Journal
PEDIATRIC BLOOD & CANCER
Volume 63, Issue 11, Pages 2054-2057Publisher
WILEY
DOI: 10.1002/pbc.26119
Keywords
arthroplasty; avascular necrosis of the hip; bisphosphonate; hyper-IgE syndrome; job syndrome; osteonecrosis of the femoral head; osteopenia; primary immunodeficiency; STAT3 deficiency; zoledronic acid
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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.
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