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

• Published in: Pediatric blood & cancer Vol. 63; no. 11; pp. 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: Journal Article
• Language: English
• Published: United States Blackwell Publishing Ltd 01.11.2016; Wiley Subscription Services, Inc
• 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; avascular necrosis of the hip; osteopenia; STAT3 deficiency; arthroplasty; zoledronic acid; osteonecrosis of the femoral head; bisphosphonate; primary immunodeficiency; job syndrome; hyper-IgE syndrome
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.26119

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.