Abstract
ABSTRACTAim: This study was planned to evaluate bone health in patients with hereditary spherocytosis.Materials and methods: In this prospective study, a total of 30 hereditary spherocytosis patients which followed in the Pediatric Hematology and Oncology Department of KSU Medical Faculty and 30 patients for control group were included. Patient and control group were chosen equal in age and sex. Hemogram and biochemical tests (serum calcium, phosphorus, alkaline phosphatase, parathormone, vitamin D) and osteocalcin were studied from the patient and control groups. Also DXA examination was performed in the patient group.Results: There was a significant difference in hemogram parameters between the two groups due to hemolytic anemia in hereditary spherocytosis patients. In the patient group, osteocalcin was 6.88 ± 4.35 ng/ml, vitamin D was 17.74 ± 7.76 ng/ml and in the control group osteocalcin was 11.93 ± 8.92 ng/ml, vitamin D was 24.04 ± 11.70 ng/ml. There was a statistically significant difference between the vitamin D and osteocalcin levels of the two groups (p = 0.017 and 0.008, respectively). Bone density was assessed in the patient group. In patients DXA results showed lower Z-scores then the normal population according to age and sex.Conclusion: Hereditary spherocytosis patients should be followed closely in terms of development, puberty, bone health as they are in other hemolytic anemias. Nutritional recommendations, vitamin D supplementation, physical activity should be advised to protect bone health.
Highlights
Chronic hemolytic anemia is a rare disease characterized by abnormal destruction of erythrocytes
There are many studies about osteoporosis in hemolytic anemia in the literature. Most of these studies have focused on thalassemia and sickle cell anemia [3,5,8,9]
Studies conducted to date with hereditary spherocytosis patients are few or have been given as a subset of other hemolytic anemias
Summary
Chronic hemolytic anemia is a rare disease characterized by abnormal destruction of erythrocytes. Extrinsic hemolytic anemias caused by autoimmunity and microangiopathy [1]. In patients with sickle cell anemia and thalassemia, the impairment in bone metabolism was demonstrated. In these patients there are changes in parameters related to bone metabolism and bone densities [2,3,4]. Vitamin D and growth hormone deficiency, chronic blood transfusion, iron toxicity, endocrine pathologies cause deterioration in bone metabolism [5]. Hemolytic anemia alone has been shown to cause bone health decline, decreased bone density, and a decrease in osteocalcin levels in the bone formation markers [6]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
