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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

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2
Q

Fused kidneys are ALWAYS ________.

A

ectopic (abnormal place of position)

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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)
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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

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5
Q

What can Horseshoe kidney be associated with?

A

may be associated with extrarenal malformations

may have normal kidney function

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6
Q

What genetic defect is Horseshoe kidney associated with?

A

trisomy 18

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7
Q

What happens in Unilateral Renal Agenesis?

A

Asymptomatic: compensatory hyperplasia of contralateral kidney, may undergo GLOMERULOSCLEROSIS

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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

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9
Q

What are symptoms of the Potter Sequence

A

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

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10
Q

Describe Renal Hypoplasia

A

Reduced number and size of renal pyramids

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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
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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
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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

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14
Q

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

A

Multicystic renal dysplasia

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15
Q

What are the 2 types of Acquired Cystic Renal Diseases

A
  1. Dialysis-associated

2. Simple (localized) cysts

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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)

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