Nephrotic syndrome
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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


Nephrotic syndrome

.

  • 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


Nephrotic syndrome

  • Steroid dependent: relapse while on steroid Rx or when u taper steroids or within 14 days of stopping steroids.

  • Steroid resistant: Failure of proteinuria to resolve after 4-6wks of 60mg/m2/day of steroid Rx. Some authors use 8wks i.e 4wks dly & 4wks alt die prednisolone.


Treatment of relapse

Treatment of relapse

  • 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 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 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 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

  • 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

  • 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

Usually multifactorial:

Drug related Toxicity- Steroids,cyclophospamide,

Frusemide,spironolactone etc.

-Due to


Complications of n s1

Complications of N/S

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

  • 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 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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