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Renal Exam > UG devo (both lectures) > Flashcards

UG devo (both lectures) Flashcards

1
Q

What is the precursor for structures below:

Kidney, Caylces, Pelvis, Ureters, Gonads, Genital Ducts

A

Intermediate mesoderm

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2
Q

What structures in UG come from Endoderm?

A

Epithelial lining of Urinary Bladder and urethra

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3
Q

Smooth Muscle & CT in the walls of Bladder and ureter are derived from

A

Splanchnic Mesoderm

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4
Q
  • The Intermediate Mesoderm is Observable at day_____
  • It eventually separates from the ________
A

18-20

paraxial mesoderm

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5
Q

Intermediate Mesoderm forms the ___________[later called the Urogenital (UG) Ridge] (covered with Coelomic Epithelium)

A

Nephrogenic Cord

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6
Q

Specification of kidney-forming ability to the intermediate mesoderm is mediated by signals from __________ and transcription factors ____,____,____ expressed in intermediate mesoderm

A

paraxial mesoderm

[Pax2, Pax8 & Lim1]

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7
Q

A Mesonephric Kidney Forms Within the__________in the Thoracolumbar Region of the Embryo

A

Nephrogenic Cord

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8
Q

Two components of mesonephric kidney (a transient functional structure)

A

Meonephric duct

mesonephric tubules

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9
Q

Mesonephric Duct is an Important Source of_______ for Kidney Structures

  • A solid cell cord which eventually canalizes
  • Extends________ through nephrogenic cord
A

Inductive signals caudally

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10
Q

Mesonephric duct fuses with _____ at day 26

A

Cloaca

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11
Q

The dilated, caudal end of the primitive hindgut; A transient, common outlet for the UG & GI systems

A

Cloaca

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12
Q

Mesonephric Tubules = Immature Nephrons

  • Inductive signals from the ________ induce tubule formation
  • Differentiation occurs in a _____ to ______ direction along the nephrogenic cord
  • All tubules induced________ present at same time
A

mesonephric duct

cranial to caudal

are NOT

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13
Q

The Metanephric (Mature) Kidney Forms in the _____of the Embryo from the Caudal Aspect of the ________

A

Pelvic Region

Nephrogenic Cord

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14
Q

2 Precursors for Metanephric kidney are derived from :

  • _________ = ureteric bud
  • ________ =metanephric lastema
A

Intermediate Mesoderm

Metanephric Diverticuclum

Metanephrogenic Mesenchyme

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15
Q

___________ interactions occur between the Metanepric
Diverticulum and the Metanephrogenic Mesenchyme

A

Reciprocal Inductive

*Metanephic diverticulm encourages differentiation of metaphrogenic mesenchyme

metaphrogenic mesenchyme encourages Branching of metanephric diverticulm

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16
Q

The Epithelial-Mesenchymal interactions between the metanephric diverticulum metanephrogenic mesenchyme are mediated by

A

growth factors, secreted factors, patterning genes & changes in the extracellular matrix

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17
Q

• A full term infant was born and had severe
respiratory distress. He developed a
pneumothorax and required mechanical
ventilation. The maternal history was positive
for oligohydramnios.
• On exam the infant was small for age. There
were unusual facial features and limb
deformities.
• The patient expired within 24 hours.

A

Potters Syndrome

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18
Q

Causes of Potters Syndrome

A

• Renal agenesis
• Severe urinary obstruction
• Features of facial compression,
growth retardation, limb deformities

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19
Q

• Too little amniotic fluid
– Associated with renal agenesis, polycystic kidney disease, urethral obstruction
– Chronic amniotic leak

A

Oligohydramnios

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20
Q

Excessive amniotic fluid
– Associated with diabetes, multiple gestation, anencephaly, esophageal atresia

A

Polyhydramnios

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21
Q

Metanephrogenic Mesenchyme (MM) signals ______ induce formation of Metanephric Diverticulm

Inductive Interactions Between the Metanephric Diverticulum
(MD) and the Metanephogenic Mesenchyme (MM) Result in:________

A

GDNF, RA

Nephron Formation

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22
Q

Expanded tips of MD called________, a key signaling center for nephron induction

A

Ampulla

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23
Q

The varying nature of signals from the ampulla directs the arrangement of ____ and ______. Ampullae begin to disappear at about ____weeks. No new nephrons are formed after all ampullae disappear

A

nephrons and collecting ducts

32

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24
Q

MD signals _____ and _____prevents MM cell apoptosis and induces a subset of
MM cells to aggregate around ampulla

A

[Fgf2, Bmp7]

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25
Q

Early Stages of Nephron Formation
Signals from the ampulla cause the metanphrogenic mesenchyme to aggregate and form an ______ and involves Differentiation and Differential Growth of the S-Shaped Tubule

A

epithelial vesicle

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26
Q

Nephron Derivatives of
the S-Shaped Tubule
Include:
• From the Proximal Part (P) = __________
• From the Middle Part (M) = ____________
• From the Distal Part (D) =____________

A
  • Distal Tubule & loop of Henle
  • Proximal Tubule
  • Renal Corpuscle
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27
Q
  • Vascular Spouts From __________are Induced to Grow Toward the Forming Kidney and form the vasculature of the kidney
  • Only induced________ secretes angiogenic growth factors [VEGF] that attracts the vascular sprouts to the forming kidney
A

Intersegmental Arteries

mesenchyme

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28
Q

A healthy one month old boy was seen in nephrology clinic for a history of his mother
having an abnormal prenatal ultrasound.
• He was well-appearing, had normal blood pressure, and was growing well. He had a right flank mass.

DDx?

A

• Hydronephrosis
– Obstruction of the urinary tract
• Cystic Kidney disease
– Polycystic kidney disease
– Multicystic dysplastic kidney disease
• Renal tumor

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29
Q

You see this on ultrasound from a little boy complaining of right flank pain.

Dx?

Is this often unilateral or bilateral?

What’s the pathology?

A

Multicystic Dysplastic Kidney
• Most often unilateral (polycystic kidney disease is bilateral)
• Multiple cysts of varying sizes and kiney is non-functioning
• Pathology—primitive ductules and cartilage seen; atretic ureter

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30
Q
A
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31
Q

What happens in MDK to the contralateral kidney?

What happens if MDK is bilateral?

A
  • Contralateral kidney hypertrophies
  • Bilateral disease is rare; fatal
32
Q

MDK

  • Potential for abnormalities of the contralateral kidney, such as vesicoureteral reflux, approx. ______
  • Hypertension is potential complication
  • Failure of a large MCKD to regress may be indication for_______
  • The cause is:
A

28%
nephrectomy

NON-genetic (polycystic is genetic)

33
Q

Remodeling & Differential Growth of the Branching Metanephric Diverticulum
results in Formation of the

A

Collecting Ducts,Calyces, Pelvis and Ureter

34
Q

There are about 15 generations of metanephric diverticulum branches; Key features of the branching process include:

  • ________ growth of early generations
  • _______ growth of polar branches
  • Expansion of the _______generations of branches to form the Calyces, Pelvis and Ureters
A

Little

Faster

3rd-6th

35
Q

Branches distal to the 5th & 6th generations form

A

Collecting Ducts

36
Q

• In the kidney, Nephrons and Collecting Ducts are organized into larger structures called _____

A

Renal Lobes

37
Q
  • Each kidney lobe ends in a pyramid shaped Renal Papilla which empties into a____
  • In the Embryonic and Fetal Kidney, the Renal Lobes are visible ______
A

Minor Caylx

externally

38
Q

• Anomalies of the Urinary System Organs are common
but _________
• Urinary System anomalies are often associated with
other anomalies, primarily ________

A

not always clinically significant

genital system anomalies

39
Q

Four anomalies seen in kidneys

A

Hypoplastic (small but may still have normal fnx~ possibly d/t abnormal devo)

Dysplastic kidneys

Duplications (of ureter or kidney~ can be partial or complete)

Horseshoe Kidney~ fusion prevents complete ascent

40
Q

The Metanephric Kidney Develops in the Pelvis and “Ascends” into the Abdomen because of Differential Growth of the __________

A

Embryonic Body

41
Q

Pelvic & Lumbar Kidneys are due to:

Extra renal vessels result from:

A

Failed or incomplete ascent

Failure to atrophy during kidney ascent

42
Q

Polycystic Kidney disorder is what kind of disease?

A

Both autosomal dominant and recessive

43
Q

Common neoplasms in children in the kidney:

What chromosome is it located?

A

Nephroblastoma = Wilms Tumor

chromosome 11

44
Q

Clincal presentation of Ureretopelvic Jnx obstruction

In infant:

In young adult:

A

– Infant: flank mass, UTI, failure to thrive, sepsis
– Older child or adult: flank pain, colicky pain, UTI, hematuria

45
Q
  • An 21year old college student presents with a history of severe right flank pain following celebration of his 21st birthday in which he drank copius amounts of an inhibitor of vasopressin. On exam, the patient was in pain and he had a unilateral right flank mass.
  • Past medical history: significant for several UTI’s as a child, which were never investigated
  • Review of systems: he has had recurrent, intermittent right sided flank pain

Dx?

What are significant findings?

A

Uretopelvic junction obstruction

*right flank pain

*hx of UTIs and he’s male

*recurrent remittent right sided flank pain

*Diarhesis enhances the obstruction of the urinary tract

46
Q

Diagnostic studies for UPJ:

Why is it important to Dx young?

A

ultrasound (anatomy) or renal scan, intravenous pyelogram (functional studies); see fluid back up to kidney

Dx young bc you increase risk of infection thus fibrosis of the kidney. While the obstruction can be fixed later, damage such as fibrosis is permanent and you end up with kidneys that don’t fnx as well

47
Q

Division of the Cloaca Provides for :
The Cloaca is Subdivided by the :

A

Separate Outlets for the UG & GI

Urorectal Septum

48
Q
  • The Primary (Primitive) UG Sinus is located______
  • The Anorectal Canal is located_______
A

ventrally

dorsally

49
Q

The Cranial or Vesicle Part of the Primary UG Sinus Enlarges:
• The cranial part of the UG sinus forms_________
• Lining derived from______
• Smooth muscle & CT of the bladder wall is derived from the surrounding _________

A

urinary bladder & urethra (part)

endoderm

splanchnic mesoderm

50
Q

Remodeling of the Posterior Wall of the Bladder Results in
Formation of the

A

Trigone Region

51
Q

• The trigone is associated with entrance of the______ & exit of the ______

A

ureters

urethra

52
Q

• In males, differential growth results in the mesonephric ducts
opening into _____ rather than _______

* in the male, the mesonephric duct distal to the metanephric diverticulum
becomes the______

A

urethra rather than urinary bladder

vas deferens

53
Q

Anomalies of the Urachus arise from

A

failure of regression of these structures; [allantois & urachus]

–patent openings: urancus doesn’t close to median ligament; you can see pee come out the belly button!

54
Q

Exstrophy of the Bladder
• A defect of the ________
• The lining of the bladder and the urethra is _______
• Associated with _______

A

ventral abdominal wall

open to the surface

UG & skeletal anomalies

55
Q

Ureteral Reflux
• The higher the grade of reflux:
– the greater the risk for ______
– the more______ the anatomy is
– spontaneous resolution is______

***surgical intervention may be necessary

A

scarring

abnormal

less likely

56
Q

What happens to the ureter and kidney over time in patient with ureteral reflux?

A

see scarring, tortuous and dialate ureters, increased risk of infection

57
Q

• A______ ureter leads to reflux, which can lead to recurrent kidney infections and
subsequent atrophy of the kidney.
• Treatment is:

A

lateralized

antibiotic prophylaxis and/or surgical repair

58
Q

• Four year old female
• Potty trained but having urgency and
dampness
• Has had three urinary tract infections in the
past year, all with fever
• Mother was told she had childhood problems,
has a low abdominal scar, has no other details

A

Ureteral reflex

59
Q

Healthy 28 year old female, G1, P0 at 20 weeks gestation
• Routine prenatal ultrasound reveals an healthy fetus but the bladder is not visualized
• Kidneys are normal
• Amniotic fluid is normal
• Sex is indeterminate
• What is the diagnosis??

A

Bladder Exstrophy

60
Q

Bladder Exstrophy
• 1 per hundred if positive ___
• Male:Female _____
• Failure of _________

A

FH

3:1

medial mesenchymal migration

61
Q

Bladder Extrophy

Males oftne with:

  • Females with :
  • Gonadal structures_____ and babies typically______
A

epispadius

shortened vagina; bifid clitoris

normal

healthy

62
Q

Exstrophy Repair: Surgery performed shortly after birth and babies hospitalized
for 10-14 days
ISSUES remaining are:

A

Incontinence is a major long term issue
• Upper tracts at risk after repair

63
Q

The Extent of the Urethra Formed from the Caudal Part of
UG Sinus Differs in Males & Females

Males:

Females:

A

Males: Proximal portion of Prostatic Urethra

Female: Most of it

64
Q

The Fate of the Definitive UG Sinus is Different in
Males & Females

Males
Pelvic portion –>

Phallic Portion –>

A

Pelvic –> Distal prostatic and Membranous urethra

Phallic Portion –> Penile urethra

65
Q

The Fate of the Definitive UG Sinus is Different in
Males & Females

Females
Pelvic portion –>

Phallic Portion –>

A

Pelvic–> lower half of vagina

phallic–> vestibule

66
Q

Agenesis & Atresia of the Urethra : Atresia is associated with

A

urinary obstruction & Prune Belly Syndrome

67
Q
  • Mucosal folds which obstruct the lumen of the urethra
  • A Common cause of renal failure in boys
A

Posterior Urethral Valves

68
Q

posterior urethral valves
• Occurs only in_____
• Obstructing valves leads to

A

males

severe obstruction of urinary tract and irreversible renal dysplasia

69
Q

mom is 22 weeks, notes baby isn’t moving around as much, get ultrasound:

• Finding include
– Massively distended bladder
– Poorly developed chest cavity
– Small, echo-bright hydronephrotic kidneys
– Oligohydramnios
• What is the diagnosis??

A

Posterior urethral vavles: only in males!

70
Q

Pathology of PUV:

  • Obstructing membrane at _______
  • Apprearane of kidneys on utlrasound:
  • Valves easily _____
  • Outcome largely predetermined
A

membranous urethra

Small echo-bright kidneys

ablated

71
Q

The kidney and ureter form from ______; the lining of the
urinary bladder and urethra are derived from _______

A

intermediate mesoderm

endoderm and the wall from splanchnic mesoderm

72
Q

A temporary kidney, the_______, provides the mesonephric duct, the
source of the metanephric diverticulum
• Formation of the metanephric (mature) kidney begins in _____

A

mesonephros

week 5

73
Q

Metanephric kidney formation depends on inductive interactions between
the _______ and _______

A

metanephric diverticulum and the metanephrogenic mesenchyme

74
Q

• The ureter, pelvis, calcyes and collecting ducts form from the

A

metanephric diverticulum

75
Q

• The nephrons form from metanephrogenic mesenchyme and glomeruli are
derived from

A

sprouts of intersegmental arteries

76
Q

The_______ part of the divided cloaca becomes the primitive UG sinus from
which the urinary bladder and urethra are formed

A

ventral

Renal Exam (22 decks)

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