Robbins Q

Question 1

71 y/o man w/ 3 day oliguria. BP 85/60, peripheral edema, diffuse rales, Cr 3.3, BUN 62, FENa less than 1%. What is it?

Answer 1

Dilated cardiomyopathy = prerenal azotemia = BUN/Cr over 20, low FENa, high urine specific gravity

Question 2

What type of hypersensitivity is Goodpasture’s syndrome?

Answer 2

Type 2

Question 3

What kind of hypersensitivity produces a granular pattern on immunofluorescence?

Answer 3

Type 3 = immune complex deposition

Eg: Membranous nephropathy, Membranoproliferative nephropathy

Question 4

Renal biopsy: hypercellular glomeruli w/ lobulation and a double-contour appearance to split basement membranes adjacent to subendothelial immune complex.
What cell type is likely proliferated?

Answer 4

Mesangial cells – Membranoproliferative nephropathy Type 1 = subendothelial deposits

Question 5

There is over 3.5g protein in 24hr urine. Dysfunction of what cell is responsible for the proteinuria?

Answer 5

Podocytes

Question 6

What is the pattern of occurence (patient population wise) for Goodpasture syndrome?

Answer 6

Bimodal = younger and older men

Question 7

Sinus problems + cANCA = ___

Answer 7

Granulomatosis with polyangiitis

Question 8

What is the only nephrotic syndrome that responds o corticosteroids and what is the pathogenic mechanism?

Answer 8

Minimal change disease (MCD) – cytokine-mediated visceral epithelial cell injury

Question 9

Hep B (HBV) is associated with what nephritic/nephrotic syndrome(s)

Answer 9

Membranoproliferative nephropathy Type 1

Membranous nephropathy

Question 10

6y/o girl, increasingly lethargic over 2wks, puffiness around eyes, Cr 0.7, BUN 12, Cholesterol 217, Urine pH 6.5, sp. gravity 1.011, 4+ proteinuria, lipiduria, no blood or glucose in urine, 24hr urine protein 3.8g.
What disease? What treatment?

Answer 10

MCD, glucocorticoid

Question 11

Corticosteroid-resistant hematuria and proteinuria leading to HTN and renal failure caused by slit diaphragm dysfunction.
What disease?

Answer 11

FSGS

Question 12

What is the most common inheritance pattern for Alport syndrome?

Answer 12

X-linked = 85% of cases

Question 13

Diffuse proliferative glomerulonephritis associated with SLE has what kind of deposits at what location?

Answer 13

Granular immune deposits of IgG and C1q in capillary loops and mesangium

“wire looping” of capillaries

Question 14

Why do patients with acute tubular injury have polyuria for weeks even after treatment?

Answer 14

Takes weeks for tubular epithelial cells to regenerate. In the mean time, they have less ability to reabsorb filtrate.

Question 15

What to suspect when you see WBC and RBC casts in a sexy lady?

Answer 15

Pyelonephritis from ascending UTI

Question 16

29 y/o woman w/ fever sore throat for 3 days is treated w/ ampicillin and recovers in 7 days. 2 weeks later, she develops fever 37.7C, erythematous rash, slight oliguria, urine pH 6, 1+ proteinuria, 1+ hematuria, no glucose or ketones, WBCs and RBCs including eosinophils in urine, no casts or crystals.
What caused this?

Answer 16

Acute drug-induced interstitial nephritis – AMPICILLIN!!!

Post-strep glomerulonephritis w/ immune complexes would not have rash or eosinophils

Question 17

A guy takes phenacetin, aspirin and acetominophen for fun a lot for 20 years. What’s he at risk for?

Answer 17

Renal papillary necrosis = chronic result of analgesic nephropathy (acute interstitial nephritis)

Question 18

Patient with 160/110 from renal artery stenosis is treated with an CEI. He takes an ibuprofen once and becomes oliguric. Why did the ibuprofen cause that?

Answer 18

Renal artery stenosis = low GFR
ACEI = efferent arteriole constriction
Physiologic prostaglandins = afferent arteriole dilation
NSAIDS = inhibit prostaglandins –> lower GFR

Question 19

5 y/o girl eats a lot at a BBQa and 4 days later develops: abdom pain, diarrhea, 90/50, occult blood in stool, Cr 2.2, BUN 20, Hb 10.8, HCT 32.4%, Plt 64k, schistocytes, D-dimer elevated, urine pH 6, 2+hematuria. Renal biopsy shows small thrombi within glomerular capillary loops.
What is it?

Answer 19

E. Coli infection (O157:H7) –> HUS –> acute renal failure

Most common cause of acute renal failure in children

Question 20

Adult autosomal dominant polycystic kidney disease often has a mutation that causes a defect in this protein component of cilia

Answer 20

Polycystin-1 – PKD1 (most)
Or
Polycystin-2 – PKD2 (15%)

Question 21

Aryl hydrocarbon receptor-interacting protein (AIP) gene mutations is associated with what disease?

Answer 21

GH-producing adenomas in young persons in familial cases –> gigantism or acromegaly

Question 22

Cyclin D1 mutations are associated with what endocrine disease?

Answer 22

Parathyroid adenomas

Question 23

Cystic dilation of inner medullary and papillary collecting ducts.
Classic presentation: renal stones, infection, or recurrent hematuria in 3rd of 4th decade.
What disease?

Answer 23

Medullary Sponge Kidney

Question 24

Autosomal recessive mutation in NPHP1 to NPHP11 genes.

MCC of end-stage renal disease in children and adolescents

Answer 24

Nephronophthisis

Question 25

What bugs can cause Ammonium magnesium phosphate stones?

Answer 25

Urease Positive: Proteus mirabilis Klebsiella S. saprophyticus

Question 26

What 2 kinds of stones can cause staghron calculi?

Answer 26

Ammonium magnesium Phosphate | Cystine

Question 27

``` 39 y/o non pregnant woman: amenorrhea for 6 months, galactorrhea, BMI 28, normal 2ndary sex characteristics, normal GH stimulation test, MRI: fluid density within a normal-sized sella turcica A) Craniopharyngioma B) Empty sella syndrome C) Hereditary hemochromatosis D) Prader-Willi syndrome E) Prolactinoma F) Sheehan syndrome ```

Answer 27

B) Empty sella syndrome = herniation of arachnoid through diaphragma sellae --> "Stalk section" effect --> loss of prolactin inhibition --> hyperprolactinemia MRI ruled out prolactinoma

Question 28

Family history of bilateral renal masses is associated with what gene?

Answer 28

MET gene mutation --> papillary renal cell carcinoma (2nd most common carcinoma of kidney)

Question 29

``` 39 y/o non pregnant woman: amenorrhea for 6 months, galactorrhea, BMI 28, normal 2ndary sex characteristics, normal GH stimulation test, MRI: fluid density within a normal-sized sella turcica A) Craniopharyngioma B) Empty sella syndrome C) Hereditary hemochromatosis D) Prader-Willi syndrome E) Prolactinoma F) Sheehan syndrome ```

Answer 29

B) Empty sella syndrome = herniation of arachnoid through diaphragma sellae --> "Stalk section" effect --> loss of prolactin inhibition --> hyperprolactinemia MRI ruled out prolactinoma

Question 30

Failure to thrive since infancy, short, coarse facial features, protruding tongue, umbilical hernia. What disease?

Answer 30

Cretinism = congenital hypothyroidism

Question 31

45 y/o woman living in the mountains has gradual and painless enlargment of thyroid gland, normal T4, slightly increased TSH. What is it?

Answer 31

Diffuse nontoxic goiter = MCC is iodine deficiency

Question 32

Tall Columnar epithelium w/ papillary infolding and scalloping of colloid. What disease?

Answer 32

Graves disease = autoantibofdies binding to TSH receptor to mimic TSH

Question 33

45 y/o woman living in the mountains has gradual and painless enlargment of thyroid gland, normal T4, slightly increased TSH. What is it?

Answer 33

Diffuse nontoxic goiter = MCC is iodine deficiency

Question 34

Chronic renal failure can lead to what kind of parathyroid dysfunction?

Answer 34

Secondary hyperparathyroidism

Question 35

Amyloid stroma is common in what type of thyroid tumor?

Answer 35

Medullary carcinoma -- look for Calcitonin

Question 36

Chronic renal failure can lead to what kind of parathyroid dysfunction?

Answer 36

Secondary hyperparathyroidism

Question 37

You see amyloid deposition in islets in Type [1/2] Diabetes

Answer 37

Type 2

Question 38

What is the mechanism for diabetic neuropathy?

Answer 38

Metabolism of Glucose--> sorbitol --> fructose uses NADPH--> prevents regeneration of glutathione-->increased oxidative stress--> neuropathy Myelin cells don't need insulin for glucose uptake

Question 39

What is Conn syndrome?

Answer 39

Adrenal cortical adenoma that secretes aldosterone --> hyperaldosteronism --> reflex low renin

Question 40

What is the mechanism for diabetic neuropathy?

Answer 40

Metabolism of Glucose--> sorbitol --> fructose uses NADPH--> prevents regeneration of glutathione-->increased oxidative stress--> neuropathy Myelin cells dont need insulin for glucose uptake

Question 41

What is Conn syndrome?

Answer 41

Adrenal cortical adenoma that secretes aldosterone --> hyperaldosteronism --> reflex low renin

Question 42

What do most adrenal nodules do?

Answer 42

NOTHING-- most are non-functional incidental findings Some can produce hyperaldosteronism or Cushing's syndrome

Question 43

What 3 neoplasms in MEN2A

Answer 43

Medullary Carcinoma of Thyroid Pheochromocytoma Parathyroid adenoma

Question 44

What 3 neoplasms in MEN1

Answer 44

Pancreatic tumors Pituitary adenomas Parathyroid adenomas