Searchable abstracts of presentations at key conferences on calcified tissues
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6th International Conference on Children's Bone Health

Rotterdam, The Netherlands
22 Jun 2013 - 25 Jun 2013

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6th International Conference on Children's Bone Health , 22 - 25 June 2013; Rotterdam, The Netherlands

ba0002op1 | (1) | ICCBH2013

Skeletal effects of hypothyroidism are mediated by thyroid hormone receptor α

Cheung Moira , Boyde Alan , Evans Holly , Bassett Duncan , Williams Graham

Childhood hypothyroidism results in delayed skeletal maturation and impaired growth. Thyroid hormones act via temporo-spatially regulated thyroid hormone receptors α (TRα) and (TRβ).In the skeleton, TRα is the predominant receptor and we hypothesise that the skeletal effects of hypothyroidism are mediated by TRα.To investigate this we assessed the response of wild-type (WT), TRα knockout (TRα...

ba0002op2 | (1) | ICCBH2013

High FSH serum levels may support the altered bone remodeling in Turner syndrome patients

Brunetti Giacomina , Ventura Anna Maria , Piacente Laura , Oranger Angela , Ciccarelli Maria , Mori Giorgio , Colucci Silvia , Cavallo Luciano , Grano Maria , Faienza Maria Felicia

Objective: Turner syndrome (TS) is a chromosomal aberration characterized by total or partial loss of one of the two X-chromosomes, and affects about 1 in every 2500 girls. TS patients can develop the bone disease with decreased bone density and selective reduction in cortical bone thickness, which probably contributes to the increased fracture risk. However, the mechanisms underlying the bone disease remain poorly understood. Thus, the aim of this study was to investigate the...

ba0002op3 | (1) | ICCBH2013

Calcimimetics as adjunct therapy in XLH: 18 months experience

Alon Uri , Haney Connie

Objective: Based on our preliminary findings, indicating a potential for Cinacalcet (due to its suppression of PTH (CJASN 2008 3 658)), to allow the use of lower doses of oral phosphate (OP) and calcitriol in treating XLH, the objective of this study was to examine its long-term effect as adjunct treatment in children with XLH.Methods: Eight children (F5), ages 7.8–20.9 years (median 13.9), who were already treated by OP an...

ba0002op4 | (1) | ICCBH2013

A new start-codon in IFITM5 causes osteogenesis imperfecta type V

Semler Oliver , Hoyer-Kuhn Heike , Garbes Lutz , Netzer Christian , Schoenau Eckhard

Background: Osteogenesis imperfecta (OI) is a rare disease characterized by increased fracture rate and bone deformities. Patients are classified by phenotype and most are affected by mutations in COL1A1/2.Patients with OI type V present with specific clinical symptoms including hyperplastic callus formation, only mildly decreased height, metaphyseal lines and a calcification of the membrane interossea of the forearm. The disease causing mutation for OI ...

ba0002op5 | (1) | ICCBH2013

Abstract withdrawn....

ba0002op6 | (1) | ICCBH2013

Children with nephrotic syndrome have increased tibial bone area but similar volumetric bone mineral density to healthy controls

Moon Rebecca , Gilbert Rodney , Page Anna , Murphy Liam , Taylor Pat , Cooper Cyrus , Dennison Elaine , Davies Justin

Objectives: An increased fracture risk is reported in children requiring recurrent courses of glucocorticoids. Reduced bone mineral density (BMD), particularly in the trabecular compartment, has also been demonstrated in a number of childhood diseases treated with glucocorticoids. The differential contribution of glucocorticoids and underlying inflammatory disease to bone demineralisation is poorly understood. Childhood nephrotic syndrome (NS) often follows a relapsing-remitti...

ba0002op7 | (1) | ICCBH2013

Bone mineralization density distribution, bone formation rate and bone turnover markers in a cohort of children with CKD before and after GH treatment

Fratzl-Zelman Nadja , Nawrot-Wawrzyniak Kamilla , Misof Barbara , Panczyk-Tomaszewska Malgorzata , Ziolkowska Helena , Roschger Paul , Klaushofer Klaus

Background and methods: A specific skeletal complication in children with CKD is growth impairment. The effects of recombinant human growth hormone (rhGH) therapy on bone turnover and bone material quality are not well understood. Bone matrix mineralization density distribution (BMDD) as assessed by quantitative backscattered electron microscopy (qBEI) is an important determinant of bone material quality and reflects bone turnover (average tissue age) and mineralization kineti...

ba0002op8 | (1) | ICCBH2013

Quantitative measurement of abnormal bone quality and strength with bone micro-architecture and rod-plate configuration in osteopenic adolescent idiopathic scoliosis

Yu Wing Sze , Chan Ka Yan , Yu Fiona Wai Ping , Lee Kwong Man , Ng Kin Wah , Lam Tsz Ping , Qin Ling , Cheng Chun Yiu Jack

Objectives: Multiple studies have documented the presence of systemic osteopenia in adolescent idiopathic scoliosis and in simulated animal models. Osteopenia was also found to be associated with severe curves and as one of the prognostic factor in predicting curve progression in AIS. The objective of this study was to compare the bone quality and its association with osteopenia between AIS and normal controls.Methods: AIS (n=234) and non-AIS gi...

ba0002op9 | (1) | ICCBH2013

Anticalciuric effect of recombinant PTH in patients with activating mutations of the calcium-sensing receptor causing autosomal dominant hypocalcaemia--hypercalciuria

Rothenbuhler Anya , Allgrove Jeremy , Coutant Regis , Kapelari Klaus , Bessenay Lucie , Isnard Myriam , Hogler Wolfgang , Linglart Agnes , ESPE Working Group on Bone and Growth Plate

Background: Most patients with hypoparathyroidism are controlled under conventional treatment with calcium and vitamin D analogues. However, this treatment may be difficult to manage, especially in patients with ADHH who have an increased risk of nephrocalcinosis and chronic renal insufficiency. ADHH is caused by activating mutations in the calcium-sensing receptor (CaSR) resulting in suppressed PTH secretion and decreased calcium reabsorption within the thick ascending limb o...

ba0002op10 | (1) | ICCBH2013

Metacarpal width, length, medullary diameter and cortical thickness in hypophosphatemic rickets

Beck-Nielsen Signe Sparre , Brixen Kim , Gram Jeppe , Poulsen Mette Ramsdal

Hand X-rays from patients with hypophosphatemic rickets (HR) were assessed to evaluate if HR influences the dimensions of the metacarpal bones and the distribution between cortical and cancellous bone. In addition, we aimed to test the hypothesis that HR caused by mutation in DMP1 has a greater impact on bone dimensions than HR caused by mutations in PHEX.Hand X-rays from 17 children with HR were evaluated. Three children had HR caused ...

ba0002op11 | (1) | ICCBH2013

Artistic gymnasts display micro-architectural advantages over trampolining and tumbling gymnasts: a high resolution peripheral quantitative computed tomography study

Burt Lauren A , Stanger Scott P , Schipilow John D , Boyd Steven K

Participation in high impact sports during growth increases bone quality. Gymnasts have previously displayed increased bone mass and strength at both the upper and lower limbs compared with controls. However, it is not yet understood how bone microarchitecture is affected by gymnastics participation, and if this differs based on gymnastics discipline. Therefore, the objective of this study was to investigate the influence of gymnastics discipline on bone microarchitecture in a...

ba0002op12 | (1) | ICCBH2013

The bone/vessels interplay in teenagers with chronic kidney disease

Bacchetta Justine , Doyon Anke , Vershelde Sophie , Vilayphiou Nicolas , Chapurlat Roland , Ranchin Bruno

Background: Bone fragility and vascular calcifications are to the two main morbidities of the mineral and bone disorders associated with chronic kidney disease (CKD), resulting from a combination of abnormalities such as impaired GH axis, vitamin D deficiency, hyperparathyroidism, increased FGF23 levels, hypogonadism, denutrition and drug toxicity.Methods: In a single-centre ancillary study of the longitudinal prospective European 4C study (Cardiovascula...

ba0002op13 | (1) | ICCBH2013

Long-term effects of bisphosphonate therapy in children with osteogenesis imperfecta

Biggin Andrew , Zheng Linda , Briody Julie , McQuade Mary , Munns Craig

Objectives: To evaluate the clinical outcomes of intravenous bisphosphonate treatment in children with mild-moderate osteogenesis imperfecta (OI) who had progressed from active bisphosphonate treatment to maintenance therapy for >2 years.Methods: A retrospective review was conducted on 17 patients with mild-moderate OI. Clinical data, fracture history, biochemistry, dual energy X-ray absorptiometry (DXA) parameters, vertebral measurements, bone age a...

ba0002op14 | (1) | ICCBH2013

Inflammation and glucocorticoid therapy impair skeletal modeling during growth following crohn disease diagnosis

Tsampalieros Anne , Shults Justine , Zemel Babette , Baldassano Robert , Leonard Mary

Objectives: Examine changes in volumetric BMD and cortical structure following Crohn Disease (CD) diagnosis, and identify associations with growth, glucocorticoid exposure and disease activity.Methods: Prospective cohort study in 76 CD participants, ages 5–21 years. Tibia pQCT scans were obtained at diagnosis, 6, 12 and a median of 42 months later. Sex, race and age-specific Z-scores were generated based on >650 controls. Cortical dimen...

ba0002op15 | (1) | ICCBH2013

Dissecting the role of osteoblast derived nitric oxide in bone remodeling

Grover Monica , Nagamani Sandesh , Erez Ayelet , Lee Brendan

Background: Bone accrual during adolescence is the main contributor to peak bone mass. Bone remodeling is a balance between bone formation and resorption as directed by osteoblast and osteoclast activity, respectively. nitric oxide (NO) is a potent regulator of bone remodeling via mediating effects of cytokines, estrogen and mechanical strain. NO is synthesized from the conversion of L-arginine to L-citrulline by the enzyme nitric oxide synthase (...