Nephrotic Syndrome

NEPHROTIC SYNDROME ✓Massive Proteinuria (735 g/24hs) Hypoalbuminemia ✓ Edemer Hyperlipidemia & lipiduria. PATHOPHYSK OGY ulomelor Glareuls copy wall-Berries/1 Mosive potencia- Permeability of GBM to proteins Massive Proteinuria (73.5 gldes Hypoobuminenia (serum Albumin (3g/100mL) Plasma oncotic pressure Plasmes volume synthesis of Lipoprotein in Liver Catabolism of Lipids Glomerulos Filkation Rate Dildosterone seaction Sodium & water Relentia. Edema Isoft, FRonorLity regions Hyperlipidemia CIPIDURIA (eakage) of Cipoproteins reabsorbal Sshed along desenrute colls Free Fat (oval Faties) Bodies Complications coronary atherosclerosis Thranbuembolic comp (Loss of Anhing) MEMBRANOUS-GLOMERULOPA THY uniform diffuse thickening of glomeruler capillary wall. This is due to the accumulation of (election-dense-deposits) along the subepithelial sick of glomerular BM. ~30% ETIOLOGY 1/Idiopathic (85%) Drugs: Penicillamine, NSAIDS •Mallignat neoplasms (akinoma of Lung & colon Autoimmune disease SLE Infections- Hep B, Hep C. PATHOGENESIS Chronic-complex-mediated diceese directed a guint Mospholipase agu Idiopathic M-site formation of immune A2 Receptor Antigen (PLAZK) complex) 2° circulating Immune Complex Endo Thyroglobe & Renal Antigen → →Hep B, Treponema Exo Mechanism No inflamy rells Complement C5b-Camembrane attack complex) damage podocyte & mesingalall liberates oxidants & protes ses cauce (Capilly wall injury lenkage of protein// MORPHOLOGY LIGHT MICROSCOPY 1. GLOMERULI = uniform, diftuse thickening of glomerulos capillary wall • Neither proliferation nur inflammation • SPECIAL STAINS Silver stain (Black colond PERIODIC ACIP SCHIFF STAIN (PINK-COLOUR) characteristic SPIKES in glomeruli blw deposits of immune-complex- 2. TUBULES Epimelial cells of PT show. PROPLETS of reabsorped Protein. 3 INTERSTITUM Mononuder cell (inflammy cells & Intersitial Fibrosis 4. BV Earl stuge vessels app. NORMAL ELECTRON MICROSCOPY Prickong of glomeruls capill wall due to irrepor electron deposits of inmene complexs bu basement membrane & subepithelium...... BM-Spikes protrudis from GBM //.. (SPIKE & POME PAT PATTERM) Jep Stag-1) Mild thicto 2) projectin of GBM SPIKES 3) Enaires DOME-LIKE PROTRUSION depositio of BM sclerosis of GLOMERULI Sonmanofloures, microsy granulis dejasit of igh& C3 CE sudden onsel of NS Non-selective protonuria Do not resp und corticosteroid therapy MINIMAL CHANGE DISEASE Glamsvile changes are absent or (minimal) and glomenli appers normal under light microscopy (Podocytes) E/M: Diffuse effacement of foot process of vis. epithelial Aji-Q-6ys D. Immunological mechanism (Lack of deposits= No immune amplex) Mechanism Resping infections or Rontinue propylactic immunizatia Responds to CORTICOSTEROIDS Ass with atopic/allergic disorders Inc. incidence with patients (Hodgkin Lymphoma) HLA haptogtes (genetic pre-disposition) →ytokine danasy 27 Non-Immung NEPHRINS & PODOCIN Protenuria for Nephringere found in Compat Mephiroth Proleniy in (FINNISH) MORPHOLONY GROSS "Kidneys-mild enlarged + smooth capselor surface. Cut surface :- pule to yellow cortex. LIGHT MICROSCOpy. Glomeruli- Normal • Tubules → Cytoplasm of epithelial cells lining tubules contain LIPID VACUOLES & glassy (ngaline) protein droplets Intersitium Mild odena • Blood vessels Normal ELECTRON MICROSCOPY •GBM app normal without of electivo deposit • Change in visceral opithelial cell :- Uniform & diffax effacement of spithefoot procuse ytoplasm => swell & Vacuolization Immunal foresanen ↳ Does not show Ig is complemat deposits. CLINICAL FEATURES ✓ preceded y ypur RT infection proteinuria - Higs selective Generalized edema, hypoalbumine, hyprlipidemia Kenal function = Normal/ PROGNOSIS EXCELLENT