Bone Abstracts

The oim/oim mouse resembles type III OI. Our study mimics clinical questions: is RANK-Fc as efficacious as ALN in reducing fracture number? Is RANK-Fc safe to administer to children? Is there any advantage to receiving RANK-Fc treatment post adolescence, following no treatment or ALN treatment from infancy through adolescence?All animal work was done under an IACUC-approval. Mice – n=200, WT or oim/oim. Treatment spanned a...

Duchenne muscular dystrophy (DMD) causes progressive muscle weakness and loss of ambulation. While glucocorticoid (GC) therapy improves motor function, many boys sustain fractures due to osteoporosis. Recently, i.v. bisphosphonate (i.v.BP) therapy has shown promise in the treatment of DMD-related osteoporosis. At the same time, bone histomorphometry revealed lowered bone volume and significant reductions in bone formation pre-i.v.BP treatment, and a further drop after 2 years&...

During early life undernutrition impairs normal weight and height gain that affects bone health. During recovering it is required an extra-amount of Ca to ensure the high demands of bone mineralization. A mixture of galactooligosaccharides (GOS) and fructooligosaccharides (FOS) would be a useful tool to increase mineral intestinal absorption to enhancing the amount of Ca to be retained into bone. Our objective was to evaluate, in an experimental model of protein restriction (P...

Background: Orally administered ibandronate is an effective agent in the treatment of postmenopausal osteoporosis. There are only scarce data regarding its use in children with impaired bone health.Objectives: To evaluate the effect of oral ibandronate on bone health in osteoporotic children and adolescents.Patients, materials and methods: We enrolled six patients (all boys; mean age 15.7±3.8 years; range 8–18 years) with...

Introduction: Bone health in patients with sickle cell disease and thalassemia is impaired. These patients feature altered parameters of bone metabolism and bone mineral density.Aim and design: To investigate bone health in patients with hemolytic anemia we conducted a cross-sectional analysis in our Hematology Outpatient Clinic at the Children’s Hospital Essen. The largest subgroups within our cohort are patients with homozygous sickle cell (HBSS) ...

Tricho-rhino-pharyngeal syndrome (TRPS) is a rare genetic disorder, which is characterized by craniofacial and skeletal abnormalities. This report presents three cases of TRPS (two sporadic and one familial). Clinical presentation included typical facial features (pear-shape nose, long flat philtrum, thin upper vermilion border and protruding ears), thin, sparse scalp hair and different skeletal abnormalities with normal mentation. Case 1: 5.5-year-old boy with short stature (...

The pediatric osteogenesis imperfecta (OI) team at our university hospital was established in 1991. The multi- and inter-disciplinary team consists of: pediatric neurologist, nurse, nursing assistant, physiotherapist, occupational therapist, orthopedic surgeon, orthotist, radiologist, dentist and geneticist. We also have a close collaboration with other specialists.Our assignment is high qualified diagnostics, functional assessments and also individualiz...