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Kidney Syndromes

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Prepared by:-
Mohammad Ali Al-shehri
Ὴ..
Supervised by :
Dr.
Nephrotic Syndrome..Ὴ(NS) Nephrotic Syndrome..Ὴ(NS)
Introduction
 Definition of NS
 Etiology of NS
 Pathology of NS
 Pathophysiology of NS
 Clinical Manifestation of NS
 Complication NS
 Laboratory Data
 Diagnosis
 Treatment
Nephrotic syndrome Nephrotic 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).
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
Nephr i tic Criteria
 -Hematuria: RBC in urine (gross hematuria)
 -Hypertension:
 ᶟ130/90 mmHg in school-age children
 ᶟ120/80 mmHg in preschool-age children
 ᶟ110/70 mmHg in infant and toddlerᾼs children
 -Azotemia (renal insufficiency ):
Increased level of serum BUN 、Cr
 -Hypo-complementemia:
Decreased level of serum c3
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 Tᾶ 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
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,
Hashimotoᾼs thyroiditis,, HSP, Vasculitis
 Metabolic disease : Diabetes mellitus
 Neoplasma : Hodgkinᾼs disease, carcinoma ( renal cell, lung,
neuroblastoma, breast, and etc)
 Genetic Disease : Alport syn, Sickle cell disease,
Amyloidosis, Congenital nephropathy
 Others : Chronic transplant rejection, congenital
nephrosclerosis
Idiopathic NS (INS): Pathology:- Pathology:-
 Minimal Change Nephropathy (MCN): < 80%
The glomeruli appear normal basically Under
Light microscopy, and Under
Immunofluorescence
*under Electron microscopy ᾶ fusion of the foot
processes of the podocytes
 (2) NonᾷMCN : <20%
*Mesangial proliferative glomerulonephritis
(MsPGN): about 10%
*Focal segmental glomerulosclerosis (FSGS): 5%
*Membranous Nephropathy (MN) : 2%
*Membrane proliferative glomerulonephritis
 (MPGN) : 1%
 *Others : rare,Cresent glomerulonephritis
 NB:-
  *Nephrotic syndrome is 15 times more common *Nephrotic syndrome is 15 times more common
in children than in adults. in children than in adults.
  *Most cases of primary nephrotic syndrome are *Most cases of primary nephrotic syndrome are
in children and are due to minimal-change in children and are due to minimal-change
disease. The age at onset varies with the type disease. The age at onset varies with the type
of nephrotic syndrome. of nephrotic syndrome.
Pathophysiology:
The Main Trigger Of primary Nephrotic
Syndrome and Fundamental and highly
important change of pathophysiology :-
Proteinuria
Pathogenesis of Proteinuria:-
 Increase glomerular permeability for proteins due to
loss of negative charged glycoprotein
 Degree of protineuria:-
 Mild less than 0.5g/m2/day
 Moderate 0.5 ᾶ 2g/m2/day
 Sever more than 2g/m2/day
 Type of proteinuria:-
 A-Selective proteinuria: where proteins of low
molecular weight .such as albumin, are excreted more
readily than protein of HMW
 B-Non selective :
 LMW+HMW are lost in urine
pathogenesis of hypoalbuminemia
*Due to hyperproteinuria----- Loss of
plasma protein in urine mainly the
albumin.

*Increased catabolism of protein during
acute phase.
pathogenesis of hyperlipidemia:-
* Response to Hypoalbuminemia   reflex to liver
--  synthesis of generalize protein (
including lipoprotein ) and lipid in the liver ,
the lipoprotein high molecular weight no loss
in urine   hyperlipidemia
*Diminished catabolism of lipoprotein
pathogenesis of edema:-
 *Reduction plasma colloid osmotic pressure  
secondary to hypoalbuminemia  Edema and
hypovolemia
 *Intravascular volume   antidiuretic hormone
(ADH ) and aldosterone(ALD)  water and
sodium retention  Edema
 *Intravascular volume   glomerular filtration
rate
 (GFR)   water and sodium retention 
Edema
How many pathological types
causes nephrotic syndrome?
Clinical Manifestation:-
IN MCNS , The male preponderance of 2:1
: 1.Main manifestations:
Edema (varying degrees) is the common symptom
Local edema : edema in face , around eyes( Periorbital swelling)
, in lower extremities.
Generalized edema (anasarca), edema in penis and scrotum.
2-Non-specific symptoms:
Fatigue and lethargy
loss of appetite, nausea and vomiting ,abdominal pain ,
diarrhea
body weight increase, urine output decrease
pleural effusion (respiratory distress)
Investigations:-
 1-Urine analysis:-
A-Proteinuria : 3-4 + SELECTIVE.
b-24 urine collection for protein
>40mg/m2/hr for children
c- volume : oliguria (during stage of edema formation)
d-Microscopically:-
microscopic hematuria 20%, large number of hyaline
cast
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-- ESR >100mm/hr during activity phase
 .
 3.Serum complemen : Vary with clinical type.
Ä
 4.Renal function
 .

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
Differential Diagnosis of NS:
 D.D of generalized edema:-
 1-Protein ᾶlosing enteropathy
 2-Hepatic Failure.
 3-HF
 4-Protein energy malnutrition
 5-Acute and chronic GN
 6-urticaria? Angio edema
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
Complication Ὴ
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.
ComplicationῊ..
 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
 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
Management of NS:
 General (non-specific )
 *Corticosteroid therapy
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 (<2gNa/ day)
only during period of edema or salt-free diet.
Severe edema: Restricting fluid intake
 Avoiding infection: very important.
 Diuresis : Hydrochlorothiazide (HCT) :2mg/kg.d
 Antisterone : 2 ~4mg/kg.d
 Dextran : 10 ~15ml/kg , after
30 ~60m,
 followed by Furosemide (Lasix) at
2mg/kg .
Induction use of albumin:-
 Albumin + Lasix (20 % salt poor)
 1-Severe edema
 2-Ascites
 3-Pleural effusion
 4-Genital edema
 5-Low serum albumin
Corticosteroidᾷprednisone 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.

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.
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.
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
Alternative agent:-
 When can be used:
 Steroid-dependent patients, frequent relapsers, and steroid-
resistant patients.
 Cyclophosphamide Pulse steroids
 Cyclosporin A
 Tacrolimus
 Microphenolate
 THE ENDῊ.
THANK YOUẐ.
THANK YOUẐ.

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