title: Living-Donor Lung Transplantation After Bone Marrow Transplantation for Chediak-Higashi Syndrome.
abstract: An 8-year-old girl with Chediak-Higashi syndrome (CHS) had pulmonary complications after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) and eventually underwent single living-donor lobar lung transplantation (LDLLT). Electron micrographic findings showed vagus nerve tissue in extracted lung having granular inclusions, which are pathognomonic for CHS. Because her mother was the donor for both hematopoietic stem cell and lung transplantations, she was weaned from immunosuppression and is doing well 3 years after lung transplantation. Furthermore, an induced pluripotent stem (iPS) cell line was established from her skin fibroblasts for investigation and potential future treatment for CHS.
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Tetsu
authors.initials: T
authors.lastname: Yamada
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Toyofumi F
authors.initials: TF
authors.lastname: Chen-Yoshikawa
authors.affiliation: Department of Clinical Application, Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto, Japan.
authors.forename: Shigeharu
authors.initials: S
authors.lastname: Oh
authors.affiliation: Department of Hematology and Oncology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Rieko
authors.initials: R
authors.lastname: Ito-Taniguchi
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Gochi
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Sato
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Aoyama
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. Electronic address: hdate@kuhp.kyoto-u.ac.jp.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Date
mesh_terms: D002609:Chediak-Higashi Syndrome / Q000601:surgery*; D002648:Child; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D019520:Living Donors; D016040:Lung Transplantation; D051359:Lymphohistiocytosis, Hemophagocytic