METABOLIC BONE DISEASES

Osteoporosis
Osteoporosis is a disease characterized by increased
porosity of the skeleton resulting from reduced bone
mass.
May be localized to a certain bone or region, as in
disuse osteoporosis of a limb, or may involve the
entire skeleton, as a manifestation of a metabolic
bone disease.
Categories of Generalized Osteoporosis:

Primary

Postmenopausal

Senile

Secondary

ENDOCRINE DISORDERS

Hyperparathyroidism

Hypo or hyperthyroidism
Hypogonadism

Pituitary tumors

Diabetes, type 1

Addison disease

NEOPLASIA

Multiple myeloma
GASTROINTESTINAL DISORDERS

Malnutrition

Malabsorption

Hepatic insufficiency

Vitamin C, D deficiencies

Idiopathic

DRUGS

Anticoagulants
The bony trabeculae are thinner and more widely
separated than usual, resulting in an increased
susceptibility to fractures .
The major microscopic changes are thinning of the
trabeculae and widening of Haversian canals.
 Pathogenesis:
In adults there is a dynamic equilibrium between
bone formation by osteoblasts, maintenance by
osteocytes, and resorption by osteoclasts .
Osteoporosis occurs when the balance tilts in favor of
resorption.
Clinical Course :
Depends on which bones are involved.
Thoracic and lumbar vertebral fractures are
extremely common, and produce loss of height and
various deformities.
Pulmonary embolism and pneumonia are common
complications of fractures of the femoral neck,
pelvis, or spine.
Osteoporosis is difficult to diagnose because
1. It remains asymptomatic until skeletal fracture
occur.
2.It cannot be reliably detected in plain radiographs
until 30%-40% of bone mass has already
disappeared.
3.Serum levels of calcium, phosphorus, and alkaline
phosphatase are normal
Bone loss estimation can be done by specialized
radiographic techniques to assess density, e.g., dual-
energy absorptiometry and quantitative
computed tomography.
Osteoporosis prevention and treatment:
1.Adequate dietary calcium intake
2.Vitamin D supplementation.
3.Regular exercise regimen-starting before the age of
30-to increase the peak bone density.
4.Bisphosphonate administration .
5.Selective estrogen receptor agonists. 6.Parathyroid
hormone administration.
Rickets and osteomalacia
Rickets affect growing children and osteomalacia
affecting adults in which there are decrease bone
mineralization .
They may result from diets deficient in calcium and
vitamin D, but probably more important is limited
exposure to sunlight.
 Less common causes of rickets and osteomalacia
include :
1.Renal disorders causing decreased synthesis of 1,25
(OH)2-D.
2.Phosphate depletion.
3.Malabsorption disorders.
 Deficiency of vitamin D tends to cause
hypocalcemia.
When hypocalcemia occurs, PTH production is
increased and uriary loss of phosphate increased.
Thus, the serum level of calcium is restored to near
normal, but hypophosphatemia persists, and so
mineralization of bone is impaired,with high bone
turnover.
The gross skeletal changes depend on the severity of
the rachitic process; its duration; and, in particular,
the stresses to which individual bones are subjected.
An excess of osteoid produces frontal bossing and
a squared appearance to the head.
Deformation of the chest results from overgrowth of
cartilage or osteoid tissue at the costochondral
junction, producing the "rachitic rosary."
The weakened metaphyseal areas of the ribs are
subject to the pull of the respiratory muscles and
thus bend inward, creating anterior protrusion of the
sternum (pigeon cheast deformity).
The inward pull at the margin of the diaphragm
creates the Harrison groove, girdling the thoracic
cavity at the lower margin of the rib cage.
 When an ambulating child develops rickets,
deformities are likely to affect the spine, pelvis, and
long bones (e.g., tibia), causing, most notably,
lumbar lordosis and bowing of the legs
Biochemical findings:
1-Low serum and urinary calcium.
2-Low serum phosphate.
3-Increase serum alkaline phosphatase.
4-Elevated parathyroid hormone.
 Paget Disease
(Osteitis Deformans)
Disease characterized by repetitive episodes of
regional osteoclastic activity and bone resorption
(osteolytic stage), followed by bone formation
(mixed osteoclastic-osteoblastic stage), and
finally by an apparent exhaustion of cellular activity
(osteosclerotic stage).
The net effect of this process is a gain in bone
mass; however, the newly formed bone is
disordered and lacks strength.
Pathogenesis:
There is suggestion that a paramyxovirus induce IL-
1 secretion from infected cells, and this cytokine-as
well as M-CSF, activate osteoclasts.
Other pathogenic mechanisms are suggested by the
observations that osteoclasts in Paget disease appear
to be intrinsically hyper-responsive to activating
agents such as vitamin D and RANK ligand