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Pediatr Blood Cancer ; 63(11): 2054-7, 2016 11.
Article in English | MEDLINE | ID: mdl-27416072

ABSTRACT

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.


Subject(s)
Bone Density Conservation Agents/therapeutic use , Diphosphonates/therapeutic use , Fractures, Bone/drug therapy , Hip/pathology , Imidazoles/therapeutic use , Osteonecrosis/drug therapy , STAT3 Transcription Factor/deficiency , Child , Fractures, Bone/etiology , Humans , Male , Zoledronic Acid
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