الفهرس 
I RON H OMEOSTASIS AND H EPCIDINOnly 14 pages are availabe for public view
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Abstract
Background: Anemia is a relevant problem frequently occurring worldwide. Despite the relative high prevalence, costs, and impact on patient’s quality of life, it is rarely considered and adequately treated. Osteogenesis imperfecta (OI) is a genetic disorder of connective tissue with a prevalence of one per (15-20000) births. It primarily affects bone with a wide spectrum of clinical expression from death in the perinatal period to normal life expectancy.
Objectives: To assess the frequency of anemia in patients with osteogenesis imperfecta (OI) and to correlate it with their iron profile levels.
Methods: Between January 2017 to December 2017, this current cross-sectional study was conducted on 56 patients diagnosed with osteogenesis imperfecta. Patients were divided into five groups: Anemia of chronic disease (ACD) group, combined ACD and iron deficiency anemia (IDA) group, no anemia group, other anemia group and IDA group. All patients were subjected to, complete blood count, serum iron, serum ferritin, and serum C - reactive protein (CRP) and total iron binding capacity (TIBC).
Results: The frequency of anemia among OI patients was 71.4%. The majority of the patients in this study showed a combined IDA and ACD (48.2%), while 12.5%, 28.6% and 10.7% of the studied OI patients represent ACD, no anemia and other anemia groups respectively. The values of RBCs indices were lower in patients with ACD whether alone or combined with iron deficiency compared to other anemia group and no anemia group. Ferritin levels were significantly higher in anemic patients than non -anemic patients (P = 0.043).
Conclusion: The high frequency of anemia in Egyptian OI patients highlights the importance of early diagnosis and management. The regular cut-off level for iron parameters in iron deficiency cannot be used due to elevated CRP. Anemia of chronic disease is undermined and overlapped with iron deficiency in patients with OI.