Misc

Question 0

Juvenile hyperuricemic nephropathy

Answer 0

AD Genetic defects in uromodulin gene (Tamm horsfall protein)
Hyperuricemia gout slowly progressive bland renal failure
Low FeUrea
Appears in 3-5th decade progresses over 10 years
Small kidneys
Medullary cysts at late stages but difficult to detect
Low proteinuria<1g
Bx may show interstitial nephritis
Distribution of Tamm horsfall protein is altered

Question 1

Cholesterol emboli

Answer 1

Fever abd pain and GI
4 days after cath
Blue toes
Eosinophilia low complement
Possibly active sediment

Question 2

Hemolytic anemia on dialysis

Answer 2

Chloramine
Copper
Hydrogen peroxide
Zinc

Question 3

Methemoglobinemia on dialysis

Answer 3

Chloramine

Nitrates

Question 4

Causes of hemolysis on hemodialysis

Answer 4

Autoimmune
Congenital thal hgn S
Mechanical- needles catheter roller pump
Drugs CNI (prior txp), those w strong oxidizing potential
Dialysate overheated, hypotonic and water impurities

Question 5

Type a dialyzer reaction

Answer 5

IgE mediated
Within first 30min, usually first 10
Most common w cellulose membranes
Can also be directed to ethylene oxide
Heparin bacterial peptide formaldehyde 
Itching urticaria wheezing headache back pain chest pain dyspnea
STOP dialysis EPI steroids histamine

Question 6

Type B dialyzer reaction

Answer 6

3-5% treatments w cellulose membranes
Complement mediated
Within first 30 usually but can occur anytime
Same sx but no allergic components- still have back pain chest pain dyspnea nause
Can continue to dialyze. Supportive tx
Unbound hydroxyl groups activate C5a w pulm deposits-> chemo taxis and activation of neutrophils

Question 7

Steroid dependent(relapses whenever tapered off) adult mcd treat with what?

Answer 7

Do well w cyc CSA mmf tk

Question 8

Renal disease associated w rheumatoid arthritis

Answer 8

Membranous
Mesangial proliferative GN w Iga deposits
Diffuse proliferative GN
Amyloidosis AA

Question 9

Renal dz due to rheumatoid rx

Answer 9

Gold (mcd, mn, ATN)
Penicillamine (mn, crescentic GN, mcd)
NSAIDs (AIN, mn, mcd, ATN)
CSA: chronic vasculopathy, tubulopathy
Mtx- ATN/crystal induced ARF
Aza:AIN

Question 10

Zebra bodies on EM

Vacillated visceral EPI cells

Answer 10

Fabry’s dz

Question 11

Renal mani of sickle

Answer 11

Hematuria painless micro or macroscopic due to papillary infarcts
Renal infarction and papillary can present w nause vomiting abd or flank pain fever htn
Hyposthenuria Nocturia polyuria early is disease
RTA distal incomplete
Abnormal proximal fxn high phos
Uti
Renal medullary carcinoma

Question 12

Mutation in alanine glyoxylate aminotransferase

Answer 12

Primary hyperoxaluria

Question 13

Podocin mutation

Answer 13

Fsgs

Question 14

Nephrin

Answer 14

Fsgs

Question 15

Alpha actinin 4

Answer 15

Fsgs

AD

Question 16

TRPC6 gene mutation is associated with

Answer 16

Fsgs AD

Question 17

Phenotype a caused by uromodulin UMOD mutations

Answer 17

Fam juvenile hyperuricemia nephropathy
Glomeruli cystic kid eye disease w hyperuricemia and isosthenuria
Medullary cystic kidney disease mckd2

Question 18

Recurrent pancreatitis
Lymphadenopathy
Irregular hypo and hyperechoic lesions in enlarged kidneys by US

Answer 18

IgG4 related disease

W chronic interstitial nephritis

Question 19

V1a receptors found where and do what

Answer 19

Vasc smooth muscle- constriction
Plts- plt agg
Hepatocytes- glycogenolysis
Baroreceptors- baroreflex-BP control
Cadiomyocytes-hypertrophic response

Question 20

V1b receptors

Answer 20

Ant pit-acth and b endorphin release

Question 21

V2 receptors

Answer 21

Renal principal cells and thick asc limb- water and NaCl absorption