Nephrotic Syndrome 2016

7/25/2019 Nephrotic Syndrome 2016

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Nephrotic Syndrome

Abdulrahman Mashi


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Nephrotic Syndrome

Nephrotic-range proteinuria : urine proteinexcretion of >3.5 g!" h or a urine protein-

creatinine ratio of >35## mgg in a $spot$morning urine sample

%ypoalbuminemia &serum albumin' 3 gd().

*demaHyperlipidemia


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+athogenesis

Underfll

Model

Overfll

Model

Permeability of glom.cap.memb. Proteinuria

Intravascular vol

ADHRenal perfusion

pressure

Water

Reabsorptn

InCollectingducts

Actv. reinin

Ang. ald. sys

Tubular reabsorp.

Of a

Hypoalbuminemia

Hepatic protein synt!esis

Plasma oncotic

pressure

HyperlipidemiaTransudation of fluid

from intravascular

comp. To interstial

space

"dema


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*le,ated lipids occur from a combinationof

increased hepatic apolipoprotein

synthesis in response to a lo plasmaoncotic pressure

decreased acti,ity of ey en/ymes suchas lipoprotein lipase and lecithin-cholesterol acyltransferase.


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Nephrotic Syndrome

NephroticSyndrom

e

+rimary&0diopathic)

Secondary


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Nephrotic Syndrome

Primary (idiopathic)Minimal change disease &M12)

Membranous nephropathy &MN)

ocal segmental glomerulosclerosis &S4S).

Mesangiocapillary &Membranoproliferati,e) 4N&M14N M+4N)

ibrillary glomerulopathy : may be assocaited emalignacy

Immunotactoid glomerulopathy : May beassociated with chronic lymphocytic leukemia or Bcelllymphomas


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Nephrotic Syndrome

Secondary

2iabetes &the most common)

0nfections

2rugs

Autoimmune diseases

Amyloidosis

Monoclonal immunoglobulin depositiondisease


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Nephrotic Syndrome

Important pointsMinimal change glomerulopathyis the most common cause of thenephrotic syndrome in children

Membranous glomerulopathy and !S"S are the most common

causes of idiopathic nephrotic syndrome in adults.

Membranous glomerulopathy is the most common orldide

!S"Sis the most common in S and no increasing to be the mostcommon orldide

!S"Sis the most common cause in blac personsMembranous glomerulopathyis the most common cause in hitepersons

Membranous glomerulopathy has the highest predilection forrenal #ein thrombosis among all causes of the nephrotic syndrome


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*,aluation


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6reatment

4eneral measures: *le,ated lipid le,els are typically

treated ith statin medications

+rophylactic anticoagulation : 0n any type of Nephrotic if albumin le,el

7!.# gd( &!# g() ith lo bleeding risregardless of cause.

0n patients ith membranousglomerulopathy ith lo ris for bleedinghas been if albumin 7!.8 gd( &!8 g().


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6reatment

4eneral measures: *dema is treated ith a lo salt diet

and loop diuretics &alone or in

combination ith a thia/ide andpotassium-sparing diuretics).

A1* and A; to reduce proteinurea

Maintain good nutrition6reatment of underlying cause


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1omplications


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S4S

1auses: 0diopathic : Some cases of idiopathic S4S are thought to

be related to a circulating plasma factor because a signi


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S4S

!i#e subtypes

not otherise speci


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S4S

linical Mani%estations :

*ither asymptomatic proteinuria or edema.

More than to thirds of patients are fully

nephrotic at presentation Subnephrotic proteinuria may occur

especially ith secondary S4S fromhyper


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S4S

iagnosis

6he hallmar of S4S is the presence ofsegmental scars in some glomeruli.

*lectron microscopy shos ,isceralepithelial cell %oot process e*acement

no immune deposits+


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-reatment and Prognosis

0n idiopathic S4S only a minority of patients

experience a spontaneous remission.

-here%ore& treatment is indicated in mostpatients+

6herapy is usually ith glucocorticoids or calcineurininhibitors both at the time of initial presentation andfor relapsing disease.

A complete or partial remission may be seen in up to"#B to C#B of patients using these treatments.

S4S recurs in the transplanted idney in 3#B or more

of cases.


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0n secondary S4S caused by infection ordrugs treatment of the infection or remo,alof the oDending agent may halt progressionof the disease and impro,e symptoms.

0n obese patients ith liely secondaryS4S eight loss is sometimes associatedith a drop in proteinuria as is the use ofA1* inhibitors or angiotensin receptorblocers &A;s) and is the preferred initialtherapy.


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Membranous 4lomerulopathy

Primary or secondary

Primary %orm &most commonorldide): antiphospholipaseA!receptor autoantibodies can be

found in E5B of cases lead to

acti,ation of complement anddamage the 4;M.


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Membranous 4lomerulopathy

May be associated with or secondary to:

S(*

0nfections: hepatitis ; and 1 ,irus infectionsF syphilisF

malaria Medication exposure: penicillamineF NSA02sF 6N-G

inhibitorsF tiopronin

Mercury or gold exposure

Malignancies: Solid organ Malignancy &bladderbreast colon lung pancreas prostate stomachcarcinoma)F carcinoidF sarcomasF lymphomasFleuemias

6hyroid disease


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1linical Manifestations

(ie other causes of the nephroticsyndrome &edema hypertensionmicrohematuria) but the propensity to

thromboembolic e,ents &particularlyrenal ,ein thrombosis) is much higher.

Secondary causes should be soughtparticularly occult malignancy in olderpatients.


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Membranous4lomerulopathy

iagnosis

(ight microscopy shos glomerular capillaryloops that often appear thicened ithout

any proliferati,e lesions.0mmuno@uorescence and electronmicroscopy sho subepithelial immunedense deposits.

.here a#ailable& P/012 antibodies

should be measured+


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6reatment

p to one third of patients ith idiopathic M4 remitspontaneously in C to H! months.

1onser,ati,e management is appropriate during

this period.

0n patients ith idiopathic M4 who ha#epersistent disease a%ter 3 to 41 months or

who ha#e worsening kidney %unction or athromboembolic e#ent&regimens containingalternating glucocorticoids ith cyclophosphamideor calcineurin inhibitors &cyclosporine or tacrolimus)may be used.


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6reatment

Ither options for relapsing or refractorydisease include mycophenolate mofetil

adrenocorticotropic hormone and theantiJ;-cell antibody rituximab.

or secondary causes of M4 treat theunderlying cause lie treatment ofhepatitis ; ,irus infection ith anti,iralagents


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Minimal 1hange 4lomerulopathy

Primary %orm &0diopathic)

secondary : Medications &such as NSA02s lithium

pamidronate rifampin and the interferons) Malignancies &such as %odgin

lymphoma other lymphoproliferati,edisorders and thymoma)

0nfections: 0nfectious Mononucleosis and 6;


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linical Mani%estations

+atients ith M14 typically presentith acute onset of edema andeight gain due to @uid retention.

rine protein le,els tend to besigni


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M12

iagnosis

Normal glomeruli on both light andimmuno@uorescence microscopy.

6he tubules may sho lipid accumulation.

6n electron microscopy& the "BM isnormal with e'tensi#e e*acement o%

#isceral epithelial %oot processes+


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6reatment

+atients typically respond to glucocorticoids &Hmggd or!mgg e,ery other day )ithin 8 to HC ees.

1omplete remission achie,ed in around E# B

elapse is common and in up to "#B of patients thecourse of M14 is one of remission folloed by relapse.

or freKuently relapsing or glucocorticoid-dependent

disease treatment options include cyclophosphamidecalcineurin inhibitors &tacrolimus or cyclosporine)mycophenolate mofetil and rituximab.


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2iabetic Nephropathy

2iabetic nephropathy &2N) is theleading cause of *SL2 in the nitedStates

6he strongest clinical indicator forprogressi,e idney disease is the

le,el of urine albumin.


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2isk %actors %or de#eloping N

older age

race &American 0ndian Mexican American

and blac)poor glycemic control

hypertension

cigarette smoingfamily history of idney disease


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linical Mani%estations

Moderately increased albuminuria &formerly nonas microalbuminuria) de


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iagnosis

Annual testing for albuminuria should begin at

the time of diagnosis in type ! diabetes and 5years after diagnosis in type H diabetes.

Lidney biopsy is not indicated unless there isa suspicion of another glomerular disease.

In electron microscopy the 4;M is normalith extensi,e eDacement of ,isceralepithelial foot processes.

Moderately increased albuminuria de


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Indications %or kidney biopsy

acute onset of the nephrotic syndrome


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+athology

2N aDects e,ery compartment of the idney.

0n the glomerulus there is e'pansion o% themesangium and thickening o% the basementmembrane folloed by focal &nodular) sclerosis&the Limmelstiel-ilson lesion) then globalsclerosis of the glomerulus.

0nterstitial


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6reatment

Achie,ing targets of glycemic control&hemoglobin AHc'EB) and blood

pressure &'H"## mm %g.

0n patients ho ha,e diabetes ithmoderately increased albuminuria or

se,erely increased albuminuria A1*inhibitors or A;s ha,e been shon toslo progression.


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ead about

M+4N

Amylodosis

Multiple Myloma

%0 associated nephropathy

%epatitis ; associated idney disease


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eferences


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Nephrotic Syndrome 2016

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