title: Primary adrenal insufficiency in a child after busulfan and cyclophosphamide-based conditioning for hematopoietic stem cell transplantation.
abstract: High rates of skeletal complications, growth disturbances, thyroid and gonadal dysfunction have been described in children undergoing stem cell transplantation. Although secondary adrenal insufficiency has been diagnosed, no primary adrenal insufficiency has been reported after busulfan and cyclophosphamide (Bu/Cy)-based conditioning regimens for stem cell transplantation in children. A 9-year-old girl with myelodysplastic syndrome was treated with stem cell transplantation of allogeneic origin. She received myeloablative conditioning chemotherapy, Bu and Cy. Her serum cortisol level was normal before stem cell transplantation. Then, 17 months after stem cell transplantation, chronic graft-versus-host disease developed and was treated with methyl prednisolone for 3 months. The control endocrinological investigation revealed low serum cortisol and high serum adrenocorticotropin (ACTH) levels 6 months after completion of methyl prednisolone treatment. The ACTH stimulation test demonstrated primary adrenal insufficiency, and the other etiologies of primary adrenal insufficiency were excluded. The patient received oral prednisolone replacement therapy. She was followed-up for 44 months and required increases in steroid doses during stress periods. Primary adrenal insufficiency which was observed in our patient after Bu/Cy-based conditioning regimen for stem cell transplantation has not been reported in children and adrenal function should be closely monitored in these patients both before stem cell transplantation and after stem cell transplantation.
authors.affiliation: Division of Pediatric Endocrinology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey. senaysavas@yahoo.com
authors.forename: Senay
authors.initials: S
authors.lastname: Savas-Erdeve
authors.forename: Merih
authors.initials: M
authors.lastname: Berberoglu
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Siklar
authors.forename: Bulent
authors.initials: B
authors.lastname: Hacihamdioglu
authors.forename: Gonul
authors.initials: G
authors.lastname: Ocal
authors.forename: Mehmet
authors.initials: M
authors.lastname: Ertem
authors.forename: Talia
authors.initials: T
authors.lastname: Ileri
authors.forename: Elif Unal
authors.initials: EU
authors.lastname: Ince
authors.forename: Zumrut
authors.initials: Z
authors.lastname: Uysal
mesh_terms: D000224:Addison Disease / Q000139:chemically induced*; D002066:Busulfan / Q000009:adverse effects*; D002648:Child; D003520:Cyclophosphamide / Q000009:adverse effects*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D019653:Myeloablative Agonists / Q000009:adverse effects; D009190:Myelodysplastic Syndromes / Q000628:therapy*; D019172:Transplantation Conditioning / Q000009:adverse effects*
chemical_list: D019653:Myeloablative Agonists; D003520:Cyclophosphamide; D002066:Busulfan