Robbins Q Flashcards
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?
Dilated cardiomyopathy = prerenal azotemia = BUN/Cr over 20, low FENa, high urine specific gravity
What type of hypersensitivity is Goodpasture’s syndrome?
Type 2
What kind of hypersensitivity produces a granular pattern on immunofluorescence?
Type 3 = immune complex deposition
Eg: Membranous nephropathy, Membranoproliferative nephropathy
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?
Mesangial cells – Membranoproliferative nephropathy Type 1 = subendothelial deposits
There is over 3.5g protein in 24hr urine. Dysfunction of what cell is responsible for the proteinuria?
Podocytes
What is the pattern of occurence (patient population wise) for Goodpasture syndrome?
Bimodal = younger and older men
Sinus problems + cANCA = ___
Granulomatosis with polyangiitis
What is the only nephrotic syndrome that responds o corticosteroids and what is the pathogenic mechanism?
Minimal change disease (MCD) – cytokine-mediated visceral epithelial cell injury
Hep B (HBV) is associated with what nephritic/nephrotic syndrome(s)
Membranoproliferative nephropathy Type 1
Membranous nephropathy
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?
MCD, glucocorticoid
Corticosteroid-resistant hematuria and proteinuria leading to HTN and renal failure caused by slit diaphragm dysfunction.
What disease?
FSGS
What is the most common inheritance pattern for Alport syndrome?
X-linked = 85% of cases
Diffuse proliferative glomerulonephritis associated with SLE has what kind of deposits at what location?
Granular immune deposits of IgG and C1q in capillary loops and mesangium
“wire looping” of capillaries
Why do patients with acute tubular injury have polyuria for weeks even after treatment?
Takes weeks for tubular epithelial cells to regenerate. In the mean time, they have less ability to reabsorb filtrate.
What to suspect when you see WBC and RBC casts in a sexy lady?
Pyelonephritis from ascending UTI
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?
Acute drug-induced interstitial nephritis – AMPICILLIN!!!
Post-strep glomerulonephritis w/ immune complexes would not have rash or eosinophils
A guy takes phenacetin, aspirin and acetominophen for fun a lot for 20 years. What’s he at risk for?
Renal papillary necrosis = chronic result of analgesic nephropathy (acute interstitial nephritis)
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?
Renal artery stenosis = low GFR
ACEI = efferent arteriole constriction
Physiologic prostaglandins = afferent arteriole dilation
NSAIDS = inhibit prostaglandins –> lower GFR
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?
E. Coli infection (O157:H7) –> HUS –> acute renal failure
Most common cause of acute renal failure in children
Adult autosomal dominant polycystic kidney disease often has a mutation that causes a defect in this protein component of cilia
Polycystin-1 – PKD1 (most)
Or
Polycystin-2 – PKD2 (15%)
Aryl hydrocarbon receptor-interacting protein (AIP) gene mutations is associated with what disease?
GH-producing adenomas in young persons in familial cases –> gigantism or acromegaly
Cyclin D1 mutations are associated with what endocrine disease?
Parathyroid adenomas
Cystic dilation of inner medullary and papillary collecting ducts.
Classic presentation: renal stones, infection, or recurrent hematuria in 3rd of 4th decade.
What disease?
Medullary Sponge Kidney
Autosomal recessive mutation in NPHP1 to NPHP11 genes.
MCC of end-stage renal disease in children and adolescents
Nephronophthisis
What bugs can cause Ammonium magnesium phosphate stones?
Urease Positive:
Proteus mirabilis
Klebsiella
S. saprophyticus
What 2 kinds of stones can cause staghron calculi?
Ammonium magnesium Phosphate
Cystine
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
B) Empty sella syndrome = herniation of arachnoid through diaphragma sellae –> “Stalk section” effect –> loss of prolactin inhibition –> hyperprolactinemia
MRI ruled out prolactinoma
Family history of bilateral renal masses is associated with what gene?
MET gene mutation –> papillary renal cell carcinoma (2nd most common carcinoma of kidney)
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
B) Empty sella syndrome = herniation of arachnoid through diaphragma sellae –> “Stalk section” effect –> loss of prolactin inhibition –> hyperprolactinemia
MRI ruled out prolactinoma
Failure to thrive since infancy, short, coarse facial features, protruding tongue, umbilical hernia.
What disease?
Cretinism = congenital hypothyroidism
45 y/o woman living in the mountains has gradual and painless enlargment of thyroid gland, normal T4, slightly increased TSH.
What is it?
Diffuse nontoxic goiter = MCC is iodine deficiency
Tall Columnar epithelium w/ papillary infolding and scalloping of colloid.
What disease?
Graves disease = autoantibofdies binding to TSH receptor to mimic TSH
45 y/o woman living in the mountains has gradual and painless enlargment of thyroid gland, normal T4, slightly increased TSH.
What is it?
Diffuse nontoxic goiter = MCC is iodine deficiency
Chronic renal failure can lead to what kind of parathyroid dysfunction?
Secondary hyperparathyroidism
Amyloid stroma is common in what type of thyroid tumor?
Medullary carcinoma – look for Calcitonin
Chronic renal failure can lead to what kind of parathyroid dysfunction?
Secondary hyperparathyroidism
You see amyloid deposition in islets in Type [1/2] Diabetes
Type 2
What is the mechanism for diabetic neuropathy?
Metabolism of Glucose–> sorbitol –> fructose uses NADPH–> prevents regeneration of glutathione–>increased oxidative stress–> neuropathy
Myelin cells don’t need insulin for glucose uptake
What is Conn syndrome?
Adrenal cortical adenoma that secretes aldosterone –> hyperaldosteronism –> reflex low renin
What is the mechanism for diabetic neuropathy?
Metabolism of Glucose–> sorbitol –> fructose uses NADPH–> prevents regeneration of glutathione–>increased oxidative stress–> neuropathy
Myelin cells dont need insulin for glucose uptake
What is Conn syndrome?
Adrenal cortical adenoma that secretes aldosterone –> hyperaldosteronism –> reflex low renin
What do most adrenal nodules do?
NOTHING– most are non-functional incidental findings
Some can produce hyperaldosteronism or Cushing’s syndrome
What 3 neoplasms in MEN2A
Medullary Carcinoma of Thyroid
Pheochromocytoma
Parathyroid adenoma
What 3 neoplasms in MEN1
Pancreatic tumors
Pituitary adenomas
Parathyroid adenomas
