33: Renal Pathology 3

Question 1

what happens with complete bilateral obstruction of the ureters

Answer 1

oliguria/anuria -> incompatible with survival unless fixed

Question 2

what happens after relief of complete urinary tract obstruction

Answer 2

post-obstructive diuresis - massive amounts of urine rich in NaCl

Question 3

common bacteria that cause magnesium ammonium phosphate stones (urea splitting bacteria)

Answer 3

proteus mirabilus**, pseudomonas, klebsiella, some staph and enterococci

Question 4

examples of overproduction of uric acid

Answer 4

dietary purine consumption (meat, beer), endogenous synthesis from malignancy, HGPRT deficiency, PRPS deficiency, glycogen storage diseases

Question 5

major cause of increased uric acid - overproduction or underexcretion?

Answer 5

underexcretion (90%)

Question 6

four major syndromes that involve RCC

Answer 6

1. Von Hippel Lindau
2. Hereditary leiomyomatosis
3. hereditary papillary carcionma
4. Birt-Hogg-Dube

Question 7

mutations in hereditary leiomyomatosis vs hereditary papillary carcinoma

Answer 7

1. leiomyomatosis: FH gene (fumarate hydratase)

2. papillary carcinoma: MET gene

Question 8

hereditary pattern for hereditary leiomyomatosis vs hereditary papillary carcinoma

Answer 8

both AD

Question 9

presentation of hereditary leiomyomatosis vs hereditary papillary carcinoma

Answer 9

1. leiomyomatosis: cutaneous and uterine leiomyomata, aggressive papillary carcinoma
2. papillary carcinoma: multiple bilateral tumors with papillary histo

Question 10

Birt-Hogg-Dube mutation and inheritance pattern

Answer 10

AD, mutation in BHD gene (produces folliculin)

Question 11

presentation of Birt-Hogg-Dube

Answer 11

1. cysts in the lungs and skin
2. renal tumors of wide variety
3. hemangioblastmoas of the CNS
4. pheochromocytomas

Question 12

RCC causes abnormal hormone production - name some things it can cause

Answer 12

1. polycythemia, anemia, eosinophilia
2. hypercalcemia
3. HTN
4. hepatic dysfunction
5. feminization / masculinization
6. Cushing
7. amyloid

Question 13

gene thats mutated in Von-Hippel Lindau + where its located

Answer 13

VHL gene on chromosome 3 short arm

Question 14

MC primary renal tumor of childhood

Answer 14

Wilm’s tumor

Question 15

WAGI/R presentation

Answer 15

1. Wilms tumor
2. Aniridia
3. Genitourinary anomalies
4. Intellectual disability

Question 16

Denys Drash syndrome presentation

Answer 16

gonadal dysgenesis, early onset nephropathy, Wilms tumor, gonadoblastomas

Question 17

Beckwith-Weidemann Syndrome

Answer 17

organomegaly, macroglossia, hemihypertrophy, omphalocele, enlarged adrenal cortex

Question 18

are metz common in kidney?

Answer 18

no - only some from melanoma, leukemia, and lymphomas