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Bone Density and Fractures in Autosomal Dominant Hyper IgE Syndrome

Journal of Clinical Immunology : Volume 34, Issue 1, January 2014

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Bone Density and Fractures in Autosomal Dominant Hyper IgE Syndrome

Content Provider	Springer Nature Link
Author	Sowerwine, Kathryn J. Shaw, Pamela A. Gu, Wenjuan Ling, Jennifer C. Collins, Michael T. Darnell, Dirk N. Anderson, Victoria L. Davis, Joie Hsu, Amy Welch, Pamela Puck, Jennifer M. Holland, Steven M. Freeman, Alexandra F.
Copyright Year	2014
Abstract	Autosomal Dominant Hyper IgE Recurrent Infection Syndrome (AD-HIES) is caused by mutations in STAT3 and characterized by eczema, recurrent bacterial infections, and skeletal and connective tissue abnormalities. To further understand the minimal trauma fractures of AD-HIES, we examined bone mineral density (BMD) and laboratory markers of bone turnover.Patients with AD-HIES enrolled in a prospective natural history study were examined with dual x-ray absorptiometry (DEXA) scans and laboratory studies of bone metabolism. The number of fractures was recorded as well as clinical features of AD-HIES including scoliosis and retained primary teeth. Patients on medications with skeletal effects, including bisphosphonates, were examined separately.Twenty-three AD-HIES children (6–18 years) and 33 AD-HIES adults (21–50 years) not receiving bone-active drugs were studied. Fourteen of the 23 children (61 %) had histories of minimal trauma fractures, as did 26 of the 33 adults (79 %). Osteopenia or osteoporosis was found in 79 % of children and adults. Only radial BMD correlated with the qualitative occurrence of fractures but it did not correlate with the numbers of fractures. Markers of bone metabolism did not correlate with minimal trauma fractures or BMD. Patients on bone-active medications had improved BMD, but still sustained fractures.Minimal trauma fractures and decreased BMD are common in AD-HIES. Low radial BMD is associated with fractures, but hip and spine BMD are not. Treatment with bisphosphonates increased BMD but its role in fracture prevention remains undefined.
Starting Page	260
Ending Page	264
Page Count	5
File Format	PDF
ISSN	02719142
Journal	Journal of Clinical Immunology
Volume Number	34
Issue Number	2
e-ISSN	15732592
Language	English
Publisher	Springer US
Publisher Date	2014-01-09
Publisher Place	Boston
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Autosomal dominant hyper IgE syndrome (AD-HIES) job’s syndrome signal transducer and activator of transcription 3 (STAT3) osteoporosis retained primary teeth Immunology Infectious Diseases Internal Medicine Medical Microbiology
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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