Bone Abstracts

Background: Asfotase alfa therapy improves clinical outcome in young children with severe form of hypophosphatasia (HPP). Treatment outcome in older children (≥12 years) has not been reported.Presenting problem: We report clinical outcome of a 16 year-old male with childhood HPP who started enzyme therapy at age 15 years.Clinical management: The patient was diagnosed with HPP at age 2 years when he presented with premature lo...

Background: Periosteal reaction can be a manifestation of various underlying medical conditions, including tumor, infection, trauma, metabolic or genetic diseases.Presenting problem: A 2 year-old male presented for evaluation of periosteal bone formation in symmetrical distribution of proximal humerus, radius, ulnar, femur, and clavicles, noted after having persistent fussiness, irritability and inability to bear weight and use arms for 3 weeks. He had a...

Objectives: Children with Duchenne muscular dystrophy (DMD) receiving long-term glucocorticoid (GC) therapy are at risk for osteoporosis and fragility fractures. Recent studies showed that cortical thickness and areas were associated with increased fracture risk. Digital X-ray measurement of the cortical thickness of the metacarpal bones has a potential role as a marker for bone health in children, but has not been evaluated in DMD. The aim of this study was to compare bone ag...