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Nephrology > Renal malignancies > Flashcards

Renal malignancies Flashcards

1
Q

ADPKD is a autosomal dominant mutation in _ or _

A

ADPKD is a autosomal dominant mutation in PKD1 (chromosome 16) or PKD2 (chromosome 4)

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2
Q

ADPKD is the development of cysts in the renal cortex and medulla (unilaterally/bilaterally)

A

ADPKD is the development of cysts in the renal cortex and medulla bilaterally
* Kidneys become enlarged

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3
Q

ADPKD will typically appear in (adults/kids)

A

ADPKD will typically appear in adults
* Kidneys will appear normal at birth

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4
Q

An adult patient presents with flank pain, hematuria, hypertension, UTI, and liver cysts; this may be a presentation of _

A

An adult patient presents with flank pain, hematuria, hypertension, UTI, and liver cysts; this may be a presentation of autosomal dominant polycystic kidney disease

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5
Q

ADPKD adults may also have associated findings including:

A

ADPKD adults may also have associated findings including:
* Liver cysts
* Pancreas cysts
* Intracranial aneurysms
* Mitral valve prolapse
* Diverticulosis

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6
Q

Autosomal dominant polycystic kidney disease will cause bilaterally enlarged kidneys with multiple cysts of (equal/varying) sizes

A

Autosomal dominant polycystic kidney disease will cause bilaterally enlarged kidneys with multiple cysts of varying sizes

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7
Q

_ are administered to prevent the progression of ADPKD to ESRD

A

ACE inhibitors, ARBs are administered to prevent the progression of ADPKD to ESRD

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8
Q

_ medication is used for ADPKD to slow the growth of renal cysts

A

Tolvaptan is used for ADPKD to slow the growth of renal cysts

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9
Q

Tolvaptan is used to slow the growth of renal cysts; it works by _ mechanism of action

A

Tolvaptan is used to slow the growth of renal cysts; it works by selective antagonism of vasopressin 2 receptors, meaning it inhibits ADH and decreases water reabsorption

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10
Q

ARPKD is an autosomal recessive mutation in _ gene

A

ARPKD is an autosomal recessive mutation in PKHD1 (chromosome 6)

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11
Q

ARPKD presents in (childhood/adulthood)

A

ARPKD presents in childhood

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12
Q

(ARPKD/ ADPKD) is associated with hepatic fibrosis

A

ARPKD is associated with hepatic fibrosis
* Can cause portal hypertension and possible liver failure

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13
Q

Autosomal recessive PKD is sometimes associated with _ in utero

A

Autosomal recessive PKD is sometimes associated with Potter sequence in utero
* Pulmonary hypoplasia, oligohydramnios, twisted face, twisted skin, extremity deficits, renal failure

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14
Q

ARPKD presents as bilaterally enlarged kidneys with cysts of (equal/varying) size on ultrasound

A

ARPKD presents as bilaterally enlarged kidneys with cysts of equal size on ultrasound

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15
Q

Both types of PKD are treated with _ for HTN and _ for cystic growth

A

Both types of PKD are treated with ACEi, ARBs for HTN and Tolvaptan for cystic growth

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16
Q

The most common renal cancer in adults is _

A

The most common renal cancer in adults is renal cell carcinoma

17
Q

Clear cell renal cell carcinoma originates in _ cells in _ region

A

Clear cell renal cell carcinoma originates in epithelial cells of the PCT

18
Q

(Sporatic/inherited) mutations that induce renal cell carcinoma are more likely to be bilateral

A

Inherited mutations that induce renal cell carcinoma are more likely to be bilateral
* Also more likely to present at younger age

19
Q

Inherited renal cell carcinoma is associated with _ gene deletion on chromosome _

A

Inherited renal cell carcinoma is associated with Von-Hippel-Lindau (VHL) gene deletion on chromosome 3
* RCC, VHL, PCT, chromosome 3

20
Q

The mutation in the VHL gene on chromosome 3 which can cause RCC is autosomal (D/R)

A

The mutation in the VHL gene on chromosome 3 which can cause RCC is autosomal dominant

21
Q

The classic triad of renal cell carcinoma (RCC) includes:

A

The classic triad of renal cell carcinoma (RCC) includes:
1. Flank pain
2. Palpable mass
3. Hematuria

Note that not all of these have to be present

22
Q

Four paraneoplastic syndromes associated with RCC:

A

Four paraneoplastic syndromes associated with RCC: PEAR
1. PTHrp: hypercalcemia
2. EPO: polycythemia
3. ACTH: cushings
4. Renin: hypertension

23
Q

Renal cell carcinoma commonly metastasizes to the _ and _

A

Renal cell carcinoma commonly metastasizes to the lungs and bones

24
Q

_ imaging should be used for renal cell carcinoma

A

Abdominal CT should be used for renal cell carcinoma

25
Q

Histopathology of RCC will show polygonal clear cells with high content of _ and _

A

Histopathology of RCC will show polygonal clear cells with high content of carbohydrates and lipids
* This explains the golden-yellow color that is seen macroscopically

26
Q

Does renal cell carcinoma have a good prognosis?

A

No, RCC has a poor response to radiation and chemo

27
Q

_ are benign epithelial tumors that originate from intercalated cells

A

Renal oncocytomas are benign epithelial tumors that originate from intercalated cells

28
Q

Renal oncocytomas originate in _ cells in _ region of the nephron

A

Renal oncocytomas originate in intercalated cells in the collecting duct
* They are benign epithelial tumors

29
Q

Renal oncocytomas may present with flank pain, abdominal mass, and _ hematuria

A

Renal oncocytomas may present with flank pain, abdominal mass, and painless hematuria

30
Q

Macroscopic pathology of a renal oncocytoma will reveal _

A

Macroscopic pathology of a renal oncocytoma will reveal a well circumscribed brown tumor with central scarring

31
Q

Microscopic pathology of renal oncocytoma will reveal _

A

Microscopic pathology of renal oncocytoma will reveal eosinophilic cells without perinuclear clearing + abunant mitochondria

32
Q

The most common renal malignancy in children is _

A

The most common renal malignancy in children is nephroblastoma (Wilms tumor)

33
Q

Wilms tumors classically present around ages _ with presence of a large, palpable unilateral flank mass and abdominal pain

A

Wilms tumors classically present around age 2-4 with presence of a large, palpable unilateral flank mass and abdominal pain
* The mass typically will not cross the midline

34
Q

Wilms tumor involves a cell type that plays a role in kidney development called _ cells

A

Wilms tumor involves a cell type that plays a role in kidney development called metanephric blastemal cells

35
Q

Wilms tumors will have a relatively (good/poor) prognosis

A

Wilms tumors will have a relatively good prognosis

36
Q

Wilms tumors can be due to a loss of function mutation of tumor suppressor gene _ or _

A

Wilms tumors can be due to a loss of function mutation of tumor suppressor gene WT1 or WT2

Nephrology (16 decks)

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