.
C-use
immunosuppressive agents.D-malnutrition
The common infection : URI, peritonitis, cellulitis and
UTI may be seen.
Organisms: encapsulated (Pneumococci, H.
influenzae), Gram negative (e.g E.coli
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Complication
Vaccines in NS;-
polyvalent pneumococcal vaccine (if not previously
immunized) when the child is in remission and off daily
prednisone therapy.
Children with a negative varicella titer should be given
varicella vaccine.
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Complication..
2-Hypercoagulability (Thrombosis).
Hypercoagulability of the blood leading to venous or arterial
thrombosis:
Hypercoagulability in Nephrotic syndrome caused by:
1-Higher concentration of I,II, V,VII,VIII,X and fibrinogen
2- Lower level of anticoagulant substance: antithrombin
III
3-decrease fibrinolysis.
4-Higher blood viscosity
5- Increased platelet aggregation
6-
Overaggressive diuresis
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3-ARF :pre-renal and renal
4- cardiovascular disease :-Hyperlipidemia, may be
a risk factor for cardiovascular disease.
5-Hypovolemic shock
6-Others: growth retardation, malnutrition,
adrenal cortical insufficiency
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Management of NS:
General (non-specific )
*Corticosteroid therapy
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General therapy:-
Hospitalization:- for initial work-up and evaluation of
treatment.
Activity: usually no restriction , except
massive edema,heavy hypertension and
infection.
Diet
Hypertension and edema: Low salt diet (
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Induction use of albumin:-
Albumin + Lasix (20 salt poor)
1-Severe edema
2-Ascites
3-Pleural effusion
4-Genital edema
5-Low serum albumin
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Corticosteroidprednisone therapy:-
Prednisone tablets at a dose of 60 mg/m 2 /day
(maximum daily dose, 80 mg divided into 2-3
doses) for at least 4 consecutive weeks.
After complete absence of proteinuria, prednisone
dose should be tapered to 40 mg/m
2
/day given
every other day as a single morning dose.
The alternate-day dose is then slowly tapered and
discontinued over the next 2-3 mo.
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Treatment of relapse in NS:
Many children with nephrotic syndrome will
experience at least 1 relapse (3-4+proteinuria
plus edema ).
daily divided-dose prednisone at the doses noted
earlier ( where he has the relapse ) until the child
enters remission (urine trace or negative for
protein for 3 consecutive days).
The pred-nisone dose is then changed to alternate-
day dosing and tapered over 1-2 mo.
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According to response to prednisone
therapy:
*Remission: no edema, urine is protein free for 5
consecutive days.
* Relapse: edema, or first morning urine sample
contains > 2 + protein for 7 consecutive days.
*Frequent relapsing: > 2 relapses within 6 months >
4/year).
*Steroid resistant: failure to achieve remission
with prednisolone given daily for 28 days.
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Side Effects With Long Term Use of
Steroids Steroid toxicity
hyperglycemia
myopathy
peptic ulcer
poor healing of wound.
Hirsutism
Thromboembolism
-Stunted growth
Cataracts
- Pseudotumor cerebri
-Psycosis
-Osteoporosis
- Cushingoid features
-Adrenal gland suppression
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Alternative agent:-
When can be used:
Steroid-dependent patients, frequent relapsers, and steroid-
resistant patients.
Cyclophosphamide Pulse steroids
Cyclosporin A
Tacrolimus
Microphenolate
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THE END.
THANK YOU.HANK YOU.