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Evaluation of Teriparatide for Treatment of Osteoporosis in Four Patients with Cystic Fibrosis: A Case Series

DOI: 10.1155/2014/893589

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Introduction. Bone disease is a common complication of cystic fibrosis (CF). To date, there have been no reports on the effectiveness of teriparatide, recombinant human parathyroid hormone, to treat CF-related bone disease. Case Presentation. We report on four patients with CF-related bone disease who were treated with teriparatide. Three patients completed two years of therapy with teriparatide, and all had significant improvements in their bone mineral density (BMD). One patient was unable to tolerate teriparatide and discontinued treatment 1 week into therapy. Conclusion. Teriparatide may be a potential treatment option for CF-related bone disease. This report highlights the need for further investigation into the use of teripartide in the CF population. 1. Introduction Cystic Fibrosis (CF), caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, is a common lethal genetic disease among Caucasians. As survival has improved, new complications from CF have emerged, including CF-related bone disease [1–3]. The major contributing factors to bone disease in CF include vitamins D and K malabsorption, poor nutritional status, physical inactivity, chronic inflammation, glucocorticoid therapy, delayed puberty, and hypogonadism [4–6]. These factors result in decreased bone mineral density (BMD), osteopenia, osteoporosis, fragility fractures, and kyphosis, which can cause significant morbidity and potential exclusion from lung transplantation candidacy [7]. Bisphosphonates, antiresorptive agents, have been shown to be efficacious in treating CF-related bone disease and currently are the mainstay of treatment. Studies on the use of teriparatide, recombinant human parathyroid hormone, in CF-related bone disease have not been reported to date. 2. Case Presentation We report on four patients with CF-related bone disease who were treated with teriparatide 20?mcg subcutaneously once a day for a two-year period at the Emory University Cystic Fibrosis clinic. The study was approved by IRB at Emory University and all patients provided written consent for presenting their data. Demographic characteristics of our patients are displayed in Table 1. Table 1: Demographic data. Case 1. Patient 1 was a 59-year-old Caucasian female with a two-year history of CF who was referred to our clinic for evaluation of osteopenia. She was diagnosed with adult onset CF based on clinical findings after she developed a pulmonary mycobacterium avium complex (MAC) infection that was very difficult to clear. She reported being treated with prednisone two

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