MTB 2 Flashcards
Alport Syndrome
Defect in?
Presentation?
Tx?
Collagen - Type IV
Sensorineural hearing loss
Visual disturbance - collagen loss for lens of eye
No therapy
Polyarteritis Nodosa
Pathophys
Ass’d with
Systemic vasculitis - small and medium arteries
MC affect kidney
Spare the lung
Ass’d with Hep B
PAN Presentation
Organ systems involved
GN +
Nonspecific sx’s = fever, malaise, weight loss, myalgias, arthralgia over wks to months
GI = Abd pain, bleeding, N/V. Pain worse w eating
Neuro: Vasc damage around big peripheral nerves = neuropathy of peroneal, ulnar, radial, brachial
Mononeuritis multiplex
Stroke in young person
Skin: Purpura and petechiae; digital gangrene, livedo reticularis
Cardiac Dz = 1/3 of pts
Livedo Reticularis seen in
Atheroemboli of kidney
PAN
Tests for PAN
Anemia, leukocytosis
High ESR and CRP
ANA and RF sometimes
Most accurate diagnostic test PAN
BX of symptomatic site
TX for PAN
Cyclophosphamide + Prednisone
Best initial test for PAN
Angiograpy shows aneurysmal dilation
Presentation of Lupus Nephritis
Any degree of renal involvement
Kidneys - normal or mild, asymptomatic proteinuria
Severe = Membranous GN
Long standing scars = Glomerulosclerosis
Most accurate test for Lupus nephritis
Kidney Bx
TX for Lupus nephritis
Mild
Severe, proliferative
Mild - glucocorticoids
Severe - Glucocorticoids + Cyclophosphamide or Mycophenolate
MOA of Mycophenolate
Inhibits enyzme needed for T and B cells to grow
Immunosuppresant
Used in RA
DDX for large kidneys seen on sonogram and CT scan
PKD
Amyloid
HIV nephropathy
Diabetes
Amyloidosis ass’d with
Abnormal protein production Myeloma Chronic Inflammatory Dz Rheumatoid Arthritis IBD Chronic Infxn
Most accurate test for Amyloidosis
Bx shows green birefringence with Congo Red stain
TX for Amyloidosis
Tx underlying dz
Melphalan + Prednisone
Massive proteinuria leads to
Nephrotic syndrome
- Edema
- Hyperlipidemia
- Thrombosis = urinary loss of Protein C, protein S, antithrombin
Nephrotic syndrome ass’d with Cancer of solid organ
Membranous
- Breast, Lung, Colon, Prostate
Nephrotic syndrome ass’d with Hep B
Membranous
Membranoproliferative
Nephrotic syndrome ass’d with SLE
Membranous
Nephrotic syndrome ass’d with Hodgkins Lymphoma
Minimal Change
Nephrotic syndrome ass’d with Children
Minimal Change