Jay R Shapiro
Affiliation: Kennedy Krieger Institute
- Osteogenesis imperfecta: effecting the transition from adolescent to adult medical careJ R Shapiro
Bone and Osteogenesis Imperfecta Department, Kennedy Krieger Institute, Johns Hopkins School of Medicine, 707 North Broadway, Baltimore, MD 21205, United States
J Musculoskelet Neuronal Interact 12:24-7. 2012..In addition, as the transition is established, the patient with OI should be encouraged to be his/her own advocate and care coordinator...
- Treatment with zoledronic acid ameliorates negative geometric changes in the proximal femur following acute spinal cord injuryJ Shapiro
Department of Physical Medicine and Rehabilitation, Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205, USA
Calcif Tissue Int 80:316-22. 2007....
- Bone mineral density and fracture rate in response to intravenous and oral bisphosphonates in adult osteogenesis imperfectaJay R Shapiro
Osteogenesis Imperfecta Program, Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205, USA
Calcif Tissue Int 87:120-9. 2010..Fracture rate decreased in type III/IV patients following pamidronate but not following oral bisphosphonate treatment. These results underscore a need to consider whether bisphosphonate treatment is appropriate for all adults with OI...
- Osteogenesis imperfecta: questions and answersJay R Shapiro
Department Physical Medicine and Rehabilitation, Johns Hopkins University, Kennedy Krieger Institute, Baltimore, Maryland 21205, USA
Curr Opin Pediatr 21:709-16. 2009..Orthopedic intervention occurs on several levels: including the immediate treatment of fractures, the treatment of scoliosis and the use of intramedullary rods...
- Microgravity and drug effects on boneJ R Shapiro
National Space Biomedical Research Institute, Department of Physical Medicine and Rehabilitation, Kennedy Krieger Institute, Johns Hopkins University, Baltimore, MD 21205, USA
J Musculoskelet Neuronal Interact 6:322-3. 2006
- Increased fracture risk and low bone mineral density in patients with loeys-dietz syndromeEric W Tan
Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, MD 21224 2780, USA
Am J Med Genet A 161:1910-4. 2013..The lowest Z- and T-scores were not associated with an increased number of fractures. Patients with Loeys-Dietz syndrome have a high risk of fracture and a high incidence of low bone mineral density...
- Phenotypic variability of osteogenesis imperfecta type V caused by an IFITM5 mutationJay R Shapiro
Department of Bone and Osteogenesis Imperfecta, Kennedy Krieger Institution, Johns Hopkins University, Baltimore, MD, USA
J Bone Miner Res 28:1523-30. 2013..The bone mineral density varied greatly, even within families. Our study thus highlights the phenotypic variability of OI type V caused by the IFITM5 mutation...
- Serum and urine markers of bone metabolism during the year after hip fractureJ A Yu-Yahiro
Department of Orthopaedic Surgery, The Union Memorial Hospital, Baltimore, Maryland, USA
J Am Geriatr Soc 49:877-83. 2001....
- Cross-sectional and longitudinal growth patterns in osteogenesis imperfecta: implications for clinical careEmily L Germain-Lee
Bone and Osteogenesis Imperfecta Department, Kennedy Krieger Institute, Baltimore, Maryland
Pediatr Res 79:489-95. 2016..There is strikingly limited information on linear growth and weight in the different types of osteogenesis imperfecta (OI). Here, we define growth patterns further with the intent of implementing appropriate adaptations proactively...
- Osteoblast function and bone histomorphometry in a murine model of Rett syndromeMary E Blue
Hugo W Moser Research Institute at Kennedy Krieger, Inc, 707 North Broadway, Baltimore, MD 21205, USA Electronic address
Bone 76:23-30. 2015..These results indicate that MeCP2 deficiency leads to altered bone growth. Osteoblast dysfunction was more marked in Mecp2-null male than in HET female mice, suggesting that expression of MeCP2 plays a critical role in bone development...
- Osteogenesis imperfecta:epidemiology and pathophysiologyElizabeth Martin
The Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205, USA
Curr Osteoporos Rep 5:91-7. 2007..Other treatment options, such as recombinant human parathyroid hormone (1-34), Rank ligand inhibitors, and stem cell technology, are being evaluated or are of future investigative interest...
- Reduced incidence of hip fracture in the Old Order AmishElizabeth A Streeten
Division of Endocrinology, Diabetes and Nutrition, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA
J Bone Miner Res 19:308-13. 2004..The incidence of hip fracture was estimated in a community of Old Order Amish and compared with available data from non-Amish whites. Hip fracture rates were 40% lower in the Amish, and the Amish also experienced higher BMD...
- Bone mass in Rett syndrome: association with clinical parameters and MECP2 mutationsJay R Shapiro
Department of Bone and Osteogenesis Imperfecta, Kennedy Krieger Institution, Johns Hopkins University, Baltimore, Maryland 21205, USA
Pediatr Res 68:446-51. 2010..Studies in a murine model of RTT confirmed low bone mass as an inherent component of this syndrome. MECP2 mutations and clinical parameters impact bone mass in RTT, but an association with a specific mutation was not demonstrable...
- The effect of intravenous pamidronate on bone mineral density, bone histomorphometry, and parameters of bone turnover in adults with type IA osteogenesis imperfectaJ R Shapiro
Kennedy Krieger Institute, Baltimore, MD 21205, USA
Calcif Tissue Int 72:103-12. 2003..These results indicate that intravenous pamidronate, 30 mg every 3 months, may have significant effects on bone density and histomorphometry in adults with type IA OI. Responses at higher doses remain to be evaluated...
- OIM and related animal models of osteogenesis imperfectaJ R Shapiro
Bone Metabolism Research Laboratory, Division of Geriatric Medicine and Gerontology, Johns Hopkins University School of Medicine, Hopkins Bayview Research Campus, Baltimore, Maryland 21224, USA
Connect Tissue Res 31:265-8. 1995..OIM duplicates the phenotype and biochemistry of human disease and has a normal life span...
- An osteopenic nonfracture syndrome with features of mild osteogenesis imperfecta associated with the substitution of a cysteine for glycine at triple helix position 43 in the pro alpha 1(I) chain of type I collagenJ R Shapiro
Bone Metabolism Research Laboratory, Johns Hopkins University School of Medicine, Baltimore, Maryland 21224
J Clin Invest 89:567-73. 1992..The prevalence of similar NH2-terminal mutations in subjects with this phenotype which clinically overlaps idiopathic osteoporosis remains to be determined...
- Defective pro alpha 2(I) collagen synthesis in a recessive mutation in mice: a model of human osteogenesis imperfectaS D Chipman
Bone Metabolism Research Laboratory, Johns Hopkins University School of Medicine, Baltimore, MD 21224
Proc Natl Acad Sci U S A 90:1701-5. 1993..The oim mouse will facilitate the study of type I collagen-related skeletal disease...
- Bone geometry and strength measurements in aging mice with the oim mutationD J McBride
Bone Metabolism Research Laboratory, Division of Geriatric Medicine and Gerontology, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21224, USA
Calcif Tissue Int 62:172-6. 1998..However, no statistical difference in body mass was detected between +/oim and +/+ mice. The absence of a gross phenotypic difference between +/oim and +/+ mice demonstrates a milder phenotype in +/oim mice...
- Expression of mutant alpha (I)-procollagen in osteoblast and fibroblast cultures from a proband with osteogenesis imperfecta type IVS D Chipman
Division of Geriatric Medicine and Gerontology, Johns Hopkins University School of Medicine, Baltimore, Maryland
J Bone Miner Res 7:793-805. 1992..Therefore, in this patient with osteogenesis imperfecta there was no qualitative alteration in the osteoblast-specific expression of this mutant alpha 2(I)-collagen allele compared to dermal fibroblasts...