Bone Abstracts

Background: Neonatal hypocalcemia is defined as S-Ca <2 mmol/l in fullterm newborns and <1.75 mmol/l in preterm newborns. Neonatal hypocalcemia is either early onset (<3 days of age) or late onset (>3 days of age). Newborns with hypocalcemia are often asymptomatic, but mays present with hypotonia, apnea, poor feeding, jitteriness, seizures, cardiac failure. Signs of hypocalcemia rarely occur unless S-Ca drops below 1.75 mmol/l.Case presen...

Objectives: The present study aims to evaluate the longitudinal association of fat mass (FM), fat free mass (FFM) with bone stiffness index (BSI) in European children and adolescents over 2 and 6 years follow-up. METHODS: We included children of the IDEFICS/I. Family cohort, who participated in repeated measurements of BSI using calcaneal quantitative ultrasound (QUS), body composition using skinfold thickness, sedentary behaviours (SB) and physical activity (PA) using self-ad...

Background: Idiopathic infantile hypercalcemia (IIH) is a rare disorder caused by CYP24A1 loss-of-function mutation, resulting in impaired degradation of 1,25-dihydroxyvitamin D3. Typical signs include muscle hypotonia, dehydration, failure to thrive, psychomotor retardation, constipation, nephrocalcinosis. IIH should be distinguished from other causes of hypercalcemia in infancy. Treatment includes low calcium diet, glucocorticoids, furosemide, calcitonin. Pamidron...

Objective: Bone metabolism was studied in patients with phosphate diabetes before and after medication.Methods: 39 patients with phosphate diabetes age 2–18 years (7.8±4 years, 64% male) and multiplanar deformities of the lower extremities were treated with monthly vitamin D (2000 IU/day), alfacalcidol (0.25 mg/every other day) and calcium glycerophosphate (600 mg/day) for 6 months.Biochemical indices were evaluated befor...

Background: Transient hyperphosphatasemia of infancy and early childhood (THI) is a benign, usually accidentally detected condition characterized by transiently increased activity of serum alkaline phosphatase (S-ALP), its bone or liver isoform, in children under five years of age, without signs of metabolic bone disease or hepatopathy. When encountered in a child with either chronic bone, liver or kidney disease, THI might concern the physician. We present a patient with urol...

Goal: The use of intramedullary telescopic constructs for osteosynthesis in surgical correction of bone deformities in children with systemic skeletal disease can be complicated by delayed bony union, and the structural and functional pathology of bone in patients with these disorders do not always make it possible to avoid displacement of bone fragments and effectively correct the deformity.Methods: Analysis of treatment of 11 patients of femoral and ti...

Background: Hypercalcemia in childhood is rare, can be of various origin and might go unrecognized until severe signs appear. We demonstrate hypercalcemia of different causes in two infants.Case no 1.: 5-month old girl with failure to thrive, constipation, muscle hypotonia, dehydration, where total calcaemia peaked to 4.25 mmol/l. Low calcium diet, application of glucocorticoids and furosemide resulted in a drop in calcaemia to values below 3 mmol/l and ...

see P407....

Clinical trials involving drug therapies for postmenopausal osteoporosis typically compare effects of the active drug combined with vitamin D (vit D) and calcium (Ca) vs vit D and Ca supplementation on its own. Bone strength is estimated based on the amount of bone, frequently expressed as bone mineral density determined by dual X-ray absorptiometry, and quality of bone, hardly measured in clinical practice.The purpose of the present study was to compare...

Objectives: Marfan syndrome (MFS) is a connective disorder caused by mutations in FBN1 gene which encodes the extracellular matrix protein fibrillin 1. Pathogenesis relies on a dysregulation of activated TGF-β. Cardiovascular, ocular and skeletal systems are involved with a variable expressivity. Findings evolve with age, making the diagnosis in children more difficult. Skeletal involvement includes disproportionate long bone overgrowth, scoliosis, and chest deformity. Al...