glomerulonefrita focala si segmentara
TRANSCRIPT
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Glomeruloscleroza focala si segmentala (GSFS) este o entitate histopatologica caracterizata de:
• prezenta unor leziuni sclerotice segmentale la unii glomeruli (dar nu la toti glomerulii)
• indus de cauze multiple prin mecanisme patogenetice multiple
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CLASIFICAREGSFS primara (idiopatica) - Leziuni podocitare de etiologie necunoscuta
• Clasica (fara alta specificare – FAS)• Varianta cu leziuni perihilare• Varianta celulara• Varianta cu leziune polara (tip lesion)• Varianta colabanta
GSFS secundara• Leziune podocitara de cauza cunoscuta
• Virala: HIV, Parvovirus B19• Droguri: Interferon alfa, Litiu, Pamidronat
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• Secundare adaptarilor structurale sau functionale• Cu numar redus de nefroni functionali
Agenezie renala unilaterala, Oligomeganefronia, Displazia renala, Polichistoza renala forma adultului, Nefropatia de reflux, Nefrite interstitiale si pielonefrita cronica, Necroza corticala partiala, Necroza papilara din nefropatia analgezica, Sickle cell anemia, Glomerulonefrite primare, Nefrectomia si ablatiile renale mai extensive, Boala ateroembolica, Hipertensiunea arteriala
• Cu numar de nefroni initial normaliObezitatea morbida, Bolile cardiace congenitale cianogene, Nefropatia diabetica, Glicogenozele, Disautonomia familiala, Acromegalia
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• Secundara unor boli genetice• Autozomal recesive: Mutatii NPHS-1 – nefrina, Mutatii NPHS-2 – podocina
• Autozomal dominante : Cromozomul 19q/13 –mutatii ACTN–4 – alfa actinina 4, Alte• Diverse: GSFS - sindromic
• Secundara glomerulonefritelor proliferative focale• Alte asocieri
Nefropatia cu C1q, Acidoza tubulara renala, Sarcoidoza, Sarcom Kaposi, Masa musculara mare, Sindrom Behcet, Sindrom Prader-Willi, Sindrom Guillain-Barre, Sindromul Turner, Limfomul non Hodgkin, LES, Filarioza Loa-loa, Schistozomiaza mansoni, Preeclampsia, Transplant renal, Abuzul de heroina
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EPIDEMIOLOGIE
• 15-20% din sindroamele nefrotice punctionate,
• 4-8% din totalul bolnavilor investigati prin punctie renala
• Incidenta si prevalenta este in crestere in ultimii 20 de ani. Motivul cresterii prevalentei este necunoscut. In SUA tinde sa devina a IIa, mai frecventa forma de GN
• Prevalenta GSFS este de 2-4 ori mai mare la rasa neagra fata de caucazieni
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PATOGENEZA
Cicatrici glomerulare segmentale si focale alcatuite din colagen IV generate de diverse afectiuni care actioneaza prin mecanisme lezionale comune.
• Virusii – leziuni podocitare directe
• Glomerulonefritele, dislipidemiile – procese inflamatorii care – exces de TGFβ si PDGF care prin modularea CTGF – leziuni cicatriciale fibroase
• Reducerea populatiei de nefroni – hipertensiune intraglomerulara – activare celulara – cicatrici fibroase
• Factorul de permeabilitate
• Modificari in sinteza proteinelor ce regleaza permeabilitatea diafragmului porilor celulelor podocitare
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Stadiu I – stergerea pedicelelor, celule podocotare crescute in dimensiune cu numeroase vacuole de incluzie proteica
Stadiul II – Podocitul se detaseaza de MBG si sintetizeaza o matrice care formeaza un halou subpodocitar
Stadiul III – Sinechie scleroasa intre MBG si capsula B denudata, capilar hialinizat
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mbB
mbG
eBV V S
mbB – membrana bazala Bowman
eB – epiteliu Bowman
V – vacuole din celula podocitara
- halou clar subpodocitar
mbG – membrana bazala
glomerulara
S – sinechie
C – capilar
M – celula mezangiala
CM
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GSFS colabanta
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SHFS / GNPM
GSFS forma CELULARA
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GSFS TIP LESION
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GSFS forma HILARA
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GSFS forma CLASICA
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MANIFESTARI CLINICE
• 70 % din cazuri – SINDROM NEFROTIC IMPUR
• asociaza de regula hematurie si HTA
• 30% din cazuri• PROTEINURIE IZOLATA
• SINROM NEFRITIC CRONIC
• Proteinurie
• HTA
• Hematurie
Biologic
• proteinurie neselectiva
• C3 normal
• CIC crescut – 30% din cazuri
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• Proteinuria nefrotica
• Creatinina ser crescuta de la prezentare
• Fibroza interstitiala si atrofiile tubulare
• Leziunile colabante
• Proteinuria refractara la corticoizi
FACTORI DE PROGNOSTIC NEGATIV
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TRATAMENT
CORTICOTERAPIE
• PREDNISON – 1 mg / kg / zi – timp de 3- 4 luni
Reaspuns favorabil Raspuns negativ
( SN se remite ) (SN nu se remite)
-se sevreaza 3 – 4 saptamani - 1,6mg/kg/48h timp 6 luni
- in caz de esec -
CICLOSPORINA
- 5mg / kg/ zi – 6 luni
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EVOLUTIE - PROGNOSTIC
• Trei tipuri de evolutie in functie de raspunsul la corticoterapie
• 37% din cazuri – remisiune durabila
• 14% din cazuri – recaderi multiple – evolutie lenta spre
IRC
• 50% din cazuri – corticorezistenta – evolutie progresiva
spre IRC