Transcript Osteoporosis In Thalassemia

Osteoporosis In Thalassemia
Dr Tarek Jawad
INT 555
Case Presentation
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Local male
Case of beta thalassemia major
He presented at 3 years of age as a
referral from Al Sagar Hospital as a case of
chronic anemia
On repeated blood transfusions
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BMD was done at 15 years of age
At this age he was well under the 3rd
centile for height and weight
Overview
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Thalassemia major is a common cause if
skeletal morbidity.
Etiology  Multifactorial  A state of
increased bone resorption and turnover
Osteoporosis
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It is a metabolic disease characterized by
low bone mass and microarchitectural
deterioration of bone tissue
This leads to enhanced bone fragility of
and increased fracture risk
Types
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Primary
Bone mass loss
related to ageing and
loss of gonadal
function in woman
and the ageing
process in men
without any other
chronic illness
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Secondary
Results from a variety
of chronic conditions
that significantly
contribute to bone
mineral loss, or from
the effects of
medications and
nutritional deficiencies
Secondary Causes
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Chronic Diseases:
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Thalassemia
Cushing’s syndrome
Anorexia nervosa
Hyperthyroidism
Hyperparathyroidism
Marfan’s syndrome
Hemochromatosis
Multiple Myeloma
Hypercalcuria
Hyperprolactinemia
Renal tubular acidosis
Chronic liver disease
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Medications:
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Steroids
Excess thyroid hormones
Gonadotropin-releasing
hormone agonists
Cyclosporine
Methotrexate
Phenobarbital
Phenytoin
Phenothiazines
Heparin
Desferal
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Conditions Causing
Nutritional
Deficiencies:
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Malabsorption
Vit D deficiency
Calcium deficiency
Gastric and bowel
resections
Alcoholism
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Other Causes:
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Athletic amenorrhea
Tobacco use
Pregnancy
WHO Definition
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WHO defines according to the Bone
Mineral Density (BMD)
T-Score of 0 is equal to a healthy normal
30 year old
T-Score between -1 to -2.5 is osteopenia
T-Score lower than -2.5 is osteoporosis
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Z-Score measures BMD compared to a
typical healthy person of the same age as
the patient
The current accepted definition of low
bone mass for all patients under 50 years
of age is Z-Score lower than -2.0
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WHO classifies
osteoporosis as the
most common
metabolic bone
disease in developed
countries
Other Tests
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Radiology  Spine X-Ray
MRI
Biochemical
Pathophysiology In Thalassemia
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The causes in thalassemia include:
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Marrow expansion
Anemia
Transfusional hemosiderosis
Delayed puberty
Use of desferal or oral chelation agents for iron overload
Multiple endocrinopathies
Lose IGF1
Low Vitamin D levels
Genetic Factors
Prevention Of Low Bone Mass
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An appropriate transfusion regimen
Maintaining adequate chelation therapy
Treatment of endocrine issues that may
affect bone mass, such as delayed
puberty/hypogonadism
Avoid Smoking
Regular Exercise  20 – 30 minutes 3 – 4
times a week
Diet Rich in Calcium and Vitamin D
Treatment
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Sex steroid replacement therapy
Calcimimetics
Anti-resorption agents
Combination therapy
Monitoring Of Treatment
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Biochemical parameters  bone and sex
steroid profiles
DEXA scan of spine and femoral neck
References
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http://www.thalassemia.com/documents/thalhan
dbook2008.final.pdf
www.thalassemia.org/updates/LowBoneMass1
.pdf
The Cleveland Clinic Center for Continuing
Education © 2000-2009. All Rights Reserved
Guidelines For The Clinical Management Of
Thalassemia 2nd Edition. TIF