Bone Abstracts

There is discussed an interrelation of BMD, progressing of erosive and destructive changes in hands and feet with development of vertebral deformations in rheumatoid arthritis (RA).The aim: To receive data on bone mineral density (BMD), erosive and destructive changes in hands and feet and vertebral deformations in patients with RA.Materials/methods: In this research, it was included 106 women, with RA, age 23–69 years. In all...

The mature human skeleton is a metabolically active organ that is continuously resorbed and rebuilt by osteoclasts and osteoblasts. Dietary supplementation of selected long chain polyunsaturated fatty acids (LCPUFAs) has shown effects on bone turnover. Most research on LCPUFAs has been done using commercially available cell lines and further clarification of the cellular effects on models relevant to humans is required. After ethical approval, peripheral blood was collected fr...

Loss of genomic maintenance contributes to aging, as exemplified by mutations in Ercc DNA repair proteins that lead to a plethora of progeroid syndromes of which some display accelerated bone loss. It is generally accepted that dietary restriction (DR) increases life span and improves organ function. We therefore assessed the impact of DR on life span and bone mass in WT and bona fide prematurely aging hypomorphic Ercc-deficient mice (Ercc1−/Δ).<...

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...

Cystic fibrosis(CF) results in low volumetric bone mineral density(vBMD), poor muscle strength and increased fracture risk in young patients. The aim of this study was to compare bone and muscle variables measured by peripheral and high-resolution QCT (pQCT and HR-pQCT) and jumping mechanography (JM) In CF children and healthy controls. We hypothesised that CF children have lower muscle force and power (Fmax and Pmax) than controls which may contribute to CF-related bone disea...

Objectives: Autosomal recessive osteopetrosis (OP) is a rare metabolic bone disease characterized by impaired osteoclast function resulting in defective bone resorption and generalized high bone mineral density (BMD). Excessive bone compromises bone marrow space, leading to marrow failure. The infantile malignant form is typically fatal within the first decade of life. The intermediate form presents later during childhood. Currently, the only potential curative therapy for OP ...

In children with spinal muscular atrophy (SMA): i) describe bone mineral density (BMD) measured by dual-energy x-ray absorptiometry (DXA); ii) examine bone health factors; iii) examine change in BMD over time and pamidronate effect.DXA results, medications, ambulation, 25-OH-Vit D levels, and fracture history were retrospectively reviewed in 14 children (five girls) with SMA. Sites scanned included whole body (WB), lumbar spine (LS), and lateral distal f...

Rett (RTT) syndrome is a neurodevelopmental disorder that affects girls almost exclusively. The majority are related to mutations in the MECP2 gene.Patients with RTT syndrome have a high incidence of fractures that can occur at a young age. One of the objectives of this study was to identify clinical, radiographic and biological parameters associated to fracture incidence.89 RTT patients bearing a MECP2 mutation who had no...

Background: Treatment of osteoporosis with bisphosphonates may result in severe suppression of bone turnover leading to adynamic bone disease. Teriparatide is now commonly used as an anabolic agent in adults with osteoporosis, and has the potential to reverse the effect of bisphosphonates. However its use in pediatrics is restricted to those with closed growth plates and information regarding its effect in this age group limited.Objective: To study the e...

Background: 3 year old boy born to non-consanguineous parents. He was diagnosed to have autism at 2 years of age. He had a history of pica.Presenting problem: He was admitted with severe carpopedal spasms of hands and feet. Investigations revealed severe hypomagnesaemia at 0.26 (0.7–1.0) mmol/l, hypocalcaemia at 1.59 (2.2–2.6) mmol/l, hypokalaemia, hyponatremia and moderately low vitamin D levels. Parathyroid hormone concentration was low. Urin...