Vitamin D deficiency and osteoporosis in hemophilic children

Abstract

The aim of this study is to investigate bone state and factors affecting it in children with hemophilia. This is a case-control study that included 37 children with hemophilia and 37 healthy controls. The patients were selected from the outpatient pediatric hematology clinic of Fayoum University Hospital, Egypt. Bone mineral density, serum vitamin D, parathormone, calcium, phosphorus, and calcium creatinine ratio levels were evaluated. Vitamin D level and bone mineral density were significantly lower in hemophiliacs than in control group (P < 0.0001). About 43.2% of cases had moderate vitamin D deficiency, whereas 35.1% had mild deficiency. Vitamin D positively correlated with bone mineral density Z-score, whereas it negatively correlated with total joint score. Positive correlation between bone mineral density and age was also found. Serum levels of urea, urinary calcium creatinine ratio, and parathormone were found to be higher in cases than in control. Also, serum calcium level was found to be lower in patients than in controls. We concluded that vitamin D deficiency is an essential cause of decreased bone mineral density in hemophilic children. Hemophilic arthropathy with consecutive immobilization plays an important role in vitamin D deficiency and decreased bone mineral density.

Effect of low‐dose factor VIII prophylaxis therapy on bone mineral density and 25(OH) vitamin D level in children with severe haemophilia A

“…In the current study, there was a significant negative correlation between 25(OH) vit D level and HJHS ( R = −.384) and ( P = .006) and a positive correlation between serum vitamin D level and DEXA z‐score ( R = .323) and ( P = .022). Similarly, Eldash et al found that vitamin D level is positively correlated with z‐score of DEXA scan and negatively correlated with J‐score in adult patients with severe haemophilia A. However, Wells et al found no correlation between 25(OH)D and BMD.…”

“…Upon comparing the laboratory and radiological data of the haemophilia children and controls, there was a significantly lower serum level of vitamin D ( P < .001) and DEXA z‐score ( P < .001) in patients with severe haemophilia A than the control group. These results are in concordance with Eldash et al , who found that both vitamin D level and BMD were significantly lower in people with haemophilia than control group ( P < .001). Also, Wells et al showed that adults with severe haemophilia A had reduced bone mineral density compared to a control group.…”

3D gait analysis, haemophilia joint health score, leg muscle laterality and biomarkers of joint damage: A cross‐sectional comparative assessment of haemophilic arthropathy

“…11 Reduced levels of vitamin D were observed in children with haemophilia. 12 The Haemophilia Joint Health Score (HJHS) was developed to assess joint damage in children with haemophilia. For teenagers and young adults, it correlates strongly with the x-ray-derived Pettersson score (rho = 0.86) and its inter-observer reliability was rated to be excellent.…”

“…High levels of matrix metalloproteinases, such as MMP‐2, have been shown to be present in osteoarthritis, and once these MMPs are fully activated, they may contribute to the destruction of cartilage 11 . Reduced levels of vitamin D were observed in children with haemophilia 12 . The Haemophilia Joint Health Score (HJHS) was developed to assess joint damage in children with haemophilia.…”

Mechanisms of Bone Remodeling Disorder in Hemophilia

“…Multiple reports show that patients with hemophilia have significantly lower vitamin D levels than healthy people, and vitamin D deficiency might result in poor bone density. [96][97][98]…”