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Nephrotic Syndrome. Outline. Definition Epidermiology Classification Ethiopathogenesis Pathophysiology Pathology/Histology. Clinical Features Lab. Investigations Treatment Complications Prognosis. Definition. N/ Synd is a Dz condition Xterized by: -Gross Proteinuria

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Nephrotic syndrome

Nephrotic Syndrome


Outline
Outline

  • Definition

  • Epidermiology

  • Classification

  • Ethiopathogenesis

  • Pathophysiology

  • Pathology/Histology

  • Clinical Features

  • Lab. Investigations

  • Treatment

  • Complications

  • Prognosis


Definition
Definition

N/Synd is a Dz condition Xterized by:

-Gross Proteinuria

-Hypoalbuminemia (2.5g/dl)

-Hypercholesterolemia

-Edema


Nephrotic range proteinuria
Nephrotic Range Proteinuria

-24 hr. urine protein >50mg/kg

-urine protein >40mg/m2/hr

-Spot Upr/Ucr ratio >3 in a first morning sample

- >3+ proteinuria on Dipstick (albustix)


Epidermiology
Epidermiology

  • 2/3 of N/S present b4 5yrs

  • Boys:Girls ratio 2:1, 1:1 by late adolescence

  • 90% of cases are Primary (not assoc.wt syst.dz.

  • 80-85% of prim. N/Synd are steroid sensitive

    or histologically minimal change type.


Classification
classification

  • PRIMARY

    -steroid sensitive

    -steroid resistance

  • SECONDARY


Contd
contd

In 2ry N/Synd. Dz occurs as part of a systemic dz.

e.g: Quartanmalaria,SLE,HSP,Chr. Hep.Binfection,PSGN


Ethiopathogenesis unsure
Ethiopathogenesis (unsure)

Hypothesis

-Immune dysfxn of T lymphocytes >Unknown mediator> neutralizes anionic charge of Glom. Carpillary > inc. permb. To Albumin

-A primary abnormality in the foot processes is also postulated. Mutations in podocyte proteins hvbn identified in many inherited N/Synd. Mut. In trans memb. Prot. Of the slit diahgram causes Finnish N/Synd.


Pathology
Pathology

MCNS:

Renal biopsy findings show:

-No abnormality on light mcpy

-Obliteration of foot processes on e-mcpy

-Immunoflourescensemcpy: No deposition of Immune rxtants.

In N/Synd due to SLE, immune comlex deposits in glom. + cellular proliferation is seen


Contd1
contd

  • Chr. Hep.Binfectn may cause membranous or membranoproliferative N/Synd


Pathophysiology
Pathophysiology

  • Loss of charge selective barrier > loss of –vely charged mol like albumin that is normally repelled

  • It is postulated that infectious stim.> cytokines toxic to glom epith and Bm.> redctn in –ve charges and loss of albumin.


Pathophysiology1
Pathophysiology

Hypoproteinemia:

-Urinary prot. loss= abt 3-5g/day

-Inc. catabolism of reabsorbed prot. By renal tube epith. Cells > amino acids= body pool

Hypercholesterolaemia:

-Reduced Albumin >gen. Inc hepatic prot. Synthesis > overprodctn of lipoprotein

-Red. Actn of lipoprotein lipase > redcd transp. Of lipids to adipose tissue.


Contd2
contd

Oedema:

Hvyproteinuria > Alb (<2.5g/dl) >Red plasma OsmP > Fluid loss to interstitium > Oedema > Hypovolemia (dec. ECFvol) > RA/ADH system = salt & H20 retention.

Thrombosis: Can occur in cerebral V or A,pulm, femoral vs. Inc. hepatic. Prot. Synth >inc level of clotting factors e.gI,II,VII,X,fibrinogen,protein C, prot.S


Contd3
contd

  • Inc spont. Platelet aggregatn in response to collagen & ADP.

  • Loss of anti thrombotic factors e.g anti thrombin III & plasminogen.

  • Inc haematocrit wt severe vol. contraction is a factor.


Infections
infections

  • Due to redctn in IgG and factor B due to urinary losses

  • Impaired lymphocyte fxn

  • Steroids and immunosuppressive Rx inc risk of infection


Clinical features
Clinical features

  • History:

    Early morning puffiness of eyelids

    Slowly progressing gen. edema

    Dec urine output

    Hx may suggest complications e.gperitonitis,thrombosis,symptoms of intravacular depletion.

    Hx may exclude diff. Diagse.gpharyngitis and skin infection =PSGN,Erythematous skin rash,athralgia/arthritis =HSP,SLE

    Hx to exclude CCF & liver pathology both causes of edema.

    Age of onset may suggst MCNS or Atypical N/S

    +vefhx of NS may suggest familial NS of FSGS type


Physical examination
Physical Examination

  • Extent of Edema

  • Search for complications of Dz or Rx + effusions, Tender jtswellings,Hepatosplenonomegaly may suggest vacsulitis,CVA etc

  • BP

  • Wt.

  • Abd girth to monitor progress


Lab investigations
Lab Investigations

  • Urinalysis=Neph. Range proteinuria

  • Up to 25% have mcpichaematuria

  • Casts= hyaline, granular & few wbc casts occas.

  • Presence of RBC casts and sig amt of RBC suggest secondary cause.

  • CBC,SEUC,C3,ASO titre,Albumin&cholesterol diff NS from other causes of edema.


Contd4
contd

  • Low C3 suggests APGN,MPGN & SLE

  • ANA screens and other collagen vascular dz.

  • + HepBsAg suggests MN or MPGN

  • Cxray= not routine >pleural effusion

  • Abd U/S > renal sz to r/o Hivan-renomegaly or CRF-shrunken kidneys.


Treatment symptomatic supportive
Treatment (symptomatic/supportive)

Indications for admission

-Anarsacacompromizingmovt or resp.

-Unstable vital signs

Fever

U.O < 1ml/kg/hr= oliguria

Severe haemoconc. i.ePcv>48%

Presence of life threatening complications


Contd5
contd

  • Stict input/output fluid chart

  • BP monitoring

  • Daily weighing

  • Daily abd. Girth measurement

  • Daily urinalysis

  • Mantoux testing bcos of impending steroid Rx to prevent disseminated TB.

  • Parental Education


.

  • Diet: No added salt diet : only in severly edematous.

  • Fluid may be restricted along wt Na in some cases of edema(inensible loss + previous days output)

  • Normal protein diet. High protein intake May cause hyperfilteration injury to kidneys.

  • Activity: No restriction on activity except if BP is raised in rare occ.


Diuretics
Diuretics

  • NB: not all NS pts require diuretics

  • Used in mod. to severe edema or oliguria in euvolemic or hypervolemic pts (n. carp refill, no orthostatic hypotension, good vol. pulse)

  • Frusemide 1-2mg/kg per dose p.o/i.v

  • Spironolactone + for k+ sparing effect

  • Thiazide 1-2mg/kg/day+ frusemide or Metazolone 0.5mg/kg/day are beneficial in pts refractory to frusemide alone


Contd6
contd

  • I.V 25% albumin is useful in pts with refractory edema,markedasciteswch impairs pulmfxn, peripheral oedema with skin breakdown, labial/severe scrotal edema.

  • Dose 1gm/kg max 25gm.Give over 2hrs +I.V frusemide after. Can rpt albumin after12hrs. If no change in U.O consider ARF.


Specific rx
Specific Rx

  • Goal is to induce and maintain remission from active NS while minimizing S/e of drugs

    Corticosteroid

    >90% of MCNS are steroid sensitive.

    Rx any systemic infectn b4 starting steroids.

    50% & 90% of MCNS achieve remission in 2wks & 4wks respectively


Corticosteroids
Corticosteroids

  • 60mg/m2/day or 2mg/kg/day oral prednis’one

    Max. dose of 60mg /day given in 2-3 divided doses x 6wks, followed by 40mg/m2/d or 1.5mg/kg/d oral pred. x 6wks.

    At the end of 6wks prednisolone is stopped and pt monitored on out pt basis afterwards 2wkly for signs of relapse.


Response to steroid rx
Response to steroid Rx.

  • Remission: Negative trace, or + proteinuria x 3 consecutive EMU samples.

  • Relapse: Urine Albustix 2+ or more for 3 consecutive EMU samples,may be assoc wt edema.

  • Frequent relapse: relapse 2 or more times in the first 6mths after presentatn or 4 or more times in any 12mths period



Treatment of relapse
Treatment of relapse taper steroids or within 14 days of stopping steroids.

  • In children with relapse: Daily pred.60mg/m2 until pt. achieves remission, followed by 4wks of 40mg/m2 alt.die pred.

  • In freq. relapsers after we achieve remission we ct 60mg/m2/d alt die which is tapererd over several wks to the min dose that maintains remission for abt 12months.


Rx of relapse contd
Rx of relapse taper steroids or within 14 days of stopping steroids.contd

  • Levamisole: Also an anti helminthic is an immunomodulator used in freq. relapers wt steroid sens. N/S. First we achieve remission then change to alt.die steroids then we stat. levam. 2-2.5mg/kg alt die on days steroid is not given x 6mths.Tx can ct x1-2yrs.Steroid should reduce to 0.25mg/kg by 1yr.

  • Levam. Is used for steroid sparing effect not for remission induction.


Rx of relapse contd1
Rx of relapse taper steroids or within 14 days of stopping steroids.contd

  • Cyclophosphamide: Where alt.die steroid with or without levam. Fails to maintain remission nxt step is use of cyclophosphasmide 2mg/kg/d x12weeks not exeeding total dose of 168mg/kg/course.

    Toxicity viz:Bmsuppression,inc risk of infectn,alopecia,haemorrhagiccystitis,gonadaltoxicity.Small risk of 2ry malig.NB @ 2m/kg/d risk of infertility does not appear to be inc.


Rx contd
Rx taper steroids or within 14 days of stopping steroids.contd

  • Chlorambucil, another alkylating agent is an alternative to cyclophosphamide. Repeat courses of both agents are usually not given.

  • Cyclosporin A: At a dose of 6mg/kg/d.It can be used when a child ct to be steroid dependent even after a course of c’phamde.

  • S/E include:hirsutism,gingival hyperplasia, wch regress when Rx is stopped.HTN & hypomagnesemia are known s/e.Long use can be nephrotoxic.Follow up renal biopsy indicated after 1yr to check for renal toxicity.


Indications for renal biopsy in n s
Indications for renal biopsy in N/S taper steroids or within 14 days of stopping steroids.

  • Steroid resistance

  • Before cytotoxic Rx

  • N/S in extremes of age i.e <6/12 or >8yrs

  • N/S assoc with persistent ARF

  • Nephritic onset of N/S

  • N/S assoc wt systemic features e.g recurrent jaundice, hepatosplenomegaly,arthritis,serositis.


Prognosis
Prognosis taper steroids or within 14 days of stopping steroids.

  • MCNS is essentially benign: approx.1/3 relapse once, another 1/3 have occassional, the remaining 1/3 bcome steroid dependent.

  • In many cases the relapses eventually cease. Many retain norm. renal fxn.

  • Most of the steroid resist. End in renal failure needing dialysis

  • Death usually results from: iatrogenic hypovolemia, sepsis and thrombosis.


Complications of n s
Complications of N/S taper steroids or within 14 days of stopping steroids.

Usually multifactorial:

Drug related Toxicity- Steroids,cyclophospamide,

Frusemide,spironolactone etc.

-Due to


Complications of n s1
Complications of N/S taper steroids or within 14 days of stopping steroids.

Usually multifactorial:

Drug related Toxicity- Steroids,cyclophospamide,

Frusemide,spironolactone etc.

-Due to Disease-Infections,Thrombosis,Anaemia,ARF,Shock etc.


Secondary n s
Secondary N/S taper steroids or within 14 days of stopping steroids.

  • This is thot to be the most common variety in the tropics. QMNS was thot to constitute up to 80% of N/S in some areas.

  • Causes include: G/nephritis, quartan malaria,

    schistosomiasis, syphilis, hydatiddz, Hep.B, Toxoplasmosis and varicella. Less common causes include: DM, SLE, anaphylactic purpura,renal vein thrombosis, heavy metals.Sickle cell anaemia,sarcoidosis.


Quartan malaria n s
Quartan taper steroids or within 14 days of stopping steroids. malaria N/S

  • MCNS

  • Temperate region

  • Idiopathic

  • Peak age, 2-5yrs

  • Haematuria-rare

  • Protein selectivity-nearly always. index.<0.1

  • Steroid response-good

  • S/e/u/c may be temp raised but later normalize

  • Histology: min. change on light mcpy.fusion of epith foot processes-e mcpy

  • QMNS

  • Tropics

  • Quartan malaria

  • Peak age,5-8yrs

  • Rare

  • Poor selectivity-index >2

  • steroid response-Poor

  • S/e/u/c persistently raised

  • Definite,gross pathologic features wt thickened carpillary walls leading to eventual glom. Sclerosis.


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