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Nephrotic syndrome

Nephrotic syndrome. Eva Albrechtsen. Skilgreining. Skemmd verður á glomeruli nýrnanna sem verða lekar  proteinuria Aðallega albúmín (selective proteinuria = stærðar-og hleðslutakmörk) Önnur einkenni secundert ve/ próteinuriu; Hypoalbumemia (<25g/L) Hyperkolesterolemia Bjúgur. Tíðni.

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Nephrotic syndrome

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  1. Nephrotic syndrome Eva Albrechtsen

  2. Skilgreining • Skemmd verður á glomeruli nýrnanna sem verða lekar  proteinuria • Aðallega albúmín (selective proteinuria = stærðar-og hleðslutakmörk) • Önnur einkenni secundert ve/ próteinuriu; • Hypoalbumemia (<25g/L) • Hyperkolesterolemia • Bjúgur

  3. Tíðni • Nýgengi • 4,4/100.000/ári hjá börnum 1-6 ára • 2,0/100.000/ári hjá börnum 6-16 ára • 80% eru yngri en 8 ára v/ greiningu • Algengari hjá strákum en stelpum yngri en 6 ára en jöfn kynjaskipting eftir það • Algengi • 16/100.000

  4. Saga og greining • Saga um veirusýkingu í efri loftvegum • Atopískt exem og ofnæmi • Leita til læknis vegna vaxandi bjúgs, sérstaklega í kringum augun á morgnanna en á fótum og í kringum kynfæri á kvöldin • Blóðþrýstingur venjulega í lagi • Þreyta, óróleiki, kviðverkir, þyngdartap, hematuria og niðurgangur algengar kvartanir • Sólarhringssöfnun á þvagi • > 40 mg/m2/klst eða > 50 mg/kg/dag

  5. Flokkun • Eftir histologiu: • Minimal change nephrotic syndrome (77%) - MCN • Focal segmental glomerulosclerosis (10%) - FSGS • Membranoproliferative glomerulonephritis (5%) - MPGN • Membranous nephropathy (2%) - MN • Annað (6%) • Mesangial proliferative glomerulonephritis, Congenital nephrotic sx, Alport sx, sec ástæður (SLE, Goodpasture sx, Wegener granulomatosis, amyloidosis, multiple myeloma, lyf, sýkingar)

  6. Meðferð • Allri nýir sj fá 4-8 vikna meðferð með sterum p.o. og eru eftir það flokkaðir í: • Steroid sensitive nephrotic syndrome (90%) • Steroid insensitive nephrotic syndrome (10%) • Ef engin svörun verður við sterameðferðinni eftir 4 vikur er tekin nýrnabiopsia • Þvagræsilyf (Lasix) og takmörkuð saltneysla ve/ bjúgsins

  7. Steroid sensitive nephrotic syndrome • Minimal change disease í langflestum tilfellum • 80% ná fullum bata fyrir 20 ára aldur á sterameðferð, en getur verið krónískur sjúkdómur • Aukaverkanir tengd steragjöf (vaxtarskerðing, cushingoid, acne, beinþynning, gláka o.fl.) • Líka hægt að gefa cyclophospamide eða cyclosporine-A

  8. Steroid sensitive nephrotic syndrome (2) Minimal-change nephrotic syndrome (MCNS) indicates glomerular morphology that on light microscopic examination is little different from normal. There may be minimal mesangial alterations, but immunoglobulins are usually absent (stundum IgM), and no deposits are observed on electron microscopy. The only significant change seen on electron microscopic examination is flattening and fusion of the epithelial cell podocytes (foot-processes)

  9. Steroid insesitive nephrotic syndrome • Focal segmental glomerulosclerosis, MPGN, MN • Svara mjög illa meðferð og enda oft í endastigs-nýrnabilun; díalýsa og ígræðslu • Háskammta sterameðferð + cytotoxísk meðferð • Háskammta cyclosporin A • 40% fá recurrence í ígrædda nýrað • Ónæmisbældir ve/ tap á immuno-globulinum og opsoninum í þvagi  peumococcal peritonitis

  10. Steroid insesitive nephrotic syndrome (2) Focal segmental glomerulosclerosis (FSGS) describes a lesion in which some glomeruli are involved with segmental sclerosis (one lobule or section within a glomerulus), with the remaining glomeruli being normal. Because this lesion is focal and is often confined to the juxtamedullary nephrons, it may be overlooked on renal biopsy examination. Immunofluorescent microscopy yields a variable picture. In some patients, all classes of immunoglobulins and complement appear to be trapped in the sclerotic area; in others, distinct immune-complex-type, particularly IgM, deposits are found .

  11. Komplicationir • Thrombosis • V. Renalis, a/v. Pulmonaris, peripherar æðar • Embolia pulm • IgG skortur  sýkingar • Skortur á plasmapróteinum; • Hypothyroidismi • D-vítamínskortur • Zink-skortur  lélegur gróandi sára og skert frumubundið ofnæmissvar

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