Development of skeletal system Flashcards

1
Q

Vertebrae form as _______ derived from adjacent sclerotomes

A

Intersegmental structures

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2
Q

Spinal nerves emerge between the vertebrae and grow out to contact _______

A

myotomes

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3
Q

Cervical spinal nerves exit _______ vertebra of same number

A

above

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4
Q

Their are originally how many cervical somites

A

8

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5
Q

Fusion of somites results in how many cervical vertebra

A

7

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6
Q

______ form neural arches surrounding the developing spinal cord

A

Sclerotomes

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7
Q

what is centrum

A

Vertebral body

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8
Q

The notochord regresses except where

A

it is incorporated into the intervertebral discs (nucleus pulposus)

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9
Q

Congenital scoliosis can be be due to what 3 causes

A

Failure of formation, failure of segmentation, or a combination of both.

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10
Q

What is scoliosis

A

Abnormal lateral curvature of the spine, resulting from disruption of normal vertebral development

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11
Q

What leads to hemivertebrae and congenital scoliosis

A

semisegmented, fully segmented, wedge vertebrae

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12
Q

What leads to block vertebrae and congential scoliosis

A

unsegmented bars

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13
Q

Ribs arise form

A

zones of condensed mesenchyme lateral to the body of the vertebra

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14
Q

Ribs develop from

A

Costal processes, in the thoracic region only

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15
Q

The sternum develops from

A

From cartilaginous sternal bars in ventral body wall
fuse with on another in crainal-caudal direction
Forms manubrium, body and xiphoid process

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16
Q

The sternum fuses with one another in what direction

A

cranial-caudal direction

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17
Q

What is Pectus Carinatum

A

an overgrowth of cartilage causing the sternum to protrude
costal cartilage overgrowth restricts the expansion of the ribs and pushes the sternum inward
can be evident at birth; most common during growth spurt.
1 in 500-1000 children; 3x males

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18
Q

What is a Sternal Foramen

A

Ossification anomaly found in 4-10% of population
can be mistaken for bullet wound or sternal disease by CT scan (note bullet would show other fractures)
Common acupuncture point (so can lead to death if not known)
Directly over the heart

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19
Q

Anomalies in appendicular may be due to

A

disturbances in specific cellular or molecular interactions

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20
Q

Initiation of limb development begins in what week

A

lat in the 4th week

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21
Q

Positioning of the limbs along the craniocaudal axis is regulated by the ____ expresed along this axis

A

HOX genes

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22
Q

HOX genes are expressed in _______ patterns from head to tail

A

overlapping patterns

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23
Q

Once positioning along the craniocaudal axis is determined, gowth must be regulated along the _____, ______, and ______ axes

A

Proximodistal, anteroposterior, dorsoventral

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24
Q

Limb outgrowth is initiated by _____ and ____ in the forelimb secreted by lateral plate mesoderm cells

A

TBX5, FGF10

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25
Q

Limb outgrowth is initiated by ___ and ___ in the hindlimb secreted by lateral plate mesoderm cells

A

TBX4, FGF10

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26
Q

What is Meromelia

A

Part of the limb is missing

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27
Q

What is amelia

A

all of the limb is missing

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28
Q

What is phocomelia

A

Feet and hands arise close to the trunk

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29
Q

what is Mesomelia

A

shortened forearm or leg elements

typically diagnosed with fetal sonography at 20 weeks

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30
Q

What is radial club hand

A

congenital absence or hypoplasia of the radial structures of the forearm and hand
hypoplastic or absent muscular structures and radial nerve
treatment is best if early passive stretching exercises and corrective casing is started

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31
Q

How is the Apical Ectodermal Ridge (AER) formed

A

once limb outgrowth is initiated, BMPs, expressed in ventral ectoderm, induce formation of the apical ectodermal ridge (AER)

32
Q

Limb bud has a strict pattern and _____

A

polarity

33
Q

Development of limb is organized around ___ axes

A

3

34
Q

The limb tissue differentiation is controlled by 2 regions called

A

apical ectodermal ridge (AER)

Zone of polarizing activity (ZPA)

35
Q

The progress zone is the site where

A

mitosis and limb lengthening occurs

36
Q

After the Apical ectodermal ridge (AER) is established, t expresses ___ and ___, which maintain the progress zone

A

FGF4, FGF8

37
Q

What is the name of the rapidly proliferating population of mesenchyme cells adjacent to the ridge

A

Progress zone

38
Q

Distal growth of the limb is affected by the rapidly proliferating cells (progress zone) which are under the influence of

A

FGFs

39
Q

The zone of polarizing activity (ZPA) is made up of what

A

mesodermal cells located at base of limb bud

40
Q

The Zone of polarizing activity (ZPA) produces ____ which initiates expression of SHH

A

Retionic acid

41
Q

Retionic acid initiates expression of ____ in the ZPA

A

SHH

42
Q

What is the function of the ZPA

A
Directs organization of limb bud and patterning of digits 
Determines anterior (preaxial/thumb), posterior (postaxial)
43
Q

Misexpression of retinoic acid or SHH results in a ____

A

mirror image duplication of the limb structures

44
Q

What is polydactyly

A

Extra digits

45
Q

What are some things that lead to duplication defects

A

misexpression of RA and or SHH

duplication of AER (apical ectodermal ridge)

46
Q

polydactyly is most common in which digit (s)

A

medial or lateral

in foot the extra digit is usually lateral

47
Q

Is polydactyly dominant or recessive

A

dominant trait

48
Q

Limb grows and develops in what direction

A

proximal to distally

49
Q

what regulates type/shape of bones

A

HOX genes

50
Q

The apical ectodermal ridge (AER) secretes FGFs that influence the closest cells (progress zone) to development into ___ structures

A

distal

51
Q

What is syndactyly

A

Webbed fingers or toes
failure of programmed apoptosis in digital ray
most common limb abnormality

52
Q

In what digits is syndactyly most common

A

between the 3rd and 4th fingers and 2nd and 3rd toes

53
Q

IS syndactyly dominant or recessive

A

simple dominant or simple recessive

54
Q

What is Brachydactyly

A

Reduction in length of phalanges
Inherited as dominant trait
short stature
uncommon

55
Q

What is cleft hand or foot

A

Uncommon
Lobster-claw
absence of one or more central digits or between digits 2 and 4
Lobster claw deformity- hand or foot is divided into 2 parts that oppose each other
-Remaining digits are partly or completely fused

56
Q

The upper limbs rotate 90 degrees _____

A

Laterally

57
Q

The lower limbs rotate 90 degrees ___

A

medially

58
Q

What is sirenomelia

A

Mermaid syndrome

59
Q

What is achondroplasia

A

Most prevalent form of dwarfism
Mutation on FGF-R3
Pathological changes at epiphyseal plate; zones of proliferation and hypertrophy are narrow and disorganized

60
Q

Achondroplasia is what form of reccessivity

A

Autosomal dominant

61
Q

Osteogenesis Imperfecta

A

also known as brittle bone disease
bone fragility that predisposes to fractures and deformities related to CT abnormalities
Blue sclera
not always evident at birth
ED must rule out first in causes of possible child abuse

62
Q

What is Talipes Equinovarus

A

Club foot
most common type of malformation
1/1000 births, two times more frequent in males
the sole of the foot is turned medially and the foot is inverted

63
Q

What is flexible club foot

A

Results from abnormal positioning or restricted movement of the lower limbs in utero
Feet are structurally normal
These abnormalities usually correct themselves spontaneously

64
Q

What is rigid club foot

A

abnormal development of the ankle and foot joints during the 6th and 7th week of development
Bony deformities particularly in the talus

65
Q

How do you treat Talipes Equinovarus

A

over 2-3 months, on a daily basis, devices systematically revolve the feet into their proper positions

66
Q

Zones of condensed mesenchyme lateral to the body of the vertebra form

A

The ribs

67
Q

Once limb outgrowth is initiated, BMPs, expressed in ventral ectoderm, induce formation of the

A

Apical ectodermal ridge (AER)

68
Q

What is the progress zone’s function in limb development

A

it is where mitosis and limb lengthening occurs

69
Q

The development of the lower limbs is how far behind that of the upper limbs

A

1 to 2 days

70
Q

On the 7th week of gestation the upper limbs rotate

A

90 degrees laterally

71
Q

On the 7th week of gestation the lower limbs rotate

A

90 degrees medially

72
Q

Misimpression of SHH in the anterior margin of a limb containing a normally expressing ZPA in the posterior border results in

A

A mirro image duplication of limb structures

73
Q

What weeks are the most sensitive period for induction of limb defects

A

The 4th and 5th weeks

74
Q

The AER directs limb bud organization along ______ while maintaining _______ axes

A

proximo-distal, dorsal/ventral axes

75
Q

No AER means

A

no limb development

76
Q

If AER is working normally but the Progress zone is not developing then if it starts developing after some time what happens

A

Distal structures form ex. like a hand