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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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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
slide22
.
  • 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
slide28
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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