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Anatomy II Test II - Jourdan > Embryology of the Urogenital Systems > Flashcards

Embryology of the Urogenital Systems Flashcards

0
Q

What does intermediate mesoderm separate from during folding and forms urogenital ridge?

A

From lateral and paraxial mesoderm

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1
Q

What do urinary and genital systems develop from?

A

Intermediate mesoderm of trilaminar disc

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2
Q

What forms pronephros?

A

Nonfunctional cervical nephrotomes

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3
Q

When do nephrotomes transitory and regress?

A

By week 5

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4
Q

When does a second primitive kidney develop?

A

During the 4th week

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5
Q

What follows the development of the second primitive kidney during the 4th week?

A
  1. functional glomeruli develops
  2. mesonephric tubules form
  3. mesonphric duct opens into cloaca
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6
Q

mesonephros are present and functional during what weeks?

A

from weeks 6-10

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7
Q

What happens to mesonephros after week 10?

A

It degenerates except for: the duct system or some vestigial structures

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8
Q

What does the duct system that remains contributes to?

A

Part of the male genital system (ductus deferens)

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9
Q

What part of the mesonephros remains after week 10 in females?

A

Some vestigial structures in the female

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10
Q

What does renogenesis involve?

A

Involves a process of reciprocal induction, which is retinoic acid dependent

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11
Q

What establishes a “renogenic” region within the intermediate mesoderm in the tail of the embryo?

A

The cranial-caudal patterning

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12
Q

What is the renogenic mesoderm called?

A

Metanephric blastema

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13
Q

What is the function of the metanephric blastema?

A

Secretes growth factors that induce growth of the ureteric bud from the caudal portion of the mesonephric duct

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14
Q

When the Ureteric bud proliferates how does it respond?

A

By secreting growth factors that stimulates proliferation and then differentiation of the metanephric blastema

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15
Q

What does the metanephric blastema differentiate into after stimulation from ureteric bud?

A

Into glomeruli and kidney tubules

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16
Q

What may cause inhibition of ureteric bud growth and renal hypoplasia or agenesis?

A

Perturbations in any aspect of renogenesis events (mutations of either metanephric or ureteric factors or disruption of retinoic acid signaling)

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17
Q

In contrast to inhibition of ureteric bud growth, what else can occur during renogenesis?

A

Duplication or over proliferation of structures

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18
Q

When does blood plasma from glomerular capillaries begin to be filtered?

A

By week 10

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19
Q

When urine is produced In utero, what is the primary function?

A

It is not to clear waste (placenta) but to supplement production of amniotic fluid

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20
Q

What may indicate bilateral renal agenesis or urethral obstruction?

A

Oligohydramnios: insufficient amount of amniotic fluid

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21
Q

Where are kidneys formed? Why do they move during development?

A

In the pelvis but ascend to lumbar region with progressive revascularization from common iliac and aorta

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22
Q

What enters the kidney independently from the primary renal vessels?

A

Aberrant renal vessels

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23
Q

Aberrant renal vessels entering the kidney occurs how often in the population? Where do the vessels originate?

A

These occur in approximately 10% of individuals and 98% arise from the abdominal aorta

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24
Q

When do aberrant renal vessels cause a problem vs pose little issues?

A

Most enter the renal pelvis and pose little problems

Aberrant renal arteries that enter the upper or lower poles of the kidney may post problems

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25
Q

Aberrant arteries to the inferior pole of the kidney (4-6%) may be the cause for what?

A

Obstruction of the ureter and may cause an intermittent or continuous obstruction to urinary drainage from the renal pelvis

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26
Q

When can potter syndrome be diagnosed?

A

In utero diagnosis

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27
Q

Who is the first to survive Potter’s syndrome diagnosis?

A

Abigail who now undergoes nightly dialysis prior to transplant. She went through experimental treatment, which involved intrauterine fluid injections to facilitate growth and development

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28
Q

What prevents normal renal rotation?

A

Renal fusion

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29
Q

What kind of blood supply do renal fusion usually have?

A

Abnormal blood supply usually from middle sacral or common iliac arteries

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30
Q

What may also be crossed with/without fusion?

A

Ectopic kidney

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31
Q

Pelvic kidney and horseshoe kidney are examples of _____

A

Renal fusion

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32
Q

What type of renal fusion is most common?

A

Horseshoe kidney
90% fused at lower pole
Usually found inferior to inferior mesenteric artery

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33
Q

What is Wilms’ tumor?

A

Cancer of the kidney

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34
Q

Wilms’ tumor usually affects who?

A

Children under the age of 5

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35
Q

What causes Wilms’ tumor?

A

Mutation in the WT1 gene

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36
Q

3 month-old previously healthy boy presented with hematuria. A renal ultrasound, and subsequent chest, abdomen, and pelvis CT exam demonstrated a large heterogeneous right renal mass: diagnosis?

A

Wilms’ tumor

37
Q

Describe the steps of the urinary bladder

A
  1. cloaca divided by the urorectal septum
  2. Dorsal (inferior) portion develops into the rectum and anal canal
  3. The ventral (superior) portion develops into the bladder and urogenital sinus
38
Q

What does the bladder and urogenital sinus give rise to?

A

The bladder and lower urogenital tracts

39
Q

What bladder and urogenital tracts are present in males?

A

Prosstatic and penile urethrae

40
Q

What bladder and lower urogenital tracts are present in females?

A

Urethra and lower vagina

41
Q

Mesonephric duct and ureteric bud incorporation into posterior wall of urinary bladder occurs between what weeks?

A

4-6

42
Q

What do ureteric buds open into?

A

The bladder wall

43
Q

Where does the mesonephric ducts open?

A

More inferiorly into the pelvic urethra.

44
Q

What does not open into the bladder?

A

Ductus deferens

45
Q

What does the triangular region of the incorporated mesonephric duct incorporated in the bladder wall form?

A

The trigone

46
Q

Bladder is continuous (superiorly/inferiorly) with allantois?

A

Superiolry

47
Q

What should happen with the allantois?

A

Collapse and become the urachus

48
Q

What abnormalities can happen with the urachus?

A
  1. may remain paten throughout its length: urachal fistula
  2. patent only at an end: urachal sinus
  3. remnants of urachal lumen may also give rise to urachal cysts: usually present as an abdominal mass
49
Q

When is the genotype of the embryo established?

A

At fertilization

50
Q

How long does the embryo remain in undifferentiated state?

A

Week 1-6

51
Q

When does phenotypic sexual differentiation begin?

A

Week 7

52
Q

When can male and female external genitalia be recognized?

A

Week 12

53
Q

When is the phenotypic differentiation complete?

A

Week 20

54
Q

Fetal sex is encoded where?

A

On the sex determining region of the Y chromosome (SRY)

55
Q

What triggers male development?

A

Synthesis of SRY protein (testis-determining factor, TDF)

56
Q

If SRY protein is not expressed what happens?

A

whether absent or defective, female path is followed

57
Q

What is the basic (default) developmental pathway?

A

Female

58
Q

What cannot be expressed in order for female path to be followed?

A

TDF

59
Q

Describe the male development pathway

A
  1. activation of SRY gene
  2. TDF
  3. Differentiation of biopotential gonad into testis
  4. Sertoli cell differentiation
  5. Mullerian inhibitory substance/factor
  6. regression of paramesonephric (mullerian) ducts
  7. urogenital sinus, forrmed from division of the cloaca
60
Q

How many pairs of genital ducts are present in both sexes during development?

A

two

61
Q

When are both pairs of genital ducts present during development?

A

The indifferent stage

62
Q

Mesonephric ducts play the most important role in (male/female)

A

male

63
Q

Paramesonephric ducts play the most important role in (male/female)

A

female

64
Q

The gonads develop from what three tissues?

A

epithelium of intraembryonic coelom
intermediate mesoderm
primordial germ cells

65
Q

What invades the dorsal mesentery and migrate to urogenital ridges?

A

primordial germ cells from mesoderm of yolk sac

66
Q

What are aggregates of supporting cells (homorne secreting cells)?

A

Primary sex cords

67
Q

When primary sex cords develop what doe gonads now have?

A

An outer cortex and inner medulla

68
Q

What does the phallus become?

A

Clitoris or penis

69
Q

What do (uro)genital folds become?

A

Labis minora or shaft of the penis

70
Q

What does the labioscrotal (genital) wellings become?

A

Either labis majora or scrotum

71
Q

What are some paramesonephric duct anomalies?

A
  1. uterus didelphys with double vagina
  2. Uterus arcuatus
  3. Uterus bicornis
  4. Uterus bicorni unicollis: 1 redimentary horn
  5. Atresia of cervix
  6. Atresia of vagina
72
Q

What can result in cervical or vaginal atresia?

A

Partial or total atresia of the distal portion of both ductus

73
Q

What can have persistence of paramesonephric ductus if the circulating levels of AMH are low or there is an abnormal response to normal AMH?

A

46, XY

74
Q

Failure of the paramesonephric ducts to develop can result in what?

A

Missing uterine tubes, uterus and variable malformations of the upper portion of the vagina

75
Q

What is MRKH or Mayer-Rokitansky-Kuster-Hauser-Syndrome?

A

Missing uterine tubes, uterus and variable malformations of the upper portion of the vagina

76
Q

What is hypospadias?

A

incomplete fusion of the urethral folds

77
Q

where does the urethra open onto in males in hypospadias?

A

The ventral aspect of the penis

78
Q

What can hypospadias result from?

A

From inadequate androgen production or inadequate receptor sites for DHT

79
Q

What are the types of hypospadias from moderate to severe?

A
  1. glandular
  2. penile
  3. penoscrotal
  4. perineal
80
Q

What do females (46, XX) with severe forms of adrenal hyperplasia have what?

A

Ambiguous genitalia at birth due to excess adrenal androgen production in utero

Female intersex

81
Q

What does abiguous genitalia range from?

A

Ranges from complete fusion of the labioscrotal folds and a phallic urethra to only clitoromegaly, partial fusion of the labioscrotal folds, or both

82
Q

Do intersex females have ovaries?

A

there are no abnormalities of ovaries

83
Q

What is androgen insensitivity syndrome (AIS) - complete ?

A

An x-linked disorder in which receptors remain unresponsive to androgens
Despite normal levels of testosterone, the male fetus fails to masculinise
External genitalia are feminine: internally they posses non-functional undescended testes
Some males may tend to be feminine in gender and behaviour (maybe)

84
Q

What can happen at puberty with someone with AIS?

A

Secondary female sexual characteristics may appear due to estradiol from testosterone aromatization

85
Q

What is common to perform on males with AIS?

A

gonadectomies at puberty and for them to receive hormone therapy, to be reared as female

86
Q

The degree of feminization of AIS depends on what?

A

Upon affected androgens and the amount of residua receptor function

87
Q

What is 5-ARD?

A

Autosomal recessive condition resulting in the inability to convert testosterone to the more physiologically active dihydrotestosterone (DHT)

88
Q

If someone has 5-ARD what happens?

A

Because DHT is required for the normal masculinization of the external genitalia in utero, genetic males with 5-ARD are born with ambiguous genitalia (underdevelopment of penis and scrotum or psuedovaginal perineoscrotal hypspadias)

89
Q

What is 5-ARD often misdiagnosed as?

A

AIS

90
Q

What derivatives are normal in 5-ARD?

A

Derivatives of mesonephric duct are normal

91
Q

What is the third gender called?

A

Guevedoche or Machinhembras

first woman, then man

Anatomy II Test II - Jourdan (9 decks)

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