Bone and joint health in children with severe hemophilia A: A single-center, prospective case-control study from North India

Abstract

Background: Poor bone and joint health, as evidenced by a reduction in bone mineral content and density (BMC, BMD) and progressive joint destruction, has been reported in hemophiliacs. Material and methods: Thirty children (4–17 y) with severe hemophilia A and 30 age and sex-matched healthy controls were included. Children with symptomatic hypocalcemia, rickets, recipients of drugs affecting BMD, and congenital skeletal malformation were excluded. Physical activity scoring (PAS), annualized bleeding rate (ABR), and annualized joint bleeding rate (AJBR) were determined in hemophiliacs. Joints were assessed by using Pettersson and Hemophilia Joint Health Score (HJHS) 2.1. BMC and BMD were estimated using a dual-energy X-ray absorptiometry (DEXA) scan. Serum vitamin D, calcium, phosphate, and alkaline phosphatase levels were estimated. Results: BMD, serum vitamin D, and calcium were lower in hemophiliacs than in controls (p < 0.05). Serum phosphorus and alkaline phosphatase were comparable. In hemophiliacs, the mean ABR and AJBR were 17.10 ± 14.9 and 15.55 ± 14.0, respectively. The knee joint was the most common site of hemarthrosis (98.33 %) and target joint (68.2 %). The majority (53.3 %) of hemophiliacs had a PAS of 5. In hemophiliacs, the mean HJHS and Pettersson scores were 12.73 ± 11.8 and 7.37 ± 7.07, respectively. A significant positive correlation was found between HJHS and Pettersson score with age. Conclusion: BMD, serum vitamin D, and calcium were lower in hemophiliacs than in the general population.

Keywords

• Hemophilia A
• HJHS 2.1
• Pettersson score
• Bone mineral density
• Vitamin D