kidney syndromes

8/11/2019 Kidney Syndromes

1/35

Prepared by:-

Mohammad Ali Al-shehri

..

Supervised by :

Dr.

Nephrotic Syndrome..(NS)

ephrotic Syndrome..(NS)


2/35

Introduction

Definition of NS

Etiology of NS

Pathology of NS

Pathophysiology of NS

Clinical Manifestation of NS

Complication NS

Laboratory Data

Diagnosis

Treatment


3/35

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).


4/35

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


5/35

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


6/35

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


7/35

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


8/35

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


20/35

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


21/35

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


22/35

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


23/35

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


24/35

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.


25/35

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


26/35

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


27/35

Management of NS:

General (non-specific )

*Corticosteroid therapy


28/35

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 (


29/35

Induction use of albumin:-

Albumin + Lasix (20 salt poor)

1-Severe edema

2-Ascites

3-Pleural effusion

4-Genital edema

5-Low serum albumin


30/35

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.


31/35

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.


32/35

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.


33/35

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


34/35

Alternative agent:-

When can be used:

Steroid-dependent patients, frequent relapsers, and steroid-

resistant patients.

Cyclophosphamide Pulse steroids

Cyclosporin A

Tacrolimus

Microphenolate


35/35

THE END.

THANK YOU.HANK YOU.

kidney syndromes

Documents