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Renal > Development of the Urinary System > Flashcards

Development of the Urinary System Flashcards

1
Q

Precursor Tissues for the Kidney and Urinary Tract:

  1. Intermediate Mesoderm
  2. Endoderm
A
  1. Intermediate Mesoderm
    • Kidney
    • Caylces
    • Pelvis
    • Ureter
  2. Endoderm
    • Epithelial Lining of:
      • Urinary Bladder
      • Urethra
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2
Q

What parts of the urinary system come from the splanchnic mesoderm?

A

Smooth Muscle & CT in the walls of urinary system organs are derived from Splanchnic Mesoderm

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3
Q

Developmental History of the Intermediate Mesoderm

  1. Intermediate Mesoderm is observable at …
  2. It eventually separates from the …
  3. It forms the …
A
  1. Intermediate Mesoderm is Observable at day 18-20
  2. It eventually separates from the paraxial mesoderm
  3. It forms the Nephrogenic Cord
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4
Q

What paraxial mesoderm transcription factors, expressed in intermediate Mesoderm, direct kidney formation?

A

Pax2, Pax8 & Lim1

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5
Q

Urogenital Derivatives from Intermediate Mesoderm:

A
  1. Kidneys
  2. Ureters
  3. Gonads
  4. Genital Ducts
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6
Q

A __________ Kidney Forms Within the Nephrogenic Cord in the Thoracolumbar Region of the Embryo

A

A **Mesonephric **Kidney Forms Within the Nephrogenic Cord in the Thoracolumbar Region of the Embryo

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7
Q

What are the 2 components of the mesonephric kidney?

A
  1. Mesonephric Duct
  2. Mesonephric Tubules
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8
Q

Why is the mesonephric duct important?

A

Important Source of Inductive Signals for Kidney Structures:

  • A solid cell cord which eventually canalizes
  • Extends caudally through nephrogenic cord
  • Fuses with Cloaca (day 26)
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9
Q

What is the cloaca?

A
  • The dilated, caudal end of the primitive hindgut
  • A transient, common outlet for the UG & GI systems
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10
Q

What are the immature nephrons?

  • Describe their differentiation:
A

Mesonephric Tubules = Immature Nephrons

  • Inductive signals from the mesonephric duct induce tubule formation
  • Differentiation occurs in a cranial to caudal direction along the nephrogenic cord
  • All tubules induced are not present at same time
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11
Q

The _________ (Mature) Kidney forms in the Pelvic Region of the Embryo from the Caudal Aspect of the Nephrogenic Cord

  • What are the 2 precursors for this structure?
A

The Metanephric (Mature) Kidney Forms in the Pelvic Region of the Embryo from the Caudal Aspect of the Nephrogenic Cord

  • 2 precursors: Both derived from the Intermediate Mesoderm
    • Metanephric Diverticuclum (ureteric bud)
    • Metanephrogenic Mesenchyme (metanephric lastema)
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12
Q

What are the reciprocal inductive interactions in the metanephric kidney?

A
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13
Q

What can lead to Renal Agenesis?

A

If the inductive interactions between the metanephric diverticulum and metanephrogenic mesenchyme are altered or absent

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14
Q
  1. When is Renal Agenesis usually asymptomatic?
  2. When is Renal Agenesis symptomatic? What syndrome is this associated with?
A
  1. Unilateral, is usually asymptomatic
  2. Bilateral
    • Incompatible with extrauterine life
    • Key features include oligohydramnios and pulmonary hypoplasia
    • Compression of the face due to decreased amniotic fluid
    • Characteristic of Potter’s syndrome
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15
Q

Oligohydramnios vs. Polyhydramnios

A
  1. Oligohydramnios
    • Too little amniotic fluid
      • Associated with renal agenesis, polycystic kidney disease, urethral obstruction
      • Chronic amniotic leak
  2. Polyhydramnios
    • Excessive amniotic fluid
      • Associated with diabetes, multiple gestation, anencephaly, esophageal atresia
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16
Q
  1. What can lead to oligohydramnios?
  2. What can oligohyramnios lead to?
  3. What can fetal compression lead to?
A
  1. defect of urinary output & chronic leak of amniotic fluid ⇒ oligohydramnios
  2. oligohydramnios ⇒ fetal compression, growth deficiency, pulmonary hypoplasia
  3. fetal compression ⇒ Potter’s facies, limb positioning defect, growth deficiency, pulmonary hypoplasia
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17
Q

Describe the signals that guide formation of the nephron:

What prevents cell apoptosis?

A
  • Signals from the MM [GDNF, RA] induce formation of MD
  • MD branches [cells express Ret a GDNF receptor]
  • Expanded tips of MD called Ampulla
    • ​key signaling center for nephron induction
  • Varying nature of signals from the ampulla directs the arrangement of nephrons and collecting ducts
  • Ampullae begin to disappear at about 32 weeks
  • No new nephrons are formed after all ampullae disappear
  • MD signals [Fgf2, Bmp7] prevents MM cell apoptosis
    • induces a subset of MM cells to aggregate around ampulla
18
Q

What are the stages of nephron formation?

A

Signals via the ampulla cause:

Segregation ⇒ Compaction ⇒ Vesicle Fusion ⇒ S-Shaped Tubule

19
Q

Nephron Derivatives of the S-Shaped Tubule Include:

  1. From the Proximal Part (P)
  2. From the Middle Part (M)
  3. From the Distal Part (D)
A
  1. From the Proximal Part (P)
    • Distal Tubule & loop of Henle
  2. From the Middle Part (M)
    • Proximal Tubule
  3. From the Distal Part (D)
    • Renal Corpuscle
20
Q

Vascular Spouts From ___________ _______ are Induced to Grow Toward the Forming Kidney

A

Vascular Spouts From Intersegmental Arteries are Induced to Grow Toward the Forming Kidney

  • They form the vasculature of the kidney
  • Only induced mesenchyme secretes angiogenic growth factors [VEGF] that attracts the vascular sprouts to the forming kidney
21
Q
  1. What is hydronephrosis?
  2. What are two types of cystic kidney disease?
A
  1. Hydronephrosis
    • Obstruction of the urinary tract
  2. Cystic Kidney disease
    • Polycystic kidney disease
    • Multicystic dysplastic kidney disease
22
Q

What are the characteristics of Multicystic Dysplastic Kidney?

A
  • Most often unilateral (polycystic kidney disease is bilateral)
  • Multiple cysts of varying sizes
  • Non-functioning
  • Pathology—primitive ductules and cartilage seen; atretic ureter
  • Contralateral kidney hypertrophies
    • potential abnormalities of the contralateral kidney
  • Bilateral disease is rare; fatal
  • Hypertension is potential complication
  • Failure of a large MCKD to regress may be indication for nephrectomy
  • Non-genetic (Polycystic kidney disease is genetic)
23
Q

Remodeling & Differential Growth of the Branching Metanephric Diverticulum results in ….

A

Formation of the Collecting Ducts, Calyces, Pelvis and Ureter

  • Little growth of early generations
  • Faster growth of polar branches
  • Expansion of the 3rd-6th generations of branches to form the Calyces, Pelvis and Ureters
  • Branches distal to the 5th & 6th generations form Collecting Ducts
24
Q
  • In the kidney, Nephrons and Collecting Ducts are organized into larger structures called _____ _____
  • Each kidney lobe ends in a pyramid shaped _____ ______ which empties into a _____ _____
A
  • In the kidney, Nephrons and Collecting Ducts are organized into larger structures called Renal Lobes
  • Each kidney lobe ends in a pyramid shaped Renal Papilla which empties into a Minor Caylx
25
Q

Anomalies That Result in Variations in Kidney Size, Histological Organization or Number:

A
  • Hypoplastic Kidneys
    • May be small & normal
    • May be small because of abnormal development
  • Dysplastic Kidneys
  • Duplications
    • Of the Ureter or Kidney which may be partial or complete
  • Horseshoe Kidney
    • Fusion prevents complete ascent
26
Q

Pelvic or Lumbar Kidneys are due to…

A

Failed or incomplete ascent

  • Ectopic location
  • Extra renal vessels result from failure to atrophy during kidney ascent
27
Q

Polycystic Disease of the Kidneys is an _______ Disorder

A

Polycystic Disease of the Kidneys is an Inherited Disorder

  1. Autosomal Recessive Polycystic Kidney Disease
  2. Autosomal Dominant Polycystic Kidney Disease
28
Q

What are the characteristics of Nephroblastoma (Wilm’s Tumor)?

A
  • A common neoplasm in children
  • The gene is located on chromosome 11
29
Q

What will enhance a Ureteropelvic Junction (UPJ) Obstruction?

A

Diuresis (increased water excretion) enhances obstruction of the urinary tract

30
Q

UPJ Obstruction:

  • Clinical Presentation:
  • Diagnostic Studies:
A
  • Clinical Presentation:
    • Infant: flank mass, UTI, failure to thrive, sepsis
    • Older child or adult: flank pain, colicky pain, UTI, hematuria
    • Prenatal diagnosis
  • Diagnostic studies: ultrasound (anatomy) or renal scan, intravenous pyelogram (functional studies)
31
Q

What Provides for Separate Outlets for the UG & GI Systems?

A

Division of the Cloaca

  • The Primary (Primitive) UG Sinus is located ventrally
  • The Anorectal Canal is located dorsally
32
Q

What does remodeling of the posterior wall of the bladder result in?

  • In males, what happens in this region?
A

Formation of the Trigone Region

  • The trigone is associated with entrance of the ureters & exit of the urethra
  • In males, differential growth results in the mesonephric ducts** opening into urethra rather than urinary bladder
    • **the mesonephric duct distal to the metanephric diverticulum becomes the vas deferens
33
Q

How can anomalies of the urinary bladder occur?

A
  • Anomalies of the Urachus [allantois & urachus]
    • Arise from failure of regression of these structures
  • Exstrophy of the Bladder
    • A defect of the ventral abdominal wall
    • The lining of the bladder and the urethra is open to the surface
    • Associated with UG & skeletal anomalies
34
Q

Ureteral Reflux

  • The higher the grade of reflux …
A
  1. the greater the risk for scarring
  2. the more abnormal the anatomy is
  3. spontaneous resolution is less likely
  4. surgical intervention may be necessary
35
Q
  • What is the consequence of a lateralized ureter?
  • How is it treated?
A
  • A lateralized ureter leads to reflux, which can lead to recurrent kidney infections and subsequent atrophy of the kidney.
  • Treatment is antibiotic prophylaxis and/or surgical repair
36
Q
  • What causes bladder exstrophy?
  • What is the difference in presentation between males and females?
  • How healthy are infants at birth?
A
  • **Failure of medial mesenchymal migration **
    • 1 per 30,000 live births
    • 1 per hundred if positive FH
  • Male:Female 3:1
    • Males with epispadius
    • Females with shortened vagina; bifid clitoris
  • Gonadal structures normal
  • Babies typically healthy
37
Q
  • What should be done to treat bladder exstrophy?
  • What are the major long term complications?
A
  • Surgery performed shortly after birth
    • Babies hospitalized for 10-14 days
  • Incontinence is a major long term issue
  • Upper tracts at risk after repair
38
Q

The Extent of the Urethra Formed from the Caudal Part of UG Sinus Differs in Males & Females. What is the difference?

A
  • Male
    • Proximal Portion of Prostatic Urethra
  • Female
    • Most of It
39
Q

How does the fate of the definitive UG sinus differ between males and females?

A
  • ​Male
    • Pelvic portion:
      • Distal Prostatic & Membranous Urethra
    • ​Phallic portion
      • ​Penile urethra
  • Female
    • Pelvic portion:
      • ​lower half of the Vagina
    • Phallic portion:
      • ​Vestibule
40
Q

How can anomalies of the urethra occur?

A
  • Agenesis & Atresia of the Urethra
    • Atresia is associated with urinary obstruction & Prune Belly Syndrome
  • Posterior Urethral Valves
    • Mucosal folds which obstruct the lumen of the urethra
    • A common cause of renal failure in boys
41
Q

What are the characteristics of Posterior Urethral Valves?

A
  • Occurs only in males
  • 1/5000 male births
  • Obstructing valves leads to severe obstruction of urinary tract and irreversible renal dysplasia

Renal (17 decks)

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