Buzzwords - pathology Flashcards
(23 cards)
Focal segmental glomerulosclerosis
LM: segmental sclerosis and hyalinosis
IF -
EM: effacement of foot process
Nephrotic
Af Am/Hispanics
Membranous Nephropathy
LM: diffuse capillary and GBM THICKENING
IF: granular - immune complex deposition
EM: “SPIKE AND DOME”
Nephrotic
Causasian adults
IgG ab to phospholipase A2
Minimal Change Disease
LM: normal
IF: -
EM: effacement of foot process
Nephrotic
Children
Abn T cell production
Amylodosis - renal
LM: congo red stain - apple green birefringence under polarized light
Nephrotic
Membrano-proliferative glomerulonephritis
Type 1: Immune complex deposits with IF; TRAM TRACK d/t GBM splitting (HBV/HCV)
Type 2: Intramembranous IC deposits - dense deposits - associated with C3 NEPHRITIC FACTOR
Nephritic & nephrotic
mesangial proliferiation
Diabetic glomerulonephropathy
LM: Kimmelsteil Wilson Lesion
Nonenzymatic glycosylation of GBM
Eosinophilic
Nephrotic
Acute PSGN
LM: glomeruli enlarged/hypercellular
IF: STARRY SKY - d/t IgG, IgM, C3 deposition
EM: SUBEPITHELIUM IMMUNE COMPLEX (IC) HUMPS
Nephritic
T3H
peripheral & periorbital edema, COLA COLORED URINE, HTN
Increased anti-DNase B titers
Decreased complement
Rapidly progressive (crescentic) glomerulonephritis (RPGN)
LM & IF: CRESCENT moon - FIBRIN & PLASMA PROTEINS
Goodpasture (T2H - pulm & renal - ab to GBM)
Granulomatosis with polyantigitis (Wegener) - C-ANCA (upper/lower airway, renal)
Microscopic polyangitis - pANCA
Diffuse proliferative glomerulonephritis
SLE/MPGN
most common cause of death in LUPUS
LM: wire loops capillaries
EM: subendothelial and intramembranous IgG based IC w/ C3 deposition
IF: granular
IgA nephropathy (Berger dz)
LM - mesangial proliferation
EM: mesangial IC deposits
IF: IgA based IC deposits in mesangium
HSP
Alport syndrome
IV collagen mutation
Ear, eye, family HX
“Can’t see, can’t pee, can’t hear a bee”
ADPKD
Autosomal dominant polycystic kidney dz
Bilateral, massive enlargement of kidneys d/t multiple large cysts
85% - PKD1 (Chromosome 16)
15% - PKD2 (Chromosome 4)
Familial adenomatous polyposis
Adenomatous polyps after pubery
APC mutation on chromosome 5
5 letters in polyp
Familial hypercholesterolemia
Elevated LDL - defective or absent LDL receptor
Hereditary hemorrhagic telangectasia
Inherited disorder of blood vessels
Telangectasia, recurrent epistaxis, skin disoloration, AVM, GI bleeding, hematuria,
Osler-Weber-Rendu
Hereditary sperocytosis
Spherocyte erythrocytes - defective spectrin or ankrin
Hemolytic anemia, increased MCHC
TX: splenectomy
Huntington Dz
Depression, progressive dementia, choreiform movements, caudate atrophy
decreased levels GABA & Ach in brain
Chromosome 4 - CAG repeat
Marfan s;yndrome
Fibrillin 1 gene mutation
CT disorder - skeleton, heart, eyes
Tall w/ long extremities, pectus excavatum, hypermobile joints, & long tapering fingers and toes
Cystic medial necrosis of aorta - aortic incompetence and dissecting aortic aneurysm, floppy mitral valve
Subluxation of lens (up and temporal)
Mutliple endocrine neoplasia
1, 2A, 2B
Famililal tumors of endocrine glands
pancreas, parathyroid, pituitary, thyroid, adrenal medulla
2A & 2B - ret gene
Neurofibromatosis type 1
Von Recklinghausen Dz
Neurocutaneous - cafe au lait spots and cutaneous neurofibromas
AD - 100% penetrance but variable expression
NF1 gene on chromosome 17
NF 2
Bilateral schwannomas
Juvenile cataracts
Meningiomas
Ependymomas
NF2 on chromosome 22
Tuberous sclerosis
Neurocutaneous
Benign hamartomas
von hippel lindau
numerous tumors (benign and malignant) deletion of VHL gene (tumor suppressor) on chromosome 3
