Searchable abstracts of presentations at key conferences on calcified tissues

ba0002p201 | (1) | ICCBH2013

Guided growth with hinge plates for lack of extension and fixed flexion of the knee

Galban Miguel , Villanueva Roceli , Carpio Annie

Lack of extension of the knee and fixed flexion of the knee may occur in patients with arthrogryposis, rheumatoid arthritis, achondroplasia, osteogenesis imperfecta, cerebral palsy and other conditions. They develop a crouch gait and this is an energy non-efficient condition that causes a compensatory flexion deformity of the hip and lumbar lordosis. Recommended treatments have included bracing, physical therapy, posterior release, distal femoral osteotomy or progressive distr...

ba0001pp295 | Muscle, physical activity and bone | ECTS2013

Proximal femur geometry as moderator factor for the effect of mechanical loading during gait: a bone remodeling analysis

Machado Miguel M , Fernandes Paulo R , Baptista Fatima

The regions of the proximal femur that are at greater risk of structural failure during a fall are those with less adaptive protection promoted by mechanical loading of the activities of daily living. Considering the associations between bone geometry of the proximal femur with bone fracture risk, we intended to examine how geometrical characteristics of the proximal femur (FNL, femoral neck length; FNW, femoral neck width; NSA, neck shaft angle) moderate the effect of mechani...

ba0004p146 | (1) | ICCBH2015

Hypovitaminosis D and factors associated in healthy children aged 2--14 years old in Mexico

Chico-Barba Gabriela , Huitron Gerardo , Guagnelli Miguel , Clark Patricia

Objectives: To estimate the status of 25-hydroxyvitamin D in healthy Mexican children and to describe factors related to vitamin D deficiency.Methods: A cross-sectional study was conducted in children 2–14 years old in Mexico City and Toluca Edo de Mexico. Trained interviewers applied a questionnaire including all relevant demographics, medical history, sun exposure, sunblock use and skin phototype. Morning fasting blood was collected in all subject...

ba0001pp10 | Clinical case posters | ECTS2013

Hadju–Cheney syndrome: report of two cases in a family

Terroso Georgina , Bernardes Miguel , Aleixo Abelha , Madureira Pedro , Vieira Romana , Fonseca Rita , Goncalves Diana , Costa Lucia

Objectives: To describe two familiar cases of Hajdu–Cheney syndrome, a rare genetic disorder associated with skeletal dysplasia, craniofacial abnormalities, short stature, acro-osteolysis and osteoporosis.Materials and methods: A 51-year-old woman (case 1) presented in our outpatient clinic with pseudo-clubbing of some fingers and toes. She was short (139 cm) and thin (34 kg). She had facial and cranial abnormalities: thin lips, long philtrum, full ...

ba0001pp12 | Clinical case posters | ECTS2013

Severe osteoporosis associated with Hajdu–Cheney syndrome: follow-up after 2 years of teriparatide therapy

Terroso Georgina , Bernardes Miguel , Aleixo Abelha , Vieira Romana , Madureira Pedro , Fonseca Rita , Goncalves Diana , Costa Lucia

Objectives: To describe the response to treatment with teriparatide for osteoporosis associated with Hajdu–Cheney syndrome after a follow-up 2 years.Material and methods: A 51-year-old woman presented in our outpatient clinic with pseudo-clubbing of some fingers and toes. She was short (139 cm) and thin (34 kg). She also had some facial and cranial abnormalities: thin lips, long philtrum, full cheeks, micrognathia, short neck, bushy eyebrows and coa...

ba0001pp211 | Cell biology: osteoblasts and bone formation | ECTS2013

Rab27a is involved in bone formation by osteoblasts

Coxon Fraser , Douglass Angela , Hughes Alun , Helfrich Miep , Seabra Miguel , Tolmachova Tanya

The Rab family GTPases Rab27a and Rab27b play an important role in the trafficking of lysosome-related organelles in specialised cells, such as melanocytes. Since secretory lysosomes, also considered a lysosome-related organelle, are important for osteoclast and osteoblast function, we hypothesised that Rab27 plays a role in bone physiology. In support of this, a recent study demonstrated impaired transport of RANK ligand to the plasma membrane in osteoblasts from mice lacking...

ba0002p187 | (1) | ICCBH2013

Juvenile idiopathic osteoporosis responsive to intravenous alendronate

Guagnelli Miguel Angel , Yeste Diego , Clemente Maria , Garrido Marta , Carrascosa Antonio

Background: Osteoporosis in otherwise healthy children demands thorough study as it may be the first manifestation of an occult illness. When other diseases can be ruled out, juvenile idiopathic osteoporosis (JIO) is the purported diagnosis.PresentationWe report the case of an 8-year -old boy with no personal or family history of chronic disease who presented with sudden ankle pain unrelated to trauma and not responsive to rest and...

ba0004p28 | (1) | ICCBH2015

Year-round cord blood vitamin D concentrations in women from diverse ethnical backgrounds in a Mediterranean area in Spain

Guagnelli Miguel Angel , Yeste Diego , Ferrer Roser , Comas Inmaculada , Carrascosa Antonio

Background: Vitamin D (Vit-D) plays a role in calcium metabolism during pregnancy, and studies have found widespread deficiency in this period. Its significance is still not fully understood, although low Vit-D levels have been associated with increased risk for preeclampsia, bacterial vaginosis, neonatal sepsis and early respiratory infections in the infant. Supplementation to the pregnant mother remains a matter of debate; thus, it is important to better understand the cause...

ba0006p069 | (1) | ICCBH2017

Multiple fractures that begun in utero in a pre-adolescent child with low ALP levels and nephrocalcinosis: clinical aproximation for the differential diagnosis of hypophosphatasia (HPP)

Fernandez Maria Isabel Gonzalez , Montesinos Berta Lopez , Marti Miguel , Calvo Inmaculada

Objectives: Differential diagnosis vs. Osteogenesis Imperfecta (OI).Methods: The parents were Moroccan origin, consanguineous. The patient is an 8 year old girl, who was visited for a first time in our hospital in October 2016, presenting a fracture of the left femur, with dramatic bone deformations, with important disability, unable to walk and with growth retardation (weight 15 kg, length: 92 cm). The first registered fracture is at birth, consisting o...

ba0006p077 | (1) | ICCBH2017

The role of bone age in the evaluation of trabecular bone score (TBS) of children and adolescents 5--19 years old

Guagnelli Miguel Angel , Ambrosi Regina , Lopez-Gonzalez Desiree , Winzenrieth Renaud , Del Rio Luis , Clark Patricia

Rationale: Trabecular Bone Score (TBS) is a texture-based tool analyzing DXA images in order to assess bone microarchitecture in the lumbar region. In pediatric population, definition of normative values has remained elusive due to the disparities of results in normal population, probably link to uncontrolled factors which impact bone microarchitecture and the nonlinear behavior of bone growth. Our objective was to evaluate TBS in healthy Mexican children and adolescents using...