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Embryology > Chapter 10 > Flashcards

Chapter 10 Flashcards

1
Q

In general, the skeletal system develops from ____ and ____ (parietal layer) mesoderm and from neural crest

A

paraxial and lateral plate

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2
Q

Paraxial mesoderm forms a ___ series of tissue blocks on each side of the neural tube, known as ___ in the head region and somites from the occipital region caudally

A

segmented - somitomeres

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3
Q

Somites differentiate into a ventromedial part, the ___, and a dorsolateral part, the ___

A

sclerotome - dermomyotome

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4
Q

At the end of the fourth week, sclerotome cells become polymorphous and form loosely organized tissue, called ___, or embryonic connective tissue

A

mesenchyme

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5
Q

Mesenchyme may become fibroblasts, chondroblasts, or ____ (bone-forming cells)

A

osteoblasts

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6
Q

In some bones, such as the flat bones of the skull, mesenchyme in the dermis differentiates directly into bone, a process known as ____

A

intramembranous ossification

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7
Q

The skull can be divided into two parts: the ____, which forms a protective case around the brain, and the ____, which forms the skeleton of the face

A

neurocranium - Viscerocranium

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7
Q

Neurocranium, Divided into two portions: (1) the membranous part, consisting of ___, which surround the brain as a vault, and (2) the ____, or _____, which forms bones of the base of the skull

A

flat bones
cartilaginous part - chondrocranium

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8
Q

The result is formation of a number of flat, membranous bones that are characterized by the presence of needle-like ___

A

bone spicules

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9
Q

At birth, the flat bones of the skull are separated from each other by narrow seams of connective tissue, the ___

A

sutures

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10
Q

Ossification centers appear in these cartilage models, and the bone gradually ossifies by ____

A

endochondral ossification

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11
Q

The most prominent of these is the ___, which is found where the two parietal and two frontal bones meet

A

anterior fontanelle

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11
Q

At points where more than two bones meet, sutures are wide and are called ___

A

fontanelles

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12
Q

Sutures and fontanelles allow the bones of the skull to overlap (____) during birth

A

molding

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13
Q

Those that lie in front of the rostral limit of the notochord, which ends at the level of the pituitary gland in the center of the sella turcica, are derived from neural crest cells. They form the ____.

A

prechordal chondrocranium

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14
Q

Those that lie posterior to this limit arise from occipital sclerotomes formed by paraxial mesoderm and form the ____

A

chordal chondrocranium

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15
Q

The first arch gives rise to a dorsal portion, the ___, which extends forward beneath the region of the eye and gives rise to the ___, the ___, and part of the ____

A

maxillary process - maxilla, zygomatic bone, temporal bone

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16
Q

The ventral portion, the ____, contains the Meckel cartilage.

A

mandibular process

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17
Q

The ventral portion, the mandibular process, contains the ___.

A

Meckel cartilage

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18
Q

Mesenchyme around the Meckel cartilage condenses and ossifies by intramembranous ossification to give rise to the ___

A

mandible

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19
Q

The Meckel cartilage disappears except in the ____ligament

A

sphenomandibular

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20
Q

The dorsal tip of the mandibular process, along with that of the second pharyngeal arch, later gives rise to the ___, ___, and the ___

A

incus, the malleus, and the stapes

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21
Q

Originating in the neuroectoderm form the facial skeleton and part of the skull. These cells also constitute a vulnerable population as they leave the neuroectoderm; Often a target for teratogens

A

Neural Crest Cells

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22
Q

The cranial vaultfails to form [___], and brain tissue exposed to amniotic fluid degenerates, resulting in anencephaly

A

[cranioschisis]

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23
Q

The cranial vaultfails to form [cranioschisis], and brain tissue exposed to amniotic fluid degenerates, resulting in _____

A

anencephaly

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24
Q

____ is caused by failure of the cranial neuropore to close

A

Cranioschisis

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25
Q

Children with relatively small defects in the skull through which meninges and/or brain tissue herniate [___ and ____] may be treated successfully

A

cranial meningocele and meningoencephalocele

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26
Q

Caused by premature closure of one or more sutures

A

Craniosynostosis

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27
Q

Loss of function mutations in EFNB1 causes ___, characterized by coronal suture synostosis and hypertelorism

A

craniofrontonasal syndrome

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28
Q

Mutations in MSX2 cause ____ that can affect a number of sutures

A

Boston-type craniosynostosis

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29
Q

mutations in TWIST1 cause ____, characterized by coronal suture synostosis and polydactyly

A

Saethre-Chotzen syndrome

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30
Q

___ and ___ play important roles in most of skeletal development

A

Fibroblast growth factors [FGFs] and fibroblast growth factor receptors [FGFRs]

31
Q

Signaling is mediated by the receptors, which are ____, each of which has three extracellular immunoglobulin domains, a transmembrane segment, and a cytoplasmic tyrosine kinase domain

A

transmembrane tyrosine kinase receptors

32
Q

___ is expressed in the cartilage growth plates of long bones and in the occipital region.

A

FGFR3

33
Q

Premature closure of the coronal sutures [20% to 25% of cases] results in a short skull called ____

A

brachycephaly

33
Q

Mutations in these receptors, which often involve only a single amino acid substitution, have been linked to specific types of ____ [FGFR1, FGFR2, and FGFR3] and several forms of ___ [FGFR3]

A

craniosynostosis - skeletal dysplasia

34
Q

The shape of the skull depends on which of the sutures closes prematurely. Early closure of the sagittal suture [57% of cases] results in frontal and occipital expansion, and the skull becomes long and narrow [____]

A

scaphocephaly

35
Q

If the coronal sutures close prematurely on one side only, then the result is an asymmetric flattening of the skull called ___

A

plagiocephaly

36
Q

___ is the most common neonatal lethal form of skeletal dysplasia [1/40,000 live births]

A

Thanatophoric dysplasia

36
Q

_____, the most common form of skeletal dysplasia [1/20,000 live births], primarilyaffects the long bones. Other skeletal defects include a large skull [megalocephaly] with a small midface, short fingers, and accentuated spinal curvature

A

Achondroplasia [ACH]

36
Q

There are two types; both are autosomal dominant. ____ is characterized by short, curved femurs with or without cloverleaf skull; ___ individuals have straight, relatively long femurs and severe cloverleaf skull caused by craniosynostosis.

A

Type I - type II

37
Q
A
38
Q

Another term for cloverleaf skull is ___. It occurs when all of the sutures close prematurely, resulting in the brain growing through the anterior and sphenoid fontanelles

A

kleeblattschadel

38
Q

___is an example of a generalized dysplasia of osseus and dental tissues that is characterized by late closure of the fontanelles and decreased mineralization of the cranial sutures resulting in bossing [enlargement] of the frontal, parietal, and occipital bones

A

Cleidocranial dysostosis

39
Q

A typical vertebra consists of a ___ and ___ (through which the spinal cord passes),___, ____, and usually a ___

A

vertebral arch and foramen, a body, transverse processes, spinous process

39
Q

____, another autosomal dominant form ofskeletal dysplasia, appears to be a mildertype of ACH

A

Hypochondroplasia

40
Q

___ is caused by congenital hyperpituitarism and excessive production of growth hormone

A

Acromegaly

41
Q

__ is usually an abnormality in which the brain fails to grow and, as a result, the skull fails to expand .

A

Microcephaly

42
Q

During the 4th week, sclerotome cells migrate around the spinal cord and notochord to merge with cells from the opposing somite on the other side of the neural tube. As development continues, the sclerotome portion of each somite also undergoes a process called ___

A

resegmentation

42
Q

Mesenchymal cells between cephalic and caudal parts of the original sclerotome segment do not proliferate but fill the space between two precartilaginous vertebral bodies. In this way, they contribute to formation of the ___

A

intervertebral disc

43
Q

Notochord regresses entirely in the region of the vertebral bodies, it persists and enlarges in the region of the intervertebral disc. Here, it contributes to the ___, which is later surrounded by circular fibers of the annulus fibrosus

A

nucleus pulposus

43
Q

Later, two secondary curves are established: the cervical curvature, as the child learns to hold up his or her head, and the ___, which forms when the child learns to walk

A

lumbar curvature

43
Q

As the vertebrae form, two primary curves of the spine are established: the ___ and ___

A

thoracic and sacral curvatures

44
Q

The process of formation and rearrangement of segmental sclerotomes into definitive vertebrae is complicated, and it is fairly common to have two successive vertebrae fuse asymmetrically or have half a vertebra missing, a cause of ___ [lateral curving of the spine]

A

scoliosis

45
Q

the number of vertebrae is frequently more or less than the norm. In ___, the cervical vertebrae are fused causing reduced mobility and a short neck

A

Klippel-Feil sequence

46
Q

A more severe abnormality is ___, in which the neural tube fails to close, vertebral arches fail to form, and neural tissue is exposed

A

spina bifida cystica

46
Q

One of the most serious vertebral defects is the result of imperfect fusion or nonunion of the vertebral arches. Such an abnormality, known as ___ [___], may involve only the bony vertebral arches, leaving the spinal cord intact.

A

cleft vertebra [spina bifida]

46
Q

the bony defect is covered by skin, and no neurological deficits occur [___]

A

spina bifida occulta

47
Q

Costal cartilages are formed by sclerotome cells that migrate across the ___ into the adjacent lateral plate mesoderm

A

lateral somitic frontier

48
Q

___ occur in approximately 1% of the population and are usually attached to the seventh cervical vertebra. Because of its location, this type of rib may impinge on the brachial plexus or the subclavian artery, resulting in varying degrees of anesthesia in the limb

A

Cervical ribs

49
Q

___ is a very rare defect and may be complete or located at either end of the sternum. Thoracic organs are covered only by skin and soft tissue. The defect arises when the sternal bands fail to grow together in the midline

A

Cleft sternum

50
Q

___ is the term for a depressed sternum that is sunken posteriorly

A

Pectus excavatum

51
Q

___ refers to a flattening of the chest bilaterally with an anteriorly projecting sternum. The projection of the sternum resembles the keel of a boat.

A

Pectus carinatum

52
Q

Craniosynostosis, broad great toes and thumbs, Cloverleaf skull, underdeveloped face

A

Pfeiffer syndrome

53
Q

Gene and chromosome of Pfeiffer syndrome

A

FGFRl -8p12

54
Q

Gene and chromosome of Pfeiffer syndrome, Apert syndrome, Jackson-weiss syndrome, and crouzon syndrome

A

FGFR2 - 10q26

54
Q

Craniosynostosis, underdeveloped face, symmetric syndactyly of hands and feet

A

Apert syndrome

54
Q

Craniosynostosis, underdeveloped face, foot anomalies, hands usually spared

A

Jackson—Weiss
syndrome

54
Q

Craniosynostosis, underdeveloped face, no foot or hand defects

A

Crouzon syndrome

55
Q

Short—limb dwarfism, underdeveloped face

A

Achondroplasia [ACH]

56
Q

Curved short femurs, with or without cloverleaf skull

A

Thanatophoric
dysplasia [type I]

57
Q

Relatively long femurs, severe cloverleaf skull

A

Thanatophoric
dysplasia [type II]

58
Q

Milder form of ACH with normal craniofacial features

A

Hypochondroplasia

59
Q

Craniosynostosis, midfacial hypoplasia, cleft palate, vertebral anomalies, hand and foot abnormalities

A

Saethre—Chotzen
syndrome

60
Q

Small, short digits, divided uterus, hypospadias

A

Hand—foot—genital
syndrome

61
Q

Fused, multiple digits

A

Synpolydactyly

62
Q

Digit defects, absent radius, limb bone hypoplasia, atrial and ventricular septal defects, conduction abnormalities

A

Upper limb and heart
defects

63
Q

Shortening, bowing, and hypomineralization of the long bones, blue sclera

A

Limb defects, blue
sclera

64
Q

Long limbs and face, sternal defects [pectus excavatum and carinatum], dilation and dissection of the ascending aorta, lens dislocation

A

Marfan syndrome

Embryology (21 decks)

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