title: Bone mineral density in children with fanconi anemia after hematopoietic cell transplantation.
abstract: Fanconi anemia (FA) is an inherited DNA repair disorder associated with short stature and bone marrow failure, usually requiring hematopoietic cell transplantation (HCT). Although low bone mineral density (BMD) has been reported in leukemia patients after HCT, little is known about BMD in FA children after HCT (FA HCT). This study's goals were to compare BMD in FA HCT to BMD in healthy controls and in children who received HCT for hematologic malignancy (cancer HCT), and to test for associations between BMD and risk factors for bone loss. This cross-sectional study included 20 FA HCT, 13 cancer HCT, and 90 healthy controls, age-matched and <18 years old at evaluation. BMD Z-scores for total body (TBMD) and lumbar spine (LBMD) were measured by dual energy x-ray absorptiometry and adjusted for height-for-age Z-score (HAZ). FA HCT had lower mean TBMDHAZ Z-score (by .8 SD) and higher fraction with Z-score ≤ -1 than healthy controls (42% versus 11%). No LBMD deficits were detected. FA HCT and cancer HCT groups did not differ significantly in TBMD or LBMD Z-scores. In FA HCT patients, lower body mass index and lower percent fat were associated with lower BMD. This study highlights the importance of monitoring BMD to optimize bone health in FA patients.
authors.affiliation: Department of Pediatrics, Division of Pediatric Endocrinology, University of Minnesota, Minneapolis, Minnesota. Electronic address: petry005@umn.edu.
authors.forename: Anna
authors.initials: A
authors.lastname: Petryk
authors.affiliation: Department of Pediatrics, Division of Pediatric Endocrinology and Metabolism, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, California.
authors.forename: Lynda E
authors.initials: LE
authors.lastname: Polgreen
authors.affiliation: Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota.
authors.forename: Jessie L
authors.initials: JL
authors.lastname: Barnum
authors.affiliation: Biostatistics Design and Analysis Center, Clinical and Translational Science Institute, University of Minnesota, Minneapolis, Minnesota.
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Division of Biostatistics, University of Minnesota, Minneapolis, Minnesota.
authors.forename: James S
authors.initials: JS
authors.lastname: Hodges
authors.affiliation: Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington.
authors.forename: K Scott
authors.initials: KS
authors.lastname: Baker
authors.affiliation: Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota.
authors.forename: John E
authors.initials: JE
authors.lastname: Wagner
authors.affiliation: Department of Pediatrics, Division of Pediatric Cardiology, University of Minnesota, Minneapolis, Minnesota.
authors.forename: Julia
authors.initials: J
authors.lastname: Steinberger
authors.affiliation: Department of Pediatrics, Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota.
authors.forename: Margaret L
authors.initials: ML
authors.lastname: MacMillan
mesh_terms: D000293:Adolescent; D000328:Adult; D064591:Allografts; D015519:Bone Density; D002648:Child; D003430:Cross-Sectional Studies; D005199:Fanconi Anemia / Q000378:metabolism* / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged
keywords: Bone marrow transplantation; Bone mineral density; Children; Dual energy x-ray absorptiometry scan (DXA); Fanconi anemia; Osteoporosis