Exam 3 Material Flashcards

(62 cards)

1
Q

The yolk sac endoderm gives rise to…

A

gut epithelium and glands.

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2
Q

The smooth muscles and connective tissue of the digestive system are derived from…

A

splanchnic mesoderm

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3
Q

Esophageal atresia

  • What is it?
  • What causes it?
  • Symptoms
A
  • Esophagus ending in closed tube
  • Caused by tracheo-esophageal septum deviating posteriorly
  • Polyhydramnios often associated with this
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4
Q

Dorsal mesogastrium forms what in the adult?

A
  • Gastrosplenic ligament
  • Splenorenal ligament
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5
Q

Hypertrophic pyloric stenosis

  • What is it?
  • What causes it?
  • Symptoms
A
  • Excessive growth of pyloric sphincter
  • Caused when pyloric smooth muscle hypertrophies, obstructing food passage
  • Symptoms include projectile vomiting after feeding
    • Small knot can be felt at right costal margin
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6
Q

Duodenal stenosis

  • What is it?
  • What causes it?
A
  • Narrowing of lumen of duodenum
  • Caused by failed recanalization
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7
Q

Duodenal atresia

  • What is it?
  • What causes it?
  • Associated with…
  • Symptoms
  • Radiograph
A
  • Occlusion of duodenal lumen
  • Caused by failed recanalization
  • Associated with Down’s syndrome
  • Symptoms:
    • Polyhydramnios due to low intestinal reabsorption of amniotic fluid
    • Bilious vomiting shortly after birth
    • No abdominal distension
  • Double-bubble sign shown on prenatal ultrasonography
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8
Q

The adult pancreas is derived from…

A

dorsal and ventral pancreatic buds that merge when ventral bud is brought posterior due to duodenal rotation.

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9
Q
  • The ventral pancreatic bud contributes to…
  • The ventral bud duct forms the…
A
  • pancreatic head, uncinate process
  • main pancreatic duct
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10
Q
  • The dorsal bud forms…
  • The dorsal bud duct may persist as…
A
  • the body and tail of the pancreas
  • the accessory pancreatic duct
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11
Q

Annular pancreas

  • What is it?
  • What causes it?
  • Symptoms
A
  • Ring of pancreatic tissue around duodenum
  • Caused when ventral pancreatic bud splits to fuse with dorsal bud both ventrally and dorsally
  • Symptoms include duodenal obstruction shortly after birth
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12
Q

Hepatic diverticulum

  • Cranial end forms what?
  • Caudal end forms what?
A
  • Cranial portion forms liver
  • Caudal portion forms gallbladder and cystic duct
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13
Q

Extrahepatic biliary atresia

  • What is it?
  • Symptoms
A
  • Obliteration of the bile ducts
  • Symptoms:
    • Jaundice soon after birth
    • Acholic stools
    • Dark urine
    • Enlarged, firm liver
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14
Q
  • Malrotation of the midgut presents with…
  • What does it predispose infant to?
A
  • Intestinal obstruction shortly after birth
    • Bilious vomiting
    • Distension
  • Can predispose infant to volvulus, where twisting of intestines around short mesentery, often interfering with intestinal blood supply.
    • Can cause necrotic bowel (presents witn peritonitis)
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15
Q

Congenital omphalocele

  • What is it?
  • What causes it?
  • Symptoms
  • What is it associated with?
A
  • Persistence of midgut herniation in umbilical cord
  • Caused by failure of intestines to return to abdominal cavity
  • The herniated gut is enclosed by umbilical cord epithelium
  • Associated with:
    • Trisomy 13, 18, 21
    • Congenital heart disease (TOF, ASD)
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16
Q

Umbilical hernia

A

Occurs when midgut hernia reduces normally, but herniates again through imperfectly closed umbilicus.

Covered by abdominal epithelium

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17
Q

Gastroschisis

  • What is it?
  • What causes it?
  • What is it associated with?
A
  • Abdominal viscera extrude through split in abdominal wall usually to right side
    • Extruded bowel appears matted, edematous, thick with shaggy membrane (“peel”) over it
  • Causedy by incomplete embryonic folding
  • Associated with prematurity
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18
Q

Meckel’s diverticulum

  • What is it?
  • Symptoms
  • What is the rule of 2’s?
A
  • Persistence of proximal yolk stalk
    • most common anomaly of GI tract
  • May become inflamed and present like appendicitis. Can produce ulceration and bleeding
  • Rule of 2’s
    • Found in 2% of population
    • Usually occurs within 2 feet of ileocolic valve
    • 2 types of ectopic tissue (gastric and pancreatic)
    • 2 yo is when most commonly diagnosed
    • 2 inches long
    • More common in males than females (2:1 ratio)
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19
Q

Anorectal agenesis

A

Condition where rectum ends too far superior, either blindly or with a fistula to the bladder, urethra, vagina, or vestibule.

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20
Q

Hirschsprung’s disease (Congenital megacolon)

  • What is it?
  • What causes it?
  • Symptoms
A
  • Neurological dysfunction that affects colon
  • Caused by absence of autonomic ganglia in distal bowel (anus) causing failure of peristalsis in aganglionic segment
  • Symptoms:
    • Enlargement of colon due to accumulation of intestinal contents can occur
    • Constipation
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21
Q

What gives rise to nephric structures of the embryo, portions of the suprarenal glands, the gonads, and teh genital duct system?

A

Intermediate mesoderm (nephrotome)

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22
Q

What part of the urogenital ridge gives rise to the urinary system? Which part gives rise to the genital system

A
  • Nephrogenic cord gives rise to the urinary system
  • Gonadal ridge gives rise to the genital system
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23
Q

What are the three sets of nephric systems developing from the nephrogenic cord?

A
  • Pronephroi: rudimentary and nonfunctional
  • Mesonephroi: functions briefly during early fetal period
  • Metanephroi: forms permanent kidneys
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24
Q

What structures form in the male and female from the mesonephric tubules?

A
  • Males: Efferent ductules of testis
    • Paradidymis
  • Females: Epoophoron
    • Paroophoron
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25
What structures in the male and female form from the mesonephric duct?
* Males: Appendix of epididymis * Duct of epididymis * Ductus deferens * Ureter, pelvis, calices, and collecting tubules * Ejaculatory duct and seminal gland * Females: Appendix vesiculosa * Duct of epoophoron * Longitudinal duct; Gartner duct * Ureter, pelvis, calices, and collecting tubule
26
**Renal agenesis** * What is it? * What causes it?
* Failure of kidney development * Caused by failure of ureteric buds to develop or primordia of ureters to degenerate
27
**Unilateral renal agenesis** * What sex does it occur most commonly in? * Which kidney is most often affected? * Symptoms
* More common in males than females * Left kidney * Often asymptomatic due to right kidney compensating and hypertrophying. * Suspected in infants with single umbilical artery
28
**Bilateral renal agenesis** Symptoms
* Oligohydramnios * Incompatible with postnatal life * Severe pulmonary hypoplasia
29
**Ectopic kidneys** * What is it? * What causes it?
* One or both kidneys in abnormal position * Most often in pelvis * Can be caused by malrotation of kidneys * Pelvic kidneys caused by failure of kidney to ascend
30
**Discoid ("pancake") kidney** What is it?
Pelvic kidneys that fuse due to being too close to each other
31
**Horseshoe kidney** * What is it? * What causes it? * Where is it located? * Symptoms
* Two kidneys fuse to form U-shaped horseshoe kidney that crosses over anterior side of aorta * Caused by fusion of inferior poles of two metanephroi during ascent * Becomes caught under IMA and does not ascend to normal site * Usually asymptomatic unless urinary flow is impeded. Then shows signs of obstruction and/or infection
32
**Autosomal recessive polycystic kidney disease** * What is it? * What causes it? * Symptoms
* Kidneys contain many small cysts * Caused by autosomal recessive gene * Results in renal failure early in life * Often die shortly after birth unless dialysis and kidney transplant is performed
33
**Autosomal dominant polycystic disease** * What is it? * What causes it? * Symptoms
* Cysts arising from all regions of nephrons * Caused by autosomal dominant gene * Renal failure usually occurs in adults. * Associated with cysts forming in kidneys as well as ductal epithelia in liver, pancreas, testis, and ovary
34
**Duplicated ureter** * What is it? * What causes it? * Symptoms
* Duplication of abdominal part of ureter with fused caudal end * Caused by premature bifurcation of ureteric bud before it reaches substance of metanephric blastema * Usually asymptomatic. Urine reflux from one branch into the other can cause urine stagnation which can lead to infections
35
**Ectopic ureter** * What is it? * Where does it usually open in males? In females? * Symptoms
* When a ureter does not enter the urinary bladder (not incorporated into the trigone) * In males, usually open into neck of bladder or prostatic part of urethra. Also possible in ductus deferens, prostatic utricle, or seminal gland * In females, usually into neck of bladder, urethra, vagina, or vestibule of vagina. * Symptoms include incontinence (urine dribbling from urethra in males and urethra and/or vagina in females) * Can predispose patient to UTI
36
The vesical part of the urogenital sinus forms what in the adult?
Urinary bladder
37
The pelvic part of the urogenital sinus forms what in the adult?
Urethra in neck of bladder, prostatic part of urethra in males, entire urethra in females
38
A patent allantois can cause what 4 conditions?
* Urachal fistula: Urine drains from umbilical orifice and increases risk of UTI * Umbilical urachal sinus: Can become infected and form abcesses * Vesicourachal diverticulum: increases risk of UTI * Urachal cyst: Usually asymptomatic unless it becomes infected. Can be life threatening if patent urachus ruptures into peritoneal cavity.
39
**Exstrophy of bladder** * What is it? * What causes it? * Correlations * Symptoms
* Exposure or protrusion of muscosal surface of posterior wall of bladder * Caused by incomplete median closure of inferior part of anterior abdominal wall. * Most often found in males * Associated with epispadius (urethra on dorsum of penis) * Associated with wide separation of pubic bones * Urine dribbles intermittently form everted bladder
40
**Congenital adrenal hyperplasia (CAH)** * What is it? * What causes it? * Symptoms
* Abnormal increase in cells of suprarenal cortex * Causes excessive androgen production * Caused by autosomal recessive disorder * Deficiency in biosynthesis of steroid hormones cause increase in release of adrenocorticotropin from pituitary gland * Causes maculinizaiton of external genitalia in females. * Both sexes can experience rapid growth and accelerated skeletal maturation
41
**Hypertrophic pyloric stenosis** * What is it? * What causes it? * Symptoms
* Lengthening and thickening of pyloric channel * Caused by hypertrophy and hyperplasia of pylorus * Symptoms: * Dilated stomach due to obstruction * Non-bilious, projectile vomiting. Hungry after vomiting (hungry vomiter) * Signs of dehydration (depressed fontanelles, dry mucous membranes, decreased tearing, poor skin turgor and lethargy)
42
**Intestinal atersia** * What is it? * What causes it?
* Closure of intestines * Caused by: * Complication of volvulus * Internal hernia * Inversion of intestine in on itself * Strangulation from omphalocele/gastroschisis * Healed intrauterine intestinal perforation
43
**Imperforate anus** * What is it? * What causes it?
* Absence of anal orifice and fistula with: * Perineum in males * Urinary tract in males * Vagina in females * Caused by failure of urorectal septum to completely separate allantois and hindgut
44
**Wilms Tumor** * What is it? * What causes it? * Symptoms
* Renal tumor of children (nephroblastoma) * Rapid growth and early metastasis * Caused by failure of metanephric tissue to differentiate into normal kidney tissue * Symptoms: * Asymptomatic abdominal mass * Blood in urine * Malaise, anemia, weight loss, hypertension * Enlarged scrotal veins due to occluded left renal vein
45
What is hypospadia? What is epispadia?
* Hypospadia: urethral opening on ventral side of penis * Caused by failure of urethra to completely cannulate * Epispadia: urethral opening on dorsum of penis
46
**Cryptorchidism** * What is it? * Symptoms
* Undescended testes (unilaterally or bilaterally) * Testes will usually descend by end of 1st year. * They will not develop properly within or just aoutside abdominal cavity due to high body heat.
47
**Ectopic Testes** * What is it? * Places they are found?
* Testes that do not descend into scrotum after descending through inguinal canal * Common alternative locations: * Superficial: External to external oblique m * Femoral: Proximal portion of medial or anterior thigh * Prepenile: Dorsal to penis * Transverse: Opposite of scrotum * Perineal: in perineum
48
**Hydrocele** * What is it? * What causes it? * Types and associations
* Peritoneal fluid from abdominal cavity in scrotum * Caused by patent processus vaginalis * Types: * Scrotal: associated with undescended testes * Spermatic cord
49
What is the female equivalent of prostatic utricle in male?
Uterovaginal primordium
50
What is the male equivalent of the epoophoron in the female?
Efferent ductules and duct of epididymis
51
What is the male equivalent of the Gartner's duct in the female?
Ductus deferens and ejaculatory duct in males
52
What causes development of a double uterus with a double vagina?
Complete lack of fusion of caudal paramesonephric ducts and sinovaginal bulbs
53
* What is a bicornuate uterus? * What causes it?
* 2 uterine horns entering common vagina * Caused by partial fusion of paramesonephric ducts
54
**Vaginal atresia** * What is it? * What causes it?
* Blocked vaginal lumen * Caused by failure of vaginal plate to canalize and form a lumen
55
**Aromatase deficiency** * What causes it? * Symptoms
* Defect in aromatase enzyme * Increase in androgens due to lack of conversion to estrogens * Causes virilization of mother and infant female
56
**Ovotesticular DSD** * What is it? * What genotype does it most commonly occur in? * What type of phenotype?
* Both ovaries and testes present (or variety of two) ("true hermaphroditism") * Mullerian and Wolffian structures present * Most commonly found in 46 XX genotype * Variable phenotype
57
**46 XX SRY+** * What is it? * What phenotype is displayed? * Symptoms
* Translocated SRY gene to X chromosome * Male phenotype * Higher rate of infertility and abnormal hormonal levels
58
**Gonadal dysgenesis** * What is it? * What genotype is most commonly associated w/this? * What phenotype is displayed? * Symptoms
* Underdeveloped and dysfunctioning gonads (streak gonads) * 46 XY * Phenotypic female - internal and external * Symptoms: * Delayed puberty * Gonadal failure
59
**5 alpha reductase deficiency** * What does it cause? * What phenotype is displayed?
* Causes no conversion of testosterone to DHT * Minimal external genital male development * At puberty, increased muscle mass, increase phallic size, onset of erections (guevedoce)
60
**Androgen insensitivity syndrome** * What is it? * What causes it? * Symptoms
* Complete or partial androgen receptor responsiveness * Caused by defect of androgen receptor * Symptoms: * Phenotypically female * No uterus and small vaginal pouch * Intra-abdominal testes * Little to no axillary or pubic hair * Normal serum testosterone levels for male
61
**Turner Syndrome** * What causes it? * Symptoms
* Complete or partial absence of second sex chromosome * Symptoms: * Short stature * Ovarian failure * Delayed puberty * Absence of puberty * Infertility
62
**Klinefelter Syndrome** * What causes it? * Symptoms
* Caused by extra X chromosome (47 XXY, 48 XXXY) * Symptoms: * Gynecomastia (enlarged breast on male) * Absence of motile sperm * Testicular failure * Small firm testes * Poor muscle development * Fat distribution more feminine than masculine * Normal amounts of pubic and axillary hair, but sparse facial hair * Taller than normal