Endocrine Abstracts

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The diagnosis of osteoporosis in the context of the growing skeleton requires both the presence of low bone mass for body size and a history of low trauma fractures. The presentation of osteoporosis may predate adolescence, or less commonly arise during that period. Primary osteoporosis is typically due to osteogenesis imperfecta or idiopathic juvenile osteoporosis. Secondary osteoporosis arises as a result of a variety of conditions, most of which fall under thematic headings...

Background: Bone mass is low and fracture risk is higher in obese children. We wished to ascertain the relationships of obesity-related changes in hormones with skeletal microstructure.Method: Children aged 8–15 years matched by gender and pubertal stage were recruited into lean and obese groups (18 pairs). We used high resolution peripheral quantitative computed tomography (HRpQCT – resolution-82 μm) to assess three-dimensional cortical a...

We present the first paediatric patient treated with Asfotase Alfa (AA) or ‘Strensiq’, for perinatal Hypophosphatasia (HPP), who has turned 16 and approaches the end of her time in paediatric care. She is the child with the longest treatment duration worldwide. We reflect on the 16 years of learning she has provided for endocrinologists globally. HPP is a rare, inherited, metabolic bone disease, characterised by defective bone and teeth mineralisation. It is caused b...

Background: Over-dosing and under-dosing of vitamin D in children and young people appears to be common, based on our audit of current practice. The contribution of ethnicity, skin colour, and vitamin D binding protein (VDBP) genotype has not been fully explored during vitamin D treatment.Objective: To investigate how ethnicity/skin colour and genetic variation affect the response to 150 000 units of vitamin D administered to young adults of white Caucas...

Background: The role of bisphosphonates in improving bone mass in patients with osteogenesis impefercta (OI) is well established. However, the impact of bisphosphonate therapy on body composition in relation to increasing bone mass remains relatively unexplored.Methods: Change in DXA-derived subtotal body (TBLH) and lumbar spine (LS: L1–L4) bone mineral content (BMC (g)), bone area (BA (cm2)), areal bone mineral density (aBMD (g/cm2<...

Introduction: Adults with Type 1 Diabetes Mellitus (T1DM) are at risk of premature osteoporosis and fractures. The onset of T1DM typically starts during childhood and adolescence thus the effects of diabetes on the skeleton may be established in this period. Studies in children primarily use DXA to evaluate the effects of T1DM on bone with conflicting results. We present the first study in children assessing the impact of T1DM on skeletal microstructure and strength, using HRp...

Objective: To describe outcomes among children with hypophosphatasia (HPP) receiving asfotase alfa.Methods: This prospective, real-world study used data from all children with HPP receiving asfotase alfa in the UK Managed Access Agreement (MAA) to assess functional, health-related quality-of-life, and safety outcomes. Visits occurred at MAA enrolment, 3 and 6 months after enrolment, and every 6 months thereafter. Assessm...