THE PATTERN OF BONE DISEASE AND ITS MORBIDITY AMONG
THALASSEMIA TREATED AT A UNIVERSITY HOSPITAL IN SAUDI ARABIA
Dr. Soad Khalil Al Jaouni, FRCPC
Hematology Department
College of Medicine
King Abdul Aziz University
Jeddah, Saudi Arabia
E-mail: saljaouni@kaau.edu.sa
Background
Bone disease is an increasingly recognized serious cause of morbidity on young adult of both
thalassemia major (TM) and thalassemia intermedia (TI). It's etiology is multifactorial,
culminating with increased bone resorption and remodeling, due to the complication of the
disease itself and other risk factors; low baseline hemoglobin, delayed puberty, hormonal
failure, high iron stones and nutritional deficiency. The lack of early diagnosis and treatment
can led to multiple problems, growth failure, osteoporosis, fractures, spinal deformities and
nerve compression.
Aims
To assess the prevalence of bone disease and its morbidity among thalassemia patients treated
at our institute.
Methods
Hundred-fourteen thalassemia patients were enrolled in the study, (104 TM and 10 TI),
patients age range from 1-40 years old (59 females & 55 males) 67% were children and
adolescent. These patients were treated at King Abdulaziz University Hospital (KAUH),
Jeddah, Kingdom of Saudi Arabia. All patients were assessed clinically. Blood and urine
samples were obtained for the determination of biochemical and hormonal profiles, included,
PTH, 25 OH vitamin D3. Bone maturation was assessed by radiological bone age. Bone
mineral density (BMD) by DEXA was determined on half of the patients. Bone formation
markers (bone-specific alkaline phosphatase and osteocalcin) and bone resorption markers
(Pyridinoline and deoxy pyridinoline) were analysed for patients whom had BMD and
referred for treatment.
Results
Indicate a high prevalence of hypovitaminosis D, 50% in thalassemic children, 80% among
adolescents, up to 70% of adolescent and young adults had dysfunction in hypogonadotropic
hypogonadism. 60% had reduced low bone mass (LBM) among adolescents, worsen by
increase in age. High prevalence of LBM among thalassemia intermedia.
Summary
Bone assessment was found to be suboptimal in children and adolescents, bone morbidity
increased with age. Adolescent period is very crucial stage in the management in chronic
illness. All thalassemia should be screened annually for bone disease. Prevention of
osteoporosis is the most important priority in managing thalassemia patients by early
diagnosis and treatment.
Keywords: Thalassemia, bone disease, low bone mass.
References
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Borgna-Pignatti etal. Survival and disease complications in thalassemia major. Ann
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2. Cunningham MJ, Mackling EA, Neufeld EJ, Colnen AR. Complications of βthalassemia major in North America. Blood 2004;104(1): 34-39.
3. Al Jaouni SK. Studies on Survival and Disease Complication of Thalassemia Major,
Experience of 14 years at King Abdulaziz University Hospital. HAEMATOLOGICA
2006; 91(supplement 1).