Kidneys

Kidneys

1. Horseshoe kidney

   • IMA

2. Renal agenesis

   • Unilateral
     • Hyperfunctionating kidney young RF
   • Bilateral
     • potter sequence

3. PKD inhereted (cyst cortex and medulla)

   • AD (APKD1 Chr 16, APKD2 Chr 4)
     • Adults. Berry aneurysm, mitral valve prolapse, bening hepatic cyst. ↑ renin
   • AR
     • infants, worsering renal failure, hypertension. Newborn Potter sequence. Liver cyst and fibrosis (portal hypertension)

4. Medullary cystic disease (cyst medulla and collecting tubules)

   • Shrunked kidneys

5. Dysplastic kidney non inhereted

   • Congenital malformation of renal parenchyma

6. Nephrotic syndrome

   • Minimal changes
     • Idiophatic or HL, HE: normal lipid PT, EM: effacement of podocytes, IF: negative. Selective proteinuria (albumin) Respond to steroids
   • FSGS
     • HIV, sickle cell, heroine abuse. HE: focal segmental sclerosis, EM: effacement podocytes, IF: negative. Doesn't respond to steroids.
   • Membranous nephropaty
     • MCC Caucasians, HB, HC, SLE (NSAIDS, penicillamine). HE: Thick glomerular BM. EM and IF: granular, spike and dome apperance. (subepithelial deposits)
   • Membranoproliferative glomerulonephritis
     • HE: thick BM, tram track apperance. IF: granular
       • Type I
         • Subendothelial. MORE in HBV, also HCV
       • Type II
         • Intermembranous. C3 nephritic factor, stabilizes C3 convertase, overactives complement
   • Diabetes mellitus
     • Hyaline arteriolosclerosis. EFFERENT more affected→ hyperfiltration (microalbuminuria). Sclerosis mesangium Kimmsteil Wilson Nodules
   • Systemic amyloidosis
     • AL Apple green birefringens

7. Nephritic syndrome

   • PSGN
     • M protein IC. S pyogenes. 2-3 w skin or pharynx infection. HE: Hypercellular, inflammed glomeruli. IF: starry sky apperance and lumpy bumpy
   • RPGN. Crescent moon shape> Fibrin plasma prot, mos
     • Linear
       • Good Pasture Syndrome
         • Ab alpha3 noncollagenous part of collagen IV. Dult males, Hematuria hemoptisis. Plasmapheresiis
     • Granular
       • PSGN and diffuse proliferative glomerulonephritis
         • SLE MC type of renal disease-- usually subendothelia or intramembranous. LM: Wire looping capillaries.
     • Negative IF paucci immune
       • Wegener
         • Granulomatous. Nasopharynx. C- ANCA/PR3-ANCA (anti proteinase 3)
       • Microscopic polyangitis p-ANCA- MPO-ANCA (anti myeloperoxidase)
       • Churg Strauss
         • Granulomatous. Asthma eosinophilia. p-ANCA
   • IgA nephropathy. Berger Sd.
     • IF: IgA IC mesangium. Henoch-Scholein purpura.
   • Alport syndrome
     • Mutation type IV collagen. X linked. Thinning, sppliting MB. Eye problems, hearing loss, hematuria. EM: Basket weave