兒科泌尿系統NS英文第1頁/共20頁

Definition

NSisanaccumulationofsymptomsandsignsandischaracterizedbyproteinuria,hypoproteinemia,edema,andhyperlipidemia.

第2頁/共20頁Thevastmajoritypatients(90%ofcases)withNSofchildhoodareprimary.

Definition

Inchildrenunderage5yearsthediseaseusuallytakestheformofidiopathic(primary)NSofchildhood.

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ConditionsOfAttack

●Secondonlytoacutenephri-

tis

●Incidenceage:atallages,but

mostcommonlybetween3~5

yearsofage第4頁/共20頁

Type

1.Clinicaltype

SimpletypeNS;

NephriticNS

2.Responsetosteroidtherapy

(P274)

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Type

Theinitialresponsetocortico-

steroidsisaguidetoprognosis

●Steroid-dependentNS

●Steroid-responsiveNS

●Steroid-resistantNS

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Type

▲

Totaleffect

▲

Partialeffect

▲Non-effect

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Type

3.

Pathologictype(P282)

●Minimalchangedisease,

MCD–80%ofallNS,little

changeonlightmicroscopy,

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Type

butelectronmicroscopy

showseffacementoffoot

processes(podocytes)of

epithelialcells

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Type

●Otherforms(Non-MCNS)

MSPGN,FSGN,MGN,

MPGN,etc.

第10頁/共20頁第11頁/共20頁第12頁/共20頁第13頁/共20頁第14頁/共20頁第15頁/共20頁Pathogenesis

?Theprimarydisorderis

anincreaseinglomerular

permeabilitytoplasma

proteins→lossofproteins,

mainlyalbumininurine

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Pathogenesis

?

Filtratedbarrier—

Chargebarrier

Molecularbarrier

?

Thelossofthenegative

chargesontheGBM

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Pathogenesis

?

Theunderlyingpathogenesis

isunknown,butevidence

stronglysupportstheimpor-

tanceofimmunemechani