CH 12--DISEASES OF BONES--Bone 1/Disorders of Growth & Maturation Flashcards
BONE CELLS
3 types of cells
1. __________: bone-forming (present on surface)
2. _________: mature, adult, main type (able to form &
resorb bone)
3. __________: bone-destroying
Osteoblasts
Osteocytes
Osteoclasts
NORMAL STRUCTURE & FUNCTION
Periosteum– (dense outer connective tissue layer)
endosteum– (lining medullary cavities)
***Arranged lamellae are seen in NORMAL healthy bone
_________bone – is haphazard arrangement of lamellae with an ↑ number of osteocytes and is usually present with pathology.
Woven
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ marrow in: 1--ribs 2--vertebrae 3--pelvic bones 4--most proximal portions of the long bones
Intramembranous = ossification (bones of the skull)
Endochondral ossification = (limb bones)
Hematopoietic
HORMONAL CONTROL
Three main hormones regulate calcium & phosphate
metabolism:
1. _____________ hormone – bone resorption
2. _____________ – inhibits movement of calcium from bone
to blood
3. Vitamin D – absorption of calcium from the intestines
↓ vitamin D leads to ________ in children &
Osteomalacia in adults
Parathyroid
Calcitonin
Rickets
DISORDERS OF GROWTH & MATURATION
4 different groups:
1. Delayed \_\_\_\_\_\_\_\_\_ maturation disorders Cretinism Morquio's disease 2. Bone Modeling disorders osteopetrosis 3. Epiphyseal plate disorders Achondroplasia Scurvy Asymmetrical cartilage growth Kyphoscoliosis Osteomalacia 4. Delayed \_\_\_\_\_\_ Maturation disorders Osteogenesis imperfecta
cartilage
Bone
CONGENITAL BONE DISORDERS (hereditary)–1
1-OSTEOGENESIS IMPERFECTA (Ol) (______ ____ disease)
Etiology:
a—-Hereditary disorder of collagen synthesis leading to
formation of abnormal lamellar collagen.
b—-Insufficient synthesis of type _____collagen
Brittle bone
I
CONGENITAL BONE DISORDERS (hereditary)–2
1-OSTEOGENESIS IMPERFECTA (Ol) (Brittle bone disease)
Prevalence:
Estimate range from 20,000 to 50,000
TREATMENT:
- No cure for OI
- A surgical procedure called “_________” is frequently considered for individuals with OI by inserting metal rod through the length of long bones to strengthen them & prevent &/or correct deformity.
- ________ as much as possible to promote muscle & bone strength.
- maintain healthy weight, nutritious diet, avoid smoking, excessive
alcohol and caffeine consumption & taking steroid medications (all
of which may deplete bone & exacerbate bone fragility)
rodding
Exercise
CONGENITAL BONE DISORDERS (hereditary)–3
2- OSTEOPETROSIS—(Alber’s Schönberg disease) or
“_______ _________ disease”
a •Hereditary abnormality of _________ (rare disease)
b •___________and sclerosis of bones = INCREASED
density of the skeleton
c • Obliteration in the medullary cavity = anemia
d • Despite the “too much” bone, it breaks easily
(pathological fractures)
e. • Less bone resorption resulting in a net bone
OVERGROWTH
f • Bone deformities and nerve compression
manifestations (reduction of neural foramina, deafness)
Two patterns:
1. Malignant—- become evident in-utero and infancy ,
autosomal recessive
2. benign—- adult, autosomal dominant
Marble bone
osteoclasts
Overgrowth
CONGENITAL BONE DISORDERS (hereditary)–4
3- ACHONDROPLASIA
a. DECREASED cartilaginous growth
b. DWARFISM
c. Long bones are SHORT and relatively THICK
Epidemiology:
- It is the most common of a group of _______ _______ characterized by abnormal body proportions
- Only 20% of cases have a positive family history
- 80% is thought to be due to genetic mutation
- 1 in every 20,000 live births
growth defects
CONGENITAL BONE DISORDERS (hereditary)–5
3- ACHONDROPLASIA
Manifestations:
- Normal membranous ossification → normal skull,
facial bones & axial skeleton
- Limbs → much ________ than normal
- Narrow foramen magnum and spinal canal may →
accumulation of too much fluid in brain (hydrocephalus)
- High risk of repeated middle ear infection → hearing
loss.
*****Treatment:
- There is NO specific treatment for achondroplasia.
- Related abnormalities, including spinal stenosis and spinal cord compression, and _________ should be treated
when they cause problems.
shorter
hydrocephalus
CONGENITAL BONE DISORDERS (hereditary)–6
4-Osteochondroma
(Hereditary Multiple Exostosis)
a. An osteochondroma is a benign (noncancerous) tumor that develops during childhood or adolescence. It is an abnormal growth that forms on the surface of a bone near the growth plate.
Features:
a—— benign masses
b——The cross-sectional appearance of an osteochondroma shows the cap of calcified cartilage overlying poorly organized __________ bone.
c—–This bone growth, though ______, can sometimes
cause problems of pain and irritation that leads to removal surgically.
cancellous
benign