nephrotic syndrome ini punya orang maaf ya

7/25/2019 Nephrotic Syndrome ini punya orang maaf ya

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Prepared by:-

Mohammad Ali Al-shehri

..Supervised by :

Dr.

Nephrotic Syndrome..(NS)Nephrotic Syndrome..(NS)


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Nephrotic *riteria:-

*Massive proteinuria:

qualitative proteinuria: 3+ or 4+,

quantitative proteinuria : more than 40 mg/m2/hr in

children (selective)

*!"po#proteinemia :

total plasma proteins $ %%g/dl and serum al&umin : $

2%g/dl

*!"perlipidemia:

serum cholesterol : ' %mmol/

*dema: pitting edema in dierent degree


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Nephritic Criteria

-+ematuria:,"* in urine ('ross hematuria)

-+ypertension: /01/ mm+' in school-a'e children

2/03/ mm+' in preschool-a'e children /04/ mm+' in inant and toddler5s children

-A$otemia renal insuiciency :

#ncreased level o serum "6N *r-+ypo-complementemia:

Decreased level o serum c


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Secondary NS

Dru'%8o=ic%Alle'y: mercury, snake venom, vaccine, pellicillamine, Heroin,

gold, NSAID, captopril, probenecid, volatile hydrocarbons

#nection: AS!N, H"#, HI#, shunt nephropathy, reflu$ nephropathy, leprosy,syphilis, Schistosomiasis, hydatid disease

Autoimmune or colla'en-vascular diseases: S%&, Hashimoto's thyroiditis,,HS, #asculitis

Metabolic disease: Diabetes mellitus

Neoplasma: Hodgkin's disease, carcinoma ( renal cell, lung, neuroblastoma,breast, and etc)

!enetic Disease: Alport syn, Sickle cell disease, Amyloidosis, Congenitalnephropathy

>thers: Chronic transplant re*ection, congenital nephrosclerosis


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Idiopathic NS (INS):Pathology:-Pathology:-

Minimal *han'e Nephropathy (M*N):+3/

8he 'lomeruli appear normal basically 6nder ?i'htmicroscopy% and 6nder #mmunoluorescence


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N":-


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Pathophysiolo'y:

8he Main 8ri''er > primary Nephrotic Syndromeand Bundamental and hi'hly important chan'e o

pathophysiolo'y :-

Proteinuria


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Patho'enesis o Proteinuria:-

#ncrease 'lomerular permeability or proteins due to loss one'ative char'ed 'lycoprotein

De'ree o protineuria:-

Mildless than /.C'0m20day

Moderate/.C 9 2'0m20day

Severmore than 2'0m20day

8ype o proteinuria:-

A-Selective proteinuria:&here proteins o lo& molecular &ei'ht.such as albumin% are e=creted more readily than protein o +M

"-Non selective :

?ME+M are lost in urine


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pathogenesis of hypoalbuminemia

Due to hyperproteinuria----- %oss of plasmaprotein in urine mainly the albumin.

Increased catabolism of protein during acutephase.


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patho'enesis o edema:-


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Ho/ many pathological types causesnephrotic syndrome0


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Clinical Manifestation:-

#N M*NS % 8he male preponderance o 2:

: .Main maniestations:

@dema (varyin' de'rees) is the common symptom?ocal edema: edema in ace % around eyes( Periorbital s&ellin')% in lo&er

e=tremities.

!enerali$ed edema(anasarca)% edema in penis and scrotum.

2-Non-speciic symptoms:

Bati'ue and lethar'yloss o appetite% nausea and vomitin' %abdominal pain % diarrhea

body &ei'ht increase% urine output decrease

pleural eusion (respiratory distress)


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Investigations:-

-6rine analysis:-

A-Proteinuria : - E S@?@*8#;@.

b-2 urine collection or protein

F/m'0m20hr or children

c- volume:oli'uria (durin' sta'e o edema ormation)

d-Microscopically:-

microscopic hematuria 2/% lar'e number o hyaline cast


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Investigations:-

2-"lood:A-serum protein: decrease FC.C'm0d? % Albumin levels are lo& ( 2.C'm0d?).

"-Serum cholesterol and tri'lycerides:*holesterol C.4mmol0? (22/m'0dl).

*-- @S, //mm0hr durin' activity phase

. .Serum complemen:;ary &ith clinical type.

.,enal unction

.

id i


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Gidney "iopsy:-

*onsidered in:

-Secondary N.S

2-BreHuent relapsin' N.S

-Steroid resistant N.S

- +ematuria

C-+ypertension

I- ?o& !B,


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Dierential Dia'nosis o NS:

D.D o 'enerali$ed edema:-

-Protein 9losin' enteropathy

2-+epatic Bailure.

-+B

-Protein ener'y malnutrition

C-Acute and chronic !N

I-urticariaJ An'io edema


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*omplications o NS:-

-#nections:#nections is a ma7or complication in children &ithNS. #t reHuently tri''er relapses.

Nephrotic pt are liable to inection because :

A-loss o immuno'lobins in urine.

"-the edema luid act as a culture medium.*-use immunosuppressive agents.

D-malnutrition

8he common inection :6,#% peritonitis% cellulitis and 68#may be seen.

>r'anisms: encapsulated (Pneumococci% +.inluen$ae)%!ram ne'ative (e.' @.coli


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Complication 1

Vaccines in NS;-

polyvalent pneumococcal vaccine (i not previouslyimmuni$ed) &hen the child is in remission and o daily

prednisone therapy.

*hildren &ith a ne'ative varicella titer should be 'iven varicella

vaccine.


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Complication1..

2-+ypercoa'ulability(8hrombosis).

Hypercoagulability of the blood leading to venous or arterial thrombosis:

Hypercoagulability in Nephrotic syndrome caused by: -+i'her concentration o #%##% ;%;##%;###%K and ibrino'en

2- ?o&er level o anticoa'ulant substance: antithrombin ###

-decrease ibrinolysis.

-+i'her blood viscosity

C- #ncreased platelet a''re'ation

I- >vera''ressive diuresis


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-A,B:pre-renal and renal

2- cardiovascular disease:-Hyperlipidemia, may be a riskfactor for cardiovascular disease.

C-+ypovolemic shocL

I->thers: 'ro&th retardation% malnutrition% adrenal cortical insuiciency


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3anagement of NS:

!eneral (non-speciic )


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!eneral therapy:-

+ospitali$ation:- or initial &orL-up and evaluation o treatment.

Activity: usually no restriction % e=cept

massive edema%heavy hypertension and inection.

Diet+ypertension and edema: ?o& salt diet (2'Na0 day) only durin'

period o edema or salt-ree diet. Severe edema: ,estrictin' luid intaLe

Avoidin' inection:very important.

Diuresis: +ydrochlorothia$ide (+*8) 2m'0L'.d Antisterone 2 m'0L'.d

De=tran / Cml0L' % ater / I/m%

ollo&ed by Burosemide (?asi=) at 2m'0L'.


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#nduction use o albumin:-

Albumin E ?asi= (2/ salt poor)

-Severe edema

2-Ascites -Pleural eusion

-!enital edema

C-?o& serum albumin


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*orticosteroidprednisone therapy:-

Prednisone tablets at a dose o I/ m'0m20day (ma=imumdaily dose% 3/ m' divided into 2- doses) or at least consecutive &eeLs.

Ater complete absence o proteinuria% prednisone doseshould be tapered to / m'0m20day 'iven every otherday as a sin'le mornin' dose.

8he alternate-day dose is then slo&ly tapered anddiscontinued over the ne=t 2- mo.


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4reatment of relapse in NS:

Many children &ith nephrotic syndrome &ill e=perienceat least relapse (-Eproteinuria plus edema).

daily divided-dose prednisone at the doses noted earlier

(&here he has the relapse) until the child entersremission (urine trace or ne'ative or protein or consecutive days).

8he pred-nisone dose is then chan'ed to alternate-daydosin' and tapered over -2 mo.


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Accordin' to response to prednisonetherapy:

*emission: no edema, urine is protein ree or % consecutive

da"s

* elapse: edema, or irst morning urine sample contains ' 2 +

protein or consecutive da"s

*-requent relapsing: ' 2 relapses .ithin months (' 4/"ear)

*teroid resistant: ailure to achieve remission .ith

prednisolone given dail" or 21 da"s


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Alternative agent:-

hen can be used:

Steroid-dependent patients% reHuent relapsers% and steroid-resistant patients.

*yclophosphamide Pulse steroids

*yclosporin A

8acrolimus

Microphenolate


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4H& &ND1.

THANK YOU.THANK YOU.

nephrotic syndrome ini punya orang maaf ya

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