Zoledronate as effective treatment for minimal trauma fractures in a child with STAT3 deficiency and osteonecrosis of the hip

Signal transducer and activator of transcription 3 (STAT3) deficiency is a primary immunodeficiency characterized by eczema, complicated recurrent infections, elevated serum immunoglobulin E (IgE), osteopenia, and minimal trauma fractures. Zoledronic acid (ZA) is a long‐acting bisphosphonate that ha...

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Bibliographic Details
Published in:Pediatric blood & cancer 2016-11, Vol.63 (11), p.2054-2057
Main Authors: Staines Boone, Aidé Tamara, Alcántara-Montiel, Julio César, Sánchez-Sánchez, Luz María, Arce-Cano, Marina, García-Campos, Jorge, Lugo Reyes, Saúl Oswaldo
Format: Article
Language:English
Subjects:
arthroplasty
avascular necrosis of the hip
bisphosphonate
Bone Density Conservation Agents - therapeutic use
Child
Children
Diphosphonates - therapeutic use
Eczema
Fractures
Fractures, Bone - drug therapy
Fractures, Bone - etiology
Hematology
Hip
Hip - pathology
Humans
hyper-IgE syndrome
Imidazoles - therapeutic use
Immunodeficiency
Immunoglobulin E
Intravenous administration
job syndrome
Male
Oncology
Osteogenesis
Osteogenesis imperfecta
Osteonecrosis
Osteonecrosis - drug therapy
osteonecrosis of the femoral head
Osteopenia
Osteoporosis
Pediatrics
Primary immunodeficiencies
primary immunodeficiency
Skin diseases
STAT3 deficiency
Stat3 protein
STAT3 Transcription Factor - deficiency
Transcription
Trauma
Zoledronic acid
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Summary:Signal transducer and activator of transcription 3 (STAT3) deficiency is a primary immunodeficiency characterized by eczema, complicated recurrent infections, elevated serum immunoglobulin E (IgE), osteopenia, and minimal trauma fractures. Zoledronic acid (ZA) is a long‐acting bisphosphonate that has been successfully used in children with secondary osteoporosis and osteogenesis imperfecta. We describe the case of a 7‐year‐old male with STAT3 deficiency and minimal trauma fractures, who also developed osteonecrosis of the hip. He responded well to intravenous ZA every 6 months for 18 months. Three years later, he walks independently and unaided, and has not suffered any other fractures. Although more studies are needed, ZA might help reduce minimal trauma fractures in patients with STAT3 deficiency.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.26119