Block 4 Md 1 Embryology Urogenital Part F Dz-S

Front 1

Clinical Considerations
5ct =

Back 1

-Ureteropelvic duplications
-Urachal fistula or cyst
-Bladder exstrophy
-ectopic opening of the ureter
-Wilms' tumor (WT)

Front 2

Ureteropelvic duplications : ***
occur when the ureteric bud

Back 2

prematurely divides before penetrating the =

This results in either a =
2ct

Hint 2

metanephric blastema.

double kidney
duplicated ureter and renal pelvis.

Front 3

Ureteropelvic duplications : ***
occur when the ureteric bud prematurely divides before penetrating the metanephric blastema.

This results in =
2ct

Back 3

double kidney
duplicated ureter and renal pelvis.

Hint 3

Front 4

Urachal fistula or cyst :

occurs when a
remnant of the
________ persists,
thereby forming a
fistula or cyst. ***

Back 4

allantois

Front 5

remnant of the
allantois persists,
thereby forming a
=

Back 5

fistula or cyst. ***

Front 6

Recall: After birth, the allantois degenerates and becomes the =

Back 6

urachus forming the
_____ _______ __________ =

Hint 6

median umbilical ligament.

Front 7

It is found along the ______ on a path from the umbilicus to the _____ of the urinary bladder.

A urachal fistula forms a direct connection between the urinary bladder and the outside of the body at the umbilicus causing urine drainage from the .

Back 7

midline

apex

umbilicus

Front 8

(Abdominal mass) is caused by

A urachal Cyst ??
or
A urachal fistula ??

Back 8

A urachal Cyst

Front 9

a congenital anomality in which, part of the bladder is present outside the body.

Back 9

*** Bladder exstrophy

Front 10

Bladder exstrophy
It is rare, occurring once every _________
live births

Back 10

40,000-50,000

with a ___ /___ male:female ratio.

Hint 10

2:1

Front 11

Bladder exstrophy
The diagnosis involves a spectrum of anomalies of =
4ct

Back 11

lower abdominal wall
bladder
anterior bony pelvis
external genitalia

Front 12

*** The cause of bladder exstrophy is

Back 12

maldevelopment of the lower abdominal wall
(lateral embryologic folds that form the abdominal wall failed to close)

leading to a rupture which causes =

Hint 12

the bladder to communicate with the amniotic fluid

(and results in protrusion of the posterior bladder wall through the lower abdominal wall).

Treatment is with surgical correction of the defect

Front 13

An ectopic opening of the ureter : ***
occurs when the =

Back 13

ureteric bud fails to separate from the =

Hint 13

mesonephric duct,

which results in the opening of the ureter to be carried to a point distal to its normal position.

Front 14

The most common ectopic opening is a lateral ureteral ectopia in which the opening is lateral to its normal position.

Back 14

Front 15

know pic

Back 15

.

Front 16

ectopic opening of the ureter

*** In males, the ectopic openings are most commonly located in the =
4ct

Back 16

prostatic urethra
ejaculatory ducts
ductus deferens
rectum.

Front 17

Because the ectopic openings all are located ________________________
boys with an ectopic opening of the ureter do not present with urine incontinence.

Back 17

above the external urethral sphincter

Front 18

► *** In females, the ectopic openings are most commonly located in the

Back 18

urethra
vestibule
vagina

Front 19

Because the ectopic openings all are located______________________, girls with an ectopic opening of the ureter generally present with _____ _______.

Back 19

below the external urethral sphincter

urine incontinence.

Front 20

is the most common renal malignancy of childhood. =

Back 20

Wilms' tumor (WT)

Front 21

Patients with _____________ present with a large, solitary, well-circumscribed mass that, on cut section, is soft, homogeneous, and tan-gray in color.

Back 21

Wilms' tumor (WT)

Front 22

Patients with WT present with a =
6ct

Back 22

large
solitary
well-circumscribed mass
is soft
homogeneous
tan-gray

Front 23

WT is interesting histologically in that this tumor tends to recapitulate different stages of embryologic formation of the kidney so that

Back 23

that three classic histologic areas are described: =
4ct

Hint 23

stromal area
blastemal area
tightly packed embryonic cells
tubular area

Front 24

Wilms’ tumor is due to mutations in the

Back 24

WT1 gene on 11p13

it may be associated with other abnormalities and syndromes. =

Hint 24

WAGR syndrome

Denys-Drash syndrome

Front 25

WAGR syndrome

Denys-Drash syndrome

Back 25

aniridia
hemihypertrophy
Wilms’ tumor


renal failure
pseudohermaphrodism
Wilms’ tumor

Front 26

Development of Suprarenal Glands

adrenal glands are located in the ___________ . They are surrounded by an _____ _____ and renal ______..

Back 26

retroperitoneum

adipose capsule
fascia

Front 27

Adrenal glands are found at the spinal level of the =

Back 27

T12


Adrenal cortex is divided into =

Hint 27

3 concentric zones

Front 28

Adrenal cortex is divided into three concentric zones:

Back 28

zona glomerulosa:
produces aldosterone, responsible for regulating salt and water metabolism

zona fasciculata:
produces glucocorticoids (cortisol)

zona reticularis:
produces adrenal androgens
(testosterone, estrogen).

Front 29

zona glomerulosa produces

Back 29

aldosterone

responsible for regulating =

Hint 29

Na
H2O

Front 30

zona fasciculata produces

Back 30

glucocorticoids aka =

Hint 30

(cortisol)

Front 31

zona reticularis produces

Back 31

adrenal androgens =
2ct

Hint 31

testosterone
estrogen

Front 32

which Adrenal cortex produces
aldosterone
=

Back 32

zona glomerulosa

Front 33

which Adrenal cortex produces
glucocorticoids (cortisol)
=

Back 33

zona fasciculata:

Front 34

which Adrenal cortex produces
adrenal androgens :
testosterone
estrogen
=

Back 34

zona reticularis

Front 35

main carbohydrate-regulating
steroid =

Back 35

Glucocorticoids

Front 36

Glucocorticoids is for maintaining =

Back 36

arbohydrate-regulating

Front 37

Aldosterone is for maintaining =

Back 37

electrolyte-regulating

Nm the 2 substances =

Hint 37

Na
H2O

Front 38

4 layers of the Sapraadrenals =

Back 38

Glomerulosa
fasciaculata
reticularis
medula

The first three are the cortex
the last is the medula

Hint 38

Front 39

The layers of the adrenaline gland produce what chemicals:

Glomerulosa
fasciaculata
reticularis
medula

Back 39

Aldosterone
cortisol
androgens
catecholamines
epinephrine
norepinephrine

Front 40

These chemicals come from what layers of the supra Adrenal glands

Aldosterone
cortisol
androgens
catecholamines
epinephrine
norepinephrine

Back 40

Glomerulosa
fasciaculata
reticularis
medula

Front 41

Suprarenal Glands

T/F
The cortex and medulla of the suprarenal (adrenal) glands have different origins

Back 41

true

Front 42

Suprarenal Glands
The cortex develops from =

Back 42

mesoderm

Front 43

Suprarenal Glands
medulla differentiates from

Back 43

neural crest cells

Front 44

The suprarenal glands become smaller as the fetal cortex involutes rapidly during the first __ weeks after birth and continues to involute during the ____ of life.

Back 44

2 wks

first year

Front 45

The zona glomerulosa and zona fasciculata of the adult cortex are present at birth, but the zona reticularis is not formed until

Back 45

age 3

Front 46

The cells that form the fetal cortex are derived from the ______________ lining the posterior abdominal wall

Back 46

mesothelium (mesodermal origin)


The cells that form the medulla are derived from an adjacent sympathetic ganglion, which is derived from =

Hint 46

neural crest cells

Front 47

Suprarenal Glands
Dz Clinical considerations:
3ct

Back 47

Neuroblastoma (NB)

Pheochromocytoma (PH)

Congenital adrenal hyperplasia (CAH)

Front 48

Neuroblastoma (NB) :
NB is a common extracranial neoplasm(tumor) containing primitive neuroblasts (small cells arranged in Homer-Wright pseudorosettes)
origin?

Back 48

NCC

NB occurs mainly in children and is found either in extra-adrenal sites usually along the sympathetic chain ganglia (__%) or within the adrenal medulla (__%).

Hint 48

60%


40%

Front 49

Neuroblastoma (NB)

NB metastasizes widely to

Back 49

bone marrow
bone
and lymph nodes

Front 50

A common laboratory finding is increased =
3ct

Back 50

urine vanillylmandelic acid
(VMA)
24-hr urinary catecholamine level
metanephrine levels.

Front 51

Pheochromocytoma (PH) :
PH is a relatively rare neoplasm that contains both

Back 51

epinephrine
norepinephrine

Front 52

Pheochromocytoma
PH occurs mainly in adults ___ - __ years old and is generally found i the region of the

Back 52

40-60 yrs

adrenal gland

it may be found in extrasuprarenal sites.

Front 53

Pheochromocytoma
PH is associated with

Back 53

persistent or paroxysmal hypertension
anxiety
tremor
profuse sweating
pallor
chest pain
abdominal pain.

Front 54

persistent or paroxysmal hypertension
anxiety
tremor
profuse sweating
pallor
chest pain
abdominal pain.

is a finding in =

Back 54

Pheochromocytoma

Laboratory findings include

Hint 54

increased urine VMA ,

24-hr urinary catecholamine and metanephrine levels.

Front 55

Pheochromocytoma
PH is treated by

Back 55

surgery

Phenoxybenzamine
an a-adrenergic antagonist).

Front 56

Congenital adrenal hyperplasia (CAH):

caused most commonly by mutations in genes for enzymes involved in

Back 56

adrenocortical steroid biosynthesis
(e.g., 21-hydroxylase deficiency).

info+

Hint 56

In 21-hydroxylase deficiency (90% of all cases), there is virtually no synthesis of the cortisol or aldosterone so that intermediates are funneled into androgen biosynthesis thereby elevating androgen levels.

Front 57

Congenital adrenal hyperplasia (CAH):
The elevated levels of androgens lead to =

Back 57

masculinization of a female fetus

Hint 57

Front 58

Congenital adrenal hyperplasia (CAH):
Female patient with CAH produces the following clinical findings:

Back 58

mild clitoral enlargement
complete labioscrotal fusion with a phalloid organ, or
macrogenitosomia (in the male fetus).

Front 59

Since cortisol cannot be synthesized, _____ _______ to the adenohypophysis does not occur, so ____ continues to stimulate the adrenal cortex resulting in ______ _______

Back 59

negative feedback

ACTH

adrenal hyperplasia.

Front 60

Congenital adrenal hyperplasia (CAH):

Since aldosterone cannot be synthesized, the patient presents with

Back 60

hyponatremia

with adjoining =
2ct

Hint 60

dehydration
hyperkalemia

Front 61

Congenital adrenal hyperplasia (CAH):

Treatment includes immediate infusion of intravenous saline and long-term steroid hormone replacement, both =
2ct

Back 61

cortisol
mineral-corticoids

Front 62

Congenital adrenal hyperplasia (CAH):

Treatment includes immediate _________________and _______________ replacement, both cortisol and mineral-corticoids.

Back 62

infusion of intravenous saline

long-term steroid hormone