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Question 1

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?

Answer 1

Autosomal Dominant (adult) PCKD

PKD1 (cell-cell or cell-matrix interactions)

Berry aneurysm

Question 2

A patient has autosomal recessive PCKD, what other malformations are they likely to have?

Answer 2

Hepatic fibrosis

Proliferation of bile ducts

Question 3

Medullary Sponge Kidney…is it a big deal?

Answer 3

Not usually…hematuria? Infection? Stones?

Question 4

A child patient presents with polyuria and polydipsia…renal images show medullary cysts: what disease is it? What causes the symptoms?

Answer 4

Nephronophthisis-Uremic medullary cystic disease complex

Cortical tubular atrophy and interstitial fibrosis

Question 5

A dialysis patient develops an asymptomatic cystic disease, what is the concern?

Answer 5

Renal cell carcinoma

Question 6

Immunofluorescence shows a linear pattern: what is the disease? What does the antibody bind to?

Answer 6

Goodpasture’s

NC1 of the alpha 3 chain of type IV collagen

Question 7

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?

Answer 7

Acute Poststreptococcal Glomerulonephritis

Just let it work itself out

Question 8

I doubt there’ll be questions on this, but: Goodpasture’s is which type of rapidly progressive (crescentic) GN?

Answer 8

Type I

Question 9

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?

Answer 9

Type II

Question 10

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?

Answer 10

Type III

Proliferation of Bowman’s capsule–fibrin and macrophages

Question 11

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?

Answer 11

Membranous Glomerular Nephritis

Complement mediated

Treat underlying cause if there is one

Question 12

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?

Answer 12

Minimal Change GN (most common GN in children)

Corticosteroids (disease is reversible)

Nothing

Question 13

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?

Answer 13

Focal Segmental Glomerulosclerosis (most common adult GN)

Mesangium

ESRD w/in 10yrs (50%) CRF w/in 2yrs (20%)

Question 14

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?

Answer 14

Membranoproliferative GN

No…will even come back after transplant

Question 15

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?

Answer 15

IgA nephropathy “Berger Disease” (most common GN worldwide)

Gluten enteropathy
Liver disease

Not really…50% recur post transplant

Question 16

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?

Answer 16

Alport syndrome (usually X-linked dominant)

Mutated COL4A5

Renal failure by age 20-50

Question 17

A patient comes in and stains with everything and has “wire loop” lesions of thickened capillary walls: what is the disease?

Answer 17

SLE nephritis

Question 18

A patient has had diabetes for over 12 years, what should be worried about with the kidney? What are some treatment options

Answer 18

Capillary BM thickening
Diffuse mesangial sclerosis
Nodular glomerulosclerosis (Kimmelstiel-Wilson disease; hyaline masses)

Long-term dialysis
Renal transplant
Pancreas transplant

Question 19

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?

Answer 19

Acute Tubular Necrosis

Ischemic/Nephrotoxic –> vasoconstriction

Salt and water overload…dialysis (patchiness allows recovery)

Question 20

A patient presents with polyuria/nocturia and mild metabolic acidosis: what is a possible diagnosis?

Answer 20

Tubulointerstitial Nephritis

Question 21

A multiple myeloma patient develops renal insufficiency: what caused the insufficiency?

Answer 21

Bence Jones + Tamm-Horsfall

Question 22

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?

Answer 22

Malignant nephrosclerosis

Pinpoint petechial hemorrhages

Onion-skinning of vessels

Question 23

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?

Answer 23

Classic (childhood) HUS

Verocytotoxin producing E. coli

Dialysis (good recovery)

Question 24

An older woman presents with fever, neurological symptoms, hemolytic anemia, and purpura: what is the disease? What is the cause? How is it treated?

Answer 24

Idiopathic Thrombotic Thrombocytopenic Purpura (TTP)

Defective von Willebrand protease

Plasma exchange and corticosteroids (<50% mortality)