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
Nephrotic syndrome ass’d with IVDA and AIDS
Focal Segmental
Nephrotic syndrome ass’d with African Americans and Hispanics
Focal Segmental
Best initial test for Nephrotic syndrome
UA
- Good for amt of protein over 24 hrs
- Albumin/Creatinine = average protein produced over 24 hrs
Most accurate test for Nephrotic syndrome
Renal BX
What are maltese crosses and when do we see them
Lipid deposits in sloughed off tubular cells
Fatty cast
Nephrotic syndrome
Labs of Nephrotic syndrome
Hyperproteinuria
Hypoproteinemia
Hyperlipidemia = loss of lipoproteins
Edema
Best initial TX for Nephrotic syndrome
Glucocorticoids No response after weeks = cyclophosphamide ACE/ARBs Edema = salt restriction + diuretics HyperLipidemia = statins
Presentation of Uremia
Metabolic Acidosis Fluid overload Encephalopathy Hyperkalemia Pericarditis
Manifestations of renal failure
Anemia = loss of EPO
Hypocalcemia = no 1,25-OH Vit D to absorb calcium from gut
Osteodystrophy = secondary hypoPTH from low Calcium
Bleeding = platelets do not work in uremic environment b/c can’t degranulate
Infxn = neutrophils don’t work well - can’t degranulate
Pruritis
Hyperphosphatemia
Hypermagnesemia
Atherosclerosis and HTN
Endocrinopathy - Men have low testosterone, ED. women are anovulatory
When do we use EPO as TX
Anemia from ESRD is the ONLY time
What is winter’s formula
pCO2 = 1.5 (HCO3) + 8
TX for hyperphosphatemia
Oral Phosphate Binders Calcium acetate Calcium carbonate Sevalamer Lanthanum
Which phosphate binders do we never use? Why?
Aluminum containing
Cause dementia
Assn’s with TTP
HIV
Cancer
Drugs = cyclosporine, ticlopidine, clopidogrel
HUS ass’d with
Children
E.Coli 0157:H7
Shigella
TTP/HUS Presentation
Intravascular hemolysis
Renal insufficiency
Thrombocytopenia
What does TTP/HUS look like on smear?
Schistocytes
Helmet cells
Fragmented RBCs
TTP ass’d with
Neuro sx’s
Fever
TX for TTP/HUS
HUS = resolves on own TTP = emergent Plasmapheresis or infusion of FFP
When do we use steroids in TTP/HUS
Never.
MCC of death in PCKD
Renal Failure from recurrent pyelonephritis and nephrolithiasis episodes = scarring, loss of renal function
Assn’s with PCKD
Liver cysts
Ovarian cysts
MVP
Diverticulosis
Causes of hypernatremia
From loss of free water Sweating Burns Fever Pneumonia Diarrhea Diuretics
Pathophys of DI
High volume water loss
Insufficient/ineffective ADH
Central v Nephrogenic DI
Central - production problem
Nephrogenic - Loss of ADH effect on collecting duct
Causes of Nephrogenic DI
Lithium Demeclocycline Chronic Kidney Dz Hypokalemia Hypercalcemia
What is Urine osmolality and sodium in NDI
Low
What is Urine osmolality and sodium in CDI
Low
Best initial test for DI
Water Deprivation Test
- Urine volume stays High
- Urine osmolality stays Low
Response to ADH admin
CDI = sharp decrease in urine volume, increase osmolality NDI = No change
Most accurate test for CDI v NDI
ADH Level
- Low in CDI
- High in NDI
TX for DI
Correct underlying cause CDI = replace ADH NDI = Correct K+ and Ca+ Stop offending drugs HCTZ or NSAIDs
What complication occurs when sodium levels are brought down too rapidly
Cerebral Edema from shift of fluids from vascular space into cells of the brain
- Worsening confusion and seizures
