Integrated anti-remodeling and anabolic therapy for the osteoporosis of Hajdu-Cheney syndrome

F E McKiernan

doi:10.1007/s00198-006-0221-z

Integrated anti-remodeling and anabolic therapy for the osteoporosis of Hajdu-Cheney syndrome

Osteoporos Int. 2007 Feb;18(2):245-9. doi: 10.1007/s00198-006-0221-z. Epub 2006 Nov 14.

Author

F E McKiernan¹

Affiliation

¹ Center for Bone Diseases, Marshfield Clinic, 1000 N. Oak Avenue, Marshfield, WI 54449, USA. mckiernan.fergus@marshfieldclinic.org

PMID: 17103298
DOI: 10.1007/s00198-006-0221-z

Abstract

Hajdu-Cheney syndrome (MIM 102500) is a rare skeletal dysplasia marked by severe generalized osteoporosis and focal bone loss (acro-osteolysis). Osteoporosis treatment outcome has been reported only once previously. Reported herein is the biochemical and densitometric response to integrated anti-remodeling and anabolic therapy in a woman with Hajdu-Cheney syndrome. Results suggest dissociation of bone formation from bone resorption resulting in dramatic increases in bone mineral density without clinical evidence of activated osteoporosis.

Publication types

Case Reports

MeSH terms

Adult
Bone Density / physiology
Bone Density Conservation Agents / therapeutic use
Bone Remodeling / drug effects
Bone Remodeling / physiology
Female
Hajdu-Cheney Syndrome / complications
Hajdu-Cheney Syndrome / drug therapy*
Hajdu-Cheney Syndrome / physiopathology
Humans
Osteoporosis / complications
Osteoporosis / drug therapy*
Osteoporosis / physiopathology
Spinal Fractures / etiology
Spinal Fractures / physiopathology
Spinal Fractures / prevention & control
Treatment Outcome

Substances

Bone Density Conservation Agents