Paediatrics Flashcards

1
Q

When does growth occur?

A

Growth runs from infancy to adolescence (embryonic to adult life)

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2
Q

What makes up the soft tissues and hard tissues?

A
Soft tissues 
•	Muscles
•	Neurovascular structures
•	Joint capsules and ligaments
Hard tissues
•	Bones 
•	Joints
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3
Q

How can we diagnose growth plate limb deformities in children early?

A

Diagnosis:

Starts antenatal (early):
•	USS – relative limb length
•	Amniocentesis
•	CVS 
•	Genetics testing of foetus and maternal blood samples
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4
Q

Describe the process of Intramembranous bone formation such as flat bones in the skull

A
  • Ossification within membranes such as the cranial bones of the skull and clavicle
  • An ossification centre appears in the fibrous connective tissue membrane
  • Selected centrally located mesenchymal cells cluster and differentiate into osteoblasts, forming an ossification centre
  • Bone matrix (osteoid) is secreted from the fibrous membrane
  • Osteoblasts begin to secrete osteoid which is mineralised within a few days
  • Trapped osteoblasts become osteocytes
  • Woven bone and periosteum form
  • Accumulating osteoid is laid down between embryonic blood vessels, which form a random network. The result is a network (instead of lamellae) of trabeculae
  • Vascularised mesenchyme condenses on the external face of the woven bone and becomes the periosteum
  • Bone collar of compact bone forms and red marrow appears
  • Trabeculae just deep to the periosteum thicken, forming a woven bone collar that is later replaced with mature lamellar bone
  • Spongy bone, consisting of distinct trabeculae, persists internally and its vascular tissue becomes red marrow
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5
Q

Describe endochondral bone ossification

A
•	From cartilage
o	Hyaline cartilage
o	Primary ossification centre in diaphysis 
o	Secondary ossification centre in epiphysis 
o	Epiphyseal plate (growth plate)
•	5 phases
o	Phase 1 and 2:
	In utero
	Hyaline cartilage template
	Osteoblasts begin depositing bone
	Bone collar forms around diaphysis 
	Cartilage cells in centre die which leaves a cavity
	Primary ossification centre forms
o	Phase 3
	In utero
	Blood vessels penetrate into the centre of the cavity
	Fibroblasts enter through the blood
	Fibroblasts convert to osteoblasts 
	Spongy bone forms along shaft
o	Phase 4
	At birth
	Elongation of diaphysis
	Secondary ossification forms in epiphysis 
	Medullary (marrow) cavity forms
o	Phase 5
	Growth and maturation 
	Complete ossification of epiphyses
	Hyaline cartilage remains at:
•	Epiphyseal growth plate 
•	Articular surface
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6
Q

What is the growth plate?

A
  • Characteristic layered structure – unchanged by age or site
  • Endochondral ossification – organised conversion of cartilage to bone
  • Allows rapid longitudinal growth
  • Growth timings is variable
  • Metaphysis at bottom and epiphysis at top
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7
Q

What are the two types of growth plate?

A
Discoid
•	Primary growth plates of long bone 
•	Apophyses
•	Tension or compression forces
Spherical
•	Secondary centre ossification 
•	Carpel or tarsal bones
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8
Q

What is the growth plate divided into?

A

The growth plate is divided into multiple zones (Figure 8). These zones include the reserve zone, proliferative zone, and the hypertrophic zone. The reserve zone is the area closest to the secondary center of ossification. It has epiphyseal vessels the pass through this area but do not provide it with oxygen and keeps the oxygen tension low in this area. It has no known function with respect to longitudinal growth.

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9
Q

What are the growth plate zones

A

On image

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10
Q

What does the reserve zone do and where it located?

A

Closest to the surface (ie, nearest the epi- physis) is the reserve zone. The cells in this zone produce cartilaginous matrix, primarily in the form of type II collagen, which is used for eventual ossification into bone. These cells do not actively divide, nor are they very metabolically active; accordingly, they have the poorest blood supply.

  • Germinal cells of stem cell origin
  • Area of low oxygen tension responds to circulating hormones
  • Contributes towards secondary centre of ossification and discoid physis
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11
Q

What does the proliferative zone do?

A

Below the reserve zone, moving toward the metaphysis, is the proliferative zone. Within this zone, the cells are stacked in columns. These columns of cells synthesize proteoglycans, thereby contributing to the extra- cellular matrix. This region, characterized by synthesis and cell division, has the most ex- tensive blood supply within the growth plate. This blood supply provides nutrition, of course, but also allows hormonal signals to reach their targets in the growth plate effectively.

  • Chondrocytes thin discs
  • Cells palisade
  • High oxygen tension
  • Cell number correlate with growth rate
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12
Q

What does the hypertrophic zone do?

A

The third zone of the growth plate, the hypertrophic zone, lies closest to the calcified bone of the metaphysis. The cells in this zone are unusually large and plump; hence, the name “hypertrophic.” This zone of the growth plate is highly active metabolically, even though it has a poor blood supply. It therefore relies on anaerobic metabolism and uses stored glycogen as its source of energy. It is also the region that participates in mineralization of the cartilage. Calcium is stored in the cells in the upper levels of the hyper- trophic zones. In the lower levels of the hypertrophic zone, these cells liberate their calcium in order to assist in matrix mineralisation.

  • Proliferation changes to hypertrophy
  • Less extracellular matrix
  • Matrix mineralisation
  • Osteoconductive septa
  • Cell death
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13
Q

What is the zone of transformation?

A
  • Vascular invasion from metaphysis
  • Chondroclasts
  • Osteoblasts
  • Matrix ossification
  • It is remodelling as primary bone is replaced
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14
Q

What happens between the hypertrophic zone and the metaphysis

A

The actual process of matrix mineraliza- tion occurs at the interface between the hy- pertrophic zone and the metaphysis: the zone of provisional calcification. Within this area, vascular invasion allows osteoblasts to arrive and replace the calcified cartilage with bone. This bone is a primitive, less-organized form called woven bone. In time, this tissue will be replaced by mature lamellar bone via the process of bone remodeling. The distinction between woven and lamellar bone is one of material orientation: the fibers of woven bone are haphazard, whereas lamellar bone aligns the structure in the direction of load. Blood does not flow easily through the physis; the intramedullary blood supply does not reach the epiphysis or secondary centers of ossification. Accordingly, in children, whose growth plates are open, the main blood supply to the epiphysis is a direct epi- physeal artery. This artery loses its promi- nence once the growth plates close at skele- tal maturity.

Numerous circulating hormones affect growth plate activity. Thyroid hormone (thy- roxine), growth hormone, parathyroid hor- mone (PTH), calcitonin, and testosterone are among the hormones used to regulate growth plate physiology. These hormones can stim- ulate matrix synthesis, cell division, and cal- cification (and thus growth plate closure). The precise method and zone of action of these hormones are beyond the scope of this text. Even without entirely understanding their mechanisms of action, however, it is clear that abnormalities of these hormones can significantly affect the development of the human skeleton

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15
Q

Are the growth mechanisms known?

What 2 factors can influence growth?

A
  • Exact mechanisms unknown – we don’t know when a bone wants to stop growing
  • We only know how much of each bone contributes towards growth. Lower limb is mostly grown by knees, upper limb is from the humorous
  • Systematic factors
  • Local factors
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16
Q

Give some systemic factors that affect growth

A
  • Genetic predisposition – sex effect
  • General health – conditions have influenced growth e.g mineral deficiency
  • Hormonal influences – GH, T4 and Vit D
  • Neuromuscular
  • We can see changes in growth charts to identify pathological changes, we can intervene to bring them back to the correct centile
17
Q

Give some local factors that affect growth

A
  • Hormonal influences – paracrine and autocrine, IGF-1,2, BMP, PTHrP
  • Genetic factors
  • Vascular supply
  • Mechanical effects
18
Q

What does Hueter Volkmann law state?

What is mechanical alignment?

A

Hueter Volkmann law – compression forces inhibit growth whilst tensile forces stimulate growth
• Mechanical alignment to measure weight and compression through the limbs
• Salenlus curve (tibio-femoral angles) – knee angle chart
• Normal mechanical alignment at age 10 or 11
• We can make interventions follow changes on the chart

19
Q

What is Chondral modelling theory?

A

Physiological loading → need correction (feedback loops) - bone remodels in line of stress (loading causes it to grow)
Pathological loading → can cause progressive deformity due to dysregulation or imbalance of this process

20
Q

Describe the growth plate blood supply

A

Where the vascular supply meet the physis
• Germinal cells
• Central blood supply -75%
• Peripheral blood supply – 25%
• Blood supply to growth plate can be damaged – Perthes disease (main concern is function of the hip joint) (covered in hip lecture)

21
Q

What is Hemihypertrophy?

Give an example

A

Hemihypertrophy is a genetic disorder characterized by excessive overgrowth of one side of the body in comparison with the other.

E.g beckwith Wiedemann

22
Q

What is chromosome 11 involved with?

What can mutations of this chromosome cause?

A

Growth

mutations can cause overgrowth or undergrowth

23
Q

What does the paternal and maternal chromosome 11 cause?

A
  • Paternal – growth promotion e.g beckwith Wiedemann
  • Material - growth suppression e.g russel silver
  • Either the paternal gene causes it or maternal does on chromosome 11
24
Q

What is Uniparental disomy (UPD)?

A

Uniparental disomy (UPD) occurs when a person receives two copies of a chromosome, or part of a chromosome, from one parent and no copies from the other parent.

25
Q

What causes Beckwith-Wiedemann syndrome?

A

There are several known genetic causes of Beckwith-Wiedemann syndrome and isolated hemihypertrophy, which generally result in changes in the expression of one or more of the genes at a region of chromosome 11 known as 11p15. -> BIG

26
Q

What causes russel silver syndrome?

A

Russell-Silver syndrome is a growth disorder characterized by slow growth before and after birth. Babies with this condition have a low birth weight and often fail to grow and gain weight at the expected rate (failure to thrive). -> SMALL
The genetic causes of Russell-Silver syndrome are complex. The disorder often results from the abnormal regulation of certain genes that control growth. Research has focused on genes located in particular regions of chromosome 7 and chromosome 11.

27
Q

What is Rickets?

A
  • Normal matrix formation
  • No calcification
  • No osteoconduction
  • Widened hypertrophic zone

Pathophysiology. Rickets arises due to decreased availability of phosphorus and calcium to mineralize the skeletal matrix, leading to growth plate disorganization and accumulation of undermineralized osteoid. This results in growth plate expansion, bone weakening, and skeletal deformities.

28
Q

What is Osteopetrosis?

A

Osteopetrosis is a bone disease that makes bones abnormally dense and prone to breakage

  • Failure of osteoclast function
  • Primary bone not resorbed
29
Q

What is Radioulnar synostosis?

What is Tarsal coalition?

A

Radioulnar synostosis is a rare condition in which the two bones of the forearm — the radius and the ulna — are abnormally connected. This limits rotation of the arm. Radioulnar synostosis is usually congenital (something your child was born with). It can also occur as the result of a forearm fracture or trauma.

Tarsal coalition – failure bones to separate, in foot no inversion in foot

30
Q

What are the treatment principles for no growth plate following a fracture?

A

Treatment principles:
• Correct deformity for normal mechanical alignment
• Support brittle and weak bones due to previous law
• Treat them surgically and medically (e.g Vitamin D)
• Equalise leg lengths
• Increase height by growth rods
Correcting the leg length deformity:
• Line should pass through midline of knee joint
• Valgus malalignment
• Need an open growth plate to remodel
• Adult – surgery
• Child – guided growth, an eight plate (screws in epiphysis and metaphysis) is put into the physis to correct alignment. We need growth plates to be open. Stop growth on one side, and allow other side to compensate
• Only corrects based on growth
How can damage to the physis occur? Damage to physis e.g trauma, vascular insult, infection can

31
Q

What is blouts disease?

What is the goal of treatment?

A

Blount’s disease is a condition found in children that affects the growth plates around the knee. The disease causes the growth plate near the inside of the knee to either slow down or stop making new bone. Meanwhile, the growth plate near the outside of the knee continues to grow normally. The result is a bowlegged appearance in one or both legs.

The goal of treatment for Blount’s disease is to correct the deformity and improve overall alignment of the legs.

32
Q

What are the non-surgical and surgical treatments for blouts disease?

A

Nonsurgical Treatment
For young patients with infantile Blount’s disease, bracing can be effective. The goal of bracing is to guide the legs into a straighter position as the child grows. An improvement is usually noticed within 12 months of treatment. If the deformity is not corrected by the age of 4, surgery may be needed.

Surgery for Blount’s Disease
Surgery may be recommended if bracing doesn’t produce desired results. Children with severe deformities and those who are no longer candidates for bracing may also need surgery. Several surgeries are available to treat Blount’s disease, including osteotomies and hemiepiphysiodeses.

An osteotomy is a procedure that involves cutting and realigning the bone to put it in a more normal position. This type of surgery usually corrects the deformity immediately.

A hemiepiphysiodesis, on the other hand, corrects the deformity over time. It involves placing plates or staples on one side of the growth plate to stop the growth on that side. The plate guides the growth of the bone into a straighter position while the nonplated side continues to grow.

33
Q

How do we intervene malalignment?

A
  • Osteochondromas can affect the growth plate and cause malalignment
  • Correct with guided growth