The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis

Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries

(2019) Nataliya Volkova et al.   Journal of Cystic Fibrosis

Bone Health in Children and Youth with Cystic Fibrosis: A Systematic Review and Meta-Analysis of Matched Cohort Studies

(2019) Esther Ubago-Guisado et al.   JOURNAL OF PEDIATRICS

CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth

(2019) Julien Braux et al.   Journal of Cystic Fibrosis

Adults with cystic fibrosis have deficits in bone structure and strength at the distal tibia despite similar size and measuring standard and relative sites

(2018) Kyle K. Nishiyama et al.   BONE

Teriparatide treatment exerts differential effects on the central and peripheral skeleton: results from the MOAT study

(2018) M. A. Paggiosi et al.   OSTEOPOROSIS INTERNATIONAL

Pediatric Bone Mineral Accrual Z-Score Calculation Equations and Their Application in Childhood Disease

(2018) Andrea Kelly et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography

(2017) C. Braun et al.   OSTEOPOROSIS INTERNATIONAL

Trabecular and cortical bone deficits are present in children and adolescents with cystic fibrosis

(2016) Andrea Kelly et al.   BONE

Age-related changes in bone strength from HR-pQCT derived microarchitectural parameters with an emphasis on the role of cortical porosity

(2016) Nicolas Vilayphiou et al.   BONE

Age- and Sex-Related Changes in Bone Microarchitecture and Estimated Strength: A Three-Year Prospective Study Using HRpQCT

(2016) Vikram V Shanbhogue et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Effects of Two Years of Teriparatide, Denosumab, or Both on Bone Microarchitecture and Strength (DATA-HRpQCT study)

(2016) J. N. Tsai et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation

(2016) Isabelle Sermet-Gaudelus et al.   Journal of Cystic Fibrosis

Young adults with cystic fibrosis have altered trabecular microstructure by ITS-based morphological analysis

(2016) M. S. Putman et al.   OSTEOPOROSIS INTERNATIONAL

Reduced bone volumetric density and weak correlation between infection and bone markers in cystic fibrosis adult patients

(2016) D. Gensburger et al.   OSTEOPOROSIS INTERNATIONAL

Enhanced F508del-CFTR Channel Activity Ameliorates Bone Pathology in Murine Cystic Fibrosis

(2014) Carole Le Henaff et al.   AMERICAN JOURNAL OF PATHOLOGY

Cystic Fibrosis and Bone Disease: Defective Osteoblast Maturation with the F508del Mutation in Cystic Fibrosis Transmembrane Conductance Regulator

(2014) Frédéric Velard et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Cystic fibrosis bone disease: is the CFTR corrector C18 an option for therapy?

(2014) Frédéric Velard et al.   EUROPEAN RESPIRATORY JOURNAL

Cortical Porosity Identifies Women With Osteopenia at Increased Risk for Forearm Fractures

(2014) Yohann Bala et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Compromised Bone Microarchitecture and Estimated Bone Strength in Young Adults With Cystic Fibrosis

(2014) Melissa S. Putman et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Osteoblast CFTR Inactivation Reduces Differentiation and Osteoprotegerin Expression in a Mouse Model of Cystic Fibrosis-Related Bone Disease

(2013) Michael S. Stalvey et al.   PLoS One

Increased cortical porosity in type 2 diabetic postmenopausal women with fragility fractures

(2012) Janina M Patsch et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Bone loss in adults with cystic fibrosis: Prevalence, associated factors, and usefulness of biological markers

(2011) Isabelle Legroux-Gérot et al.   JOINT BONE SPINE

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

Reproducibility of direct quantitative measures of cortical bone microarchitecture of the distal radius and tibia by HR-pQCT

(2010) Andrew J. Burghardt et al.   BONE

Site Specificity of Bone Architecture Between the Distal Radius and Distal Tibia in Children and Adolescents: An HR-pQCT Study

(2010) Danmei Liu et al.   CALCIFIED TISSUE INTERNATIONAL

A longitudinal HR-pQCT study of alendronate treatment in postmenopausal women with low bone density: Relations among density, cortical and trabecular microarchitecture, biomechanics, and bone turnover

(2010) Andrew J Burghardt et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Finite element analysis performed on radius and tibia HR-pQCT images and fragility fractures at all sites in men

(2010) Nicolas Vilayphiou et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Osteopenia in Cftr-deltaF508 mice

(2010) Josée Paradis et al.   Journal of Cystic Fibrosis

Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation

(2010) Frank J. Accurso et al.   NEW ENGLAND JOURNAL OF MEDICINE

The Prevalence of Osteoporosis, Osteopenia, and Fractures Among Adults with Cystic Fibrosis: A Systematic Literature Review with Meta-Analysis

(2009) J. Paccou et al.   CALCIFIED TISSUE INTERNATIONAL

Cystic Fibrosis-Related Diabetes: Current Trends in Prevalence, Incidence, and Mortality

(2009) A. Moran et al.   DIABETES CARE

Cystic fibrosis transmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone

(2009) Louis Le Heron et al.   Journal of Cystic Fibrosis

Relation of bone mineral density with clinical and laboratory parameters in pre-pubertal children with cystic fibrosis

(2009) Nazan Cobanoglu et al.   PEDIATRIC PULMONOLOGY

Murine Model for Cystic Fibrosis Bone Disease Demonstrates Osteopenia and Sex-Related Differences in Bone Formation

(2009) Troy D Pashuck et al.   PEDIATRIC RESEARCH

Site-Specific Variation of Bone Micro-Architecture in the Distal Radius and Tibia

(2008) Steven K. Boyd   JOURNAL OF CLINICAL DENSITOMETRY

Prevalence of Low Bone Mass and Deficiencies of Vitamins D and K in Pediatric Patients With Cystic Fibrosis From 3 Canadian Centers

(2008) V. Grey et al.   PEDIATRICS

Persistent Osteopenia in Adult Cystic Fibrosis Transmembrane Conductance Regulator–deficient Mice

(2007) Christina K. Haston et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE