title: Management of advanced-stage neuroblastoma in a patient with 21-hydroxalase deficiency.
abstract: A 2-year-old boy presented with a 21-hydroxylase deficiency, associated with advanced-stage neuroblastoma primarily occurring in the left adrenal gland. He required intensive chemotherapy with polypharmacy, followed by cord blood stem cell transplantation to treat the neuroblastoma. The precise adjustment of cortisol levels was crucial in this patient to prevent adrenal crisis. We administered hydrocortisone by continuous infusion while monitoring blood cortisol levels. As there are no published reports on the target cortisol levels for children, we used two control infants with advanced-stage neuroblastoma, also undergoing chemotherapy and cord blood stem cell transplantation, to guide the continuous hydrocortisone therapy. The daily dose of hydrocortisone during chemotherapy required about threefold the normal treatment to avoid adrenal insufficiency. Continuous hydrocortisone therapy is feasible for preventing adrenal crisis and this report may provide an effective management for hydrocortisone replacement in 21-hydroxylase-deficient patients undergoing chemotherapy and hematopoietic stem cell transplantation.
authors.affiliation: Department of Pediatrics, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima, Japan. akari-ped@hiroshima-u.ac.jp
authors.forename: Akari N
authors.initials: AN
authors.lastname: Utsunomiya
authors.forename: Satoshi
authors.initials: S
authors.lastname: Okada
authors.forename: Keiichi
authors.initials: K
authors.lastname: Hara
authors.forename: Mizuka
authors.initials: M
authors.lastname: Miki
authors.forename: Yoko
authors.initials: Y
authors.lastname: Mizoguchi
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Miyagawa
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Jinno
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Nakamura
authors.forename: Takashi
authors.initials: T
authors.lastname: Sato
authors.forename: Masao
authors.initials: M
authors.lastname: Kobayashi
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000628:therapy*; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000175:diagnosis; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009447:Neuroblastoma / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation / Q000379:methods*; D014057:Tomography, X-Ray Computed
keywords: 21-hydroxylase deficiency; cord blood stem cell transplantation; hydrocortisone; neuroblastoma