Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Nephrology > Renal Developmental Pathology > Flashcards

Renal Developmental Pathology Flashcards

1
Q

What is the incidence of GU tract maldevelopment? what does is commonly cause?

A

10%

common cause of ESRD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Fused kidneys are ALWAYS ________.

A

ectopic (abnormal place of position)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are 3 characteristics of Horseshoe Kidney?

A
  1. Fusion of upper or lower poles of kidney
  2. Typically located at midline
  3. Lie anterior to great vessels (if above bifurcation for common iliac)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What can happen to Ureters with a Horseshoe kidney and what happens to the kidney as a result?

A

Ureters may become kinked (@ inferior pole) - may result in hydronephrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What can Horseshoe kidney be associated with?

A

may be associated with extrarenal malformations

may have normal kidney function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What genetic defect is Horseshoe kidney associated with?

A

trisomy 18

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What happens in Unilateral Renal Agenesis?

A

Asymptomatic: compensatory hyperplasia of contralateral kidney, may undergo GLOMERULOSCLEROSIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What happens in Bilateral Renal Agenesis? What birth defect is it associated with?

A

Incompatible with life

Associated with oligohydramnios (little amniotic fluid) + POTTER SEQUENCE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are symptoms of the Potter Sequence

A

flattened nose, low-set ears, recessed chin, limb contractures, pulmonary hypoplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Describe Renal Hypoplasia

A

Reduced number and size of renal pyramids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Where is an Ectopic kidney usually located and is it associated with?

A 
often located in pelvis
associated w:
urine outflow obstruction
hydronephrosis
pyelonephritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the 3 categories of Cystic Renal Diseases? and what can it result in?

A
  1. Hereditary
  2. Developmental (sporadic)
  3. Acquired
    can result in CKD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the 2 types of hereditary cystic renal diseases? What are the populations that they occur in?

A
  1. Autosomal Dominant PKD - Adult

2. Autosomal Recessive PKD-children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What kind of Cystic renal Disease is Developmental (Sporadic)?

A

Multicystic renal dysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the 2 types of Acquired Cystic Renal Diseases

A
  1. Dialysis-associated

2. Simple (localized) cysts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is a cyst?

A

membrane (epithelial) bound sac of fluid - renal TUBULAR epithelium

17
Q

What are the gross differences between ADPKD and ARPKD?

A

ADPKD - kidney is larger, cobblestone appearance

ARPKD - cysts are thin/long, oriented as spokes around kidney. Cysts within CORTEX+MEDULLA

18
Q

What are the two genetic defects in ADPKD? which one is more prominent/has worse prognosis?

A
  1. PKD1 (16p13.3) - polycystin-1
    - –80% of cases, worse prognosis
  2. PKD2 (4q21) - polycystin-2
19
Q

What does PKD1 gene affect?

A

renal tubular epithelial cells; cell-cell, cell-matrix interactions

20
Q

What does PKD2 gene affect?

A

Renal tubular epithelial cells, Ca2+ channels

21
Q

What is the initial presentation of ADPKD

A
  1. renal insufficiency - slowly progressive
    (hematuria, pain, sensation of abdominal/pelvic mass)
  2. Extrarenal congenital malformations (liver cysts, pancreatic cysts, BERRY ANEURYSM)
22
Q

What genetic mutation is associated with ARPKD? what does it code for?

A

PKHD1 (6p21-23) - codes for fibrocystin

23
Q

What is the progression of PKHD1 mutation

A

PKHD1 codes for fibrocystin
congenital hepatic fibrosis
=portal hypertension, splenomegaly

24
Q

What are characteristics of Multicystic Renal Dysplasia

A
  1. abnormal lobules of IMMATURE metanpehric structures

2. Disorganized mesenchymal tissue (immature cartilage)

25
Q

What is the gross appearance of Multicystic Renal Dysplasia?

A

Enlarged kidney with large, irregular cysts and compression of residual parenchyma - barely looks like a kidney anymore

26
Q

What are the cysts like in Multicystic Renal dysplasia and what is the histology?

A

Cysts retain a single lobule

histology - no glomeruli = nonfxnal kidney

27
Q

What does Dialysis-associated Acquired Cystic Disease look like?

A

Multiple, variabled sized
THIN-WALLED
UNILOCULATED cysts in CORTEX

28
Q

What is Dialysis-associated Acquired Cystic Disease associated with?

A 
papillary neoplasia (adenoma, RCC)
hematuria
29
Q

Describe Simple cysts

A

most common kidney cyst
Cortical, smooth, thin-walled, UNILOCULATED
hemorrhagic

30
Q

What is the clinical triad associated with Prune Belly Syndrome?

A
  1. Deficiency of abdominal musculature (loose ab wall)
  2. Urinary tract anomalies
  3. Bilateral cryptorchidism
31
Q

What is the etiology of Prune Belly Syndrome?

A

Abnormal mesodermal development

32
Q

What are the Prune Belly Syndrome clinical features of the kidney, collecting system, GI tract?

A
  1. kidney - dysplastic changes
  2. collecting system - ureteral elongation/dilation + urinary bladder dilatation
    - –obstruction = recurrent infections(CKD->ESRD)
  3. GI tract - malrotation

Nephrology (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions