REVIEW OF

MUSCULOSKELETAL
SYTEM
Biochemistry of Bone
Cartilage & Muscle

Standar Kompetensi
Dokter
Permasalahan / Keluhan:

Nyeri sendi
Sakit punggung
Nyeri Pinggang
Nyeri otot
Bengkak
Sendi (Kaku,bengkak, kelainan bentuk
Gerakan terbatas
Patah tulang, dll

Standar kompetensi
Dokter
DAFTAR PENYAKIT :

Duchene muscular Dystrophy 1

Arthritis 4
Marfan Syndrom 1
Osteogenesis imperfecta 1
Achondroplasia 1
Rheumatoid Arthritis 2
Osteoporosis 3A
Rikets, Osteomalacia 1
Gout 3 A ,dll

ACHONDROPLASIA

DUCHENNE MUSCULAR
DYSTROPHY

Duchenne Muscular
Dystrophy
A severe recessive X-linked

Rapid progression of muscle degeneration

Eventually loss of ambulation death
Prevalent is one in 4000 males
In general male. Carriers female
Female if father & mather affected
Mutation in the dystrophin gene Xp 21

codes for the protein dystrophin

structure stability to dystroglycan complex
(DGC) located on the cell membrane

Duchenne Muscular
Dystrophy
Symptoms:

Appear in male, before age 5

Progressive proximal muscle weakness of the

legs & pelvis loss of muscle mass first

Early sign s pseudohypertrophy, low
undurance
Last : muscle tissue wasting fibrosis
By age 10 aid in walking wheelchair by age
12
Late symptoms: skeletal deformities, intelectual
impairment
Surviving teens to mild 20s, rare age 40 th

MARFAN SYNDROME
Genetic disorder off connectic tissue

(fibrilin-1)
Unussually tall with long limbs & long
thin finger
Dominant gen FBN1
Defects on the heart valve & aorta,
lung,eyes,spinal.skeleton & hard palate
Indicate 1/ 3000 -5000,1/20.000 birth
Long limbs, dislocated lenses, aortic
root dilation,arachnodactily

Myasthenia Gravis
An autoimmune neuromuscular disease
Fluctuating muscle weakness & fatiguability
Weakness is caused by circulating antibody

block Ach reseptor at post synaptiv NMJ

Inhibiting the stimulate effect of the NT Ach
Incidence 3- 30 cases / million
R/ cholinesterase inhibitors or
immunosuppressants
But not response to Cong.myasthenic synd.

Myasthenia Gravis
Sign & Symptoms
Muscle become progressively

weakness during activity & improve

after periods of rest
Eye &eyelid movement, facial
expressions, chewing, talking &
swallowing
The onset sudden,
Often symtoms are intermittent

OSTEOGENESIS IMPERFECTA
Called = Brittle bone disease

substaining multiple broken bones in

child
Causes a defect in the production of
collagen type I
Type I collagen is found in bone, sclera,
ligament & teeth
Its a dominant mutation
Is due a genetic mutation in the gene to
produce Collagen

OSTEOGENESIS IMPERFECTA
Signs & Symptoms
Bone fragility, scoliosis
Tooth defect, loose ligaments
Bluish sclera, hearing deficits
Dll
R/: Biphosphanates & Ca suplements
R/ genetic & Growth Hormon

Regulation of Calcium
Homeostasis

COMPARISON OF TWO TYPES OF DENSE

CONNECTIVE TISSUE BONE & CARTILAGE
Characteristics

Mechanical
properties

Cartilage

Bone

Rigid but flexible

Hard and strong

Chondrocytes

Osteocytes

Composition of
Matrix

Chondroitin
sulfate

Hydroxyapatite

Vascularization

Avascular

Vascular

No

Yes

Perichondrium

Periosteum

Cell type

Innervation
Covering

19

berfungsi menurunkan absorpsi kalsium di usus halus

adalah:
a. Kalsitonin
c. Kalsitriol
b.Vit D
d. PTH
e.ADH
2.Komponen utama tulang terdiri atas :
a.Kolagen tipe I dan Ca Hidroksiapatit
b Kolagen tipe I dan Ca Hidroksiapatit
c.Kolagen tipe III dan Ca Hidroksiapatit
d.Kolagen tipe IV dan Ca Hidroksiapatit
e.kolafen tipe II dan Proteoglikan
3.Tempat simpanan dinamis Kalsium di tubuh kita adalah :
a. Otak
c. Otot
e.Tulang
b, Hati
e. Usus halus
1.Yang

TERIMA KASIH