BIg picture Flashcards
Patient comes in with bilaterally enlarged kidneys that look like bags of cysts, have a cystic liver, and mitral valve prolapse: What is the disease? What is the most likely mutated gene? What is a common (4-10%) cause of death in these patients?
Autosomal Dominant (adult) PCKD
PKD1 (cell-cell or cell-matrix interactions)
Berry aneurysm
A patient has autosomal recessive PCKD, what other malformations are they likely to have?
Hepatic fibrosis
Proliferation of bile ducts
Medullary Sponge Kidney…is it a big deal?
Not usually…hematuria? Infection? Stones?
A child patient presents with polyuria and polydipsia…renal images show medullary cysts: what disease is it? What causes the symptoms?
Nephronophthisis-Uremic medullary cystic disease complex
Cortical tubular atrophy and interstitial fibrosis
A dialysis patient develops an asymptomatic cystic disease, what is the concern?
Renal cell carcinoma
Immunofluorescence shows a linear pattern: what is the disease? What does the antibody bind to?
Goodpasture’s
NC1 of the alpha 3 chain of type IV collagen
A patient was recently ill, and now histology shows subepithelial “camel” humps and has “smoky” urine: what is the disease? What should be done about it?
Acute Poststreptococcal Glomerulonephritis
Just let it work itself out
I doubt there’ll be questions on this, but: Goodpasture’s is which type of rapidly progressive (crescentic) GN?
Type I
I doubt there’ll be questions on this, but: immune complex mediated GN (PSGN, SLE, IgA nephropathy) are which type of rapidly progressive (crescentic) GN?
Type II
I doubt there’ll be questions on this, but: what type of rapidly progressive (crescentic) GN is Wegener’s (ANCA related)? What causes the crescents?
Type III
Proliferation of Bowman’s capsule–fibrin and macrophages
A patient has chronic proteinuria, thick capillary walls that look like cheerios, and have spikes on silver stain: what is the disease? What causes the damage? What is the treatment?
Membranous Glomerular Nephritis
Complement mediated
Treat underlying cause if there is one
A child comes in with eczema and massive proteinuria post respiratory infection: What is the disease? What is the treatment? What is seen on microscopy/IF/EM?
Minimal Change GN (most common GN in children)
Corticosteroids (disease is reversible)
Nothing
An adult, obese, HIV+, African-American heroin addict comes in with a nephrotic syndrome, HTN, and reduced GFR, the patient is given corticosteroids (maybe it’s MCGN) but it doesn’t improve: what is the disease? Where are deposits/what gets bigger? What is the usual outcome?
Focal Segmental Glomerulosclerosis (most common adult GN)
Mesangium
ESRD w/in 10yrs (50%) CRF w/in 2yrs (20%)
A patient comes in who has some kind of chronic immune complex disease (or one of many other diseases) and presents with nephrotic +/- nephritic symptoms, silver stains shows “tram-track”: what is the disease? Is it treatable?
Membranoproliferative GN
No…will even come back after transplant
A patient comes in with recurrent isolated hematuria, sensory hearing loss and ocular disturbances: what is the disease? What other diseases is it associated with? Is it treatable?
IgA nephropathy “Berger Disease” (most common GN worldwide)
Gluten enteropathy
Liver disease
Not really…50% recur post transplant
A younger deaf male patient with optical issues comes in with hematuria +/- proteinuria: what is the possible disease? What causes the disease? What is the usual outcome?
Alport syndrome (usually X-linked dominant)
Mutated COL4A5
Renal failure by age 20-50
A patient comes in and stains with everything and has “wire loop” lesions of thickened capillary walls: what is the disease?
SLE nephritis
A patient has had diabetes for over 12 years, what should be worried about with the kidney? What are some treatment options
Capillary BM thickening
Diffuse mesangial sclerosis
Nodular glomerulosclerosis (Kimmelstiel-Wilson disease; hyaline masses)
Long-term dialysis
Renal transplant
Pancreas transplant
A patient presents with Acute Renal Failure (decreased urine w/increased BUN): what is the most likely cause? What is the pathogenesis? What is the treatment?
Acute Tubular Necrosis
Ischemic/Nephrotoxic –> vasoconstriction
Salt and water overload…dialysis (patchiness allows recovery)
A patient presents with polyuria/nocturia and mild metabolic acidosis: what is a possible diagnosis?
Tubulointerstitial Nephritis
A multiple myeloma patient develops renal insufficiency: what caused the insufficiency?
Bence Jones + Tamm-Horsfall
A young black man presents with headache, nausea, vomiting, scotomas, a diastolic pressure greater than 130, proteinuria and hematuria: what is the diagnosis? What is the main gross feature? What is the main micro feature?
Malignant nephrosclerosis
Pinpoint petechial hemorrhages
Onion-skinning of vessels
A child presents with sudden onset of bleeding, oliguria, hematuria, MAHA, and possibly neurologic changes: What is the disease? What is the likely causative agent? What is the treatment?
Classic (childhood) HUS
Verocytotoxin producing E. coli
Dialysis (good recovery)
An older woman presents with fever, neurological symptoms, hemolytic anemia, and purpura: what is the disease? What is the cause? How is it treated?
Idiopathic Thrombotic Thrombocytopenic Purpura (TTP)
Defective von Willebrand protease
Plasma exchange and corticosteroids (<50% mortality)
